Pre-Operative Assessment and Planning

Aims Preparation
• Informed consent
• Assess risk vs. benefits NBM
• Optimise fitness of patient • ≥2h for clear fluids, ≥6h for solids
• Check anaesthesia / analgesia type c̄ anaesthetist

Bowel Prep
Pre-op Checks: OP CHECS • May be needed in left-sided ops
• Operative fitness: cardiorespiratory comorbidities § Picolax: picosulfate and Mg citrate
• Pills § Klean-Prep: macrogol
• Consent • Not usually needed in right-sided procedures
• History • Necessity is controversial as benefit of minimising
§ MI, asthma, HTN, jaundice post-op infection might not outweigh risks
§ Complications of anaesthesia: DVT, § Liquid bowel contents spilled during surgery
anaphylaxis § Electrolyte disturbance
• Ease of intubation: neck arthritis, dentures, loose § Dehydration
teeth § ↑ rate of post-op anastomotic leak
• Clexane: DVT prophylaxis
• Site: correct and marked
Prophylactic Abx
• Use
§ GI surgery (20% post-op infection if elective)
Drugs § Joint replacement
• Give 15-60min before surgery
Anti-coagulants • Regimens: (see local guidelines)
• Balance risk of haemorrhage c̄ risk of thrombosis § Biliary: Cef 1.5g + Met 500mg IV
• Avoid epidural, spinal and regional blocks § CR or appendicetomy: Cef+Met TDS
§ Vascular: co-amoxiclav 1.2g IV TDS
AED § MRSA+ve: vancomycin
• Give as usual
• Post-op give IV or via NGT if unable to tolerate orally
DVT Prophylaxis
OCP / HRT • Stratify pts according to patient factors and type of
• Stop 4wks before major / leg surgery surgery.
• Restart 2wks post-op if mobile • Low risk: early mobilisation
• Med: early mobilisation + TEDS + 20mg enoxaparin
β-Blockers • High: early mobilisation + TEDS + 40mg enoxaparin +
• Continue as usual intermittent compression boots perioperatively.
• Prophylaxis started @ 1800 post-op
• May continue medical prophylaxis at home (up to
1mo)
Pre-op Investigations

Bloods ASA Grades

• Routine: FBC, U+E, G+S, clotting, glucose • Normally healthy
• Specific • Mild systemic disease
§ LFTs: liver disease, EtOH, jaundice • Severe systemic disease that limits activity
§ TFT: thyroid disease • Systemic disease which is a constant threat to life
§ Se electrophoresis: Africa, West Indies, Med
• Moribund: not expected to survive 24h even c̄ op
• Cross-match
§ Gastrectomy: 4u
§ AAA: 6u

Cardiopulmonary Function
• CXR: cardiorespiratory disease/symptoms, >65yrs
• Echo: poor LV function, Ix murmurs
• ECG: HTN, Hx of cardiac disease, >55yrs
• Cardiopulmonary Exercise Testing
• PFT: known pulmonary disease or obesity

Other
• Lat C-spine flexion and extension views: RA, AS
• MRSA swabs

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 Specific Pre-operative Complications
Diabetes Jaundice
• Best to avoid operating in jaundiced pts.
↑ Risk of post-operative complications • Use ERCP instead
• Surgery → stress hormones → antagonise insulin
• Pts. are NBM Risks
• ↑ risk of infection • Pts. c̄ obstructive jaundice have ↑ risk of post-op renal
• IHD and PVD failure \ need to maintain good UO.
• Coagulopathy
Pre-op • ↑ infection risk: may → cholangitis
• Dipstick: proteinuria
• Venous glucose Pre-op
+
• U+E: K • Avoid morphine in pre-med
• Check clotting and consider pre-op vitamin K
IDDM • Give 1L NS pre-op (unless CCF) → moderate diuresis
• Urinary catheter to monitor UPO
Practical Points • Abx prophylaxis: e.g. cef+met
• Put pt. first on list and inform surgeon and
anaesthetist Intra-op
• Some centres prefer to use GKI infusions • Hrly UO monitoring
• Sliding scale may not be necessary for minor ops • NS titrated to output
§ If in doubt, liaise c̄ diabetes specialist nurse
Post-op
Insulin • Intensive monitoring of fluid status
• ± stop long-acting insulin the night before • Consider CVP + frusemide if poor output despite NS
• Omit AM insulin if surgery is in the morning
• Start sliding scale
§ 5% Dex c̄ 20mmol KCl 125ml/hr Anticoagulated Patients
§ Infusion pump c̄ 50u actrapid • Balance risk of haemorrhage c̄ risk of thrombosis
§ Check CPG hrly and adjust insulin rate • Consult surgeon, anaesthetist and haematologist
• Check glucose hrly: aim for 7-11mM • Very minor surgery may be undertaken w/o stopping
• Post-op warfarin if INR <3.5.
§ Continue sliding-scale until tolerating food • Avoid epidural, spinal and regional blocks if
§ Switch to SC regimen around a meal anticoagulated,
• In general, continue aspirin/clopidogrel unless risk of
NIDDM bleeding is high – then stop 7d before surgery
• If glucose control poor (fasting >10mM): treat as
IDDM Low thromboembolic risk: e.g. AF
• Omit oral hypoglycaemics on the AM of surgery • Stop warfarin 5d pre-op: need INR <1.5
• Eating post-op: resume oral hypoglycaemics c̄ meal • Restart next day
• No eating post-op
§ Check fasting glucose on AM of surgery High thromboembolic risk: valves, recurrent VTE
§ Start insulin sliding scale • Need bridging c̄ LMWH
§ Consult specialist team ore. restarting PO Rx § Stop warfarin 5d pre-op and start LMWH
§ Stop LMWH 12-18h pre-op
Diet Controlled § Restart LMWH 6h post-op
• Usually no problem § Restart warfarin next day
• Pt. may be briefly insulin-dependent post-op § Stop LMWH when INR >2
§ Monitor CPG
Emergency Surgery
• Discontinue warfarin
Steroids • Vit K .5mg slow IV
• Request FFP or PCC to cover surgery
Risks
• Poor wound healing COPD and Smoking
• Infection
• Adrenal crisis Risks
• Basal atelectasis
Mx • Aspiration
• Need to ↑ steroid to cope c̄ stress • Chest infection
• Consider cover if high-dose steroids w/i last yr
• Major surgery: hydrocortisone 50-100mg IV c̄ pre- Pre-op
med then 6-8hrly for 3d. • CXR
• Minor: as for major but hydrocortisone only for 24h • PFTs
• Physio for breathing exercises
• Quit smoking (at least 4wks prior to surgery)
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Anaesthesia
Principals and Practical Conduct
• Aims: hypnosis, analgesia, muscle relaxation
• Induction: e.g. IV propofol
• Muscle Relaxation
§ Depolarising: suxamethonium
§ Non-depolarising: vecuronium, atracurium
• Airway Control: ET tube, LMA
• Maintenance
§ Usually volatile agent added to N2O/O2 mix
§ E.g. halothane, enflurane
• End of Anaesthesia
§ Change inspired gas to 100% O2
§ Reverse paralysis: neostigmine + atropine
(prevent muscarinic side effects)
Analgesia
Necessity
• Pain → autonomic activation → arteriolar constriction →
↓ wound perfusion → impaired wound healing
• Pain → ↓ mobilisation → ↑ VTE and ↓ function
• Pain → ↓ respiratory excursion and ↓ cough →
atelectasis and pneumonia
• Humanitarian considerations
General Guidance
• Give regular doses at fixed intervals
• Consider best route: oral when possible
• PCA should be considered: morphine, fentanyl
• Follow stepwise approach
• Liaise c̄ Acute Pain Service

Pre-medication: 7As Pre-Op

• Anxiolytics and Amnesia: e.g. temazepam
• Analgesics: e.g. opioids, paracetamol, NSAIDs
• Anti-emetics: e.g. ondansetron 4mg / metoclop 10mg
• Antacids: e.g. lansoprazole
• Anti-sialogue e.g. glycopyrolate (↓ secretions)
• Antibiotics
• Epidural anaesthesia: e.g. c̄ bupivacaine
End-Op
• Infiltrate wound edge c̄ LA
• Infiltrate major regional nerves c̄ LA
Post-Op: stepwise approach

Regional Anaesthesia 1. Non-opioid ± adjuvants

• May be used for minor procedures or if unsuitable for § Paracetamol
GA § NSAIDs
• Nerve or spinal blocks - Ibuprofen: 400mg/6h PO max
§ CI: local infection, clotting abnormality - Diclofenac: 50mg PO / 75mg IM
• Use long-acting agents: e.g. bupivacaine
2. Weak opioid + non-opioid ± adjuvants
§ Codeine
Complications of Anaesthesia § Dihydrocodeine
§ Tramadol
Propofol Induction
3. Strong opioid + non-opioid ± adjuvants
• Cardiorespiratory depression § Morphine: 5-10mg/2h max
§ Oxycodone
Intubation § Fentanyl
• Oro-pharyngeal injury c̄ laryngoscope
• Oesophageal intubation Spinal or Epidural Anaesthesia
• ↓ SE as drugs more localised
Loss of pain sensation •
st
1 line for major bowel resection
• Urinary retention • Caution
• Pressure necrosis § Respiratory depression
• Nerve palsies § Neurogenic shock → ↓BP

Loss of muscle power

• Corneal abrasion
• No cough → atelectasis + pneumonia

Malignant Hyperpyrexia
• Rare complication ppted by halothane or
suxamethonium
• AD inheritance
• Rapid rise in temperature + masseter spasm
• Rx: dantrolene + cooling

Anaphylaxis
• Rare
• Possible triggers
§ Antibiotics
§ Colloid
§ NM blockers: e.g. vecuronium

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Enhanced Recovery After Surgery Surgical Complications
ERAS Immediate (<24h)
• Commonly employed in colorectal and orthopaedic • Intubation → oropharyngeal trauma
surgery • Surgical trauma to local structures
• Primary or reactive haemorrhage
Aims
• Optimise pre-op preparation for surgery Early (1d-1mo)
• Avoid iatrogenic problems (e.g. ileus) • Secondary haemorrhage
• Minimise adverse physiological / immunological • VTE
responses to surgery • Urinary retention
§ ↑ cortisol and ↓ insulin (absolute or relative) • Atelectasis and pneumonia
§ Hypercoagulability • Wound infection and dehiscence
§ Immunosuppression • Antibiotic association colitis (AAC)
• ↑ speeded of recovery and return to function
• Recognise abnormal recovery and allow early
Late (>1mo)
intervention
• Scarring
• Neuropathy
• Failure or recurrence
Pre-op: optimisation
• Aggressive physiological optimisation
§ Hydration
§ BP (↑ / ↓)
§ Anaemia
§ DM
§ Co-morbidities
• Smoking cessation: ≥4wks before surgery
• Admission on day of surgery, avoidance of prolonged fast
• Carb loading prior to surgery: e.g. carb drinks
• Fully informed pt., encouraged to participate in recovery

Intra-op: ↓ physical stress

• Short-acting anaesthetic agents
• Epidural use
• Minimally invasive techniques
• Avoid drains and NGTs where possible

Post-op: early return to function and

mobilisation
• Aggressive Rx of pain and nausea
• Early mobilisation and physiotherapy
• Early resumption of oral intake (inc. carb drinks)
• Early discontinuation of IV fluids
• Remove drains and urinary catheters ASAP

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 Post-op Complications: General
Haemorrhage Classification Wound Infection
• Primary: continuous bleeding starting during • 5-7d post-op
surgery • Organisms: S. aureus and Coliforms
• Reactive
§ Bleeding at the end of surgery or early Operative Classification
post-op • Clean: incise uninfected skin w/o opening viscus
O
§ 2 to ↑ CO and BP • Clean/Cont: intra-op breach of viscus (not colon)
• Secondary • Contaminated: breach of viscus + spillage or opening of
§ Bleeding >24h post-op colon
§ Usually due to infection • Dirty: site already contaminated – faeces, pus, trauma

Risk Factors
Post-op Urinary Retention • Pre-operative
§ ↑ Age
Causes § Comorbidities: e.g. DM
• Drugs: opioids, epidural/spinal, anti-AChM § Pre-existing infection: e.g. appendix perforation
• Pain: sympathetic activation → sphincter § Pt. colonisation: e.g. nasal MRSA
contraction • Operative
• Psychogenic: hospital environment § Op classification and wound infection risk
§ Duration
§ Technical: pre-op Abx, asepsis
Risk Factors
• Post-operative
• Male
§ Contamination of wound from staff
• ↑ age
• Neuropathy: e.g. DM, EtOH
Mx
• BPH
• Regular wound dressing
• Surgery type: hernia and anorectal
• Abx
• Abscess drainage
Mx
• Conservative
§ Privacy
§ Ambulation Wound Dehiscence
§ Void to running taps or in hot bath
§ Analgesia Presentation
• Catheterise ± gent 2.5mg/kg IV stat • Occurs ~10d post-op
• TWOC = Trial w/o Catheter • Preceded by serosanguinous discharge from wound
§ If failed, may be sent home c̄ silicone
catheter and urology outpt. f/up. Risk Factors
• Pre-Operative Factors
§ ↑ age
Pulmonary Atelectasis § Smoking
• Occurs after every nearly every GA § Obesity, malnutrition, cachexia
• Mucus plugging + absorption of distal air → § Comorbs: e.g. BM, uraemia, chronic cough, Ca
collapse § Drugs: steroids, chemo, radio
• Operative Factors
Causes § Length and orientation of incision
• Pre-op smoking § Closure technique: follow Jenkin’s Rule
• Anaesthetics ↑ mucus production ↓ mucociliary § Suture material
clearance • Post-operative Factors
• Pain inhibits respiratory excursion and cough § ↑ IAP: e.g. prolonged ileus → distension
§ Infection
§ Haematoma / seroma formation
Presentation
• w/i first 48hrs
Mx
• Mild pyrexia
• Replace abdo contents and cover c̄ sterile soaked gauze
• Dyspnoea
• IV Abx: cef+met
• Dull bases c̄ ↓AE
• Opioid analgesia
• Call senior and arrange theatre
Mx • Repair in theatre
• Good analgesia to aid coughing § Wash bowel
• Chest physiotherapy § Debride wound edges
§ Close c̄ deep non-absorbable sutures (e.g. nylon)
• May require VAC dressing or grafting

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 Post-op Complications: Specific
General Surgery Vascular

Cholecystectomy Arterial Surgery

• Conversion to open: 5% • Thrombosis and embolisation
• CBD injury: 0.3% • Anastomotic leak
• Bile leak • Graft infection
• Retained stones (needing ERCP)
• Fat intolerance / loose stools Aortic Surgery
• Gut ischaemia
Inguinal Hernia Repair • Renal failure
• Early • Aorto-enteric fistula
• Haematoma / seroma formation: 10% • Anterior spinal syndrome (paraplegia)
• Intra-abdominal injury (lap) • Emboli → distal ischaemia (trash foot)
• Infection: 1%
• Urinary retention Breast
• Late • Arm lymphoedema
§ Recurrence: 0.5% • Skin necrosis
§ Ischaemic orchitis: 0.5% • Seroma
§ Chronic groin pain / paraesthesia: 10-20%
Urological
Appendicectomy • Sepsis
• Abscess formation • Uroma: extravasation of urine
• Fallopian tube trauma
• Right hemicolectomy (e.g. for carcinoid, caecal Prostatectomy
necrosis)
• Urinary incontinence
• Erectile dysfunction
Colonic Surgery • Retrograde ejaculation
• Early • Prostatitis
§ Ileus
§ Anastomotic leak
§ Enterocutaneous fistulae ENT
§ Abdominal or pelvic abscess
• Late Thyroidectomy
§ Adhesions → obstruction • Wound haematoma → tracheal obstruction
§ Incisional hernia • Recurrent laryngeal N. trauma → hoarse voice
§ Transient in 1.5%
Post-op Ileus § Permanent in 0.5%
• Causes § R commonest (more medial)
§ Bowel handling • Hypoparathyroidism → hypocalcaemia
§ Anaesthesia • Thyroid storm
§ Electrolyte imbalance • Hypothyroidism
• Presentation
§ Distension Tracheostomy
§ Constipation ± vomiting • Stenosis
§ Absent bowel sounds • Mediastinitis
• Rx • Surgical emphysema
§ IV fluids + NGT
§ TPN if prolonged Orthopaedic Surgery
Anorectal Surgery Fracture Repair
• Anal incontinence
• Mal-/non-union
• Stenosis
• Osteomyelitis
• Anal fissure
• Avascular necrosis
• Compartment syndrome
Small Bowel Surgery
• Short gut syndrome (≤250cm)
Hip Replacement
• Deep infection
Splenectomy • VTE
O
• Gastric dilatation (2 gastric ileus) • Dislocation
§ Prevent c̄ NGT • Nerve injury: sciatic, SGN
• Thrombocytosis → VTE • Leg length discrepancy
• Infection: encapsulated organisms
Cardiothoracic Surgery
• Pneumo-/haemo-thorax
• Infection: mediastinitis, empyema

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 Post-op Pyrexia
Causes Pneumonia

Early: 0-5d post-op Cause

• Blood transfusion • Anaesthesia → atelectasis
•
O
Physiological: SIRS 2 to trauma, 0-1d • Pain → ↓ cough
• Pulmonary atelectasis: 24-48hrs • Surgery → immunosuppression
• Infection: UTI, superficial thrombophlebitis, cellulitis
• Drug reaction Rx
• Chest physio: encouraging coughing
Delayed: >5d post-op • Good analgesia
• Pneumonia • Abx
• VTE: 5-10d
• Wound infection: 5-7d
• Anastomotic leak: 7d Collection
• Collection: 5-20d
Presentation
• Malaise
Examination of Post-Op Febrile Pt. • Swinging fever, rigors
• Observation chart, notes and drug chart • Localised peritonitis
• Wound • Shoulder tip pain (if subphrenic)
• Abdo + DRE
• Legs Locations
• Chest • Pelvic
• Lines • Subphrenic
• Urine • Paracolic gutters
• Stool • Lesser sac
• Hepatorenal recess (Morrison’s space)
• Small bowel (interloop spaces)
Ix
• Urine: dip + MCS Ix
• Blood: FBC, CRP, cultures ± LFTs • FBC, CRP, cultures
• Cultures: wound swabs, CVP tip for culture • US, CT
• CXR • Diagnostic lap

Rx
• Abx
• Drainage / washout

Cellulitis
• Acute infection of the subcutaneous connective tissue

Cause: β-haemolytic Streps + staph. aureus

Presentation
• Pain, swelling, erythema and warmth
• Systemic upset
• ± lymphadenopathy

Rx
• Benpen IV
• Pen V and fluclox PO

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 Deep Venous Thrombosis
Epidemiology Preventing DVT
• DVTs occur in 25-50% of surgical patients without
thromboprophylaxis Pre-Op
• Pre-op VTE risk assessment
Risk Factors: Virchow’s Triad • TED stockings
• Blood Contents • Aggressive optimisation: esp. hydration
§ Surgery → ↑ plats and ↑ fibrinogen • Stop OCP 4wks pre-op
§ Dehydration
§ Malignancy Intra-Op
§ Age: ↑ • Minimise length of surgery
• Blood Flow • Use minimal access surgery where possible
§ Surgery • Intermittent pneumatic compression boots
§ Immobility
§ Obesity
Post-Op
• Vessel Wall
• LMWH
§ Damage to veins: esp. pelvic veins
§ Previous VTE • Early mobilisation
• Good analgesia
• Physio
Signs • Adequate hydration
• Peak incidence @ 5-10d post-op
• 65% of below knee DVTs are asymptomatic
• Calf warmth, tenderness, erythema, swelling
• Mild pyrexia
• Pitting oedema

Differential
• Cellulitis
• Ruptured Baker’s cyst

Ix
• D-Dimers: sensitive but not specific
• Compression US (clot will be incompressible)
• Thrombophilia screen if:
§ No precipitating factors
§ Recurrent DVT
§ Family Hx

Dx
• Assess probability using Wells’ Score
• Low-probability → perform D-dimers
§ Negative → excludes DVT
§ Positive → Compression US
• Med / High probability → Compression US

Rx

Anticoagulate
• Therapeutic LMWH: enoxaparin 1.5mg/kg/24h SC
• Start warfarin using Tait model: 5mg OD for first 4d
• Stop LMWH when INR 2.5
• Duration
§ Below knee: 6-12wks
§ Above knee: 3-6mo
§ On-going cause: indefinite

Graduated Compression Stockings

• Consider for prevention of post-phlebitic syndrome

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 Other Common Post-Operative Presentations
Dyspnoea / Hypoxia Hypotension

Causes Immediate Mx
• Previous lung disease • Tilt bed head down, give O2
• Atelectasis, aspiration, pneumonia • Assess fluid status
• LVF
• PE Causes: CHOD
• Pneumothorax (e.g. due to CVP line insertion) • Cardiogenic
• Pain → hypoventilation § MI
§ Fluid overload
Ix • Hypovolaemia
• FBC, ABG § Inadequate replacement of fluid losses
• CXR § Haemorrhage
• ECG • Obstructive
§ PE
• Distributive
Rx
§ Sepsis
• Sit up, give O2, monitor SpO2
§ Neurogenic shock
• Rx cause
Mx
• Hypovolaemia → fluid challenge
Reduced Urine Output § 250-500ml colloid over 15-30min
• Haemorrhage → return to theatre
Causes • Sepsis → fluid challenge, start Abx
• Post-renal • Overload → frusemide
§ Commonest cause • Neurogenic → NA infusion
§ Blocked / malsited catheter
§ Acute urinary retention
• Pre-renal: hypovolaemia Hypertension
• Renal: NSAIDs, gentamicin • Continue anti-hypertensives during peri-operative
• Anuria usually = blocked or malsited catheter period
• Oliguria usually = inadequate fluid replacement
Causes
Mx • Pain
• Information • Urinary retention
§ Op Hx • Previous HTN
§ Obs chart: UO
§ Drug chart: nephrotoxins
Rx
• Examination
• Rx cause
§ Assess fluid status
§ Examine for palpable bladder • May use labetalol 50mg IV every 5min (200mg max)
§ Inspect drips, drains, stomas, CVP
• Action
§ Flush c̄ 50ml NS and aspirate back Acute Confusional State
§ Fluid challenge • Agitation, disorientation, attempts to leave hospital

Common Causes: DELIRIUM

Nausea and Vomiting • Drugs: opiates, sedatives, L-DOPA
• Causes • Eyes, ears and other sensory deficits
§ Obstruction • Low O2 states: MI, stroke, PE
§ Ileus • Infection
§ Emetic drugs: e.g. opioids • Retention: stool or urine
• Consider NGT, AXR and ondansetron 4mg IV TDS • Ictal
• Under- hydration / -nutrition
• Metabolic: Na, AKI, glucose, EtOH withdrawal
↓ Na
• What was pre-op level? Mx
• Common Causes: • May need sedation: midazolam / haldol
§ S(I)ADH: pain, nausea, opioids, stress • Nurse in well-lit environment
§ Over administration of IV fluids • Rx cause
• Correct slowly
§ Acute: 1mM/h
§ Chronic:15mM/d

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 Fluid Homeostasis
Body Composition Fluid Balance
• Total water: 60% of 70kg = 42L
§ 2/3 intracellular = 28L Input Output
§ 1/3 extracellular = 14L Water 1500 Urine 1500
- Plasma = 3L (21% of ECF) Food 1000 Stool 300
- Interstitial = 10L Metabolism 300 Insensible 1000
- Transcellular = 1L
- lungs
- sweating
Total 2800ml Total 2800ml
Starling’s Forces = =
40ml/kg/d 40ml/kg/d
Osmotic Pressure
• Pressure which needs to be applied to prevent the Other Values
inflow of water across a semipermeable membrane. • Minimum UO = 0.5ml/kg/h = ~30ml/h
• i.e. the ability of a solute to attract water. • Na requirement = 1.5-2mmol/kg/d = 100mmol/d
• Oncotic pressure: form of osmotic pressure exerted • K requirement = 1mmol/kg/d = 60mM/d
by proteins.
Fluid Regimens
Hydrostatic Pressure
• Pressure exerted by a fluid at equilibrium due to the
Daily Requirements
force of gravity. +
• 3L dex-saline c̄ 20mM K in each bag
+
• 1L NS + 2L dex c̄ 20mM K in each bag
• Each bag over 8h = 125ml/h
Distribution
• Distribution between the ECF and ICF is driven by
Replace other losses
differences in osmotic pressure only.
• Vomiting and Diarrhoea
• Distribution w/i the ECF is determined by Starling’s
• NGT
forces.
• Drains
§ Capillary and interstitial oncotic pressure. O
§ Capillary and interstitial hydrostatic pressure. • Fever (+500ml for each C)
§ Filtration coefficient (capillary permeability) • Tachypnoea
• High-output stomas

3rd Space Losses → ↓ ECF

•
rd
Bowel obstruction → ↓ fluid reabsorption → 3 space
CVP Monitoring
loss • Indicates RV preload and depends on
• Sudden diuresis on day 2-3 post op = recovery of § Venous return
ileus § Cardiac output
•
rd
Peritonitis → ascites → 3 space loss • ↑ CVP
§ ↑ circulating volume
§ ↓CO: i.e. pump failure
• ↓ CVP
§ ↓ circulating volume
• Normal value: ~5-10cmH2O
• Single reading is not as useful as serial measurements
before and after fluid challenge.
§ Unchanged: hypovolaemic
§ ↑ that reverses after 30min: euvolaemic
§ Sustained ↑ >5cmH2O: overload / failure
• Passive leg raising may be more useful than fluid
challenge in determining response to fluids.
§ Sustained ↑ in CVP suggests heart failure.

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© Alasdair Scott, 2018
Crystalloid Colloid
Normal Saline Physiology
• Contain large molecular wt. molecules
Contents • Gelatin
• 0.9% NaCl = 9g/L • Dextrans
• 154mmol NaCl • Preserves oncotic pressure \ remains intravascular →
preferential ↑ in intravascular volume
pH: 5-6
Synthetic
Use • Gelofusin
• Normal daily fluid requirements + replace losses • Volplex
• Haemaccel
• Voluven
5% Dextrose
Natural
Contents • Albumin
• 50g dextrose /L • Blood

Use Use
• Normal daily fluid requirements • Fluid challenge: 250-500ml over 15-30min
• Hypovolaemic shock
• Mount Vernon Formula
§ (wt. x %burn)/2 = ml colloid per unit time
Dextrose-Saline
Problems
Contents • Anaphylaxis
• 4% dextrose = 40g/L
• Volume overload
• 0.18% NaCl = 31mM NaCl • Can interfere c̄ cross-matching therefore take blood for
x-match before using.
Use
• Normal daily fluid requirements

Hartmann’s / Ringer’s Lactate

Contents
• Na: 131mM
• Cl: 111mM
• K: 5mM
• Ca: 2.2mM
• Lactate / HCO3: 29mM

Use
• Resuscitation in trauma pts.
st
• Parkland’s formula: 4 x wt x %burn = mL in 1 24hrs

pH
• pH = 6.5 but Hartmann’s is an alkalinising solution
• Lactate is not an acid in itself: it’s a conjugate base
• Given exogenously as sodium lactate
• Lactate metabolised in liver → HCO3 production
• The Cori Cycle

Daily Requirements
+
• 3L dex-saline c̄ 20mM K in each bag
+
• 1L NS + 2L dex c̄ 20mM K in each bag
• Each bag over 8h = 125ml/h

Problems
• Give 1L NS → ~210ml remaining intravascularly
• Give 1L D5W → ~70ml remaining intravascularly
• Acidosis or electrolyte disturbances
• Fluid overload

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 Fluid Problems
Assessing Fluid Status Ileostomy
• Hx: balance chart, surgery, other losses, thirsty • Ileal fluid composition
• Impression: drowsy, alert § Na: 130mM
• Inspection: drips, drains, stomas, catheters, CVP § Cl: 110mM
§ K: 10mM
Examination § HCO3: 30mM
• Normal output: 10-15mL/Kg/d = ~700ml/d
• IV volume
§ CRT • High output = >1000ml/d
§ HR • Ileum will adapt to limit fluid and electrolyte losses
§ BP lying and standing • Fluids
§ JVP § 0.9% NS +KCl
• Tissue perfusion § Daily requirements + replaces losses, titrated to
§ Skin turgor UO
§ Oedema: ankle, pulmonary, ascites § Serial U+Es guide electrolyte replacement
§ Mucus membranes • High Output
• End-organ § Loperamide
§ UO, ↑U+Cr § Codeine
§ Consciousness
§ Lactate
Reduced Urine Output Post-op
Other Tests
• PCWP: indirect measure of left atrial pressure Causes
• CVP • Post-renal
§ Commonest cause
Post-operative Fluids § Blocked / malsited catheter
§ Acute urinary retention
Problems • Pre-renal: hypovolaemia
• Renal: NSAIDs, gentamicin
• ↑ADH, ↑aldosterone, ↑cortisol → Na +H2O conservation
+ • Anuria usually = blocked or malsited catheter
• ↑ K : tissue damage, transfusion, stress hormones
• Oliguria usually = inadequate fluid replacement
Solutions
Mx
• Use UO (aim>30ml/h) to guide fluid replacement but
may need to ↓ maintenance fluids to 2L first 24h post- • Information
op § Op Hx
+ § Obs chart: UO
• Avoid K supplementation for first 24h post-op
§ Drug chart: nephrotoxins
• Examination
Cardiac or Renal Failure § Assess fluid status
§ Examine for palpable bladder
Problem § Inspect drips, drains, stomas, CVP
• RAS activation → Na and H2O retention • Action
§ Flush c̄ 50ml NS and aspirate back
Solution § Fluid challenge
• Avoid fluids c̄ Na → give 5% dextrose
Suspect Catheter Problem
• Flush c̄ 50ml NS and aspirate back
Bowel Obstruction
• Pts. have significant third space losses c̄ loss of both
water and electrolytes. Suspect Pre-renal Problem
• Likely to need significantly more than standard daily • Fluid challenge
requirements. § 250-500ml colloid bolus over 15-30min
• Regimen § Look for CVP or UO response w/i minutes
§ 0.9% NS c̄ 20-40mm KCl added to each bag
§ Titrate rate of fluid therapy to clinical findings on
serial examination.
§ Serial U+Es guide electrolyte replacement

Pancreatitis
• Inflammation → significant fluid shift into the abdomen.
• Pts require aggressive fluid resuscitation and
maintenance
§ Insert urinary catheter and consider CVP
monitoring
§ 0.9% NS c̄ 20-40mm KCl added to each bag
§ Keep UO >30ml/h
§ Serial U+Es guide electrolyte replacement

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 Nutrition
Assessment Parenteral Nutrition
• May be “Total” or used to supplement enteral feeding
Clinical • Combined c̄ H2O to deliver total daily requirements
• Hx
§ Wt. loss
§ Diet Indications
• Examination • Prolonged obstruction or ileus (>7d)
§ Skin fat • High output fistula
§ Dry hair • Short bowel syndrome
§ Pressure sores • Severe Crohn’s
§ Cheilitis • Severe malnutrition
2
§ Wt. and BMI (<20kg/m )
• Severe pancreatitis
• Unable to swallow: e.g. oesophageal Ca
Anthropometric
• Skin-fold thickness
• Arm circumference
Delivery
• Delivered centrally as high osmolality is toxic to veins
Ix § Short-term: CV catheter
• Albumin § Long-term: Hickman or PICC line
• Transthyretin (prealbumin) • Sterility is essential: use line only for PN
• Phosphate
Monitoring
Requirements (/kg/24h) • Standard
• Calories: 20-40 Kcal § Wt., fluid balance and urine glucose daily
• Carb: 2g § Zn, Mg weekly
• Fat: 3g • Initially
• Protein: 0.5-1g § Blood glucose, FBC, U+E + PO4 3x /wk
• Nitrogen:0.2-0.4g § LFTs 3x /wk
• Once stable
Enteral Nutrition § Blood glucose, FBC, U+E + PO4 daily
§ LFTs weekly
Delivery
• PO is best
§ Consider semi-solid diet if risk of aspiration Contents
• Fine bore NGT (9 Fr) • 2000Kcal: 50% fat, 50% carb
• Percutaneous Endoscopic Gastrostomy • 10-14g nitrogen
• Jejunostomy • Vitamins, minerals and trace elements
• Build up feeds gradually to prevent diarrhoea

Feeds Complications
• Oral supplements • Line-related
• Polymeric: e.g. osmolite, jevity § Pneumothorax / haemothorax
§ Intact proteins, starches and long-chain FAs § Cardiac arrhythmia
• Disease-specific § Line sepsis
§ e.g. ↓ branched chain AAs in hepatic § Central venous thrombosis → PE or SVCO
encephalopathy • Feed-related
• Elemental § Villous atrophy of GIT
§ Simple AAs and oligo/monosaccharides § Electrolyte disturbances
§ Require minimal digestion and used if abnormal - Refeeding syndrome
GIT: e.g. in Crohn’s - Hypercapnoea from excessive CO2
production
Indications § Hyperglycaemia and reactive hypoglycaemia
§ Line sepsis: ↑ risk c̄ TPN
• Catabolic: sepsis, burns, major surgery
§ Vitamin and mineral deficiencies
• Coma/ITU
• Malnutrition
• Dysphagia: stricture, stroke

Complications
• NGT
§ Nasal trauma
§ Malposition or tube blockage
• Feeding
§ Feed intolerance → diarrhoea
§ Electrolyte imbalance
§ Aspiration
§ Refeeding syndrome
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Refeeding Syndrome
Definition
• Life-threatening metabolic complication of refeeding
via any route after a prolonged period of starvation.

Pathophysiology
• ↓ carbs → catabolic state c̄ ↓insulin, fat and protein
catabolism and depletion of intracellular PO4
• Refeeding → ↑ insulin in response to carbs and ↑
cellular PO4 uptake.
• → hypophosphataemia
§ Rhabdomyolysis
§ Respiratory insufficiency
§ Arrhythmias
§ Shock
§ Seizures

Chemistry
• ↓K, ↓Mg, ↓PO4

At-risk Patients
• Malignancy
• Anorexia nervosa
• Alcoholism
• GI surgery
• Starvation

Prevention
• Identify and monitor at-risk patients
• Liaise c̄ dietician

Rx
• Identify at-risk pts in advance and liaise c̄ dietician
• Parenteral and oral PO4 supplementation

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© Alasdair Scott, 2018
 Primary Survey
ADDRESS PROBLEMS IN ORDER CABDE Circulation
• Two-large bore cannulae (14/16G) in each ACF
Airway and C-Spine • FBC, U+E, x-match (6U), clotting, VBG

Airway Assessment
• Check for airway compromise • Inspection: pale, sweaty, active bleeding
§ Ask pt. a question • Vascular status: BP, HR, JVP, heart sounds, cardiac
§ Stridor mon
§ Orofacial injury or burns • End-organ: consciousness, UO
§ Visualise airway and use suction if necessary
• Manoeuvres to open airway Sites of Haemorrhage
§ Jaw thrust • Chest
• Adjuncts if compromise / potential compromised • Abdomen
§ NPA: gag reflex present • Pelvis: use pelvic binder
§ OPA: no gag reflex (stop tongue swallowing) • Floor
• Emergency airways
§ Needle cricothyroidotomy or surgical cric Mx
• Definitive airways (no risk of aspiration) • If haemodynamic compromise give 2L warmed
§ Endotracheal tube Hartmann’s stat.
§ Tracheostomy • Consider further colloid / blood
• Insert CVP and catheter (after PR) to guide resus
C-Spine
• Maintain in-line cervical support to keep neck stable Response
• Place pt. in hard-collar and sandbags c̄ tape • Assess response to fluids using UO, lactate, BP

Rapid
Breathing • Usually <20% loss
• Start 15L O2 via non-rebreathe mask (Hudson) • Slow fluid to maintenance if haemodynamically
stable
Assessment
• SpO2 Transient
• Inspection of chest • 20-40% loss
• Position of trachea • On-going losses or inadequate resuscitation
• RR and chest expansion
• Breath sounds, vocal resonance None
• Percussion • Exsanguinating haemorrhage → theatre
• ABG • Consider non-haemorrhagic shock
§ Tamponade
Tension Pneumothorax § Pneumothorax
• Signs
§ Respiratory distress
§ ↑JVP and ↓BP Disability
§ Tracheal deviation + displaced apex
§ ↓ air entry and ↓ VR Assessment
§ Hyperresonant percussion
• Assess consciousness using AVPU or GCS
• Rx: immediate decompression
nd • Pupil responses
§ Insert large-bore venflon into 2 ICS, mid-
clavicular line.
§ Insert ICD later
Exposure
Open Sucking Chest Wounds
• Convert to closed wound by covering with damp Assessment
occlusive dressing stuck down on 3 sides. • Completely undress pt.
• Perform log-role and PR
§ Feel for high riding prostate (urethral rupture)
§ Look for bleeding
• Prevent hypothermia

REPEAT 1O SURVEY AGAIN!

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© Alasdair Scott, 2018
Secondary Survey Shock
History
Haemorrhagic Shock
• Allergies
• Circulating blood volume = 7% body mass
• Medication

HTTHW
• PMH
• Last ate / drunk % ml RR HR BP UO Mental
• Events 1 0-15 750 ↔ ↔ ↔ ↔ Normal
2 15-30 750-1500 >20 >100 ↔ <30 Anxious++
3 30-40 1500-2000 >30 >120 ↓ 5-20 Confused
Examination 4 >40 >2000 >35 >140 ↓↓ <5 Lethargic
• Head-to-toe examination
• Examine every system

Ix Neurogenic Shock
• Trauma series • Disruption of sympathetic nervous system
§ C-spine: lat + peg
§ CXR Causes
§ Pelvis • Spinal anaesthesia
• FAST scan (Focussed Assessment c̄ Sonography in • Hypoglycaemia
Trauma) • Cord injury above T5
• CT: when pt. is stable. • Closed head injuries

Presentation
Assessing C-spine Radiographs • Hypotension
• Views • Bradycardia
§ Lateral • Warm extremities
§ AP
§ Open-mouth Peg view Mx
• Adequacy • Vasopressors: vasopressin and norad
§ Must see C7-T1 junction • Atropine: reverse the bradycardia
§ May need swimmer’s view c̄ abducted arm
• Alignment: 4 lines
§ Ant. vertebral bodies
§ Ant. vertebral canal Spinal Shock
§ Post. vertebral canal • Acute spinal cord transection
§ Tips of spinous processes • Loss of all voluntary and reflex activity below the level of injury
• Bones: shapes of bodies, laminae, processes
• Cartilage: IV discs should be equal height Presentation
• Soft tissue • Hypotonic paralysis
§ Width of soft tissue shadow anterior to upper • Areflexia
vertebrae should be 50% of vertebral width. • Loss of sensation
• Urinary retention

Clearing the C-Spine

Clinical Clearance
• Indication: NEXUS Criteria
§ Fully alert and orientated
§ No head injury
§ No drugs or alcohol
§ No neck pain
§ No abnormal neurology
§ No distracting injury
• Method
§ Examine for bruising or deformity
§ Palpate for deformity and tenderness
§ Ensure pain-free active movement

Radiological Clearance
• Indications
§ Pt. doesn’t meet criteria for clinical clearance
• Modalities
§ Radiograph initially
- Clear if normal XR and clinical exam
§ CT C-spine if abnormal XR or clinical exam

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© Alasdair Scott, 2018
Life-Threatening Chest Injuries 2O Survey Chest Injuries
Differential: ATOMIC Rib #
th th
• Airway obstruction • Usually 5 -9 ribs
• Tension Pneumothorax • # of upper 4 ribs = high energy trauma
• Open pneumothorax (sucking) • Complications
• Massive haemothorax § Pneumothorax
• Intercostal disruption and pulmonary contusion § Lacerate thoracic or abdominal viscera
• Cardiac Tamponade • Rx: good analgesia
§ NSAIDs + opioids
§ Intrapleural analgesia
Massive Haemothorax § Intercostal block
• Accumulation of >1.5L of blood in chest cavity
• Usually caused by disruption of hilar vessels
Sternal #
• Usually MVA driver vs. steering wheel
Presentation
• Risk of mediastinal injury
• Signs of chest wall trauma
• Rx
• ↓BP
§ Analgesia, admit, observe
• ↓ expansion
§ Cardiac monitor
• ↓ breath sounds and ↓VR § Troponin: rule out myocardial contusion
• Stony dull percussion
Pulmonary Contusion
Mx
• Usually due to rapid deceleration injury or shock waves
• X-match 6u
• May → ARDS
• Large-bore chest drain c̄ hep saline for autotransfusion
• Pres: dyspnoea, haemoptysis, respiratory failure
• Thoracotomy if >1.5L or >200ml/h • Ix
§ CXR: opacification
Flail Chest § Serial ABGs: ↓ PaO2:FiO2 ratio
• Ant. or lat. # of ≥2 adjacent ribs in ≥2 places • Rx: O2, ventilate if necessary
• Flail segment moves paradoxically c̄ respiration
• ↓ Oxygenation Myocardial Contusion
§ Underlying pulmonary contusion • Direct blunt trauma over precordium
§ ↓ Ventilation of affected segment • Ix
§ ECG: abnormal, arrhythmias
Ix § ↑ troponin
• CXR / CT chest: pulmonary contusion (white) • Rx: bed rest, cardiac monitoring, Rx arrhythmias
• Serial ABGs: ↓PaO2:FiO2 ratio
Contained Aortic Disruption
Rx • Rapid deceleration injury (80% immediately fatal)
• O2 • Pres: initially stable but → hypotensive
• Good analgesia: PCA, epidural • Ix
• Persistent respiratory failure: PPV § CXR: wide mediastinum, deviation of NGT
§ CT
Cardiac Tamponade • Rx: cardiothoracic consult
• Disruption of myocardium or great vessels → blood in the
pericardium → ↓ filling and contraction → shock Diaphragmatic Injury
th
• Usually results from penetrating trauma • Consider in penetrating injuries below 5 rib or high
energy compression.
Presentation • Ix: CXR (visceral herniation), CT
• Beck’s Triad
§ ↑ JVP / distended neck veins Oesophageal Disruption
§ ↓ BP • Usually penetrating trauma
§ Muffled heart sounds • → mediastinitis
• Pulsus paradoxus: SBP fall of >10mmHg on inspiration • Ix
• Kussmaul’s sign: ↑ JVP on inspiration § CXR: pneumomediastinum, surgical
• Intensely restless pt. emphysema
§ CT
Ix
• US: FAST or transthoracic echo Tracheobronchial Disruption
• CXR: enlarged pericardium • Presentation
• ↑CVP >12mmHg § Persistent pneumothorax
• ECG: low voltage QRS ± electrical alternans § Pneumomediastinum
• Rx: thoracotomy
Mx
• Pericardiocentesis: spinal needle in R subxiphoid space
O
aiming at 45 towards the R tip of left scapula
• Thoracotomy may be needed

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© Alasdair Scott, 2018
 Abdominal Trauma
Mechanisms Damage Control Surgery
• Penetrating
§ All require exploration as tract may be deeper Aim
than it appears. • Early Mx of abdominal trauma should focus on “damage
• Blunt control” to limit physiological stress.
§ Have a high index of suspicion for taking to § Control haemorrhage: ligation and packing
theatre. § Control contamination
§ Stabilise in ITU
Specific Ix
Spleen
Urine Dip • Kehr’s Sign
O
• Haematuria suggests injury to renal tract § Shoulder tip pain 2 to blood in the peritoneal
cavity.
§ Left Kehr sign is classic symptom of ruptured
FAST Scan spleen
• Replacing DPL in most centres • Classification
• Check for fluid in the abdomen, pelvis and pericardium. § 1: capsular tear
§ 90% sensitive for free fluid § 2: Tear + parenchymal injury
• Can be extended to look for pneumothoraces § 3: Tear up to the hilum
§ 4: Complete fracture
Diagnostic Peritoneal Lavage • Mx
• Advantages and Disadvantages § Haemodynamically unstable: laparotomy
§ 98% sensitive for intra-abdominal haemorrhage § Stable 1-3: observation in HDU
§ Useful if FAST unavailable § Stable 4: consider laparotomy
§ May be better for identifying injury to hollow viscus - Suture lac or partial / complete
§ Unable to identify retroperitoneal injury splenectomy
• Insert urinary catheter and NGT
§ Decompression to minimise risk of injury Liver
• Midline incision through skin and fascia @ 1/3 distance • Conservative if capsule is intact
form umbilicus to pubic symphysis (arcuate line). • Suture laceration
• Carefully dissect to the peritoneum and insert a urinary • Partial hepatectomy
catheter. • Packing
• Instil 10ml/kg warmed Hartmann’s
• Drain fluid back into bag and send sample to lab.
3 Bowel
• +ve = >100,000 RBCs/mm , bile/intestinal contents
• Resection may be required

Indications for Laparatomy Bladder (often assoc. c̄ pelvic injury)

• Unexplained shock
• Intraperitoneal rupture requires laparoscopic repair c̄
• Peritonism: rigid silent abdomen
urethral and suprapubic drainage
• Evisceration: bowel or omentum
• Extraperitoneal rupture can be treated conservatively c̄
• Radiological evidence of intraperitoneal gas
urethral drainage.
• Radiological evidence of ruptured diaphragm
• Give prophylactic Abx
• Gunshot wounds
• +ve DPL or CT
Urethra
• Classification
§ Anterior
- Spongy urethra (penile + bulbar)
- Occur following straddling injuries or
instrumentation
§ Posterior
- Membranous urethra
- Occur following pelvic #s
• Presentation
§ Often assoc. c̄ pelvic fracture
§ Blood in the urethral meatus or scrotum
§ Perineal bruising
§ High-riding prostate
§ Inability to micturate + palpable bladder
• Ix
§ Retrograde urethrogram
• Mx
§ Suprapubic catheter
§ Surgical repair

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© Alasdair Scott, 2018
 Head Injury
Epidemiology History
• Head injury, alone or in combination c̄ other injuries, is • LOC
the commonest cause of trauma death (50%) • Amnesia: anterograde worse
• Nausea / vomiting
Primary Brain Injury • Fits
• Occurs at time of injury and is a result of direct or indirect • Focal neurology
injury to brain tissue. • Mechanism
• Drugs: e.g. antiplats, warfarin
Diffuse
• Concussion / Mild Traumatic Brain Injury Examination
§ Temporary ↓ in brain function • GCS: E4, V5, M6
§ Headache, confusion, visual symptoms, amnesia, § 3-8 = coma
nausea § 9-12 = moderate head injury
• Diffuse Axonal Injury § 13-15 = mild head injury
§ Shearing forces disrupt axons • Scalp lacerations
§ May → coma and persistent vegetative state
§ Autonomic dysfunction → fever, HTN, sweating Basal Skull #
• CSF rhinorrhoea or otorrhoea (Test: halo sign)
Focal • Battle sign: bruised mastoid
• Contusion • Pando sign: bilateral orbital bruising
§ E.g. coup and contra-coup • Haemotympanum
§ May have focal neurological deficit
Ix
• Intracranial Haemorrhage • C-spine
§ Extradural • CT Head
§ Subdural
§ Basal or other skull #
§ Subarachnoid
§ Amnesia: > 30min retrograde (before event)
§ Parenchymal haemorrhage and laceration
§ Neurological deficit: e.g. seizures
§ GCS: <13 @ scene, <15 2h later
Secondary Brain Injury § Sick: vomiting > 1
• Occurs after primary injury. • Bloods: FBC, U+E, glucose, clotting, EtOH level, ABG

Causes Mx
• Hypoxia • Neurosurgical consult if +ve CT
• Hypercapnoea • Admit if
• Hypotension § LOC >5min
• ↑ ICP § Abnormalities on imaging
• Infection § Difficult to assess: EtOH, post-ictal
§ Not returned to GCS 15 after imaging
Monroe-Kelly Doctrine § CNS signs: persistent vomiting, severe headache
•
Cranium is rigid box \ total volume of intracranial • Neuro obs: half hrly until GCS 15/15
contents must remain constant if ICP is not to change. § GCS, pupils, TPR, BP
• ↑ in volume of one constituent → compensatory ↓ in • Analgesia: codeine phosphate 30-60mg PO/IM QDS
another: • Suture scalp lacs
§ CSF • Abx: if open / base of skull #
§ Blood (esp. venous)
• These mechanisms can compensate for a volume Intubate if
change of ~100ml before ICP ↑. • GCS ≤ 8
§ As autoregulation fails, ICP ↑ rapidly → herniation. • PaO2 <9KPa on air / <13KPa on O2 or PCO2 >6KPa
• Spontaneous hyperventilation: PCO2 <4KPa
Cerebral Blood Flow • Respiratory irregularity
• CBF µ CPP x radius of vessels
• CPP = MABP – ICP Rx ↑ ICP
• ↑ ICP → ↓CPP → ↓CBF • Elevate bed
§ Autoreg → vasodilatation → ↑ volume → ↑ICP… • Good sedation, analgesia ± NM block
• Prevent or attenuate this vicious circle by • Neuroprotective ventilation
§ Ventilate to normocapnoea: 4.5KPa • Mannitol or hypertonic saline
§ IV fluid to normovolaemia
§ Mannitol bolus acutely
Discharge Advice
• Stay with someone for first 48hrs
Cushing Reflex: imminent herniation • Give advice card advising return on:
• Hypertension § Confusion, drowsiness, LOC, fits
• Bradycardia § Visual problems
• Irregular breathing § V. painful headache that won’t go away
§ Vomiting

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© Alasdair Scott, 2018
 Burns
Risk Factors Mx
• Age: children and elderly • Based on ATLS principals
• Co-morbidities: epilepsy, CVA, dementia, mental illness • Specific concerns c̄ burns
• Occupation § Secure airway
§ Manage fluid loss
Classification § Prevent infection

Superficial Airway
• Erythema • Examine for respiratory burns
• Painful § Soot in oral or nasal cavity
• E.g. sunburn § Burnt nasal hairs
§ Hoarse voice, stridor
• Flexible laryngoscopy can be helpful
Partial Thickness
• Consider early intubation + dexamethasone (↓ inflam)
• Heal w/i 2-3wks if not complicated
• Superficial
§ No loss of dermis
Breathing
§ Painful • 100% O2
§ Blisters • Exclude constricting burns
• Deep • Signs of CO poisoning
§ Loss of dermis but adnexae remain § Headache
§ Healing from adnexae: e.g. follicles § n/v
§ V. painful § Confusion
§ Cherry red appearance
Full Thickness • ABG
§ COHb level
• Complete loss of dermis
§ SpO2 unreliable if CO poisoning
• Charred, waxy, white, skin
• Anaesthetic
Circulation
• Heal from the edges → scar
• Fluid losses may be huge
• 2x large-bore cannulae in each ACF
• Bloods: FBC, U+E, G+S/XM
Complications • Start 2L warmed Hartmann’s immediately
• Formula guide additional fluid requirements in burns pts.
Early
• Infection: loss of barrier function, necrotic tissue, SIRS st
Parkland Formula to guide replacement in 1 24hrs
• Hypovolaemia: loss of fluid in skin + ↑ cap permeability • 4 x wt. (kg) x % burn = mL of Hartmann’s in 24h
• Metabolic disturbance: ↑↑K, ↑↑myoglobin, ↑Hb → AKI • Replace fluid from time of burn
• Compartment syndrome: circumferential burns •
st
Give half in 1 8h
• Peptic ulcers: Curling’s ulcers • Best guide is UO: 30-50mL/h
• Pulmonary: laryngeal oedema, CO poisoning, ARDS
• Renal and hepatic impairment Muir and Barclay Formula to guide fluid replacement
• (wt. x % burn)/2 = mL of Colloid per unit time
Intermediate • Time units: 4, 4, 4, 6, 6, 12 = 36hrs total
• VTE • May need to use blood
• Pressure sores
Burn Treatments
Late • Analgesia: morphine
• Scarring • Dress partial thickness burns
• Contractures § Biological: e.g. cadaveric skin
• Psychological problems § Synthetic
§ Cream: e.g. Flamazine (silver sulfadiazine) +
sterile film
Assessment • Full thickness burns
§ Tangential excision debridement
§ Split-thickness skin grafts
Wallace Rule of 9s: % body surface area burnt • Circumferential burns may require escharotomy to
• Head and neck: 9% prevent compartment syndrome.
• Arms: 9% each • Anti-tetanus toxoid (0.5ml ATT)
• Torso: 18% front and back • Consider prophylactic Abx: esp. anti-pseudommonal
• Legs: 18% each
• Perineum: 1%
• (Palm: 1%)

NB. may also use Lund and Browder charts

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© Alasdair Scott, 2018
 Hypothermia
Definition Ix
•
O
Core (rectal) temperature <35 C • Rectal / ear temperature
• FBC, U+E, glucose
• TFTs, blood gas
Pathophysiology • ECG
• Body heat is lost via 4 mechanisms § J waves: between QRS and T wave
§ Arrhythmias
1. Radiation: 60%
§ Infra-red emissions Mx
• Cardiac monitor
2. Conduction: 15% • Warm IVI 0.9% NS
§ Direct contact • Urinary catheter
O
§ 1 means in cold water immersion • Consider Abx for prevention of pneumonia
§ Routine if temp <32 and >65yrs
3. Convection: 15%
§ Removes warmed air from around the body Slowly Rewarm
§ ↑d in windy environments • Reheating too quickly → peripheral vasodilatation and
shock.
4. Evaporation: 10% •
O
Aim for 0.5 C /hr
§ Removal of warmed water • Passive external: blankets, warm drinks
§ ↑ in dry, windy environments
• Active external: warm water or warmed air
• Active internal: mediastinal lavage and CPB
§ Severe hypothermia only
Aetiological Classification
• Primary: environmental exposure
• Secondary: change in temperature set-point Complications
§ E.g.: age-related, hypothyroidism, autonomic
• Arrhythmias
neuropathy
• Pneumonia
• Coagulopathy
• Acute renal failure
Presentation
O
Mild: 32 – 35 C
• Shivering
• Tachycardia
• Vasoconstriction
• Apathy
O
Moderate: 28 – 32 C
• Dysrhythmia, bradycardia, hypotension
• J waves
• ↓ reflexes, dilated pupils, ↓ GCS
O
Severe: <28 C
• VT → VF → Cardiogenic shock
• Apnoea
• Non-reactive pupils
• Coagulopathy
• Oliguria
• Pulmonary oedema

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© Alasdair Scott, 2018
 Dysphagia
Oesophageal Anatomy Achalasia
• 25cm long muscular tube (40cm from GOJ → lips) • Pathophysiology
• Starts at level of cricoid cartilage (C6) § Degeneration of myenteric plexus (Auerbach’s)
• In the neck lies in the visceral column § ↓ peristalsis
• Runs in posterior mediastinum and passes through § LOS fails to relax
right crus of diaphragm @ T10. • Cause
O
• Continues for 2-3cm before entering the cardia § 1 / idiopathic: commonest
O
• 3 locations of narrowing § 2 : Chagas’ disease (T. cruzii)
§ Level of cricoid • Presentation
§ Posterior to left main bronchus and aortic arch § Dysphagia: liquids then solids
§ LOS § Regurgitation (esp. at night)
rds
• Divided into 3 : reflects change in musculature from § Substernal cramps
striated → mixed → smooth. § Wt. loss
• Lined by non-keratinising squamous epithelium. • Comps: Chronic → oesophageal SCC in 3-5%
• Z-line: transition from squamous → gastric columnar • Ix
§ Ba swallow: dilated tapering oesophagus
- Bird’s beak
§ Manometry: failure of relaxation + ↓ peristalsis
Causes § CXR: widened mediastinum, double RH border
§ OGD: exclude malignancy
Inflammatory • Rx:
• Tonsillitis, pharyngitis § Med: CCBs, nitrates
• Oesophagitis: GORD, candida § Int: botox injection, endoscopic balloon
• Oral candidiasis dilatation
• Aphthous ulcers § Surg: Heller’s cardiomyotomy (typically lap)

Neurological / Motility Disorders Pharyngeal Pouch: Zenker’s Diverticulum

• Local • Outpouching between crico- and thyro-pharyngeal
§ Achalasia components of the inf. pharyngeal constrictor.
§ Diffuse oesophageal spasm § Area of weakness = Killian’s dehiscence.
§ Nutcracker oesophagus • Defect usually occurs posteriorly but swelling usually
§ Bulbar / pseudobulbar palsy (CVA, MND) bulges to left side of neck.
• Systemic • Food debris → pouch expansion → oesophageal
§ Systemic sclerosis / CREST compression → dysphagia.
§ MG • Pres: Regurgitation, halitosis, gurgling sounds
• Rx: excision, endoscopic stapling
Mechanical Obstruction
• Luminal Diffuse Oesophageal Spasm
§ FB • Intermittent severe chest pain ± dysphagia
§ Large food bolus • Ba swallow shows corkscrew oesophagus
• Mural
§ Benign stricture
§ Web (e.g. Plummer-Vinson) Nutcracker Oesophagus
§ Oesophagitis • Intermittent dysphagia ± chest pain
§ Trauma (e.g. OGD) • ↑ contraction pressure c̄ normal peristalsis
§ Malignant stricture
§ Pharynx, oesophagus, gastric Plummer-Vinson Syndrome
§ Pharyngeal pouch • Severe IDA → hyperkeratinisation of upper 3 of
rd

• Extra-Mural oesophagus → web formation

§ Retrosternal goitre • Pre-malignant: 20% risk of SCC
§ Rolling hiatus hernia
§ Lung Ca
§ Mediastinal LNs (e.g. lymphoma) Oesophageal Rupture
§ Thoracic aortic aneurysm • Iatrogenic (85-90%): endoscopy, biopsy, dilatation
• Violent emesis: Boerhaave’s syndrome
• Carcinoma
• Caustic ingestion
Ix
• Trauma: surgical emphysema ± pneumothorax
• Upper GI endoscopy
• Ba swallow
Features
• Manometry
• Odonophagia
• Mediastinitis: tachypnoea, dyspnoea, fever, shock
• Surgical emphysema

Mx
• Iatrogenic: PPI, NGT, Abx
• Other: resus, PPI, Abx, antifungals, debridement +
formation of oesophago-cutaneous fistula c̄ T-tube
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 Oesophageal Cancer
Staging: TNM
Epidemiology
• Tis: carcinoma in situ
• Incidence: 12/100,000, increasing (↑ Barrett’s)
• T1: submucosa
• Age: 50-70 yrs
• T2: muscularis propria (circ / long)
• Sex: M>F = 5:1
• T3 adventicia
• Geo: ↑ Iran, Transkei, China
• T4: adjacent structures
• N1: regional nodes
Risk Factors • M1: distant mets
• EtOH
• Smoking
• Achalasia
Rx
• Discuss in an MDT
• GORD → Barrett’s
§ Upper GI surgeon + gastroenterologist
• Plummer-Vinson
§ Radiologist
• Fatty diet
§ Pathologist
• ↓ vit A+C § Oncologist
• Nitrosamine exposure § Specialist nurses
§ Macmillan nurses
Pathophysiology § Palliative care
• 65% adenocarcinoma
rd
§ Lower 3 Surgical: oesophagectomy
§ GORD → Barrett’s → dysplasia → Ca • Only 25-30% have resectable tumours
• 35% SCC • May be offered neo-adjuvant chemo before surgery to
rds
§ Upper and middle 3 downstage tumour: e.g. cisplatin + 5FU
§ Assoc. c̄ EtOH and smoking • Approaches
§ Commonest type worldwide § Ivor-Lewis (2 stage): abdominal + R
thoracotomy
Presentation § McKeown (3 stage): abdominal + R
• Progressive dysphagia: solids → liquids (esp. bread) thoracotomy + left neck incision
§ Often alter dietary habit → soft food → § Trans-hiatal: abdominal incision
exacerbation of wt. loss. • Prognosis
• Wt. loss § Stage dependent
• Retrosternal chest pain § ~15% 5ys
• Lymphadenopathy
• Upper 3 :
rd Palliative
§ Hoarseness: recurrent laryngeal N. invasion • Majority of pts.
§ Cough ± aspiration pneumonia • Laser coagulation
• Alcohol injection + ↓ Ascites (spiro)
Spread • Stenting and Secretion reduction (e.g. hyoscine patch)
• Direct extension, lymphatics and blood • Analgesia: e.g. fentanyl patches
• 75% of pts have mets @ Dx • Radiotherapy: external or brachytherapy
• Referral
§ Palliative care team
Ix § Macmillan nurses
• Bloods
• Prognosis
§ FBC: anaemia
§ 5ys <5%
§ LFTs: hepatic mets, albumin
§ Median: 4mo
• Diagnosis
§ Upper GI endoscopy: allows biopsy
§ Ba swallow: not often used, apple-core
stricture Benign Tumours
• Staging: TNM • Leiomyoma
§ CT • Lipomas
§ EUS • Haemangiomas
§ Laparoscopy / mediastinoscopy: mets • Benign polyps

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 GORD
Pathophysiology Rx
• LOS dysfunction → reflux of gastric contents →
oesophagitis. Conservative
• Lose wt.
Risk Factors • Raise head of bed
• Hiatus hernia • Small regular meals ≥ 3h before bed
• Smoking • Stop smoking and ↓ EtOH
• EtOH • Avoid hot drinks and spicy food
• Obesity • Stop drugs: NSAIDs, anti-AChM, nitrates, CCB, TCAs
• Pregnancy
• Drugs: anti-AChM, nitrates, CCB, TCAs Medical
• Iatrogenic: Heller’s myotomy • OTC antacids: Gaviscon, Mg trisilicate
• 1: Full-dose PPI for 1-2mo
Symptoms § Lansoprazole 30mg OD
• 2: No response → double dose PPI BD
Oesophageal • 3: No response: add an H2RA
• Retrosternal pain: heartburn § Ranitidine 300mg nocte
§ Related to meals • Control: low-dose acid suppression PRN
§ Worse lying down (e.g. @ night) / stooping
§ Relieved by antacids Surgical: Nissen Fundoplication
• Belching • Indications: all 3 of:
• Regurgitation § Severe symptoms
• Acid brash, water brash § Refractory to medical therapy
• Odonophagia § Confirmed reflux (pH monitoring)

Extra-oesophageal Nissen Fundoplication

• Nocturnal asthma • Aim: prevent reflux, repair diaphragm
• Chronic cough • Usually laparoscopic approach
• Laryngitis, sinusits • Mobilise gastric fundus and wrap around lower
oesophagus
Complications • Close any diaphragmatic hiatus
• Oesophagitis • Complications
• Ulceration: rarely → haematemesis, melaena, ↓Fe § Gas-bloat syn.: inability to belch / vomit
§ Dysphagia if wrap too tight
• Benign stricture: dysphagia
• Barrett’s oesophagus
§ Intestinal metaplasia of squamous epithelium Hiatus Hernia
§ Metaplasia → dysplasia → adenocarcinoma
• Oesophageal adenocarcinoma
Classification
Differential Dx
Sliding (80%)
• Oesophagitis
• Gastro-oesophageal junction slides up into chest
§ Infection: CMV, candida
§ IBD • Often assoc. c̄ GORD
§ Caustic substances / burns
• PUD Rolling (15%)
• Oesophageal Ca • Gastro-oesophageal junction remains in abdomen but
part of stomach rolls into chest alongside oesophagus
Ix • LOS remains intact so GORD uncommon
• Isolated symptoms don’t need Ix • Can → strangulation
• Bloods: FBC
• CXR: hiatus hernia may be seen Mixed (5%)
• OGD if:
§ >55yrs Ix
§ Persistent symptoms despite Rx • CXR: gas bubble and fluid level in chest
§ Anaemia • Ba swallow: diagnostic
§ Loss of wt. • OGD: assess for oesophagitis
§ Anorexia • 24h pH + manometry: exclude dysmotility or
§ Recent onset progressive symptoms achalasia
§ Melaena
§ Swallowing difficulty
§ OGD allows grading by Los Angeles
Rx
• Lose wt.
Classification
• Ba swallow: hiatus hernia, dysmotility • Rx reflux
• 24h pH testing ± manometry • Surgery if intractable symptoms despite medical Rx.
§ pH <4 for >4hrs § Should repair rolling hernia (even if asympto)
as it may strangulate.

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 Peptic Ulcer Disease
Presentation Surgery for PUD
• Epigastric pain
§ DU Concepts
- Before meals and at night • No acid → no ulcer
- Relieved by eating • Acid secretion stimulated by gastrin (from antral G
§ GU cells) and vagus N.
- Worse on eating (→ ↓ wt.)
- Relieved by anatacids Vagotomy
• Truncal
Risk Factors § ↓ acid secretion directly and via ↓ gastrin
• H. pylori § Prevents pyloric sphincter relaxation
• NSAIDs, steroids § \ must be combined c̄ pyloroplasty (widening
• Smoking, EtOH of pylorus) or gastroenterostomy
• Stress (GU) • Selective
§ Cushing’s ulcers: head injury § Vagus nerve only denervated where it supplies
§ Curling’s: ulcers: burns lower oesophagus and stomach
§ Nerves of Laterjet (supply pylorus) left intact
Pathology
• Punched out ulcers Antrectomy c̄ Vagotomy
• Usually background of chronic inflammation • Distal half of stomach removed.
• DU • Anastomosis:
§ 4x commoner cf. GU § Billroth 1: directly to duodenum
st
§ 1 part of duodenum (cap) § Billroth 2 /Polya: to small bowel loop c̄
• GU duodenal stump oversewn
§ Lesser curvature of gastric antrum
Subtotal Gastrectomy c̄ Roux-en-Y
Complications • Occasionally performed for Zollinger-Ellison
• Haemorrhage
§ Haematemesis or melaena
§ Fe deficiency anaemia Physical Complications
• Perforation: peritonitis • Ca: ↑ risk of gastric Ca
• Gastric Outflow Obstruction • Reflux or bilious vomiting (improves c̄ time)
§ Vomiting • Abdominal fullness
§ Colic • Stricture
§ Distension • Stump leakage
• Malignancy
§ ↑ risk c̄ H. pylori infection Metabolic Complications
§ Actual malignant transformation probably • Dumping syndrome
doesn’t occur
§ Abdo distension, flushing, n/v, fainting,
sweating
Ix § Early: osmotic hypovolaemia
• Bloods: FBC, urea (↑ in haemorrhage) § Late: reactive hypoglycaemia
13
• C breath test • Blind loop syndrome → malabsorption, diarrhoea
• OGD (stop PPIs >2wks before) § Overgrowth of bacteria in duodenal stump
§ CLO / urease test for H. pylori • Vitamin deficiency
§ Biopsy all ulcers to check for malignancy § ↓ parietal cells → B12 deficiency
• Gastrin levels if Zollinger-Ellison suspected § Bypassing proximal SB → Fe + folate
deficiency
Mx § Osteoporosis
• Wt. loss: malabsorption of ↓ calories intake
Conservative
• Lose wt.
• Stop smoking and ↓ EtOH
• Avoid hot drinks and spicy food
• Stop drugs: NSAIDs, steroids
• OTC antacids

Medical
• OTC antacids: Gaviscon, Mg trisilicate
• H. pylori eradication: PAC 500 / PMC 250
• Acid suppression
§ PPIs: lansoprazole 30mg/d
§ H2RAs: ranitidine 300mg nocte

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 Upper GI Bleeding
Hx
• Previous bleeds
Management
• Dyspepsia, known ulcers
• Liver disease or oesophageal varices Resuscitate
• Dysphagia, wt. loss
• Head down
• Drugs and EtOH
• 100% O2, protect airway
• Co-morbidities
• 2 x 14G cannulae + IV crystalloid infusion up to 1L.
• Bloods: FBC, U+E (↑ urea), LFTs, clotting, x-match 6u,
o/e
ABG, glucose
• Signs of CLD
• PR: melaena
• Shock?
§ Cool, clammy, CRT>2s Blood if remains shocked
§ ↓BP (<100) or postural hypotension (>20 drop) • Group specific or O- until x-matched
§ ↓ urine output (<30ml/h)
§ Tachycardia
§ ↓GCS
Variceal Bleed
Common Causes • Terlipressin IV (splanchnic vasopressor)
• PUD: 40% (DU commonly) • Prophylactic Abx: e.g. ciprofloxacin 1g/24h
• Acute erosions / gastritis:20%
• Mallory-Weiss tear: 10%
• Varices: 5%
• Oesophagitis: 5% Maintenance
• Ca stomach / oesophagus:<3% • Crystalloid IVI, transfuse if necessary (keep Hb≥10)
• Catheter + consider CVP (aim for >5cm H2O)
Rockall Score: (Prof T Rockall, St. Mary’s) • Correct coagulopathy: vit K, FFP, platelets
• Prediction of re-bleeding and mortality • Thiamine if EtOH
• 40% of re-bleeders die • Notify surgeons of severe bleeds
• Initial score pre-endoscopy
§ Age
§ Shock: BP, pulse
§ Comorbidities Urgent Endoscopy
• Final score post-endoscopy Haemostasis of vessel or ulcer
§ Final Dx + evidence of recent haemorrhage • Adrenaline injection
§ Active bleeding
• Thermal / laser coagulation
§ Visible vessel
§ Adherent clot • Fibrin glue
• Initial score ≥3 or final >6 are indications for surgery • Endoclips

Oesophageal Varices Variceal bleeding:

• Portal HTN → dilated veins @ sites of porto-systemic
anastomosis: L. gastric and inferior oesophageal
veins
• 30-50% c̄ portal HTN will bleed from varices
• Overall mortality 25%: ↑ c̄ severity of liver disease.
• 2 of: banding, sclerotherapy, adrenaline, coagulation
• Balloon tamponade c̄ Sengstaken-Blakemore tube
§ Only used if exsanguinating haemorrhage or failure
of endoscopic therapy
• TIPSS if bleeding can’t be stopped endoscopically

Causes of portal HTN

• Pre-hepatic: portal vein thrombosis
• Hepatic: cirrhosis (80% in UK), schisto (commonest
worldwide), sarcoidosis.
• Post-hepatic: Budd-Chiari, RHF, constrict pericarditis
Bleed Prevention
•
O
1 : β-B, repeat endoscopic banding
O
• 2 : β-B, repeat banding, TIPSS
Transjuglar Intrahepatic Porto-Systemic Shunt
(TIPSS)
• IR creates artificial channel between hepatic vein and
portal vein → ↓ portal pressure.
• Colapinto needle creates tract through liver
parenchyma which is expand using a balloon and
maintained by placement of a stent.
• Used prophylactically or acutely if endoscopic therapy
fails to control variceal bleeding.
After endoscopy
• Omeprazole IV + continuation PO (↓s re-bleeding)
• Keep NBM for 24h → clear fluids → light diet @ 48h
• Daily bloods: FBC, U+E, LFT, clotting
• H. pylori testing and eradication
• Stop NSAIDs, steroids et.c.
Indications for Surgery
• Re-bleeding
• Bleeding despite transfusing 6u
• Uncontrollable bleeding at endoscopy
• Initial Rockall score ≥3, or final >6.
• Open stomach, find bleeder and underrun vessel.
NA. Avoid 0.9% NS in uncompensated liver disease (worsens
ascites). Use blood/albumin for resus and 5% dex for
maintenance.

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Perforated Peptic Ulcer Gastric Outlet Obstruction
Pathophysiology Cause
• Perforated duodenal ulcer is commonest • Late complication of PUD → fibrotic stricturing
st
§ 1 part of the duodenum: highest acid conc • Gastric Ca
§ Ant. perforation → air under diaphragm
§ Post. perforation can erode into GDA → bleed Presentation
§ ¾ of duodenum retroperitoneal \ no air under • Hx of bloating, early satiety and nausea
diaphragm if perforated.
• Outlet obstruction
• Perforated GU § Copious projectile, non-bilious vomiting a few
• Perforated gastric Ca hrs after meals.
§ Contains stale food.
Presentation § Epigastric distension + succussion splash
• Sudden onset severe pain, beginning in the
epigastrium and then becoming generalised. Ix
• Vomiting • ABG: Hypochloraemic hypokalaemic met alkalosis
• Peritonitis • AXR
§ Dilated gastric air bubble, air fluid level
Differential § Collapsed distal bowel
• Pancreatitis • OGD
• Acute cholecystitis • Contrast meal
• AAA
• MI Rx
• Correct metabolic abnormality: 0.9% NS + KCl
Ix • Benign
• Bloods § Endoscopic balloon dilatation
§ FBC, U+E, amylase, CRP, G+S, clotting § Pyloroplasty or gastroenterostomy
§ ABG: ? mesenteric ischaemia • Malignant
• Urine dipstick § Stenting
• Imaging § Resection
§ Erect CXR
- Must be erect for ~15min first
- Air under the diaphragm seen in 70% Hypertrophic Pyloric Stenosis
- False +ve in Chailaditi’s sign
§ AXR Epidemiology
- Rigler’s: air on both sides of bowel wall • Sex: M>F=4:1
• Race: ↑ in Caucasians
Mx
Presentation
Resuscitation • 6-8wks
• NBM • Projectile vomiting minutes after feeding
• Aggressive fluid resuscitation • RUQ mass: olive
§ Urinary Catheter ± CVP line • Visible peristalsis
• Analgesia: morphine 5-10mg/2h max
§ ± cyclizine Dx
• Abx: cef and met • Test feed: palpate mass + see peristalsis
• NGT • Hypochloraemic hypokalaemic metabolic alkalosis
• US
Conservative
• May be considered if pt. isn’t peritonitic Mx
• Careful monitoring, fluids + Abx • Resuscitate and correct metabolic abnormality
• Omentum may seal perforation spontaneously • NGT
preventing operation in ~50% • Ramstedt pyloromyotomy: divide muscularis propria

Surgical: Laparotomy
• DU: abdominal washout + omental patch repair
• GU: excise ulcer and repair defect
• Partial / gastrectomy may rarely be required
§ Send specimen for histo: exclude Ca

Test and Treat

• 90% of perforated PU assoc. c̄ H. pylori

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 Gastric Cancer
Epidemiology Spread
• Incidence: 23/100,000 • w/i stomach: linitis plastica
• Age: 50s • Direct invasion: pancreas
• Sex: M>F=2:1 • Lymphatic: Virchow’s node
• Geo: ↑ in Japan, Eastern Europe, China, S. America • Blood: liver and lung
• Transcoelomic
Risk Factors § Ovaries: Krukenberg tumour (Signet ring morph)
• Atrophic gastritis (→ intestinal metaplasia) § Sister Mary Joseph nodule: umbilical mets
§ Pernicious anaemia / AI gastritis
§ H. pylori Ix
• Diet: ↑ nitrates – smoked, pickled, salted (↑ Japan) • Bloods
§ Nitrates → carcinogenic nitrosamines in GIT § FBC: anaemia
• Smoking § LFTs and clotting
• Blood group A • Imaging
• Low SEC § CXR: mets
• Familial: E. cadherin abnormality § USS: liver mets
• Partial gastrectomy § Gastroscopy + biopsy
§ Ba meal
• Staging
Pathology § Endoluminal US
• Mainly adenocarcinomas § CT/MRI
• Usually located on gastric antrum § Diagnostic laparoscopy
• H. pylori may → MALToma
Mx
Classification
Medical Palliation
Depth of Invasion • Analgesia: e.g. fentanyl patch
• Early gastric Ca: mucosa or submucosa • PPI
• Late gastric Ca: muscularis propria breached • Secretion control
• Chemo: epirubicin, 5FU, cisplatin
Microscopic Appearance • Palliative care team package
• Intestinal: bulky, glandular tumours, heaped
ulceration Surgical Palliation
• Diffuse: infiltrative c̄ signet ring cell morphology • Pyloric stenting
• Bypass procedures
Borrmann Classification
• Polypoid / fungating Curative Surgery
• Excavating • EGC may be resected endoscopically
• Ulcerating and raised • Partial or total gastrectomy c̄ roux-en-Y to prevent bile
• Linitis plastica: leather-bottle like thickening c̄ flat reflux.
rugae § Spleen and part of pancreas may be removed

Symptoms Prognosis
• Usually present late • Overall: 5ys <10%
• Wt. loss + anorexia • Surgery: 20-50% 5ys
• Dyspepsia: epigastric or retrosternal pain/discomfort
• Dysphagia
• n/v

Signs
• Anaemia
• Epigastric mass
• Jaundice
• Ascites
• Hepatomegaly
• Virchow’s node (= Troisier’s sign)
• Acanthosis nigricans

Complications
• Perforation
• Upper GI bleed: haematemesis, melaena
• Gastric outlet obstruction → succession splash

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Other Gastric Neoplasms Zollinger-Ellison Syndrome
Benign Pathophysiology
• Benign polyps: adenomas • Gastrin-secreting tumour (gastrinoma) most
• Leiomyoma commonly found in the small intestine or pancreas.
• Lipomas • ↑ Gastrin → ↑HCL→ PUD + chronic diarrhoea
• Haemangiomas § Diarrhoea due to inactivation of panc enzymes
• Schwannomas • ECL proliferation can → carcinoid tumours
• 60-90% of gastrinomas are malignant
Malignant • 25% assoc. c̄ MEN1
• Lymphoma
• Carcinoid Presentation
• GIST • Abdominal pain and dyspepsia
• Chronic diarrhoea / Steatorrhoea
• Refractory PUD
Gastrointestinal Stromal Tumour
• Commonest mesenchymal tumour of the GIT Ix
• >50% occur in the stomach. • ↑ gastrin c̄ ↑↑ HCl (pH<2)
• MRI/CT
Epidemiology • Somatostatin receptor scintigraphy
• M=F
• ~60yrs Rx
• ↑ c̄ NF1 • High dose PPI
• Surgery
Pathology § Tumour resection
• Arise from intestinal cells of Cajal § May do subtotal gastrectomy c̄ Roux en Y
§ Located in muscularis propria
§ Pacemaker cells
• OGD: well-demarcated spherical mass c̄ central
punctum

Presentation
• Mass effects: abdo pain, obstruction
• Ulceration: → bleeding

Poor Prognosticators
• ↑ size
• Extra-gastric location
• ↑ mitotic index

Mx
• Medical
§ Unresectable, recurrent or metastatic disease
§ Imatinib: kit selective tyrosine kinase inhibitor
• Surgical
§ Resection

Carcinoid Tumours
• Diverse group of neuroendocrine tumours of
enterochromaffin cell origin
• May secrete multiple hormones
• 10% occur in the stomach

Gastric Carcinoids
• Atrophic gastritis → ↓ acid production → ↑ gastrin →
ECL hyperplasia → carcinoid tumour
• Gastrinomas may also → carcinoid

Gastric Lymphoma
• Commonest site for extranodal lymphoma
• Most commonly MALToma due to chronic H. pylori
gastritis
• H. pylori eradication can be curative

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Bariatric Surgery
Benefits
• Sustained wt. ↓
• Symptom improvement
§ Sleep apnoea
§ Mobility
§ HTN
§ DM

Indications
• All the criteria must be met
§ BMI ≥40 or ≥35 c̄ significant co-morbidities that
could improve c̄ ↓ wt.
§ Failure of non-surgical Mx to achieve and
maintain clinically beneficial wt. loss for 6mo.
§ Fit for surgery and anaesthesia
§ Integrated program providing guidance on diet,
physical activity, psychosocial concerns and
lifelong medical monitoring
§ Well-informed and motivated pt.
st
• If BMI >50, surgery is 1 -line Rx

Laparoscopic Gastric Banding

• Inflatable silicone band around proximal stomach →
small pre-stomach pouch.
§ Limits food intake
§ Slows digestion
• At 1yr 46% mean excess wt. loss

Roux-en-Y Gastric Bypass

• Oesophagojejunostomy allows bypass of stomach,
duodenum and proximal jejunum.
• Alters secretion of hormones influencing glucose
regulation and perception of hunger / satiety.
• Greater wt. loss and lower reoperation rates.
• Complications
§ Dumping syndrome
§ Wound infection
§ Hernias
§ Malabsorption
§ Diarrhoea

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Gallstones
Epidemiology
• ~8% of the population >40yrs
• Incidence ↑ over last 20yrs: western diet
• Slightly ↑ incidence in females
• 90% of gallstones remain asymptomatic
Formation
General Composition
• Phospholipids: lecithin
• Bile pigments (broken down Hb)
• Cholesterol
Biliary Colic
Pathogenesis
• Gallbladder spasm against a stone impacted in the neck of
the gallbladder – Hartmann’s Pouch.
• Less commonly, the stone may be in the CBD
Presentation
• Biliary colic
§ RUQ pain radiating → back (scapular region)
§ Assoc. c̄ sweating, pallor, n/v
§ Attacks may be ppted. by fatty food and last <6h
• o/e may be tenderness in right hypochondrium
• ± jaundice if stones passes in to CBD

Aetiology Differential
• Lithogenic bile: Admirand’s Triangle • Cholecystitis / other gallstone disease
• Biliary sepsis • Pancreatitis
• GB hypomotility → stasis • Bowel perforation
§ Pregnancy, OCP
§ TPN, fasting Ix
• Same work up as cholecystitis as may be difficult to
Cholesterol Stones: 20% differentiate clinically.
• Large • Urine: bilirubin, urobilinogen, Hb
• Often solitary • Bloods: FBC, U+E, amylase, LFTs, G+S, clotting, CRP
• Formation ↑ according to Admirand’s • Imaging
Trangle § AXR: 10% of gallstones are radio-opaque
§ ↓ bile salts § Erect CXR: look for perforation
§ ↓ lecithin § US:
§ ↑ cholesterol - Stones: acoustic shadow
• Risk factors - Dilated ducts: >6mm
§ Female - Inflamed GB: wall oedema
§ OCP, pregnancy • If Dx uncertain after US
§ ↑ age § HIDA cholescintigraphy: shows failure of GB filling
§ High fat diet and obesity (requires functioning liver)
§ Racial: e.g. American Indian tribes • If dilated ducts seen on US → MRCP
§ Loss of terminal ileum (↓ bile salts)
Rx
Pigment Stones: 5% • Conservative
• Small, black, gritty, fragile § Rehydrate and NBM
• Calcium bilirubinate § Opioid analgesia: morphine 5-10mg/2h max
• Associated c̄ haemolysis § High recurrence rate \ surgical Rx favoured
• Surgical
Mixed Stones: 75% § Laparoscopic cholecystectomy
• Often multiple
• Cholesterol is the major component

Complications

In the Gallbladder
• Biliary Colic
• Acute cholecystitis ± empyema
• Chronic cholecytsitis
• Mucocele
• Carcinoma
• Mirizzi’s syndrome

In the CBD
• Obstructive jaundice
• Pancreatitis
• Cholangitis

In the Gut
• Gallstone ileus

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Acute Cholecystitis Chronic Cholecystitis
Pathogenesis Symptoms: Flatulent Dyspepsia
• Stone or sludge impaction in Hartmann’s pouch • Vague upper abdominal discomfort
• → chemical and / or bacterial inflammation • Distension, bloating
• 5% are acalculous: sepsis, burns, DM • Nausea
• Flatulence, burping
Sequelae • Symptoms exacerbated by fatty foods
• Resolution ± recurrence § CCK release stimulates gallbladder
• Gangrene and rarely perforation
• Chronic cholecystitis Differential
• Empyema • PUD
• IBS
Presentation • Hiatus hernia
• Severe RUQ pain • Chronic pancreatitis
§ Continuous
§ Radiates to right scapula and epigastrium Ix
• Fever • AXR: porcelain gallbladder
• Vomiting • US: stones, fibrotic, shrunken gallbladder
• MRCP
Examination
• Local peritonism in RUQ Mx
• Tachycardia c̄ shallow breathing • Medical
• ± jaundice § Bile salts (not very effective)
• Murphy’s sign • Surgical
§ 2 fingers over the GB and ask pt. to breath in § Elective cholecystectomy
§ → pain and breath catch. Must be –ve on the § ERCP first if US shows dilated ducts and stones
L
• Phlegmon may be palpable
§ Mass of adherent omentum and bowel
• Boas’ sign
§ Hyperaesthesia below the right scapula Rarer Gallstone Disease
Ix Mucocele
• Urine: bilirubin, urobilinogen
• Neck of gallbladder blocked by stone but contents remains
• Bloods sterile
§ FBC: ↑ WCC
• Can be very large → palpable mass
§ U+E: dehydration from vomiting
• May become infected → empyema
§ Amylase, LFTs, G+S, clotting, CRP
• Imaging
§ AXR: gallstone, porcelain gallbladder
§ Erect CXR: look for perforation Gallbladder Carcinoma
§ US • Rare
- Stones: acoustic shadow • Associated c̄ gallstones and gallbladder polyps.
- Dilated ducts (>6mm) • Calcification of gallbladder → porcelain GB
- Inflamed GB: wall oedema • Incidental Ca found in 0.5-1% of lap choles.
• If Dx uncertain after US
§ HIDA cholescintigraphy: shows failure of
GB filling (requires functioning liver) Mirizzi’s Syndrome
• MRCP if dilated ducts seen on US
• Rare
• Large stone in GB presses on the common hepatic duct
Mx → obstructive jaundice.
• Conservative • Stone may erode through into the ducts
§ NBM
§ Fluid resuscitation
§ Analgesia: paracetamol, diclofenac, codeine
§ Abx: cefuroxime and metronidazole
Gallstone Ileus
§ 80-90% settle over 24-48h • Large stone (>2.5cm) erodes from GB → duodenum
O
§ Deterioration: perforation, empyema through a cholecysto-duodenal fistula 2 to chonic inflam.
• Surgical • May impact in distal ileum → obstruction
§ May be elective surgery @ 6-12wks (↓ • Rigler’s Triad:
inflam) § Pneumobiliia
§ If <72h, may perform lap chole in acute § Small bowel obstruction
phase § Gallstone in RLQ
• Empyema • Rx: stone removal via enterotomy
§ High fever • NB. Bouveret’s syn. = duodenal obstruction
§ RUQ mass
§ Percutaneous drainage: cholecystostomy

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Obstructive Jaundice Pancreatic Carcinoma
Causes Risk Factors: SINED
• 30% stones • Smoking
• 30% Ca head of the pancreas • Inflammation: chronic pancreatitis
• 30% Other • Nutrition: ↑fat diet
§ LNs @ porta hepatis: TB, Ca • EtOH
§ Inflammatory: PBC, PSC • DM
§ Drugs: OCP, sulfonylureas, flucolox
§ Neoplastic: Cholangiocarcinoma
§ Mirizzi’s syndrome
Pathology
• 90% ductal adenocarcinomas
• Present late, metastasise early
Clinical Features § Direct extension to local structures
• Jaundice § Lymphatics
§ Clinically evident at ~50mM § Blood → liver and lungs
§ Seen at tongue frenulum first (affinity for elastin) • 60% located in head, 25% body, 15% tail
• Dark urine, pale stools
• Itch (bile salts)
Presentation
• Typically male >60yrs
Ix • Painless obstructive jaundice: dark urine, pale stools
• Urine • Epigastric pain: radiates to back, relieved sitting
§ Dark forward
§ ↑ bilirubin • Anorexia, wt. loss and malabsorption
§ ↓ urobilinogen
• Acute pancreatitis
• Bloods
• Sudden onset DM in the elderly
§ FBC: ↑ WCC in cholangitis
§ U+E: hepatorenal syndrome
§ LFT: ↑cBR, ↑↑ ALP, ↑AST/ALT Signs
§ Clotting: ↓ vit K → ↑ INR • Palpable gallbladder
§ G+S: may need ERCP • Jaundice
§ Immune: AMA, ANCA, ANA • Epigastric mass
• Imaging • Thrombophlebitis migrans (Trousseau sign)
§ AXR • Splenomegaly: PV thrombosis → portal HTN
- May visualise stone • Ascites
- Pneumobilia suggests gas forming
infection Courvoisier’s Law
§ US • In the presence of painless obstructive jaundice, a
- Dilated ducts >6mm palpable gallbladder is unlikely to be due to stones.
- Stones (95% accurate)
- Tumour Ix
§ MRCP or ERCP
• Bloods: cholestatic LFTs, ↑Ca19-9 (90% sens), ↑Ca
§ Percutaneous Transhepatic Cholangiography
• Imaging
§ US: pancreatic mass, dilated ducts, hepatic
Mx of Stones mets, guide biopsy
• Conservative § EUS: better than CT/MRI for staging
§ Monitor LFTs: passage of stone may → § CXR: mets
resolution § Laparoscopy: mets, staging
§ Vitamins ADEK • ERCP
§ Analgesia § Shows anatomy
§ Cholestyramine § Allows stenting
• Interventional § Biopsy of peri-ampullary lesions
§ If no resolution, worsening LFTs or cholangitis
§ ERCP c̄ sphincterotomy and stone extraction Rx
• Surgical • Surgery
§ Open / lap stone removal c̄ T tube placement § Fit, no mets, tumour ≤3cm (≤10% of pts)
- T tube cholangiogram 8d later to confirm § Whipple’s pancreaticoduodenectomy
stone removal. § Distal pancreatectomy
§ Delayed cholecystectomy to prevent recurrence § Post-op chemo delays progression
§ 5ys = 5-14%
Ascending Cholangitis • Palliation
• May complicate CBD obstruction § Endoscopic / percutaneous stenting of CBD
• Charcot’s triad: fever/rigors, RUQ pain, jaundice § Palliative bypass surgery:
• Reynolds pentad: Charcot’s triad + shock + confusion (a) cholecystojejunostomy +
• Mx gastrojejunostomy
§ Cef and met § Pain relief – may need coeliac plexus block
st
§ 1 : ERCP
nd
§ 2 : Open or lap stone removal c̄ T tube drain Prognosis
• Mean survival <6mo
• 5ys = <2%
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 Acute Pancreatitis
Pathophysiology Ix
• Pancreatic enzymes released and activated in vicious • Bloods
circle → multi-stage process. § FBC: ↑WCC
• Oedema + fluid shift + vomiting → hypovolaemic § ↑amylase (>1000 / 3x ULN) and ↑lipase
shock while enzymes → autodigestion and fat - ↑ in 80%
necrosis - Returns to normal by 5-7d
• Vessel autodigestion → retroperitoneal haemorrhage § U+E: dehydration and renal failure
• Inflammation → pancreatic necrosis § LFTs: cholestatic picture, ↑AST, ↑LDH
2+
• Super-added infection: 50% of pts. c̄ necrosis § Ca : ↓
§ Glucose: ↑
§ CRP: monitor progress, >150 after 48hrs = sev
Epidemiology § ABG: ↓O2 suggests ARDS
• 1% of surgical admissions
th th • Urine: glucose, ↑cBR, ↓urobilinogen
• 4 and 5 decades
• Imaging
• 10% mortality § CXR: ARDS, exclude perfed DU
§ AXR: sentinel loop, pancreatic calcification
Aetiology § US: Gallstones and dilated ducts, inflammation
• Gallstones (45%) § Contrast CT: Balthazar Severity Score
• Ethanol (25%)
• Idiopathic (20%): ?microstones Conservative Mx
• Trauma • Manage at appropriate level: e.g. ITU if severe
• Steroids • Constant reassessment is key
• Mumps + other infections: Coxsackie B § Hrly TPR, UO
• Autoimmune: e.g. PAN 2+
§ Daily FBC, U+E, Ca , glucose, amylase ABG
• Scorpion (Trinidadian)
• Hyperlipidaemia (I and V), ↑Ca, Hypothermia Fluid Resuscitation
• ERCP: 5% risk • Aggressive fluid resus: keep UO >30ml/h
• Drugs: e.g. thiazides, azathioprine • Catheter ± CVP

Symptoms Pancreatic Rest

• Severe epigastric pain → back • NBM
§ May be relieved by sitting forward • NGT if vomiting
• Vomiting • TPN may be required if severe to prevent catabolism

Signs Analgesia
• ↑HR, ↑RR • Pethidine via PCA
• Fever • Or morphine 5-10mg/2h max
• Hypovolaemia → shock
• Epigastric tenderness Antibiotics
• Jaundice • Not routinely given if mild
• Ileus → absent bowel sounds • Used if suspicion of infection or before ERCP
• Ecchymoses • Penems often used: e.g. meropenem, imipenem
§ Grey Turners: flank
§ Cullens: periumbilical (tracks up Falciform) Mx Complications
• ARDS: O2 therapy or ventilation
Differential • ↑ glucose: insulin sliding scale
• Perforated DU • ↑/↓Ca
• Mesenteric infarction • EtOH withdrawal: chlordiazepoxide
• MI
Interventional Mx: ERCP
Modified Glasgow Criteria •
O
If pancreatitis c̄ dilated ducts 2 to gallstones
• Valid for EtOH and gallstones • ERCP + sphincterotomy → ↓ complications
• Assess severity and predict mortality
• Ranson’s criteria are only applicable to EtOH and can
only be fully applied after 48hrs.
Surgical Mx

PANCREAS Indications
• • Infected pancreatic necrosis
PaO2 <8kPa 1 = mild
• Pseudocyst or abscess
• Age >55yrs 2 = mod
9 • Unsure Dx
• Neutrophils >15 x10 /L 3 = severe
2+
• Ca <2mM
Operations
• Renal function U>16mM
• Laparotomy + necrosectomy (pancreatic debridement)
• Enzymes LDH>600iu/L • Laparotomy + peritoneal lavage
AST>200 iu/L • Laparostomy: abdomen left open c̄ sterile packs in ITU
• Albumin <32g/L
• Sugar >10mM
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Complications of Acute Chronic Pancreatitis
Pancreatitis
Causes: AGITS
• Alcohol (70%)
Early: Systemic • Genetic
• Respiratory: ARDS, pleural effusion § CF
• Shock: hypovolaemic or septic § HH
• Renal failure • Immune
• DIC § Lymphoplasmacytic sclerosing pancreatitis
• Metabolic (↑IgG4)
2+
§ ↓ Ca • Triglycerides ↑
§ ↑ glucose • Structural
§ Metabolic acidosis § Obstruction by tumour
§ Pancreas divisum

Late (>1wk): Local Presentation

• Pancreatic necrosis • Epigastric pain
• Pancreatic infection § Bores through to back
• Pancreatic abscess § Relieved by sitting back or hot water bottle →
§ May form in pseudocyst or in pancreas erythema ab igne
§ Open or percutaneous drainage § Exacerbated by fatty food or EtOH
• Bleeding: e.g. from splenic artery • Steatorrhoea and wt. loss
§ May require embolisation • DM: polyuria, polydipsia
• Thrombosis • Epigastric mass: pseudocyst
§ Splenic A., GDA or colic branches of SMA
- May → bowel necrosis
§ Portal vein → portal HTN
Ix
• ↑ glucose
• Fistula formation
§ Pancreato-cutaneous → skin breakdown • ↓ faecal elastase: ↓ exocrine function
• US: pseudocyst
• AXR: speckled pancreatic calcifications
Pancreatic Pseudocyst
• CT: pancreatic calcifications
• Collection of pancreatic fluid in the lesser sac
surrounded by granulation tissue
• Occur in 20% (esp. in EtOH pancreatitis) Rx
• Presentation • Diet
§ 4-6wks after acute attack § No EtOH
§ Persisting abdominal pain § ↓ fat, ↑ carb
§ Epigastric mass → early satiety • Drugs
• Complications § Analgesia: may need coeliac plexus block
§ Infection → abscess § Enzyme supplements: pancreatin (Creon)
§ Obstruction of duodenum or CBD § ADEK vitamins
• Ix § DM Rx
§ Persistently ↑ amylase ± LFTs • Surgery
§ US / CT § Indications
• Rx - Unremitting pain
§ <6cm: spontaneous resolution - Wt. loss
§ >6cm - Duct blockage
- Endoscopic cyst-gastrostomy § Procedures
- Percutaneous drainage under US/CT - Distal pancreatectomy, Whipple’s
- Pancreaticojejunostomy: drainage
- Endoscopic stenting

Complications
• Pseudocyst
• DM
• Pancreatic Ca
• Pancreatic swelling → biliary obstruction
• Splenic vein thrombosis → splenomegaly

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© Alasdair Scott, 2018
Pancreatic Endocrine Neoplasia Pancreatic Malformations
Epidemiology Ectopic Pancreas
• 30-60yrs • Meckel’s diverticulum
• ~15% assoc. c̄ MEN1 • Small bowel

Insulinoma
• Fasting / exercise-induced hypoglycaemia Pancreas Divisium
• Confusion, stupor, LOC • Failure of fusion of dorsal and ventral buds
• ↑ insulin + ↑ c-peptide + ↓glucose • → bulk of pancreas drains through smaller accessory
duct.
Gastrinoma → Zollinger-Ellison • Usually asymptomatic
• Hypergastrinaemia → hyperchlorhydria → PUD and • May → chronic pancreatitis
chronic diarrhoea (inactivation of pancreatic enzymes)

Glucagonoma Annular Pancreas

• ↑ se glucagon → mild DM • Fusion of dorsal and ventral buds around duodenum
• Characteristic blistering rash • May present c̄ infantile duodenal obstruction.
§ Necrolytic migratory erythema

VIPoma / Verner-Morrison / WDHA Syn.

• Watery Diarrhoea
• Hypokalaemia
• Achlorhydria
• Acidosis

Somatostatinoma
• Somatostatin
§ Inhibits glucagon and insulin release
§ Inhibits pancreatic enzyme secretion
• Features
§ DM
§ Steatorrhoea
§ Gallstones
• Usually v. malignant tumour c̄ poor prognosis

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Cholangiocarcinoma Hydatid Cyst
Pathology Pathophysiology
• Rare bile duct tumour • Zoonotic infection by Echinococcus granulosus
• Adenocarcinoma • Occurs in sheep-rearing communities
• Typically occur @ confluence of right and left hepatic • Parasite penetrates the portal system and infects the
ducts: called “Klatskin” tumours liver → calcified cyst

Risk Factors Presentation

• PSC • Mostly asymptomatic
• Ulcerative colitis • Pressure effects
• Choledocholithiasis § Non-specific pain
• Hep B/C § Abdominal fullness
• Choledochal cysts § Obstructive jaundice
• Lynch 2 • Rupture
• Flukes § Biliary colic
§ Jaundice
§ Urticaria
Presentation § Anaphylaxis
• Progressive painless obstructive jaundice •
O
2 infection
§ Gallbladder not palpable
• Steatorrhoea
• Wt. loss
Ix
• Eosinophilia
• CT
Ix
• Cholestatic LFTs
• CA 19-9
Rx
• Medical: albendazole
• Surgical cystectomy
Rx § Indicated for large cyst
• Poor prognosis: no curative Rx
• Palliative stenting by ERCP

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 Paediatric Conditions
Meckel’s Diverticulum Intussusception
• Ileal remnant of vitellointestinal duct • Portion of intestine (the intussusception) is
§ Joins yoke sac to midgut lumen invaginated into its own lumen (the intussuscipiens)

Features Cause
• A true diverticulum • Hypertrophied Peyer’s patch
• 2 inches long • Meckel’s
• 2 ft from ileocaecal valve on antimesenteric border • HSP
• 2% of population • Peutz-Jeghers
• 2% symptomatic • Lymphoma
• Contain ectopic gastric or pancreatic tissue
Presentation
Presentation of Symptomatic Meckel’s • 6-12mo
• Rectal bleeding: from gastric mucosa • Colicky abdo pain:
• Diverticulitis mimicking appendicitis § Episodic inconsolable crying, drawing up legs
• Intussusception § ± bilious vomiting
• Volvulus • Redcurrent jelly stools
• Malignant change: adenocarcinoma • Sausage-shaped abdominal mass
• Raspberry tumour: mucosa protruding at umbilicus
§ A vitello-intestinal fistula Mx
• Littre’s Hernia: herniation of Meckel’s • Resuscitate, x-match, NGT
• US + reduction by air enema
Dx • Surgery if not reducible by enema
• Tc pertechnecate scan +ve in 70% (detects gastric
mucosa) NB. Intussusception rarely occurs in an adult
• If it does, consider neoplasm as lead-point
Rx
• Surgical resection
Mesenteric Adenitis
• Viral infection / URTI → enlargement of mesenteric
LNs
• → pain, tenderness and fever
• Differentiating features
§ Post URTI
§ Headache + photophobia
§ Higher temperature
§ Tenderness is more generalised
§ Lymphocytosis

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Small Bowel Neoplasms Carcinoid Tumours
Benign: 35% Pathology
• Lipoma • Diverse group of neuroendocrine tumours of
• Leiomyoma enterochromaffin cell origin capable of producing 5HT
• Neurofibroma • May be derived from
• Haemangioma § Foregut: respiratory tract
• Adenomatous polyps (FAP, Peutz-Jeghers) § Midgut: stomach, ileum, appendix
§ Hindgut: colorectum
Malignant: 65% (only 2% of GI malignancies) • May secrete: 5-HT, VIP, gastrin, glucagon, insulin, ACTH
§ Hind gut tumours rarely secrete 5-HT
• Adenocarcinoma (40% of malignant tumours)
• Carcinoid syndrome suggest bypass of first-pass
• Carcinoid (40% of malignant tumours)
metabolism and is strongly assoc. c̄ metastatic disease.
• Lymphoma (esp. c̄ Coeliac disease: EATL)
• 10% part of MEN1
• GIST
• Sites
§ Appendix: 45%
Presentation § Ileum: 30%
• Often non-specific symptoms so present late § Colorectum: 20%
• N/V, obstruction § Stomach: 10%
• Wt. loss and abdominal pain § Elsewhere in GIT
• Bleeding § Bronchus: 10%
• Jaundice from biliary obstruction or liver mets. • Consider all as malignant

Imaging
• AXR: SBO Presentation
• CT
Local
Endoscopy • Appendicitis
• Push enteroscopy • Intussusception or obstruction
• Wireless capsule endoscopy • Abdominal pain

Carcinoid Syndrome: FIVE HT

• Flushing: paroxysmal, upper body ± wheals
• Intestinal: diarrhoea
• Valve fibrosis: tricuspid regurg and pulmonary stenosis
• whEEze: bronchoconstriction
st
• Hepatic involvement: bypassed 1 pass metabolism
• Tryptophan deficiency → pellagra (3Ds)

Ix
• ↑ urine 5-hydroxyindoleacetic acid
• ↑ plasma chromogranin A
• CT/MRI: find primary

Rx
• Symptoms: octreotide or loperamide
• Curative
§ Resection: tumours are v. yellow
§ Give octreotide to avoid carcinoid crisis

Carcinoid Crisis
• Tumour outgrows blood supply or is handled too much →
massive mediator release
• Vasodilatation, hypotension, bronchoconstriction,
hyperglycaemia
• Rx: high-dose octreotide

Prognosis
• Median survival is 5-8yrs (~3yrs if mets present)

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© Alasdair Scott, 2018
 Acute Appendicitis
Definition Differential
• Inflammation of the vermiform appendix ranging from • Surgical
oedema to ischaemic necrosis and perforation. § Cholecystitis
§ Diverticulitis
§ Meckel’s diverticulitis
Epidemiology
• Gynae
• Incidence: 6% lifetime incidence, commonest surgical
§ Cyst accident: torsion, rupture, haemorrhage
emergency
§ Salpingitis / PID
• Age: rare <2yrs, maximal peak during child, ↓ § Ruptured ectopic
thereafter
• Medical
§ Mesenteric adenitis
Pathogenesis § UTI
• Obstruction of the appendix § Crohn’s
§ Faecolith most commonly
§ Lymphoid hyperplasia post-infection Ix
§ Tumour (e.g. caecal Ca, carcinoid) • Dx is principally clinical
§ Worms (e.g. Ascaris lumbicoides, Schisto)
• Bloods: FBC, CRP, amylase, G+S, clotting
• Gut organisms → infection behind obstruction
• Urine
• → oedema → ischaemia → necrosis → perforation
§ Sterile pyuria: may indicate bladder irritation
§ Peritonitis
§ Ketones: anorexia
§ Abscess
§ Exclude UTI
§ Appendix mass
§ β-HCG
• Imaging
Pattern of Abdominal Pain § US: exclude gynae path, visualise inflamed
appendix
Early inflammation → appendiceal irritation § CT: can be used
• Visceral pain is not well localised cf. somatic pain. • Diagnostic lap
• Nociceptive info travels in the sympathetic afferent
fibres that supply the viscus Mx
• Pain referred to the dermatome corresponding to the • Fluids
spinal cord entry level of these sympathetic fibres. • Abx: cef 1.5g + met 500g IV TDS
• Append = midgut = lesser splanch (T10/11) = umb • Analgesia: paracetamol, NSAIDs, codeine phosphate
• Certain Dx → appendicectomy (open or lap)
Late inflammation → parietal peritoneum irritation • Uncertain Dx → active observation
• Pain localised in RIF
Complications
Symptoms
• Colicky abdo pain Appendix Mass
§ Central → localised in RIF • Inflamed appendix c̄ adherent covering of omentum
§ Worse c̄ movement and small bowel
• Anorexia • Dx: US or CT
• Nausea (vomiting is rarely prominent) • Mx
• Constipation / diarrhoea § Initially: Abx + NBM
§ Resolution of mass → interval appendicectomy
Signs § Exclude a colonic tumour: colonoscopy
• Low-grade pyrexia: 37.5 – 38.5
• ↑HR, shallow breathing Appendix Abscess
• Foetor oris • Results if appendix mass doesn’t resolve
• Guarding and tenderness: @ McBurney’s point • Mass enlarges, pt. deteriorates
§ +ve cough / percussion tenderness • Mx
• Appendix mass may be palpable in RIF § Abx + NBM
• Pain PR suggests pelvic appendix. § CT-guided percutaneous drainage
§ If no resolution, surgery may involve right
Special Signs hemicolectomy.

Rovsing’s Sign Perforation

• Pressure in LIF → more pain in RIF
Psoas Sign
• Pain on extending the hip: retrocaecal appendix
• Commoner if faecolith present and in young children
(as Dx is often delayed)
• Deteriorating pt. c̄ peritonitis.

Cope Sign
• Flexion + internal rotation of R hip → pain
§ Appendix lying close to obturator internus

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 Inflammatory Bowel Disease: Pathology and Presentation
Epidemiology Pathology

UC Crohn’s UC Crohn’s

Prev 100-200 50-100 /100,000 Macroscopic

/100,000 Location Rectum + colon Mouth to anus
Age 30s 20s ± backwash ileitis esp. terminal ileum
Sex F>M (just) Distribution Contiguous Skip lesions
Aet Concordance = Concordance = Strictures No Yes
10% 70%
Smoking Smoking ↑ risk Microscopic
protective TH1/TH17- Inflammation Mucosal Transmural
TH2-mediated mediated Ulceration Shallow, broad Deep, thin, serpiginous
→ cobblestone mucosa
Fibrosis None Marked
Granulomas None Present
Pseudoplyps Marked Minimal
Fistulae No Yes

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© Alasdair Scott, 2018
Presentation

UC Crohn’s

Symptoms
Systemic Fever, malaise, anorexia, wt. loss in active disease
Abdominal • Diarrhoea • Diarrhoea (not usually bloody)
• Blood ± mucus PR • Abdominal pain
• Abdominal discomfort • Wt. loss
• Tenesmus, faecal urgency

Signs
Abdominal • Fever • Aphthous ulcers, glossitis
• Tender, distended abdomen • Abdominal tenderness
• RIF mass
• Perianal abscesses, fistulae, tags
• Anal / rectal strictures
Extra- Skin Joints
abdominal - Clubbing • Arthritis (non-deforming, asymmetrical)
- Erythema nodosum • Sacroiliitis
- Pyoderma gang (esp. UC) • Ank spond
HPB
Eyes • PSC + cholangiocarcinoma (esp. UC)
- Iritis • Gallstones (esp. Crohn’s)
- Conjunctivitis • Fatty liver
- Episcleritis Other
- Scleritis • Amyloidosis
• Oxalate renal stones (esp. Crohns)

Complications • Toxic megacolon • Fistulae

§ Diameter >6cm § Entero-enteric/colonic → diarrhoea
§ Risk of perforation § Enterovesical → frequency, UTI
• Bleeding § Enterovaginal
• Malignancy § Perianal → “pepperpot” anus
§ CRC in 15% c̄ pancolitis for 20yrs • Strictures → obstruction
• Cholangiocarcinoma • Abscesses
• Strictures → obstruction § Abdominal
• Venous thrombosis § Anorectal
• Malabsorption
§ Fat → Steatorrhoea, gallstones
§ B12 → megaloblastic anaemia
§ Vit D → osteomalacia
§ Protein → oedema
• Toxic megacolon and Ca may occur (< cf. UC)

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 Ulcerative Colitis: Management
Ix Inducing Remission in Mild / Mod Disease
• Bloods: • OPD-based
§ FBC: ↓Hb, ↑WCC
§ LFT: ↓albumin Oral Therapy
§ ↑CRP/ESR •
st
1 line: 5-ASAs
§ Blood cultures •
nd
2 line: prednisolone
• Stool
§ MCS: exclude Campy, Shigella, Salmonella…
Topical Therapy: mainly left-sided disease
§ CDT: C. diff may complicate or mimic
• Proctitis: suppositories
• Imaging
• More proximal disease: enemas or foams
§ AXR: megacolon (>6cm), wall thickening
§ CXR: perforation • 5-ASAs ± steroids (prednisolone or budesonide)
§ CT
§ Ba / gastrograffin enema Additional Therapy: steroid sparing
§ Lead-pipe: no haustra • Azathioprine or mercaptopurine
§ Thumbprinting: mucosal thickening • Infliximab: steroid-dependent pts
§ Pseudopolyps: regenerating mucosal island
• Ileocolonoscopy + regional biopsy: Baron Score Maintaining Remission
st
• 1 line: 5-ASAs PO – sulfasalazine or mesalazine
Severity § Topical Rx may be used in proctitis
nd
• 2 line: Azathioprine or mercaptopurine
Truelove and Witts Criteria § Relapsed on ASA or are steroid-dependent
§ Give 6-mercaptopurine if azathioprine intolerant
rd
Mild Mod Severe • 3 line: Infliximab / adalimumab

Motions <4 4-6 >6

Emergency Surgery
PR bleed small moderate large • 20% require surgery at some stage
Temp Apyrexic 37.1-37.8 >37.8 • 30% c̄ colitis require surgery w/i 5yrs
HR <70 70-90 >90
Indications
Hb >11 10.5-11 <10.5
• Toxic megacolon
ESR <30 >30 • Perforation
• Massive haemorrhage
Acute Severe UC • Failure to respond to medical Rx
• Resus: Admit, IV hydration, NBM
• Hydrocortisone: IV 100mg QDS + PR Procedures
• Transfuse if required • Total / subtotal colectomy c̄ end ileostomy ± mucus
• Thromboprophylaxis: LMWH fistula
• Monitoring • Followed after ~3mo by either
§ Bloods: FBC, ESR, CRP, U+E § Completion proctectomy + Ileal-pouch anal
§ Vitals + stool chart anastomosis (IPAA) or end ileostomy
§ Twice daily examination § Ileorectal anastomosis (IRA)
§ ± AXR • Panproctocolectomy + permanent end ileostomy
• Acute colitis op mortality: 7% (30% if perforated)
NB. RCTs show no benefit of Abx: not routinely
recommended Elective Surgery
• May use: megacolon, perforation, uncertain Dx
Indications
Acute Complications • Chronic symptoms despite medical therapy
• Perforation • Carcinoma or high-grade dysplasia
• Bleeding
• Toxic megacolon (>6cm) Procedures
• VTE • Panproctocolectomy c̄ end ileostomy or IPAA
• Total colectomy c̄ IRA
Improvement → oral therapy
• Switch to oral pred + a 5-ASA Surgical Complications
• Taper pred after full remission • Abdominal
§ SBO
No Improvement → rescue therapy § Anastomotic stricture
• On day 3: stool freq >8 or CRP >45 § Pelvic abscess
§ Predicts 85% chance of needing a colectomy • Stoma: retraction, stenosis, prolapse, dermatitis
during the admission • Pouch
• Discussion between pt, physician and surgeon § Pouchitis (50%): metronidazole + cipro
• Medical: ciclosporin, infliximab or visilizumab (anti-T § ↓ female fertility
cell) - Faecal leakage
• Surgical
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© Alasdair Scott, 2018
 Crohn’s Disease: Management
Ix Inducing Remission in Mild / Mod Disease
• Bloods: (top 3 are severity markers) • OPD treatment
§ FBC: ↓Hb, ↑WCC
§ LFT: ↓albumin Supportive
§ ↑CRP/ESR • High fibre diet
§ Haematinics: Fe, B12, Folate • Vitamin supplements
§ Blood cultures
• Stool
Oral Therapy
§ MCS: exclude Campy, Shigella, Salmonella… st
• 1 line
§ CDT: C. diff may complicate or mimic
§ Ileocaecal: budesonide
• Imaging
§ Colitis: sulfasalazine
§ AXR: obstruction, sacroileitis nd
• 2 line: prednisolone (tapering)
§ CXR: perforation rd
§ MRI • 3 line: methotrexate
th
§ Assess pelvic disease and fistula • 4 line: infliximab or adalimumab
§ Assess disease severity
§ Small bowel follow-through or enteroclysis Perianal Disease
§ Skip lesions • Occurs in ~50%
§ Rose-thorn ulcers • Ix: MRI + EUA
§ Cobblestoning: ulceration + mural oedema • Rx
§ String sign of Kantor: narrow terminal ileum § Oral Abx: metronidazole
• Endoscopy § Immunosuppression ± infliximab
§ Ileocolonoscopy + regional biopsy: Ix of § Local surgery ± seton insertion
choice
§ Wireless capsule endoscopy Maintaining Remission
§ Small bowel enteroscopy •
st
1 line: azathioprine or mercaptopurine
nd
• 2 line: methotrexate
rd
• 3 line: Infliximab / adalimumab
Severe Attack
Surgery
Assessment • 50-80% need ≥1 operation in their life
• ↑temp, ↑HR, ↑ESR, ↑CRP, ↑WCC, ↓albumin • Never curative
• Should be as conservative as possible
Management
• Resus: Admit, NBM, IV hydration Indications
• Hydrocortisone: IV + PR if rectal disease • Emergency
• Abx: metronidazole PO or IV § Failure to respond to medical Rx
• Thromboprophylaxis: LMWH § Intestinal obstruction or perforation
• Dietician Review § Massive haemorrhage
§ Elemental diet • Elective
- Liquid prep of amino acids, glucose and § Abscess or fistula
fatty acids § Perianal disease
§ Consider parenteral nutrition § Chronic ill health
• Monitoring § Carcinoma
§ Vitals + stool chart
§ Daily examination Procedures
• Limited resection: e.g. ileocaecal
Improvement → oral therapy • Stricturoplasty
• Switch to oral pred (40mg/d) • Defunction distal disease c̄ temporary loop ileostomy

No Improvement → rescue therapy Complications

• Discussion between pt, physician and surgeon • Stoma complications
• Medical: methotrexate ± infliximab • Enterocutaneous fistulae
• Surgical • Anastomotic leak or stricture

Short gut
• <1-2m small bowel
• Features
§ Steatorrhoea
§ ADEK and B12 malabsorption
§ Bile acid depletion → gallstones
§ Hyperoxaluria → renal stones
• Rx
§ Dietician
§ Supplements or TPN
§ Loperamide

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 Diverticular Disease
Definitions Mx of Acute Diverticulitis
• Diverticulum = out-pouching of tubular structure • Mild Attacks
• True = composed of complete wall (e.g. Meckel’s) § Can be treated at home c̄ bowel rest (fluids
• False = composed of mucosa only (pharyngeal, only) and co-amoxiclav ± metronidazole
colonic) • Admit if
• Diverticular disease: symptomatic diverticulosis § Unwell
• Diverticulitis: inflammation of diverticula § Fluids can’t be tolerated
§ Pain can’t be controlled
Epidemiology • Medical
§ NBM
• 30% of Westerner’s have diverticulosis by 60yrs
§ IV fluids
• F>M
§ Analgesia
§ Antibiotics: cefuroxime + metronidazole
Pathophysiology § Most cases settle
• Assoc. c̄ ↑ intraluminal pressure • Surgical
§ Low fibre diet: no osmotic effect to keep stool § Indications
wet - Perforation
• Mucosa herniates through muscularis propria at points - Large haemorrhage
of weakness where perforating arteries enter. - Stricture → obstruction
• Most commonly located in sigmoid colon § Procedure
• Commoner in obese pts. - Hartmann’s to resect diseased bowel
§ Uniting factor in Saint’s Triad? - May consider lap washout for Hinchey 3
- Hiatus Hernia
- Cholelithiasis Other Complications
- Diverticular disease
Perforation
Symptoms of Diverticular Disease • Sudden onset pain (± preceding diverticulitis)
• Altered bowel habit ± left-sided colic • Generalised peritonitis and shock
§ Relieved by defecation • CXR: free air under diaphragm
• Nausea • Rx: Hartmann’s
• Flatulence
• Rx Haemorrhage
§ High fibre diet, mebeverine may help • Sudden, painless bright red PR bleed
§ Elective resection for chronic pain
• Ix: mesenteric angiography or colonoscopy
• Rx
Diverticulitis § Usually stops spontaneously
• Inspissated faeces → obstruction of diverticulum § May need transfusion
• Elderly pt. c̄ prev Hx of constipation § Colonoscopy ± diathermy / adrenaline
§ Embolisation
Presentation § Resection
• Abdominal pain and tenderness
§ Typically LIF Abscess
§ Localised peritonitis • Walled-off perforation
• Pyrexia • Swinging fever
• Localising signs: e.g. boggy rectal mass
Ix • Leukocytosis
• Bloods • Rx: Abx + CT/US-guided drainage
§ FBC: ↑WCC
§ ↑CRP/ESR Fistulae
§ Amylase • Enterocolic
§ G+S/x-match • Colovaginal
• Imaging • Colovesicular: pneumaturia + intractable UTIs
§ Erect CXR: look for perforation • Rx: resection
§ AXR: fluid level / air in bowel wall
§ Contrast CT
Strictures
• Endoscopy
• After diverticulitis, colon may heal c̄ fibrous strictures
§ Flexi Sig
§ Colonoscopy: not in acute attack • Rx
O
§ Resection (usually c̄ 1 anastomosis)
Hinchey Grading of Perforated Diverticulitis § Stenting

1 Small confined pericolic Surgery rarely

abscesses needed
2 Large abscess extending into May resolve w/o
pelvis surgery
3 Generalised purulent peritonitis Surgery needed
4 Generalised faecal peritonitis Surgery needed
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 Bowel Obstruction: Causes and Investigation
Classification Presentation
• Simple • Abdominal Pain
§ 1 obstructing point + no vascular compromise § Colicky
§ May be partial or complete § Central but level depends on gut region
• Closed Loop § Constant / localised pain suggests
§ Bowel obstructed @ two points strangulation or impending perforation
- Left CRC c̄ competent ileocaecal valve • Distension
- Volvulus § ↑ c̄ lower obstructions
§ Gross distension → perforation • Vomiting
• Strangulated § Early in high obstruction
§ Compromised blood supply § Late or absent in low obstructions
§ Localised, constant pain + peritonism • Absolute Constipation: flatus and faeces
§ Fever + ↑WCC
Examination
Commonest Causes • ↑HR: hypovolaemia, strangulation
• Dehydration, hypovolaemia
SBO • Fever: suggests inflammatory disease or strangulation
• Adhesions: 60% • Surgical scars
• Hernia • Hernias
• Mass: neoplastic or inflammatory
LBO • Bowel sounds
• Colorectal Neoplasia: 60% § ↑: mechanical obstruction
• Diverticular stricture: 20% § ↓: ileus
• Volvulus: 5% • PR
§ Empty rectum
§ Rectal mass
§ Hard impacted stool
Other Causes § Blood from higher pathology

Non-Mechanical = Paralytic Ileus (usually SB) Ix

• Post-op • Bloods
• Peritonitis § FBC: ↑WCC
• Pancreatitis or any localised inflammation § U+E: dehydration, electrolyte abnormalities
• Poisons / Drugs: anti-AChM (e.g. TCAs) § Amylase: ↑↑ if strangulation/perforation
• Pseudo-obstruction § VBG: ↑ lactate in strangulation
• Metabolic: ↓K, ↓Na, ↓Mg, uraemia § G+S, clotting: may need surgery
• Mesenteric ischaemia • Imaging
§ Erect CXR
Mechanical § AXR: ± erect film for fluid levels
• Intraluminal § CT: can show transition point
§ Impacted matter: faeces, worms, bezoars • Gastrograffin studies
§ Intussusception § Look for mechanical obstruction: no free
§ Gallstones flow
• Intramural § Follow through or enema
§ Benign stricture § Follow through may relieve mild
- IBD mechanical obstruction: usually adhesional
- Surgery • Colonoscopy
- Ischaemic colitis § Can be used in some cases
- Diverticulitis § Risk of perforation
§ May be used therapeutically to stent
- Radiotherapy
§ Neoplasia
§ Congenital atresia AXR Findings
• Extramural
§ Hernia SBO LBO
§ Adhesions Diameter ≥3cm ≥6cm (caecum ≥9)
§ Volvulus (sigmoid, caecal, gastric) Location Central Peripheral
§ Extrinsic Compression Markings Valvulae coniventes Haustra
- Pseudocyst - completely across - partially across
- Abscess LB Gas Absent Present
- Haematoma - not in rectum
- Tumour: e.g. ovarian No. of loops Many Few
- Congenital bands (e.g. Ladd’s) Fluid levels Many, short Few, long

Ileus
• Both small and large bowel may be visible
• No clear transition point

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 Bowel Obstruction: General Management
Medical Surgical

Resuscitate: “Drip and Suck” Indications

• NBM
• IV fluids: aggressive as pt. may be v. dehydrated
• NGT: decompress upper GIT, stops vomiting,
prevents aspiration
• Catheterise: monitor UO
Therapy
• Analgesia: may require strong opioid
• Antibiotics: cef+met if strangulation or perforation
• Gastrograffin study: oral or via NGT
• Consider need for parenteral nutrition
Monitoring
• Regular clinical examination is necessary to ensure
that the pt. is not deteriorating.
§ ↑ distension
§ ↑ pain or tenderness
§ ↑ HR/ ↑RR
• Repeat imaging and bloods
• Non-operative Mx successful in ~80% of pts. c̄ SBO
w/o peritonitis
• Pts. c̄ LBO are more likely to need surgery.
• Closed loop obstruction
• Obstructing neoplasm
• Strangulation / perforation → sepsis, peritonitis
• Failure of conservative Mx (up to 72h)
Principals
• Aim to treat the cause
• Typically involves resection of the obstructing lesion
• Colon has not been cleansed therefore most surgeons
utilise a proximal ostomy post-resection.
• Pts. c̄ substantial comorbidity or unresectable tumours
may be offered bypass procedures.
• Endoscopically placed expanding metal stents offer
palliation or a bridge to surgery allowing optimisation.
Procedures
• Must consent pt. for possible resection ± stoma
• SBO: adhesiolysis
• LBO
§ Hartmann’s
O
§ Colectomy + 1 anastomosis + on table lavage
§ Palliative bypass procedure
§ Transverse loop colostomy or loop ileostomy
§ Caecostomy

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 Bowel Obstruction: Specific Management
Sigmoid Volvulus (80% of volvulus) Paralytic Ileus

Pathophysiology Presentation
O
• Long mesentery c̄ narrow base predisposes to torsion • Adynamic bowel 2 to the absence of normal
O
• Usually due to sigmoid elongation 2 to chronic peristalsis
constipation • Usually SBO
• ↑ risk in neuropsych pts.: MS, PD, psychiatric • Reduced or absent bowel sounds
§ Disease or Rx interferes c̄ intestinal motility • Mild abdominal pain: not colicky
• → closed loop obstruction
Cause
Presentation • Post-op
• Commoner in males • Peritonitis
• Often elderly, constipated, co-morbid pts. • Pancreatitis or any localised inflammation
• Massive distension c̄ tympanic abdomen • Poisons / Drugs: anti-AChM (e.g. TCAs)
• Pseudo-obstruction
AXR • Metabolic: ↓K, ↓Na, ↓Mg, uraemia
• Characteristic inverted U / coffee bean sign • Mesenteric ischaemia

Mx Prevention
• Often relieved by sigmoidoscopy and flatus tube • ↓ bowel handling
insertion • Laparoscopic approach
§ Monitor for signs of bowel ischaemia following • Peritoneal lavage after peritonitis
decompression.
• Sigmoid colectomy occasionally required Mx
§ Failed endoscopic decompression • Conservative “drip and suck” Mx
§ Bowel necrosis • Correct any underlying causes
• Often recurs \ elective sigmoidectomy may be § Drugs
needed § Metabolic abnormalities
• Consider need for parenteral nutrition
• Exclude mechanical cause if protracted
Caecal Volvulus
• Assoc. c̄ congenital malformation where caecum is
not fixed in the RIF. Colonic Pseudo-obstruction / Ogilvie’s Syn.
• Only ~10% of pts. can be detorsed c̄ colonoscopy
§ \ typically requires surgery Presentation
•
O
Right hemi c̄ 1 ileocolic anastomosis • Clinical signs of mechanical obstruction but no
• Caecostomy obstructing lesion found
• Usually distension only: no colic

Cause
Gastric Volvulus • Aetiology unknown
• Assoc. c̄
Triad of gastro-oesophageal obstruction § Elderly
• Vomiting → retching c̄ regurgitation of saliva § Cardiorespiratory disorders
• Pain § Pelvic surgery: e.g. hip arthroplasty
• Failed attempts to pass an NGT § Trauma

Risk Factors Ix
• Congenital • Gastrograffin oral/enema: exclude mechanical
§ Bands cause
§ Rolling / Paraoesophageal hernia
§ Pyloric stenosis Mx
• Acquired • Neostigmine: anti-cholinesterase
§ Gastric / oesophageal surgery • Colonoscopic decompression: 80% successful
§ Adhesions

Ix
• Gastric dilatation
• Double fluid level on erect films

Mx
• Endoscopic manipulation
• Emergency laparotomy

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 Colorectal Carcinoma: Pathology and Presentation
Epidemiology Pathology
rd
• 3 commonest cancer • 95% adenocarcinoma
nd
• 2 commonest cause of cancer deaths (16,000/yr) § Others: lymphoma, GIST, carcinoid
• Age: peak in 60s • Location
• Sex: rectal Ca commoner in men § Rectum: 35%
• Geo: Western disease § Sigmoid: 25%
§ Caecum and ascending colon: 20%
§ Transverse: 10%
Pathophysiology
§ Descending: 5%
• Proximal tumours: sessile or polypoid
Colonic Adenomas • Distal tumours: annular stenosing
• Benign precursors to CRC
• Characterised by dysplastic epithelium
• Classification Spread
§ Tubular: small, pedunculated, tubular glands • Local
§ Villous: large, sessile, covered by villi • Lymphatic
§ Tubulovillous: mixture • Blood (liver, lungs)
• Presentation • Transcoelomic
§ Typically asymptomatic
§ Large polyps can bleed → IDA Presentation
§ Villous adenomas can → ↓K +
hypoproteinaemia Left
• Malignant potential • Altered bowel habit
§ ↑ size
• PR mass (60%)
§ ↑ dysplasia
• Obstruction (25%)
§ ↑ villous component
• Bleeding / mucus PR
• Tenesmus
APC et al.
• -ve regulator of β-catenin (component of WNT
Right
pathway)
• Anaemia
• APC binds to and promotes degradation of β-catenin.
• Wt. loss
• APC mutation → ↑ β-catenin → ↑ transcription of
genes which promote cell proliferation. • Abdominal pain
• Proliferation → mutation of other genes which
promote growth and prevent apoptosis Either
§ KRAS (proto-oncogene) • Abdominal mass
§ p53 (TSG) • Perforation
• Haemorrhage
Adenoma → Carcinoma Sequence (Vogelstein) • Fistula
• First hit: mutation of one APC copy
• Second hit: mutation of second APC copy Examination
§ → adenoma formation • Palpable mass: per abdomen or PR
• Additional mutations in adenoma → malignant • Perianal fistulae
transformation: e.g KRAS, p53 • Hepatomegaly
• Anaemia
Other Aetiological Factors • Signs of obstruction
• Diet: ↓ fibre + ↑ animal fat / protein
• IBD: CRC in 15% c̄ pancolitis for 20yrs
• Familial: FAP, HNPCC, Peutz-Jeghers
• Smoking
• Genetics
§ No relative: 1/50 CRC risk
st
§ One 1 degree: 1/10
• NSAIDs / Aspirin (300mg/d): protective

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 Colorectal Carcinoma: Ix, Mx and Prevention
Ix Surgery
• Bloods • Use ERAS pathway
§ FBC: Hb • Pre-operative bowel prep (except R sided lesions)
§ LFTs: mets § E.g. Kleen Prep (Macrogol: osmotic laxative)
§ Tumour Marker: CEA (carcinoembryonic Ag) the day before and phosphate enema in the
• Imaging AM.
§ CXR: lung mets • Consent: discuss stomas
§ US liver: mets • Stoma nurse consult for siting
§ CT and MRI
- Staging Principals
- MRI best for rectal Ca and liver mets • Excision depends on lymphatic drainage which follows
§ Endoanal US: staging rectal tumours arterial supply.
§ Ba / gastrograffin enema: apple-core lesion
• Mobility of bowel and blood supply at cut ends is also
• Endoscopy + Biopsy important.
§ Flexi sig: 65% of tumours accessible
• Hartmann’s often used if obstruction.
§ Colonoscopy
• Laparoscopic approach is the standard of care

Rectal Ca
Staging • Neo-adjuvant radiotherapy may be used to ↓ local
recurrence and ↑5ys
Dukes (Sir Cuthbert Dukes: St. Mark’s pathologist) • Anterior resection: tumour 4-5cm from anal verge
§ Defunction c̄ loop ileostomy
Spread % 5ys • AP resection: <4cm from anal verge
A Confined to bowel wall 90 • + Total mesorectal excision for tumours of the
B Through bowel wall but no LNs 60 middle and lower third.
C Regional LNs 30 § Aims to ↓ recurrence
§ ↑ anastomotic leak and faecal incontinence
D Distant mets <10
Other Tumours
TNM
• Sigmoid: high anterior resection or sigmoid colectomy
• TIS: carcinoma in situ
• Left: left hemicolectomy
• T1: submucosa
• Transverse: extended right hemicolectomy
• T2: muscularis propria
• Caecal / right: right hemicolectomy
• T3: subserosa
• T4: through the serosa to adjacent organs
• N1: 1-3 nodes
• N2: >4 nodes Other Rx
• Local excision: e.g. Transanal Endoscopic Microsurg
• Bypass surgery: palliation
• Hepatic resection: if single lobe mets only
Grading
• Stenting: palliation or bridge to surgery in obstruction
• Grading from low to high
• Chemo
• Based on cell morphology
§ Adjuvant 5-FU for Dukes’ C ↓ mortality by 25%
• Dysplasia, mitotic index, hyperchromatism
- i.e. LN +ve pts.
§ High grade tumour
§ Palliation of metastatic disease
Mx
• Manage in an MDT
• Confirmation of Dx
• Stage c̄ CT or MRI NHS Screening for CRC
• 60% amenable to radical surgery
FOB Testing
• Introduced in 2006
• 60-75yrs
• Home FOB testing every 2yrs: ~1/50 have +ve FOB
• Colonoscopy if +ve: ~1/10 have Ca
• Lindholm et al. BJS 2008
§ Screening ↓ risk of dying from CRC by 25%

Flexible Sigmoidoscopy
• Introduced in 2011/12
• 55-60yrs
• Once only flexi Sig
• Atkin et al. Lancet 2010
§ ↓ CRC incidence by 33%
§ ↓ CRC mortality by 43%

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Familial CRC Syndromes GI Polyps
Familial Adenomatous Polyposis Inflammatory Pseudopolyps
• Autosomal dominant • Regenerating islands of mucosa in UC
• APC gene on 5q21
Hyperplastic Polyps
Presentation • Piling up of goblet cells and absorptive cells
• 100-1000s of adenomas by ~16yrs • Serrated surface architecture
§ Mainly in large bowel • No malignant potential
§ Also stomach and duodenum (near ampulla)
• 100% develop CRC, often by ~40yrs Hamartomatous
• May be assoc. c̄ congenital hypertrophy of the retinal • Tumour-like growths composed of tissues present at
pigment epithelium (CHPRE) site where they develop.
• Sporadic or part of familial syndromes
Variants • Juvenile polyp: solitary hamartoma in children
• Attenuated FAP: <100 adenomas, later CRC (>50yrs) § “Cherry on a stalk”
• Gardener’s (TODE)
§ Thyroid tumours Neoplastic
§ Osteomas of the mandible, skull and long • Tubular or villous adenomas
bones • Usually asymptomatic
§ Dental abnormalities: supernumerary teeth • May have blood / mucus PR, tenesmus
§ Epidermal cysts
• Turcot’s: CNS tumours: medullo- and glio-blastomas
Other Hamartomatous Polyposis Syndromes
Mx
• Prophylactic colectomy before 20yrs
• Total colectomy + IRA
Juvenile Polyposis
§ Requires life-long rectal stump surveillance • Autosomal dominant
• Proctocolectomy + IPAA • >10 hamartomatous polyps
• Remain @ risk of Ca in stomach and duodenum • ↑ CRC risk: need surveillance and polypectomy
§ Regular endoscopic screening
Cowden Syndrome
• Auto dominant
• Macrocephaly + skin stigmata
Hereditary Non-Polyposis Colorectal Cancer • Intestinal hamartomas
• Autosomal dominant
• ↑ risk of extra-intestinal Ca
• Mutation of mismatch repair enzymes
§ E.g. MSH2 on Chr 2p
• Commonest cause of hereditary CRC: 3% of all CRC

Presentation
• Lynch 1: right sided CRC
• Lynch 2: CRC + gastric, endometrial, prostate, breast

Dx: “3, 2, 1, rule”

• ≥3 family members over 2 generations c̄ one <50yrs

Peutz-Jeghers Syndrome
• Autosomal dominant
• STK11 mutation

Presentation
• ~ 10-15yrs
• Mucocutaneous hyperpigmentation
§ Macules on palms, buccal mucosa
• Multiple GI hamartomatous polyps
§ Intussusception
§ Haemorrhage
• ↑ Ca risk
§ CRC, pancreas, breast, lung, ovaries, uterus

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Mesenteric Ischaemia Lower GI Bleed
Acute Causes

Causes Common / Important

• Arterial: thrombotic (35%), embolic (35%) • Rectal: haemorrhoids, fissure
• Non-occlusive (20%) • Diverticulitis
O
§ Splanchnic vasoconstriction: e.g. 2 to shock • Neoplasm
• Venous thrombosis (5%)
• Other: trauma, vasculitis, strangulation Other
• Inflammation: IBD
Presentation • Infection: shigella, campylobacter, C. diff
• Nearly always small bowel • Polyps
• Triad • Large upper GI bleed (15% of lower GI bleeds)
§ Acute severe abdominal pain ± PR bleed • Angio: dysplasia, ischaemic colitis, HHT
§ Rapid hypovolaemia → shock
§ No abdominal signs Ix
• Degree of illness >> clinical signs • Bloods: FBC, U+E, LFT, x-match, clotting, amylase
• May be in AF • Stool: MCS
• Imaging
Ix § AXR, erect CXR
• Bloods § Angiography: necessary if no source on
§ ↑Hb: plasma loss endoscopy
§ ↑WCC § Red cell scan
§ ↑ amylase • Endoscopy
§ Persistent metabolic acidosis: ↑lactate st
§ 1 : Rigid proctoscopy / sigmoidoscopy
• Imaging nd
§ 2 : OGD
rd
§ AXR: gasless abdomen § 3 : Colonoscopy: difficult in major bleeding
§ Arteriography / CT/MRI angio
Mx
Complications • Resuscitate
• Septic peritonitis • Urinary catheter
• SIRS → MODS • Abx: if evidence of sepsis or perf
• PPI: if upper GI bleed possible
Mx • Keep bed bound: need to pass stool may be large
• Fluids bleed → collapse
• Abx: gent + met • Stool chart
• LMWH • Diet: keep on clear fluids (allows colonoscopy)
• Laparotomy: resect necrotic bowel • Surgery: only if unremitting, massive bleed

Chronic Small Bowel Ischaemia

• Cause: atheroma + low flow state (e.g. LVF) Angiodysplasia
• Presentation: • Submucosal AV malformations
§ Severe, colicky post-prandial abdo pain • 70-90% occur in right colon
- “gut claudication” • Can affect anywhere in GIT
§ PR bleeding
§ Malabsorption Presentation
§ Wt. loss
• Elderly
• Mx: angioplasty
• Fresh PR bleeding

Chronic Large Bowel Ischaemia Ix

• Cause: follows low flow in IMA territory • Exclude other Dx
• Presentation § PR exam
§ Lower, left-sided abdominal pain § Ba enema
§ Bloody diarrhoea § Colonoscopy
§ Pyrexia • Mesenteric angiography or CT angiography
§ Tachycardia
• Tc-labelled RBC scan: identify active bleeding
• Ix
§ ↑WCC
§ Ba enema: thumb-printing Rx
§ MR angiography • Embolisation
• Complications • Endoscopic laser electrocoagulation
§ May → peritonitis and septic shock • Resection
§ Strictures in the long-term
• Mx
§ Usually conservative: fluids and Abx
§ Angioplasty and endovascular stenting

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Key Anatomy Minor Anal Conditions
Rectum
Perianal Haematoma
• 12cm
• Sacral promontory to levator ani muscle
• The 3 tenia coli fuse around the rectum to form a
Definition
continuous muscle layer. • Subcutaneous bleeding from a burst venule caused
by straining or the passage of hard stool.
• Also called an external pile: a misnomer
Anal Canal
• 4cm Presentation
• Levator ani muscle to anal verge
• Tender blue lump at the anal margin
• Upper 2/3 of canal
• Pain worsened by defecation or movement
§ Lined by columnar epithelium
§ Insensate
§ Sup. rectal artery and vein Rx
§ Internal iliac nodes • Analgesia + spontaneous resolution
• Lower 1/3 of canal • Evacuation under LA
§ Lined by squamous epithelium
§ Sensate
§ Middle and inf. rectal arteries and veins Proctalgia Fugax
§ Superficial inguinal nodes • Young, anxious men
• Dentate line = squamomucosal junction • Crampy anorectal pain, worse @ night
• White line = where anal canal becomes true skin • Unrelated to defecation
• Assoc. c̄ trigeminal neuralgia
• Rx
Anal Sphincters § Reassurance
§ GTN cream
Internal
• Thickening of rectal smooth muscle
• Involuntary control Perineal Warts
• Commonly seen in MSM
External • Condylomata accuminata
• Three rings of skeletal muscle § HPV
§ Deep § Rx: podophyllin paint, cryo, surgical excision
§ Superficial • Condylomata lata
§ Subcutaneous § Syphilis
• Voluntary control § Rx: penicillin

Anorectal Ring
• Deep segment of external sphincter which is Pruritis Ani
continuous c̄ puboretalis muscle (part of levator ani) • 50% idiopathic
• Palpable on PR ~5cm from the anus • Poor hygiene
• Demarcates junction between anal canal and rectum. • Haemorrhoids
• Must be preserved to maintain continence • Anal fissure
• Anal fistula
• Fungi, worms
• Crohn’s
• Neoplasia

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 Haemorrhoids
Definition Symptoms
• Disrupted and dilated anal cushions • Fresh painless PR bleeding
§ Bright red
§ On paper, on stool, may drip into pan
Pathophysiology
• Pruritis ani
• Anal cushion: mass of spongy vascular tissue
• Lump in perianal area
• Positioned @ 3, 7 and 11 O’clock
• Severe pain = thrombosis
§ Where the three major arteries that feed the
vascular plexuses enter the anal canal
• Gravity, straining → engorgement and enlargement of Examination
anal cushions • Full abdo exam, palpating for masses
• Hard stool disrupts connective tissue around • Inspect perianal area: masses, recent bleeding
cushions. • DRE: can’t palpate piles unless thrombosed
• Cushions protrude and can be damaged by hard stool • Rigid sig to identify higher rectal pathology
§ → bright red (capillary) bleeding. • Proctoscopy (also allows Rx)
• Haemorrhoids arise above dentate line \ not painful
• May be gripped by anal sphincter → thrombosis Differential
§ Strangulated piles are acutely painful • Perianal haematoma
§ May ulcerate or infarct • Fissure
• Abscess
Causes • Tumour (must exclude in all cases)
• Constipation c̄ prolonged straining
• Venous congestion may contribute Mx
§ Pregnancy
§ Abdominal tumour Conservative
§ Portal HTN
• ↑ fibre and fluid intake
• Stop straining @ stool
Classification
st
• 1 degree: never prolapse Medical
nd
• 2 : prolapse on defecation but spontaneously reduce • Topical preparations
rd
• 3 : prolapse on defecation but require digital § Anusol: hydrocortisone
reduction § Topical analgesics
th
• 4 : remain permanently prolapsed • Laxatives: lactulose, fybogel

Interventional
• Injection c̄ sclerosant (5% phenol in Almond oil)
§ Injection above dentate line
§ SE: impotence, prostatitis
• Banding → thrombosis and separation
§ SE: bleeding, infection
• Cryotherapy
§ SE: watery discharge post-procedure
• Infra-red coagulation

Surgical: Haemorrhoidectomy
• Excision of piles + ligation of vascular pedicles
• Discharge c̄ laxatives post-op
• SE: bleeding, stenosis

Mx of Thrombosed Piles
• Analgesia
• Ice-packs
• Stool softeners
• Topical lignocaine jelly
• Pain usually resolves in 2-3wks
• Haemorrhoidectomy is not usually necessary

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Anal Fissure Fistula in Ano
Definition Definition
• Tear of squamous epithelial lining in lower anal canal • Abnormal connection between ano-rectal canal and
the skin.
Causes
•
O
Mostly trauma 2 to the passage of hard stool Pathogenesis
O
§ Assoc. c̄ constipation • Usually occur 2 to perinanal sepsis
§ Spasm of internal anal sphincter contributes to § Blockage of intramuscular glands → abscess
pain and → ischaemia + poor healing § Abscess discharges to form a fistula
§ Commoner in women • Associations
• Rarer causes, often → multiple ± lateral fissures § Crohn’s
§ Crohn’s § Diverticular disease
§ Herpes § Rectal Ca
§ Anal Ca § Immunosuppression

Presentation Classification
• Intense anal pain • High: cross sphincter muscles above dentate line
§ Especially on defecation • Low: cross sphincter muscles below dentate line
§ May prevent pt. from passing stools
• Fresh rectal bleeding Goodsall’s Rule
§ On paper mostly • Determines path of fistula tract
• Fistula anterior to anus track in a straight line (radial)
Examination • Fistula posterior to anus always have internal opening
• PR often impossible at the 6 o’clock position → curved track
• Midline ulcer is seen
Anterior
§ Usually posterior @ 6 O’clock
§ May be anterior
• May be a mucosal tag – sentinel pile
§ Usually posterior @ 6 O’clock
• Groin LNs suggest complicating factor: e.g. HIV

Mx
• If fissure recurrent, chronic or difficult to Rx the patient
requires EUA.

Conservative
• Soaks in warm bath Posterior
• Toileting advice
• Dietary advice: ↑ fibre and fluids
Presentation
Medical • Persistent anal discharge
• Laxatives: lactulose + fybogel • Perianal pain or discomfort
• Topical → resolution in 75%
§ Lignocaine Examination
§ GTN ointment • May visualise external opening: may be pus
§ Diltiazem cream • Induration around the fistula on DRE
• EUA + botulinum toxin injection • Proctosopy may reveal internal opening

Surgical: Lateral partial sphincterotomy Ix

• Division of internal anal sphincter @ 3 O’clock • MRI
• Pre-op anorectal US and manometry • Endoanal US
• V. rarely performed
• Complications Rx
§ Minor faecal/flatus incontinence (= GTN)
• Extent of fistula must first be delineated by probing the
§ Perianal abscess
fistula @ EUA
NB. Lord’s operation (anal dilatation under
Low Fistula
anaesthesia) is no longer used due to high rates of
• Fistulotomy and excision
incontinence. O
§ Laid open to heal by 2 intention

High Fistula
• Fistulotomy could damage the anorectal ring
• Suture – a seton – passed through fistula and
gradually tightened over months
§ Stimulates fibrosis of tract
§ Scar tissue holds sphincter together

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Peri-anal Sepsis / Abscess Pilonidal Sinus
Pathogenesis Definition
• Anal gland blockage → infection → abscess • Pilonidal: lat “nest of hair”
§ E.g. coli, bacteroides • Sinus: blind ending tract, lined by epithelial or
• May develop from skin infections granulation tissue, which opens onto an epithelial
§ E.g. sebaceous gland or hair follicle surface.
§ Staphs
Pathophysiology
Associations • Hair works its way beneath skin → foreign body
• Crohn’s reaction → formation of abscess
• DM • Usually occur in the natal cleft.
• Malignancy
Risk Factors
Classification • M>F=4:1
• Perianal: 45% • Geo: Mediterranean, Middle east, Asians
§ Discrete local red swelling close to the anal • Often overweight c̄ poor personal hygiene
verge • Occupations c̄ lots of sitting: e.g. truck drivers
• Ischiorectal: ≤30%
§ Systemic upset
§ Extremely painful on DRE
Presentation
• Interphincteric / intermuscular: >20% • Persistent discharge of purulent or clear fluid
• Pelvirectal / supralevator: ~5% • Recurrent pain
§ Systemic upset • Abscesses
§ Bladder irritation
Rx
Presentation
• Throbbing perianal pain Conservative
§ Worse on sitting • Hygiene advice
• Occasionally a purulent anal discharge • Shave / remove hair from affected area

Examination Surgical
• Perianal mass or cellulitic area • Incision and drainage of abscesses
• Fluctuant mass on PR • Elective sinus excision
• Septic signs: fever, tachycardia § Methylene blue to outline tract
§ Recurrence in 4-15%
Rx
• Abx may suffice if Rx instigated v. early in course
• Most cases require EUA c̄ I&D
§ Wound packed
O
§ Heals by 2 intention
§ Daily dressing for 7-10d
• Look for an anal fistula which complicates ~30% of
abscesses.

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Anal Carcinoma Rectal Prolapse
Epidemiology Definition
• Relatively uncommon: 250-300 cases/yr in the UK • Protrusion of rectal tissue through the anal canal.

Pathology Classification
• 80% SCCs
§ Others: melanomas, adenocarcinomas Type 1: Mucosal prolapse
• Anal margin tumours • Partial prolapse of redundant mucosa
§ Well differentiated keratinising lesions • Common in children: esp. c̄ CF
§ Commoner in men • Essentially large piles \ same Rx
§ Good prognosis
• Anal canal tumours
Type 2: Full thickness prolapse
§ Arise above dentate line
§ Poorly differentiated, non-keratinising • Commoner cf. type 1
§ Commoner in women • Usually elderly females c̄ poor O&G Hx
§ Worse prognosis
• Spread Presentation
§ Above dentate line → internal iliac nodes • Mass extrudes from rectum on defecation
§ Below dentate line → inguinal nodes § May reduce spontaneously or require manual
reduction
Aetiology • May become oedematous and ulcerated
• HPV (16, 18, 31, 33) is important factor § → pain and bleeding
§ ↑ incidence in MSM • Faecal soiling
§ ↑ incidence if perianal warts • Assoc. c̄ vaginal prolapse and urinary incontinence

Presentation Examination
• Perianal pain and bleeding • Visible prolapse: brought out on straining
• Pruritis ani • ± excoriation and ulceration
• Faecal incontinence • ↓ sphincter tone on PR
§ 70% have sphincter involvement @ • Assoc. uterovaginal prolapse
presentation
• May → rectovaginal fistula Ix
• Sigmoidoscopy to exclude proximal lesions
Examination • Anal manometry
• Palpable lesion in only 25% • Endoanal US
• ± palpable inguinal nodes • MRI

Ix Rx
• ↓ Hb (ACD)
• Endoanal US Partial Prolapse
• Rectal EUA + biopsy • Phenol injection
• CT / MRI: assess pelvic spread • Rubber band ligation
• Surgery: Delorme’s Procedure
Rx
• Chemoradiotherapy: most pts Complete Prolapse
§ 50% 5ys • Conservative
• Surgery: reserved for § Pelvic floor exercises
§ Tumours that fail to respond to radiotherapy § Stool softeners
§ GI obstruction • Surgery
§ Small anal margin tumours w/o sphincter § Abdominal Approach: Rectopexy
involvement - Lap or open
- Mobilised rectum fixed to sacrum c̄
mesh
§ Perineal Approach: Delorme’s Procedure
- Resect mucosa and suture the two
mucosal boundaries.

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 Inguinal Hernia
Epidemiology Clinical Features
• 3% of adults will require hernioplasty
• ~4% of male neonates have hernia (↑ c̄ prems) Children
• M>>F = 9:1 (descent of testes) • Lump in groin which may descend into scrotum
• Majority present in 50s. • Exacerbated by crying
• Commonly obstruct

Pathology Adults
• Lump in groin, exacerbated by straining/cough
• Commoner on R (?damage to ilioinguinal N. @
• May be clear ppting event: e.g. heavy lifting
appendicectomy → muscle weakness)
• Dragging pain radiating to groin
• 5% bilateral
• 8-15% present as emergency c̄ • May present c̄ obstruction/strangulation
strangulation/obstruction
Questions
• Reducible?
• Ever episodes of obstruction / strangulation?
Aetiology • Predisposing factors: cough, straining, lifting?
• Occupation and social circumstances?
Congenital: patent processus vaginalis
• Processus vaginalis should obliterate following
descent of the testes.
• If it stays patent it may fill with Mx
§ Fluid → hydrocele
§ Bowel/omentum → indirect hernia Non-surgical
• Rx RFs: cough, constipation
Acquired: mainly things which ↑IAP • Lose weight
• Chronic cough: COPD, asthma • Truss
• Prostatism
• Constipation Surgical
• Severe muscular effort: e.g. heavy lifting • Tension-free mesh (e.g. Lichtenstein repair) better
• Previous incision/repair cf. suture repair (e.g. Shouldice repair)
• Ascites / obesity § Recurrence ~0.5% vs. 10%
• Appendicectomy • Open approach can be done under LA or GA
• Lap approach allows bilateral repair and improved
cosmesis. Also preferred for recurrent hernias.
Classification • O
1 unilateral repairs should be open (NICE)
• Children only require sac excision (herniotomy)
Indirect
• 80%: commoner in young Complications
• Congenital patent processus vaginalis • Early
• Emerge through deep ring § Haematoma / seroma formation: 10%
• Same 3 coverings as cord and descend into the § Intra-abdominal injury (lap)
scrotum § Infection: 1%
• Can strangulate § Urinary retention
• Late
Direct § Recurrence (~0.5%)
§ Ischaemic orchitis: 0.5%
• 20%: commoner in elderly
§ Chronic groin pain / paraesthesia: 5-10%
• Acquired
• Emerge through Hesselbach’s triangle
• Can acquire internal and external spermatic fascia
• Rarely descend into scrotum
• Rarely strangulate

Ix
• US if Dx in doubt

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Femoral Hernia Incisional Hernia
Definition Definition
• Protrusion of viscus through the femoral canal • Hernia arises through a previously acquired defect

Epidemiology Epidemiology
• F>M • 6% of surgical incisions
§ Inguinal hernias still more common in F
• Middle aged and elderly
Risk Factors

Aetiology Pre-operative
• Acquired: ↑ intra-abdominal pressure • ↑ age
• Femoral canal larger in females due to shape of pelvis • Obesity or malnutrition
and changes in its configuration due to childbirth • Comorbidities: DM, renal failure, malignancy
• Drugs: steroids, chemo, radio

Clinical features Intra-operative

• Surgical technique/skill (major factor)
§ Too small suture bites
Painless groin lump
§ Inappropriate suture material
• Neck inferior (and lateral) to the pubic tubercle. • Incision type (e.g. midline)
• Cough impulse. • Placing drains through wounds
• Often irreducible (tight boarders)
Post-operative
Commonly present c̄ obstruction or strangulation • ↑ IAP: chronic cough, straining, post-op ileus
• Tender, red and hot • Infection
• Abdo pain, distension, vomiting, constipation • Haematoma

Mx Mx
• 50% risk of strangulation w/i 1mo • Surgery is not appropriate for many patients.
• Urgent surgery • Must balance risk of operation and recurrence c̄ risk of
obstruction / strangulation and pt. choice.
Elective: Lockwood Approach • Usually broad-necked \ low risk of strangulation
• Low incision over hernia c̄ herniotomy and
herniorrhaphy (suture ing. ligt. to pectineal ligt.) Conservative
• Manage RFs: e.g. constipation, cough
Emergency: McEvedy Approach • Weight loss
• High approach in inguinal region to allow inspection • Elasticated corset or truss
and resection of non-viable bowel.
• Then herniotomy and herniorrhaphy Surgical
• Pre-op
§ Optimise cardiorespiratory function
§ Encourage wt. loss
• Mesh repair: open or lap

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Other Hernia Types
Umbilical Features Management
• Congenital • Usually resolves by 2-3yrs
• 3% of live births • Surgical repair if no closure.
• Defect in the umbilical scar • Can recur in adults: pregnancy or gross
ascites
Risk Factors
• Afro-Caribbean
• Trisomy 21
• Congenital hypothyroidism

Paraumbilical Features Management

• Acquired: middle aged obese men • Mayo (double-breast linea alba c̄ sutures) /
• Defect through linea alba just above or mesh repair
below umbilicus
• Small defect ® strangulation (often
omentum)

Risk Factors
• Chronic cough
• Straining

Epigastric Features Management

• Young M>F • Mesh/suture repair
• Pea-sized swelling caused by defect in
linea alba above the umbilicus.
• Usually contains omentum: can strangulate

Spigelian Features
• Hernia through linea semilunaris
• Hernia lies between layers of abdo wall
• Palpable mass more likely to be colon Ca

Obturator Features
• Old aged F>M
• Sac protrudes through obturator foramen
• Pain on inner aspect of thigh or knee
• Frequently present obstructed / strangulated

Lumbar Features
• Middle-aged M>F
• Typically follow loin incisions
• Hernias through sup./inf. lumbar triangles

Sciatic Features
• Hernia through lesser sciatic foramen
• Usually presents as SBO + gluteal mass

Gluteal Features
• Hernia through greater sciatic foramen
• Usually presents as SBO + gluteal mass

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 Malignant and Pre-malignant Skin Lesions
Malignant Melanoma Squamous Cell Carcinoma
• Ulcerated lesion c̄ hard, raised everted edges
Epidemiology • Sun exposed areas
• F>M = 1.5:1
• UK incidence = 10 000/yr and 2000 deaths/yr Causes
§ ↑ 80% in 20yrs • Sun exposure: scalp, face, ears, lower leg
• May arise in chronic ulcers: Marjolin’s Ulcer
Features • Xeroderma pigmentosa
• Asymetry
• Boarder: irregular Evolution
• Colour: non-uniform • Solar/actinic keratosis → Bowen’s → SCC
• Diameter >6mm • Lymph node spread is rare
• Evolving / Elevation
Rx
Risk Factors • Excision + radiotherapy to affected nodes
• Sunlight: esp. intense exposure in early years.
• Fair skinned (low Fitzpatrick skin type)
• ↑ no. of common moles
• +ve FH
Actinic Keratoses
• ↑ age • Irregular, crusty warty lesions.
• Immunosuppression • Pre-malignant (~1%/yr)

Classification Rx
• Superficial Spreading: 80% • Cautery
§ Irregular boarders, colour variation • Cryo
§ Commonest in Caucasians • 5-FU
§ Grow slowly, metastasise late = better • Imiquimod
prognosis • Photodynamic phototherapy
• Lentigo Maligna Melanoma
§ Often elderly pts.
§ Face or scalp Bowen’s Disease
• Acral Lentiginous • Red/brown scaly plaques
§ Asians/blacks • Typically on the legs of older women
§ Palms, soles, subungual (c̄ Hutchinson’s sign) • SCC in situ
• Nodular Melanoma
§ All sites Rx
§ Younger age, new lesion • As for AKs
§ Invade deeply, metastasis early = poor prog
• Amelanotic
§ Atypical appearance → delayed Dx
Basal Cell Carcinoma
Staging and Prognosis • Commonest cancer
• Pearly nodule c̄ rolled telangiectactic edge
• Breslow Depth
§ Thickness of tumour to deepest point of dermal • May ulcerate
invasion • Typically on face in sun-exposed area
§ <1mm = >75% 5ys § Above line from tragus → angle of mouth
§ >4mm = 50% 5ys
• Clark’s Staging Behaviour
• Stratifies depth by 5 anatomical levels • Low-grade malignancy → very rarely metastasise
§ Stage 1: Epidermis • Locally invasive
§ Stage 5: sc fat
Rx
Metastasis • Excision
• Liver § Mohs: complete circumferential margin
• Eye assessment using frozen section histology
• Cryo/radio may be used.
Mx
O
• Excision + 2 margin excision depending on Bres
depth
• ± lymphadenectomy
• ± adjuvant chemo (may use isolated limb perfusion)

Poor prognostic indicators

• Male sex (more tumours on trunk cf. females)
• ↑ mitoses
• Satellite lesions (lymphatic spread)

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Neck Lumps: General Approach Anterior Triangle Lumps
Diagnosis Branchial Cysts
nd
• 85% of neck lumps are LNs: esp. if present < • Embryological remnant 2 branchial cleft
3wks
§ Infection: EBV, tonsillitis, HIV Presentation
§ Ca: lymphoma or mets • Age <20yrs
• 8% are goitres • Ant. margin of SCM at junction of upper and middle 3
rd

• 7% other: e.g. sebaceous cyst or lipoma • May become infected → abscess

• May be assoc. c̄ branchial fistula
Ix: Triple Assessment
• Clinical assessment Pathology
• Imaging: US • Lined by squamous epithelium
• Cyto/Histo: aspiration or biopsy • Contain “glary” fluid c̄ cholesterol crystals

Anterior triangle Rx
• Ant. margin of SCM • Med
• Midline § Abx for infection
• Ramus of the mandible § Sclerotherapy c̄ OK-432 can be used
• Roof: investing fascia • Surgical excision
• Floor: pre-vertebral fascia § Definitive Rx
§ May be difficult due to proximity of carotids
Causes
• Pulsatile
§ Carotid artery aneurysm Branchial sinus or fistula
rd
§ Tortuous carotid artery • Small opening in lower 3 of neck on ant. margin of SCM
§ Carotid body tumour (chemodectoma) • Between tonsillar fossa and ant. border of SCM
• Non-pulsatile • May discharge mucus
§ Branchial cysts
§ Laryngocele
§ Goitre Carotid Body Tumour: Chemodectoma
§ Parotid tumour (lump in postero-superior
• Very rare
area)
• Carotid bodies
§ Located @ carotid bifurcation
+
§ Detect pO2, pCO2 and H
Submandibular Triangle
• Mental process Presentation
rd
• Ramus of the mandible • Just anterior to upper 3 of SCM.
• Line between two angles of the mandible • Pulsatile
• Move laterally but not vertically
Causes • May be bilateral
• Salivary stone • Pressure may → dizziness and syncope
• Sialadenitis • Mostly benign (5% malignant)
• Salivary tumour
Ix: Doppler or angio: splaying of bifurcation

Posterior Triangle Rx: extirpation by a vascular surgeon

• Post. margin of SCM
• Ant. margin of trapezius
• Mid 1/3 of clavicle. Laryngocele
• Cystic dilatation of the laryngeal saccule
Causes • Congenital or acquired
• LNs • Exacerbated by blowing
• Cervical ribs
• Pharyngeal pouch
• Cystic hygromas
• Pancoast’s tumour

Midline Lumps
• <20yrs
§ Thyroglossal cyst
§ Dermoid cyst
• >20yrs
§ Thyroid isthmus mass
§ Ectopic thyroid tissue

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Midline Neck Lumps Posterior Triangle Lumps
Dermoid Cyst Cervical ribs
• Developmental inclusion of epidermis along lines of • Overdevelopment of transverse process of C7
skin fusion. • Occur in 1:150

Presentation Presentation
• Common <20yrs • Mostly asymptomatic
• Found at junctions of embryological fusion • Hard swelling
§ Neck midline • ↓ radial pulse on abduction and external rotation of arm
§ Lateral angles of eyebrow • Can → vascular symptoms
§ Under tongue § Compresses subclavian A
• Contains ectodermal elements § Raynaud’s
§ Hair follicles, sebaceous glands § Subclavian steal
§ ↓ venous outflow → oedema
Rx: excision • Can → neurological symptoms
§ Compresses lower trunk of brachial plexus, T1
nerve root or stellate ganglion.
Thyroglossal Cyst § Wasting of intrinsic hand muscles
§ Paraesthesia along medial border of arm
• Cyst formed from persistent thyroglossal duct
§ Path of thyroid descent from base of tongue

Presentation Pharyngeal Pouch: Zenker’s Diverticulum

• Can be located anywhere between foramen caecum • Herniation of pharyngeal mucosa through its muscular
and the thyroid coat at its weakest point.
§ Usually just inferior to the hyoid: subhyoid § Pulsion diverticulum
§ Or, just above the hyoid: suprahyoid • Killian’s dehiscence
• Fluctuant lump that moves up c̄ tongue protrusion § Between thyro- and crico-pharyngeal muscles that
form the inferior constrictor
• Can become infected → thyroglossal fistula

Presentation
Rx
• Swelling on left side of neck
• Sistrunk’s Op: excision of cyst and thyroglossal
duct with segment of hyoid bone • Regurgitation and aspiration
• Halitosis
• Gurgling sounds
• Food debris → pouch expansion → oesophageal
compression → dysphagia.

Ix: barium swallow

Rx
• Excision and cricopharyngeal myotomy
• Endoscopic stapling

Cystic Hygroma
• Congenital multiloculated lymphangioma arising from the
jugular lymph sac

Presentation
• Infants
• Lower part of post. triangle but may extend to axilla.
• ↑ in size when child coughs/cries
• Transilluminates brilliantly

Rx: excision or hypertonic saline sclerosant

• May recur

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Cervical Lymphadenopathy Hypertrophic and Keloid Scars
Key Features Features
• Consistency • Scar more prominent than surrounding skin
• Number
• Fixation Hypertrophic
• Symmetry • Scar confined to wound margins
• Tenderness • Across flexor surfaces and skin creases
• Appear soon after injury and regress spontaneously
Additional Examination • Any age: commonly 8-20yrs
• Face and scalp for infection or neoplasm • M=F
• Chest exam: infection or neoplasm • All races
• Breast examination
• Formal full ENT examination Keloid
• Rest of reticuloendothelial system • Scar extends beyond wound margins
• Earlobes, chin, neck, shoulder, chest
History • Appear months after injury and continue to grow
• Symptoms from the lumps • Puberty to 30yrs
§ E.g. EtOH-induced pain • F>M
• General symptoms • Black and Hispanic
§ Fever, malaise, wt. loss
• Systemic disease
§ PMH Wound Associations
§ Previous operations • Infection
• Social history • Trauma
§ Ethnic origin • Burns
§ HIV risk factors • Tension
• Certain body areas

Causes: LIST
• Lymphoma and Leukaemia Mx
• Infection
• Sarcoidosis
Non-surgical
• Tumours
• Mechanical-pressure therapy
• Topical silicone gel sheets
Infection • Intralesional steroid and LA injections
• Bacterial
§ Tonsillitis, dental abscess
§ TB
Surgical
§ Bartonella henselae (Cat-scratch disease) • Revision of scar c̄ closure by direct suturing
• Viral
§ EBV
§ HIV
• Protozoal
§ Toxoplasmosis

Ix

Blood
• FBC, ESR, film (atypical lymphocytes)
• TFTs, serum ACE
• Monospot test, HIV test

Radiological
• US
• CT scan

Pathology
• FNAC
• Excision biopsy

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 Thyroid Lumps
Congenital Thyroid Disease Ix
• Lingual thyroid
• Ectopic thyroid tissue Bloods
• Thyroglossal cyst • TFTs: TSH, fT3, fT4
2+
• Other: FBC, Ca , LFTs, ESR
Embryology • Antibodies: anti-TPO, TSH
• Thyroid migrates from its origin at the foramen
caecum at the base of the tongue. Imaging
§ Passes behind the hyoid bone • CXR: goitres and mets
rd th
§ Lies anterior to 3 -4 tracheal rings in the • High resolution US
pretracheal fascia. • CT
• Leaves behind the thyroglossal cyst which atrophies • Radionucleotide (Tc or I) scan (hot vs. cold)
§ Persistence → thyroglossal cyst
• Ectopic thyroid tissue can be found anywhere along Histology or cytology
this descent
• FNAC (can’t distinguish adenoma vs. follicular Ca)
• Biopsy
Goitre Differential
Laryngoscopy
Diffuse • Pre-operative assessment of vocal cords
• Simple colloid goitre
§ Endemic: iodine deficiency
§ Sporadic: autoimmune, hereditary,
goitrogens (e.g. sulphonylureas)
• Graves’
• Thyroiditis
§ Hashimoto’s
§ De Quervain’s
§ Subacute lymphocytic (e.g. post-partum)
• (multinodular goitre c̄ nodules too small to palpate)

Multinodular
• Multinodular colloid goitre (commonest)
• Multiple cysts
• Multiple adenomas

Solitary nodule
• Dominant nodule in multinodular goitre
• Adenoma (hot or cold)
• Cyst
• Malignancy

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 Benign Thyroid Disease
Disease Clinical Features Cause Management
Simple goitre Diffuse painless goitre Endemic Thyroxine
Mass effects: - iodine deficiency
- dysphagia Total or subtotal
- stridor Sporadic thyroidectomy if
- SVC obstruction - autoimmune pressure symptoms.
- goitrogens (e.g.
Usually euthyroid, may →hypothyroid sulphonylureas)
- hereditary (dyshormogenic)
Multinodular Evolves from long-standing simple As for simple goitre Thyroxine
goitre goitre.
Mass effects.
Euthyroid (or subclinical hyperthyroid) Total or subtotal
thyroidectomy if
pressure symptoms.
Plummer’s Multinodular goitre Autonomous nodule develops on Carbimazole
Toxic Thyrotoxicosis background multinodular goitre Radioiodine
multinodular Uneven iodine uptake with hot nodule Total or subtotal
goitre thyroidectomy.
Graves’ Diffuse goitre c̄ bruit Autoimmune (T2 hypersensitivity) Propanolol
Ophthalmopathy - anti-TSH Carbimazole
Dermopathy Radioiodine
Thyrotoxicosis Thyroidectomy
Assoc. c̄ other AI disease (T1DM, PA)
↑ uptake on radionucleotide scan
Hashimoto’s Diffuse painless goitre Autoimmune (T2 and T4) Levothyroxine
thyroiditis May have transient thyrotoxicosis, then - anti-TPO, -Tg
hypothyroidism
Assoc. c̄ other AI disease (T1DM, PA)
de Quervain’s Diffuse painful goitre Viral: Coxsackie common Self-limiting
Preceding viral URTI common
Thyrotoxicosis → hypo → eu Autoimmune
↓ iodine uptake
Subacute Diffuse painless goitre Autoimmune Self-limiting
lymphocytic May occur post-partum
Thyrotoxicosis → hypo → eu
Riedel’s Firm, fixed, irregular thyroid mass Autoimmune fibrosis Conservative
thyoiditis Mass effects
Assoc. c̄ retroperitoneal fibrosis
Follicular Single thyroid nodule Hemithyroidectomy
adenoma ± thyrotoxicosis (majority are cold)
May get pressure symptoms
Thyroid cysts Solitary thyroid nodule Aspiration or excision
Asympto or pressure symptoms
Can → localised pain due to cyst bleed

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 Malignant Thyroid Disease
Disease Frequency Age Cell Origin Spread Mx
Papillary 80% 20-40 Follicular cells Nodes and lung Total thyroidectomy
- JDG node = ± node excision
Assoc. c̄ Tg tumour marker lateral aberrant ± radioiodine
irradiation thyroid T4 to suppress TSH

Often multifocal >95% 10ys

Follicular 10% 40-60 Follicular cells Blood → bone and Total thyroidectomy +
F>M = 3:1 lungs T4 suppression +
Tg tumour marker Radioiodine

>95% 10ys
Medullary 5% MEN2: young Parafollicular C-cells Do phaeo screen pre-op
30% familial Sporadic: 40-50 Thyroidectomy +
- e.g. MEN2 CEA and calcitonin Node clearance
markers Consider radiotherapy
Anaplastic Rare >60 Undifferentiated Rapid growth Usually palliative
F>M = 3:1 follicular cells
Aggressive: local, May try thyroidectomy +
LN and blood. radiotherapy

<1% 10ys
Lymphoma 5% Lymphocytes Chemoradiotherapy
- MALToma in Hashi’s

Presentation Complications of Thyroid Surgery

• Non-functional (cold)
• Painless neck mass Early
• Cervical mets • Reactionary haemorrhage → haematoma (<1%)
• Compression symptoms § Can → airway obstruction.
§ Dysphagia § Call anaesthetist and remove wound clips
§ Stridor § Evacuate haematoma and re-explore wound
§ SVC obstruction • Laryngeal oedema
§ Damage during intubation or surgical
Risk factors for malignancy in thyroid manipulation
§ Can → airway obstruction
nodules • Recurrent laryngeal nerve palsy (0.5%)
• Solitary § Right RLN more common (oblique ascent)
• Solid § Damage to one → hoarse voice
• Younger § Damage to both → obstruction needing
• Male tracheostomy
• Cold • Hypocalcaemia (3-5%)
• Radiation exposure § Usually parathyroid dysfunction but may be
permanent if parathyroids removed.
§ Presents @ 24-48hrs
- Tingling in fingers and lips
Thyroid Surgery - Wheeze / stridor → airway obstruction
- Chvostek’s and Trousseau’s
Indications: 5 Ms § Rx: 10ml 10% Ca gluconate IV
• Mechanical obstruction • Thyroid storm
• Malignancy § Severe hyperthyroidism
• Marred beauty: cosmetic reasons § Rx: propranolol, antithyroid drugs, Lugol’s
• Medical Rx failure: thyrotoxicosis iodine and hydrocortisone sodium succinate
• Mediastinal extension: can’t monitor changes
Late
Practicalities • Hypothyroidism
• Render euthyroid pre-op c̄ antithyroid drugs • Recurrent hyperthyroidism
§ Stop 10 days prior to surgery (they ↑ • Keloid scar
vascularity)
§ Alternatively just give propronalol
• Check for phaeo pre-op in medullary carcinoma
• Laryngoscopy: check vocal cords pre- and post-op

Procedure
• Collar incision

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 Salivary Glands
History Salivary Gland Neoplasms
• Swelling/pain related to food: calculi • 80% are in the parotid (80% are superficial)
• Malaise, fever, mumps • 80% are pleiomorphic adenomas
• Dry eyes/mouth: Sjogrens (Sicca, Mickulicz) • Deflection of ear outwards is classic sign
• CN VII palsy = malignancy
Causes of Salivary Gland Enlargement
• Whole gland Classification
§ Parotitis • Benign
st
§ Sjogren’s / Sicca Syndrome § 1 : Pleiomorphic adenoma
nd
§ Sarcoid § 2 : Adenolymphoma (Warthin’s)
§ Amyloid • Malignant (CN7 palsy + fast growing)
st
§ ALL § 1 : Mucoepidermoid
nd
§ Chronic liver disease § 2 : Adenoid cystic
§ Anorexia or bulimia
• Localised Ix
§ Tumours • ENT examination
§ Stones • US ± CT
• FNAC
Acute Parotitis
• Viral: mumps, coxsackie A, HIV Pleiomorphic Adenoma
• Bacterial: S. aureus • Commonest salivary gland neoplasm (80%)
- Assoc. c̄ calculi and poor oral hygiene • Presentation
§ 90% occur in parotid
Calculi § Occur in middle age
• Recurrent unilateral swelling and pain § F>M
§ Benign and slow growing
• Worse on eating
• Histo: different tissue types (hence name)
• Red, tender, swollen gland (80% submandibular)
• Rx: superficial parotidectomy
• Ix: plain x-ray or sialography
• Rx: gland excision
Adenolymphoma (Warthin’s tumour)
• Benign soft cystic tumour
• Older men
• Rx: enucleation

Adenoid-cystic carcinoma
• One of the commonest malignant salivary tumours
• Highly malignant and often incurable
• Rapid growth
• Hard fixed mass
• Pain
• Facial palsy

Surgery
• Superficial or radical parotidectomy
• Facia lata face lift for facial palsy

Complications
• Facial palsy
• Salivary fistula
• Frey’s Syndrome (gustatory sweating)
§ Redness and sweating skin over parotid area
§ Occurs in relation to food (inc. thinking)
§ Auriculotemporal branch of CN V3 carries
sympathetic fibres to sweat glands over parotid
area and parasympathetic fibres to the parotid
§ Reinervation of divided sympathetic nerves by
fibres from the secretomotor branch of
auriculotemporal branch of CN V3

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 Breast Cancer: Pathology, Presentation and Assessment
Epidemiology Presentation
• Affects 1/10 women • Lump: commonest presentation of Ca breast
• 20 000 cases/yr in UK § Usually painless
• Commonest cause of cancer death in females 15-54 § 50% in upper outer quadrant
• Second commonest cause of cancer deaths overall § ± axillary nodes
• Skin changes
§ Paget’s: persistent eczema
Aetiology / Risk Factors § Peau d’orange
• Nipple
Family Hx § Discharge
• 10% Ca breast is familial § Inversion
st
§ One 1 degree relative = 2x risk • Mets
• 5% assoc. c̄ BRCA mutations § Pathological #
§ BRCA1 (17q) → 80% breast Ca, 40% + ov Ca § SOB
§ BRCA2 (13q) → 80% breast Ca § Abdominal pain
§ Seizures
Oestrogen Exposure • May present through screening
• Early menarche, late menopause
• HRT, OCP (Million Women Study) Differential
• First child >35yrs • Cysts
• Obesity • Fibroadenomas
• DCIS
Other Factors • Duct ectasia
• Proliferative breast disease c̄ atypia
• Previous Ca breast Triple Assessment: any breast lump
• ↑ age (v. rare <30) • Hx and Clinical Examination
• Breast feeding is protective • Radiology
§ <35yrs: US
Pathology § >35ys: US + mammography
• Pathology
§ Solid lump: tru-cut core biopsy
DCIS/LCIS
§ Cystic lump: FNAC (green / 18G needle)
• Non-invasive pre-malignant condition
- Reassure if clear fluid
• Microcalcification on mammography
- Send cytology if bloody fluid
• 10x ↑ risk of invasive Ca
- Core biopsy residual mass
- Core biopsy if +ve cytology
Invasive Ductal Carcinoma, NST/NOS
• Commonest: ~70% of cancers
• Feels hard (scirrhous) Other Ix
• Bloods
§ FBC, LFTs, ESR, bone profile
Other subtypes
• Imaging: help staging
• Invasive lobular: ~20% of cancers
§ CXR
• Medullary: affects younger pts, feels soft
§ Liver US
• Colloid/mucinous: occur in elderly § CT scan
• Inflammatory: pain, erythema, swelling, peau d’orange § Breast MRI: multifocal disease or c̄ implants
• Papillary § Bone scan and PET-CT
• May need wire-guided excision biopsy
Phyllodes Tumour
• Stromal tumour
• Large, non-tender mobile lump
Clinical Staging
• Stage 1: confined to breast, mobile, no LNs
• Stage 2: Stage 1 + nodes in ipsilateral axilla
Spread • Stage 3: Stage 2 + fixation to muscle (not chest wall)
• Direct extension → muscle and/or skin LNs matted and fixed, large skin involvement
• Lymph → p’eau d’orange + arm oedema • Stage 4: Complete fixation to chest wall + mets
• Blood →
§ Bones: bone pain, #, ↑Ca
§ Lungs: dyspnoea, pleural effusion
TNM Staging
§ Liver: abdo pain, hepatic impairment • Tis (no palpable tumour): CIS
§ Brain: headache, seizures • T1: <2cm, no skin fixation
• T2: 2-5cm, skin fixation
• T3: 5-10cm, ulceration + pectoral fixation
Screening • T4: >10cm, chest wall extension, skin involved
• Every 3yrs from 47-73 • N1: mobile nodes
• Craniocaudal and oblique views • N2: fixed nodes
• ↓ breast Ca deaths by 25%
• 10% false negative rate.

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 Breast Cancer: Management
Principals Nottingham Prognostic Index
• Manage in an MDT c̄ an individual approach • Predicts survival and risk of relapse
§ Oncologist • Guides appropriate adjuvant systemic therapy
§ Breast surgeon • (0.2 x tumour size) + histo grade + nodal status
§ Breastcare nurse § Histo grade: Bloom-Richardson system (1-3)
§ Radiologist
§ Histopathologist Systemic Rx
• Try to enrol pts. in a trial
• Factors: age, fitness, wishes, clinical stage Radiotherapy
§ 1-2: surgical • Post-WLE: ↓ local recurrence
§ 3-4: chemo and palliation
• Post-mastectomy: only if high risk of local recurrence
§ Large, poorly differentiated, node +ve
Surgery • Axillary: node +ve disease
• Aim = gain local control • Palliation: bone pain
• Two options
§ WLE + radiotherapy (80% treated like this) Chemotherapy
§ Mastectomy • Pre-menopausal, node +ve, high grade or recurrent
- Typically large tumours >4cm tumours.
- Multifocal or central tumours • Neo-adjuvant chemo improves survival in large tumours
- Nipple involvement • 6 x FEC: 5-FU, Epirubicin, Cyclophosphamide
- Pt. choice • Trastuzumab (anti-Her2) is used if Her2+ve
- Not radical: no longer used § SE: cardiac toxicity
• Same survival, but WLE has ↑ recurrence rates
Endocrine Therapy
Sentinel Node Biopsy: gold standard • Used in ER or PR +ve disease: ↓ recurrence, ↑ survival
• 5yrs of adjuvant therapy
Rational • Tamoxifen
• SN = first node that a section of breast drains to. § SERM: antagonist @ breast, agonist @ uterus
• If clinically –ve axillary LNs, no need for further § SE: menopausal symptoms, endometrial Ca
dissection if SN is clear. • Anastrazole / Letrozole
§ Aromatase inhibitor → ↓ oestrogen
Procedure § Better cf. tamoxifen if post-men (ATAC trial)
• Blue dye / radiocolloid injected into tumour § SE: menopausal symptoms
• Visual inspection / gamma probe @ surgery to ID SN • If pre-menopausal and ER+ve may consider ovarian
• SN removed and sent for frozen section ablation or GnRH analogues (e.g. goserelin)
• If node +ve → axillary clearance or radiotherapy
Treating Advanced Disease (Stage 3-4)
Evidence • Tamoxifen if ER+ve
• 2 RCTs compared SNB vs. SNB + axillary clearance • Chemo for relapse
• No differences in overall or disease-free survival • Her2 +ve tumours may respond to trastuzumab
• ↓ morbidity c̄ SNB alone
§ Lymphoedema Supportive
§ Pain • Bone pain: DXT, bisphosphonates, analgesia
§ Numbness • Brain: occasional surgery, DXT, steroids, AEDs
• Lymphoedema: decongestion, compression
Other Axillary Options
• For clinically -ve axilla options include Reconstruction
O
§ Axillary sampling • Offered either at 1 surgery or as delayed procedure.
- Removal of lower nodes
- Clearance or DXT if +ve Implants: silastic or saline inflatable
§ Axillary clearance
- Can be done to various levels Lat dorsi myocutaneous flap
• Pedicled flap: skin, fat, muscle and blood supply
Surgical Complications • Supplied by thoracodorsal A. via subscapular A.
• Haematoma, seroma • Usually used c̄ an implant
• Frozen shoulder
• Long-thoracic nerve palsy Transverse rectus abdominis myocutaneous flap
• Lymphoedema • Gold-standard
• Upper inner arm numbness • Pedicled (inf. epigastric A.)
§ Intercostobrachial nerve injury
• Or free: attached to internal thoracic A
• No implant necessary and combined tummy tuck
• CI if poor circulation: smokers, obese, PVD, DM
• Risk of abdominal hernia

Nipple Tattoo
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 Other Breast Disease
Congenital Mastalgia
• Amastia: complete absence of breast and nipple
• Hypoplasia more common: some asymmetry Cyclical
normal • ~35yrs
• Accessory nipples • Pre-menstrual pain
§ Can occur anywhere along the milk line • Relieved by menstruation
§ Present in 1% • Commonly in upper outer quadrants bilaterally

Gynaecomastia Non-cyclical
• Occurs in 30% of boys at puberty • ~45yrs
• Hormone secreting tumours: e.g. sex-cord • Severe lancing breast pain (often left)
testicular • May be assoc. c̄ back pain
• Chronic liver disease: hypogonadism + ↓E2
metabolism
Rx
• Drugs: spironolactone, digoxin, cimetidine
• Reassurance + good bra for most
st
• 1 line: EPO (contains gamma-linoleic acid)
• OCP
• Topical NSAIDs (e.g. ibuprofen)
• Bromocriptine
• Danazol
• Tamoxifen

Inflammatory

Disease Patient Presentation Treatment

Acute Mastitis Usually lactating Painful, red breast Fluclox alone in early stages
May → Abscess (lump near nipple) Fluclox + I&D if fluctuant
abscess
Fat Necrosis Associated with previous trauma Analgesia
Painless, palpable, non-mobile mass No f/up necessary
May calcify simulating Ca
Duct Ectasia Post-menopausal Slit-like nipple Need to distinguish from Ca
Duct dilatation - 50-60yrs Often bilateral
± peri-areolar mass Surgical duct excision if mass
Thick white/green discharge present or discharge
May be calcified on mammography troublesome

Close f/up
Periductal Smokers Painful, erythematous sub-areolar mass Broad-spectrum Abx
Mastitis 30yrs Assoc. with inverted nipple ± purulent discharge
May → abscess or discharging fistula

Benign Epithelial Lesions

Disease Patient Presentation Treatment

Benign 30-50yrs Pre-menstrual breast nodularity and pain Triple assessment.
Mammary Often in upper outer quadrant
Dysplasia - Tender “lumpy-bumpy” breasts Reassurance
Analgesia
Aberration of Normal Development and Good bra
Involution (ANDI) ± evening primrose oil
- Fibroadenosis
- Cyst formation Danazol may occasionally be
- Epitheliosis (hyperplasia) used
- Papillomatosis
Cystic Disease Peri-menopausal >40 Distinct, fluctuant round mass Aspiration: green-brown fluid
Often painful
Persistence or blood → triple
assessment.
Duct Papilloma 40-50yrs Common cause of bloody discharge Triple assessment.
Not usually palpable Excise due to ↑ risk of Ca

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Stromal Tumours

Disease Patient Presentation Treatment

Fibroadenoma <35yrs Commonest benign tumour Reassurance + f/up if <2.5cm
Rare post-menopause Painless, mobile, rubbery mass Shell-out surgically
↑ in Blacks Often multiple and bilateral - >2.5cm
- FH of breast Ca
Popcorn calcification - Pt. choice
Phyllodes tumour 50s+ Large, fast growing mass WLE
Mobile, non-tender
Epithelial and connective tissue
elements

Malignant Conditions

Disease Clinical Treatment

DCIS Presents as Microcalcification on mammogram WLE + radiotherapy
Rarely assoc. with symptoms:
- lump Extensive or multifocal → mastectomy + reconstruction
- discharge + SLNB
- eczematous change = Paget’s disease
→ Ca @ 1%/yr (10x ↑ risk) in ipsilateral breast

ri
Paget’s

LCIS
-
Unilat, scaly, erythematous, itchy
+/- palpable mass (invasive carcinoma)
Incidental biopsy finding (no calcification)
_
Usually underlying invasive or DCIS breast cancer.
Mastectomy + radio ± chemo/endo
Bilateral prophylactic mastectomy or close watching c̄
Often bilat (20-40%) mammographic screening
Young women
↑ risk Ca risk (x10) in both breasts

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 Chronic Limb Ischaemia: Presentation and Classification
Incidence Presentation
• 5% of males >50yrs have intermittent claudication
Intermittent Claudication
• Cramping pain after walking a fixed distance
Cause • Pain rapidly relieved by rest
• Atherosclerosis • Calf pain = superficial femoral disease (commonest)
§ Typically asymptomatic until 50% stenosis • Buttock pain = iliac disease (internal or common)
• (vasculitis and fibromuscular dysplasia are v. rare
causes) Critical Limb Ischaemia: Fontaine 3 or 4
• European working group definition (1991)
• Ankle pressure <50mmHg (toe <30mmHg) and
Atherosclerosis Summary either:
• Endothelial injury: haemodynamic, HTN, ↑ lipids § Rest pain requiring analgesia for ³2 wks
• Chronic inflammation - Especially @ night
§ Lipid-laden foam cells produce GFs, cytokines, - Usually felt in the foot
ROS and MMPs - Pt. hangs foot out of bed
§ → lymphocyte and SMC recruitment - Due to ↓ CO and loss of gravity help
• SM proliferation: conversion of fatty streak to § Ulceration or gangrene
atherosclerotic plaque
Leriche’s Syndrome: Aortoiliac Occlusive Disease
NB. Arteriosclerosis = general arterial hardening • Atherosclerotic occlusion of abdominal aorta and iliacs
Atherosclerosis = arterial hardening specifically • Triad
due to atheroma. § Buttock claudication and wasting
§ Erectile dysfunction
§ Absent femoral pulses
Atheroma Pathology
• Fibrous cap: SM cells, lymphocytes, collagen Buerger’s Disease: Thromboangiitis Obliterans
• Necrotic centre: cell debris, cholesterol, Ca, foam • Young, male, heavy smoker
cells • Acute inflammation and thrombosis of arteries and
veins in the hands and feet → ulceration and
gangrene
Risk Factors
Signs
Modifiable Non-modifiable • Pulses: loss of pulses and ↑ CRT (norm ≤2sec)
• Smoking • FH and PMH • Ulcers: painful, punched-out, on pressure points
• BP • Male • Nail dystrophy / Onycholysis
• DM control • ↑ age • Skin: cold, white, atrophy, absent hair
• Hyperlipidaemia • Genetic • Venous guttering
• ↓ exercise • Muscle atrophy
• ↓ Buerger’s Angle
§ ≥90: normal
§ 20-30: ischaemia
Associated Vascular Disease § <20: severe ischaemia
• IHD: 90% • +ve Buerger’s Sign
• Carotid stenosis:15% § Reactive hyperaemia due to accumulation of
• AAA deoxygenated blood in dilated capillaries
• Renovascular disease
• DM microvascular disease
Clinical Classification

Fontaine
• Asympto (subclinical)
• Intermittent claudication
§ >200m
§ <200m
• Ischaemic rest pain
• Ulceration / gangrene
Rutherford
• Mild claudication
• Moderate claudication
• Severe claudication
• Ischaemic rest pain
• Minor tissue loss
• Major tissue loss

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 Chronic Limb Ischaemia: Investigation and Management
Ix Conservative Mx
• Most pts. c̄ claudication can be managed conservatively
Doppler Waveforms • ↑ exercise and employ exercise programs
• Normal: triphasic • Stop smoking
• Mild stenosis: biphasic • Wt. loss
• Severe stenosis: monophasic • Foot care
• Prog: 1/3 improve, 1/3 stay the same, 1/3 deteriorate
ABPI
Medical Mx
Clinical Fontaine ABPI • Risk factors: BP, lipids, DM
Calcification: CRF, DM >1.4 § β-B don’t worsen intermittent claudication but use
Normal ≥1 c̄ caution in CLI
Asymptomatic Fontaine 1 0.8-0.9 • Antiplatelets: aspirin / clopidogrel
Claudication Fontaine 2 0.6-0.8 • Analgesia: may need opiates
Rest pain Fontaine 3 0.3-0.6 • (Parenteral prostanoids ↓ pain in pts. unfit for surgery)
Ulceration and gangrene Fontaine 4 <0.3
Endovascular Mx
NB. Falsely high results may be obtained in DM / CRF • Percutaneous Transluminal Angioplasty ± stenting
due to calcification of vessels: mediasclerosis • Good for short stenosis in big vessels: e.g. iliacs, SFA
• Use toe pressure with small cuff: <30mmHg • Lower risk for pt.: performed under regional anaesthesia
as day case
Walk test • Improved inflow → ↓ pain but restoration of foot pulses is
• Walk on treadmill @ certain speed and incline to required for Rx of ulceration / gangrene.
establish maximum claudication distance.
• ABPI measured before and after: 20% ↓ is sig Surgical Reconstruction
Bloods Indications
• FBC + U+E: anaemia, renovascular disease • V. short claudication distance (e.g. <100m)
• Lipids + glucose • Symptoms greatly affecting pts. QoL
• ESR: arteritis • Development of rest pain
• G+S: possible procedure
Pre-op assessment
Imaging: assess site, extent and distal run-off • Need good optimisation as likely to have cardiorespiratory
• Colour duplex US co-morbidities.
• CT / MR angiogram
• Digital subtraction angiography Practicalities
§ Invasive \ not commonly used for Dx only. • Need good proximal supply and distal run-off
§ Used when performing therapeutic • Saphenous vein grafts preferred below the IL
angioplasty or stenting
• More distal grafts have ↑ rates of thrombosis

Other Classification
• ECG: ischaemia
• Anatomical: fem-pop, fem-distal, aortobifemoral
• Extra-anatomical: axillo-fem / -bifem, fem-fem crossover

Other
• Endarterectomy: core-out atheromatous plaque
• Sympathectomy: chemical (EtOH injection) or surgical
§ Caution in DM c̄ neuropathy
• Amputation

Prognosis

1yr after onset of CLI

• 50% alive w/o amputation
• 25% will have had major amputation
• 25% dead (usually MI or stroke)

Following amputation
• Perioperative mortality
§ BK: 5-10%
§ AK: 15-20%
• 1/3 → complete autonomy
• 1/3 → partial autonomy
• 1/3 → dead

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 Acute Limb Ischaemia
Definitions
• Acute: ischaemia <14d
• Acute on chronic: worsening symptoms and signs
<14d
• Chronic: ischaemia stable for >14d
Severity
• Incomplete: limb not threatened
• Complete: limb threatened
§ Loss of limb unless intervention w/i 6hrs
• Irreversible: requires amputation
General Mx
• In an acutely ischaemic limb discuss immediately c̄ a
senior as time is crucial.
• NBM
• Rehydration: IV fluids
• Analgesia: morphine + metoclopramide
• Abx: e.g. co-amoxiclav if signs of infection
• Unfractionated heparin IVI: prevent extension
• Complete occlusion?
§ Yes: urgent surgery: embolectomy or bypass
§ No: angiogram + observe for deterioration

Causes
• Thrombosis in situ (60%)
§ A previously stenosed vessel c̄ plaque rupture
§ Usually incomplete ischaemia
• Embolism (30%)
§ 80% from left atrium in AF
§ Valve disease
§ Iatrogenic from angioplasty / surgery
§ Cholesterol in long bone #
§ Paradoxical (venous via PFO)
§ Typically lodge at femoral bifurcation
§ Often complete ischaemia
• Graft / stent occlusion
• Trauma
• Aortic dissection
Angiography
• Not performed if there is complete occlusion as it
introduces delay: take straight to theatre.
• If incomplete occlusion, pre-op angio will guide any
distal bypass.
Embolus Mx
1. Embolectomy
§ Under regional anaesthesia or GA
§ Wire fed through embolus
§ Fogarty catheter fed over the top
§ Balloon inflated and catheter withdrawn,
removing the embolism.
§ Adequacy confirmed by on-table angiography

2. Thrombolysis
Presentation: 6Ps § Consider if embolectomy unsuccessful
• Pale § E.g. local injection of TPA
• Pulseless
• Perishingly cold 3. Other options
• Painful § Emergency reconstruction
• Paraesthesia § Amputation
• Paralysis
Post-embolectomy
• Anticoagulate: heparin IVI → warfarin
Thrombosis vs. Embolus • ID embolic source: ECG, echo, US aorta, fem and
pop
Thrombosis Embolus • Complications
Onset Hrs-days Sudden § Reperfusion injury
Severity Less severe Profound - Local swelling → compartment
- collaterals ischaemia syndrome
O
Embolic source Present - Acidosis and arrhythmia 2 to ↑K
- often AF - ARDS
Claudication Hx Present Absent - GI oedema → endotoxic shock
Contralat Absent Present § Chronic pain syndromes
pulses
Dx Angiography Clinical
Rx Thrombolysis Embolectomy + Thrombosis Mx
Bypass surgery Warfarin • Emergency reconstruction if complete occlusion
• Angiography + angioplasty
Ix • Thrombolysis
• Blood • Amputation
§ FBC, U+E, INR, G+S
§ CK
• ECG
Manage Cardiovascular Risk Factors
• Imaging
§ CXR
§ Duplex doppler
§ CT angio

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Carotid Artery Disease Aneurysms
Definitions Definition
• Stroke: sudden neurological deficit of vascular origin • Abnormal dilatation of a blood vessel >50% of its
lasting >24h normal diameter.
• TIA: sudden neurological deficit of vascular origin
lasting <24h (usually lasts <1h) c̄ complete recovery Classification
• True Aneurysm
Pathogenesis § Dilatation of a blood vessel involving all layers
• Turbulent flow → ↓ shear stress @ carotid bifurcation of the wall and is >50% of its normal diameter
promoting atherosclerosis and plaque formation. § Two different morphologies
• Plaque rupture → complete occlusion or distal emboli - Fusiform: e.g AAA
• Cause 15-25% of CVA/TIA - Saccular: e.g Berry aneurysm
• False Aneurysm
Presentation § Collection of blood around a vessel wall that
• Bruit communicates c̄ the vessel lumen.
• CVA/TIA § Usually iatrogenic: puncture, cannulation
• Dissection
§ Vessel dilatation caused by blood splaying
Ix apart the media to form a channel w/i the
• Duplex carotid Doppler vessel wall.
• MRA
Causes
Mx • Congenital
§ ADPKD → Berry aneurysms
Conservative § Marfan’s, Ehlers-Danlos
• Aspirin or clopidogrel • Acquired
• Control risk factors § Atherosclerosis
§ Trauma: e.g. penetrating trauma
Surgical: Endarterectomy § Inflammatory: Takayasu’s aortitis, HSP
• Symptomatic (ECST, NASCET) § Infection
§ ≥70% (5% stroke risk per yr) - Mycotic: SBE
§ ≥50% if low risk (<3%, typically <75yrs) - Tertiary syphilis (esp. thoracic)
§ Perform w/i 2wks of presentation
• Asymptomatic (ACAS, ACST) Complications
§ ≥60% benefit if low risk • Rupture
• Thrombosis
Complications of Endarterectomy • Distal embolization
• Stroke or death: 3% • Pressure: DVT, oesophagus, nutcracker syndrome
• HTN: 60% • Fistula (IVC, intestine)
• Haematoma
• MI
• Nerve injury
• Hypoglossal: ipsilateral tongue deviation Popliteal Aneurysm
• Great auricular: numb ear lobe • Less common cf. AAA
• Recurrent laryngeal: hoarse voice, bovine cough • 50% of pts. c̄ popliteal aneurysm also have AAA

Stenting Presentation
• Less invasive: ↓ hospital stay, ↓ infection, ↓ CN injury • Very easily palpable popliteal pulse
• There is concern over ↑ stroke risk, esp. pts. >70yrs • 50% bilateral
• Meta-analysis shows no sig difference in mortality vs. • Rupture is relatively rare
CEA @ 120d • Thrombosis and distal embolism is main complication
• Younger pts. have best risk / benefit ratio § → acute limb ischaemia

Mx
• Acute: embolectomy or fem-distal bypass
• Stable
§ Elective grafting + tie off vessel
§ Stenting

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 Abdominal Aortic Aneurysms
Epidemiology AAA Rupture
• Prevalence: ~5% >50yrs
• Mortality: 10,000 deaths/yr Rupture Rates
• Sex: M>F=3:1 • <5.5cm = 1% / yr
• >6cm = 25% / yr
Pathology • ↑ risk if:
• Dilatation of the abdominal aorta ≥3cm § ↑BP
• 90% infrarenal § Smoker
• 30% involve the iliac arteries § Female
§ Strong FH
Presentation Rupture Presentation
• Usually asympto: discovered incidentally
• Sudden onset severe abdominal pain
• May → back pain or umbilical pain radiating to groin
§ Intermittent or continuous
• Acute limb ischaemia § Radiates to back or flanks (don’t dismiss as
• Blue toe syndrome: distal embolisation colic)
• Acute rupture • Collapse → shock
• Expansile abdominal mass
Examination
• Expansile mass just above the umbilicus Mx: a surgical emergency
• Bruits may be heard • High flow O2
• Tenderness + shock suggests rupture • 2 x large bore cannulae in each ACF
§ Give fluid if shocked but keep SBP
Ix <100mmHg
• AXR: calcification may be seen § Give O- blood if desperate
• Abdo US: screening and monitoring § Blood: FBC, U+E, clotting, amylase, xmatch
• CT / MRI: gold-standard 10u
• Angiography • Instigate the major haemorrhage protocol
§ Won’t show true extent of aneurysm due to • Call vascular surgeon, anaesthetist and warn theatre
endoluminal thrombus. • Analgesia
§ Useful to delineate relationship of renal arteries • Abx prophylaxis: cef + met
• Urinary catheter + CVP line
Mx • If stable + Dx uncertain: US or CT may be feasible
• Take to theatre: clamp neck, insert dacron graft
Conservative
Mortality
• Manage cardiovascular risk factors: esp. BP
• 100% w/o surgery
• UK Small Aneurysm Trial suggested that AAA <5.5cm
• 50% c̄ surgery
in maximum diameter can be monitored by US (/CT)
§ <4cm: yearly monitoring
§ 4-5.5cm: 6 monthly monitoring

Surgical
• Aim to treat aneurysm before it ruptures.
§ Elective mortality: 5%
§ Emergency mortality: 50%
• Operate when risk of rupture > risk of surgery
• Indications
§ Symptomatic (back pain = imminent rupture)
§ Diameter >5.5cm
§ Rapidly expanding: >1cm/yr
§ Causing complications: e.g. emboli
• Open or EVAR
§ EVAR has ↓ perioperative mortality
§ No ↓ mortality by 5yrs due to late endograft
failures.
§ EVAR not better than medical Rx in unfit pts.

Screening
• MASS trial revealed 50% ↓ aneurysm-related mortality
in males aged 65-74 screened c̄ US.
• UK men offered one-time US screen @ 65yrs

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Thoracic Aortic Dissection Gangrene
Definition Definition
• Blood splays apart the laminar planes of the media to • Death of tissue from poor vascular supply.
form a channel w/i the aortic wall.
Classification
Aetiology • Wet: tissue death + infection
• Atherosclerosis and HTN cause 90% • Dry: tissue death only
• Minority caused by connective tissue disorder • Pregangrene: tissue on the brink of gangrene
§ Marfan’s, Ehlers Danlos
§ Vitamin C deficiency Presentation
• Black tissues ± slough
Presentation • May be suppuration ± sepsis
• Sudden onset, tearing chest pain
§ Radiates through to the back Gas Gangrene
O
§ Tachycardia and hypertension (1 + • Clostridium perfringes myositis
sympathetic)
• RFs: DM, trauma, malignancy
• Distal propagation → sequential occlusion of branches
• Presentation
§ Left hemiplegia
§ Toxaemia
§ Unequal arm pulses and BP
§ Haemolytic jaundice
§ Paraplegia (anterior spinal A.)
§ Oedema
§ Anuria
§ Crepitus from surgical emphysema
• Proximal propagation § Bubbly brown pus
§ Aortic regurgitation
• Rx
§ Tamponade
§ Debridement (may need amputation)
• Rupture into pericardial, pleural or peritoneal cavities § Benzylpenicillin + metronidazole
§ Commonest cause of death § Hyperbaric O2

Stanford Classification Synergistic Gangrene

Type A: Proximal • Involves aerobes + anaerobes
• 70% • Fournier’s: perineum
• Involves ascending aorta ± descending • Meleney’s: post-op ulceration
• Higher mortality due to probable cardiac involvement • Both progress rapidly to necrotising fasciitis + myositis
• Usually require surgery
Mx
Type B: Distal • Take cultures
• 30% • Debridement (including amputation)
• Involves descending aorta only: distal to L SC artery • Benzylpenicillin ± clindamycin
• Usually best managed conservatively

Ix
• ECG: exclude MI
• TTE / TOE: can use in haemodynamically unstable pts
• CT/MRI: not suitable for unstable pts.

Mx

Resuscitate

Investigate
• Bloods: x-match 10u, FBC, U+E, clotting, amylase
• ECG: 20% show ischaemia due to involvement of the
coronary ostia
• Imaging
§ CXR
§ CT/MRI: not if haemodynamically unstable
§ TOE: can be used if haemodynamically unstable

Treat
• Analgesia
• ↓SBP
§ Labetalol or esmolol (short t½)
§ Keep SBP 100-110mmHg
• Type A: open repair
§ Acute operative mortality: <25%
• Type B: conservative initially
§ Surgery if persistent pain or complications
§ Consider TEVAR if uncomplicated
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 Varicose Veins
Definition CEAP Classification
• Tortuous, dilated veins of the superficial venous • Chronic venous disease can be classified according to
system § Clinical signs (1-6 + sympto or asympto)
§ Etiology
Pathophysiology § Anatomy
§ Pathophysiology
• One-way flow from sup → deep maintained by valves
• Valve failure → ↑ pressure in sup veins → varicosity
• 3 main sites where valve incompetence occurs: Conservative Mx
§ SFJ: 3cm below and 3cm lateral to pubic • Treat any contributing factors
tubercle § Lose weight
§ SPJ: popliteal fossa § Relieve constipation
§ Perforators: draining GSV • Education
- 3 medial calf perforators (Cockett’s) § Avoid prolonged standing
- 1 medial thigh perforator (Hunter’s) § Regular walks
• Class II Graduated Compression Stockings
Causes § 18-24mmHg
§ Symptomatic relief and slows progression
• Skin care
Primary § Maintain hydration c̄ emollients
• Idiopathic (congenitally weak valves)
§ Treat ulcers rapidly
§ Prolonged standing
§ Pregnancy
§ Obesity Minimally Invasive Therapies
§ OCP
§ FH Indications
• Congenital valve absence (v. rare) • Small below knee VVs not involving GSV or SSV

Secondary Techniques
• Valve destruction → reflux: DVT, thrombophlebitis • Local or GA
• Obstruction: DVT, foetus, pelvic mass • Injection sclerotherapy: 1% Na tetradecyl sulphate
• Constipation • Endovenous laser or radiofrequency ablation
• AVM • Glue ablation
• Overactive pumps (e.g. cyclists)
• Klippel-Trenaunay Post-Operatively
§ PWS, varicose veins, limb hypertrophy • Compression bandage for 24hrs
• Compression stockings for 1mo
Symptoms
• Cosmetic defect Surgical Mx
• Pain, cramping, heaviness
• Tingling Indications (rarely performed nowdays)
• Bleeding: may be severe • SFJ incompetence
• Swelling • Major perforator incompetence
• Symptomatic: ulceration, skin changes, pain
Signs
• Skin changes Procedures
§ Venous stars • Trendelenberg: saphenofemoral ligation
§ Haemosiderin deposition • SSV ligation: in the popliteal fossa
§ Venous eczema • LSV stripping: no longer performed due to potential
§ Lipodermatosclerosis (paniculitis) for saphenous nerve damage.
§ Atrophie blanche • Multiple avulsions
• Ulcers: medial malleolus / gaiter area • Perforator ligation: Cockett’s operation
• Oedema • Subfascial endoscopic perforator surgery (SEPS)
• Thrombophlebitis
Post-op
Ix • Bandage tightly
• Duplex ultrasonography • Elevate for 24h
§ Anatomy • Discharged c̄ compression stockings and instructed to
§ Presence of incompetence walk daily.
§ Caused by obstruction or reflux
• Surgery: FBC, U+E, clotting, G+S, CXR, ECG
Complications
• Haematoma (esp. groin)
Referral Criteria • Wound sepsis
• Bleeding • Damage to cutaneous nerve (e.g. long saphenous)
• Pain • Superficial thrombophlebitis
• Ulceration • DVT
• Superficial thrombophlebitis • Recurrence: may approach 50%
• Severe impact on QoL
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Leg Ulcers Leg Swelling
Definition Differential
• Interruption in the continuity of an epithelial surface
Bilateral
Causes • ↑ Venous Pressure
• Venous: commonest • RHF
• Arterial: large or small vessel • Venous insufficiency → stasis
• Neuropathic: EtOH, DM • Drugs: e.g. nifedipine
• Traumatic: e.g. pressure • ↓ Oncotic Pressure
• Systemic disease: e.g. pyoderma gangrenosum • Nephrotic syndrome
• Neoplastic: SCC • Hepatic failure
• Protein losing enteropathy
Venous: 75% • Lymphoedema
• Painless, sloping, shallow ulcers • Myxoedema
• Usually on medial malleolus: “gaiter area” • Hyper- / hypo-thyroidism
• Assoc. c̄ haemosiderin deposition and
lipodermatosclerosis Unilateral
• RFs: venous insufficiency, varicosities, DVT, obesity • Venous insufficiency
• DVT
Arterial: 2% • Infection or inflammation
• Lymphoedema
• Hx of vasculopathy and risk factors
• Painful, deep, punched out lesions
• Occur @ pressure points Lymphoedema
§ Heal • Collection of interstitial fluid due to blockage or
§ Tips of, and between, toes absence of lymphatics
th
§ Metatarsal heads (esp. 5 )
• Other signs of chronic leg ischaemia Primary
• Congenital absence of lymphatics
Neuropathic • May or may not be familial
• Painless c̄ insensate surrounding skin • Presentation
• Warm foot c̄ good pulses § Congenital: evident from birth
§ Praecox: after birth but <35yrs
§ Tarda: >35yrs
Complications
• Osteomyelitis Milroy’s Syndrome
• Development of SCC in the ulcer (Marjolin’s ulcer) § Familial AD subtype of congenital
lymphoedema
Ix § F>M
• ABPI if possible
• Duplex ultrasonography Secondary: FIIT
• Biopsy may be necessary • Fibrosis: e.g. post-radiotherapy
§ Look for malignant change: Marjolin’s ulcer • Infiltration
§ Ca: prostate, lymphoma
Mx of Venous Ulcers § Filariasis: Wuchereria bancrofti
• Refer to leg ulcer community clinic • Infection: TB
• Focus on prevention • Trauma: block dissection of lymphatics
§ Graduated compression stockings
§ Venous surgery Ix
• Optimise risk factors: nutrition, smoking • Doppler US
• Lymphoscintigraphy
Specific Rx • CT / MRI
• Analgesia
• Bed Rest + Elevate leg Mx
• 4 layer graded compression bandage (if ABPI >0.8) • Conservative
• Pentoxyfylline PO § Skin care
§ ↑ microcirculatory blood flow § Compression stocking
§ Improves healing rates § Physio
§ Treat or prevent comorbid infections
Other Options (no proven benefit) • Surgical: debulking operation (rarely performed)
• Desloughing: e.g. larval therapy, hydrogel
• Topical antiseptics: iodine, Manuka honey
• Split-thickness skin grafting may be considered

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Urinary Tract Obstruction Urethral Stricture
Causes Aetiology
• Trauma
Luminal § Instrumentation
• Stones § Pelvic #s
• Blood clots • Infection: e.g. gonorrhoea
• Sloughed papilla • Chemotherapy
• Balantitis xerotica obliterans
Mural
• Congenital / acquired stricture Presentation: voiding difficulty
• Tumour: renal, ureteric, bladder • Hesitancy
• Neuromuscular dysfunction • Strangury
• Poor stream
Extramural • Terminal dribbling
• Prostatic enlargement • Pis en deux
• Abdo / pelvic mass / tumour
• Retroperitoneal fibrosis Examination
• PR: exclude prostatic cause
Presentation • Palpate urethra through penis
Acute • Examine meatus
• Upper Urinary Tract
§ Loin pain → groin Ix
• Lower Urinary Tract • Urodynamics
§ Bladder outflow obstruction precedes § ↓ peak flow rate
suprapubic pain c̄ distended palpable bladder § ↑ micturition time
• Urethroscopy and cystoscopy
Chronic • Retrograde urethrogram
• Upper Urinary Tract
§ Flank pain Mx
§ Renal failure (may be polyuric) • Internal urethrotomy
• Lower Urinary Tract • Dilatation
§ Frequency, hesitancy, poor stream, terminal • Stent
dribbling, overflow incontinence
§ Distended, palpable bladder ± large prostate
PR

Ix Obstructive Uropathy
• Bloods: FBC, U+E
• Urine: dip, MC+S Pathogenesis
• Imaging • Acute retention on a chronic background may go
§ US: hydronephrosis or hydroureter unnoticed for days due to lack of pain.
§ Anterograde / retrograde ureterograms • Se Cr may be up to 1500uM
- Allow therapeutic drainage • Renal function should return to normal over days
§ Radionucleotide imaging: renal function • Some background impairment may remain.
§ CT / MRI
Complications
Mx
Upper Urinary Tract Hyperkalaemia
• Nephrostomy Metabolic acidosis
• Ureteric stent
Post-obstructive diuresis
Lower Urinary Tract • Kidneys produce a lot of urine in the acute phase
• Urethral or suprapubic catheter after relief of obstruction.
§ May be a large post-obstructive diuresis • Must keep up c̄ losses to avoid dehydration.

Complications of Ureteric Stents Na and HCO3 losing nephropathy

Common • Diuresis may → loss of Na and HCO3
• Infection • May require replacement c̄ 1.26% NaHCO3
• Haematuria
• Trigonal irritation
Infection
• Encrustation

Rare
• Obstruction
• Ureteric rupture
• Stent migration

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 Urinary Retention
Causes Chronic Urinary Retention (CUR)

Obstructive Classification
• Mechanical • High Pressure
§ BPH § High detrusor pressure @ end of micturition
§ Urethral stricture § Typically bladder outflow obstruction
§ Clots, stones § → bilateral hydronephrosis and ↓ renal function
§ Constipation • Low Pressure
• Dynamic: ↑ smooth muscle tone (α-adrenergic) § Low detrusor pressure @ end of micturition
§ Post-operative pain § Large volume retention c̄ very compliant
§ Drugs bladder
§ Kidney able to excrete urine
Neurological § No hydronephrosis \ normal renal function
• Interruption of sensory or motor innervation
§ Pelvic surgery Presentation
§ MS • Insidious as bladder capacity ↑↑ (>1.5L)
§ DM • Typically painless
§ Spinal injury / compression • Overflow incontinence / nocturnal enuresis
• Acute on chronic retention
Myogenic • Lower abdo mass
• Over-distension of the bladder • UTI
§ Post-anaesthesia • Renal failure
§ High EtOH intake
Mx

Acute Urinary Retention (AUR) High-Pressure

• Catheterise if
Clinical Features § Renal impairment
• Suprapubic tenderness § Pain
• Palpable bladder § Infection
§ Dull to percussion • Hrly UO + replace: post-obstruction diuresis
§ Can’t get beneath it • Consider TURP before TWOC
• Large prostate on PR
§ Check anal tone and sacral sensation Low-Pressure
• <1L drained on catheterisation • Avoid catheterisation if possible
§ Risk of introducing infection
Ix • Early TURP
• Blood: FBC, U+E, PSA (prior to PR) § Often do poorly due to poor detrusor function
• Urine: dip, MC+S § Need CISC or permanent catheter
• Imaging
§ US: bladder volume, hydronephrosis
§ Pelvic XR Suprapubic Catheterisation

Mx Advantages
• ↓ UTIs
Conservative • Avoids risk of urethral stricture formation
• Analgesia • TWOC w/o catheter removal
• Privacy • Pt. preference: ↑ comfort
• Walking • Maintain sexual function
• Running water or hot bath
Disadvantages
Catheterise • More complex
• Use correct catheter: e.g. 3-way if clots • Serious complications can occur
• ± STAT gentamicin cover
• Hrly UO + replace: post-obstruction diuresis CI
• Tamsulosin: ↓ risk of recatheterisation after retention • Known or suspected bladder carcinoma
• TWOC after 24-72h • Undiagnosed haematuria
§ May d/c and f/up in OPD • Previous lower abdominal surgery
§ More likely to be successful if predisposing § → adhesion of small bowel to abdo wall
factor and lower residual volume (<1L)

TURP Clean Intermittent Self-Catheterisation

• Failed TWOC • Alternative to indwelling catheter in AUR and CUR
• Impaired renal function • Also useful in pts. who fail to void after TURP
• Elective

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Haematuria Peri-Aortitis
False Aetiology
• Beetroot • Idiopathic retroperitoneal fibrosis
• Rifampicin • Inflammatory AAAs
• Porphyria • Perianeurysmal RPF
O
• PV bleed • RPF 2 to malignancy: e.g. lymphoma

Idiopathic Retroperitoneal Fibrosis

True • Autoimmune vasculitis
• Fibrinoid necrosis of vasa vasorum
General • Affects aorta and small/medium sized retroperitoneal
• HSP vessels.
• Bleeding diathesis • Ureters are embedded in dense, fibrous tissue →
bilateral obstruction
Renal
• Infarct Associations
• Trauma: inc. stones • Drugs: β-B, bromocriptine, methysergide, methyldopa
• Infection • AI disease: thyroiditis, SLE, ANCA+ vasculitis
• Neoplasm • Smoking
• GN • Asbestos
• Polycystic kidneys
Presentation
Ureter • Middle–aged male
• Stone • Vague loin, back or abdo pain
• Tumour • ↑ BP
• Chronic urinary tract obstruction
Bladder
• Infection Ix
• Stones • Blood: ↑U and Cr, ↑ESR/CRP, ↓Hb
• Tumour • US: bilateral hydronephrosis + medial ureteric
• Exercise deviation
• CT/MRI: peri-aortic mass
Prostate § Biopsy: exclude Ca
• BPH
• Prostatitis Rx
• Tumour • Relieve obstruction: retrograde stent placement
• Ureterolysis: dissection of ureters from retroperitoneal
Urethra tissue.
• Infection • ± immunosuppression
• Stones
• Trauma
• Tumour

Clinical Features
• Timing?
§ Beginning of stream: urethral
§ Throughout stream: renal / systemic, bladder
§ End of stream: bladder stone, schisto
• Painful or painless?
• Obstructive symptoms?
• Systemic symptoms: wt. loss, appetite

Ix
• Bloods: FBC, U+E, clotting
• Urine: dip, MC+S, cytology
• Imaging
§ Renal US
§ IVU
§ Flexible cystoscopy + biopsy
§ CT/MRI
§ Renal angio

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 Urolithiasis: Pathophysiology and Presentation
Epidemiology Presentation
• Lifetime incidence: 15%
• Young men Ureteric Colic
§ Peak age: 20-40yrs • Severe, sudden onset loin pain radiating to the groin
§ Sex: M>F=3:1 • Assoc. c̄ n/v
• Pt. cannot lie still

Pathophysiology Bladder or Urethral Obstruction

• ↑ concentration of urinary solute • Bladder irritability: frequency, dysuria, haematuria
• ↓ urine volume • Strangury: painful urinary tenesmus
• Urinary stasis • Suprapubic pain radiating → tip of penis or in labia
• Pain and haematuria worse at the end of micturition

Common Anatomical Sites Other Possible Features

• Pelviureteric junction • UTI
• Crossing the iliac vessels at the pelvic brim • Haematuria
• Under the vas or uterine artery • Sterile pyuria
• Vesicoureteric junction • Anuria

Stone Types Examination

• Calcium oxalate: 75% • Usually no loin tenderness
§ ↑ risk in Crohn’s • Haematuria
• Triple phosphate (struvite): 15%
§ Ca Mg NH4 – phosphate
§ May form staghorn calculi
§ Assoc. c̄ proteus infection
• Urate: 5% (radiolucent)
§ Double if confirmed gout
• Cystine: 1% (faint)
§ Assoc. c̄ Fanconi Syn.

Associated Factors
• Dehydration
O
• Hypercalcaemia: 1 HPT, immobilisation
• ↑ oxalate excretion: tea, strawberries
• UTIs
• Hyperuricaemia: e.g. gout
• Urinary tract abnormalities: e.g. bladder diverticulae
• Drugs: frusemide, thiazides

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 Urolithiasis: Ix and Mx
Urine Initial Rx
• Dip: haematuria • Analgesia
• MC+S § Diclofenac 75mg PO/IM or 100mg PR
§ Opioids if NSAIDs CI: e.g. pethidine
Blood • Fluids: IV if unable to tolerate PO
• FBC, U+E, Ca, PO4, urate • Abx if infection: e.g. cefuroxime 1.5mg IV TDS

Conservative: <5mm in lower 1/3 of ureter

• 90-95% pass spontaneously
Imaging • Can discharge pt. c̄ analgesia
• Sieve urine to collect stone for OPD analysis
Non-contrast CT-KUB
• 99% of stones visible
• Gold standard Medical Expulsive Therapy (MET)

KUB XR Indications
• 90% of stones radio-opaque • Stone 5-10mm
• Urate stones are radiolucent, cysteine stones are faint • Stone expected to pass

USS: hydronephrosis Drugs

• Nifedipine or tamsulosin
• ± prednisolone
IVU • Most pass w/i 48h, 80% w/i 30d
• 600x radiation dose of KUB XR
• IV contrast injected and control, immediate and serial Active Stone Removal
films taken until contrast @ level of obstruction
• Abnormal findings Indications
§ Failure of flow to the bladder • Low likelihood of spontaneous passage: e.g. >10mm
§ Standing column of contrast • Persistent obstruction
§ Clubbing of the calyces: back pressure • Renal insufficiency
§ Delayed, dense nephrogram: no flow from • Infection
kidney
• CI Extracorporeal Shockwave Lithotripsy (SWL)
§ Contrast allergy • Stones <20mm in kidney or proximal ureter
§ Severe asthma
• SE: renal injury may → ↑BP
§ Metformin
• CI: pregnancy, AAA, bleeding diathesis
§ Pregnancy
Ureterorenoscopy (URS) + Dormier Basket Removal
Functional Scans
• Stone >10mm in distal ureter or if SWL failed
• DMSA: dimercaptosuccinic acid
• Stone >20mm in renal pelvis
• DTPA: diethylenetriamene penta-acetic acid
• MAG-3
Percutaneous Nephrolithotomy (PNL)
• Stone >20mm in renal pelvis
• E.g. staghorn calculi: do DMSA first
Prevention
• Drink plenty Lap or Open Surgery: rare
• Treat UTIs rapidly
• ↓ oxalate intake: chocolate, tea, strawberries
Febrile c̄ Renal Obstruction
• Surgical emergency
• Percutaneous nephrostomy or ureteric stent
• IV Abx: e.g. cefuroxime 1.5g IV TDS

Rx Summary
• Conservative: stone <5mm in distal ureter
• MET: stone 5-10mm and expected to pass
• Active: stones >10mm, persistent pain, renal
insufficiency

st nd
Location Size 1 line 2 line
Pelvis >20mm PNL or URS SWL
<20mm SWL URS, PNL
Proximal >10mm URS or SWL
ureter <10mm SWL URS
Distal ureter >10mm URS SWL
<10mm URS or SWL

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 Renal Tumours
Renal Cell Carcinoma / Hypernephroma Transitional Cell Carcinoma

Epidemiology Epidemiology
nd
• 90% of renal cancers • 2 commonest renal cancer
• Age: 55yrs • Age: 50-80yrs
• Sex: M>F=2:1 • Sex: M>F=4:1

Risk Factors Risk Factors

• Obesity • Smoking
• Smoking • Amine exposure (rubber industry)
• HTN • Cyclophosphamide
• Dialysis (15% of pts. develop RCC)
• 4% heritable: e.g. VHL syndrome Pathology
• Highly malignant
Pathology • Locations
•
Adenocarcinoma from proximal renal tubular epithelium § Bladder: 50%
•
Subtypes § Ureter
§ Clear Cell (glycogen): 70-80% § Renal pelvis
§ Papillary: 15%
§ Chromophobe: 5% Presentation
§ Collecting duct: 1% • Painless haematuria
• Frequency, urgency, dysuria
Presentation • Urinary tract obstruction
• 50% incidental finding
• Triad: Haematuria, loin pain, loin mass Ix
• Systemic: anorexia, malaise, wt. loss, PUO • Urine cytology
• Clot retention • CT/MRI
• Invasion of L renal vein → varicocele (1%) • IVU: pelviceal filling defect
• Cannonball mets → SOB
Mx
Paraneoplastic Features • Nephrouretectomy
• EPO → polycythaemia • Regular f/up: 50% develop bladder tumours
• PTHrP → ↑ Ca
• Renin → HTN
• ACTH → Cushing’s syn. Nephroblastoma: Wilm’s Tumour
• Amyloidosis
• Childhood tumour of primitive renal tubules and
mesenchymal cells
Spread • May be assoc. c̄ Chr 11 mutation
• Direct: renal vein • May be assoc. c̄ WAGR syndrome
• Lymph § Wilms, Aniridia, GU abnormalities, Retardation
• Haematogenous: bone, liver and lung
Presentation
Ix • 2-5yrs
• Blood: polycythaemia, ESR, U+E, ALP, Ca • 5-10% bilat
• Urine: dip, cytology • Abdo mass (doesn’t cross the midline)
• Imaging • Haematuria
§ CXR: cannonball mets
• Abdo pain
§ US: mass
• HTN
§ IVU: filling defect
§ CT/MRI

Robson Staging Other Neoplasms

• Confined to kidney
• Involves perinephric fat, but not Gerota’s fascia Benign
• Spread into renal vein • Cysts: very common
• Spread to adjacent / distant organs • Renal papillary adenomas
• Oncocytoma: eosinophilic cells c̄ numerous
Mx mitochondria
• Medical • Angiomyolipoma: seen in tuberous sclerosis
§ Reserved for pts. c̄ poor prognosis
§ Temsirolimus (mTOR inhibitor) Malignant
• Surgical • SCC: assoc. c̄ chronic infected staghorn calculi
§ Radical nephrectomy
§ Consider partial if small tumour or 1 kidney NB. Benign tumours commonly require nephrectomy to
exclude malignancy.
Prognosis: 45% 5ys
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 Bladder Tumours
Epidemiology Ix
• Incidence: 1:5000/yr • Urine: dip (sterile pyuria), cytology
• Sex: M>F=4:1 • IVU: filling defects
• Cystoscopy c̄ biopsy: diagnostic
Pathology • Bimanual EUA: helps to assess spread
• Transitional cell carcinomas account for 90% • CT/MRI: helps stage
• SCCs: assoc. c̄ schistosomiasis
• Adenocarcinoma Mx
• Depends on histological grade and the presence of
Natural Hx dissemination.
• Low-Grade Tumours
§ 80% TIS, Ta and T1 (Superficial)
§ Non-invasive, generally not life-threatening • 80% of all pts.
§ High rate of recurrence • Diathermy via transurethral cystoscopy / Transurethral
• High-Grade Tumours Resection of Bladder Tumour (TURBT)
§ 20% • Intravesicular chemo: mitomycin C
§ Invasive and life-threatening • Intravesicular immunotherapy: Bacille Calmette-
§ High recurrence rates Guérin

Risk Factors T2, T3 (Invasive)

• Smoking • Radical cystectomy c̄ ileal conduit is gold standard
• Amine exposure (rubber industry) • Radiotherapy: worse 5ys but preserves bladder
• Previous renal TCC § Salvage cystectomy can be performed
• Chronic cystitis • Adjuvant chemo: e.g. M-VAC
• Schistosomiasis (SCC) • Neoadjuvant chemo may have a role
• Urechal remnants (adenocarcinomas)
§ Embryological remnant of communication T4
between umbilicus and bladder • Palliative chemo / radiotherapy
• Pelvic irradiation • Long-term catheterisation
• Urinary diversions
Presentation
• Painless haematuria Complications
• Voiding irritability: dysuria, frequency, urgency • Massive bladder haemorrhage
• Recurrent UTIs • Cystectomy → Sexual and urinary malfunction
• Retention and obstructive renal failure
Follow-Up
Examination • Up to 70% of bladder tumours recur therefore
• Anaemia intensive f/up is required.
• Palpable bladder mass • History, examination and regular cystoscopy
• Palpable liver • High-risk tumours: every 3mo for 2yrs, then every 6mo
• Low-risk tumours: @ 9mo, then yrly
TNM Staging
• 80% confined to mucosa Prognosis
• 20% penetrate muscle (↑ mortality) • Depends on age and stage
• TIS, Ta and T1: 95% 5ys
TIS Carcinoma in situ Not felt at EUA • T2: 40-50% 5ys
Ta Confined to epithelium Not felt at EUA • T3: 25% 5ys
T1 Tumour in lamina propria Not felt at EUA • T4: <1yr median survival
T2 Superficial muscle involved Rubbery
thickness
T3 Deep muscle involved Mobile mass
T4 Invasion of prostate, uterus or Fixed mass
vagina

Spread
• Local → pelvic structures
• Lymph → iliac and para-aortic nodes
• Haem → bones, liver and lungs

Histological Classification
• Grade 1: well differentiated
• Grade 2: intermediate
• Grade 3: poorly differentiated

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 Benign Prostatic Hypertrophy
Epidemiology Mx
• 70% @ 60yrs
• 90% @ 80yrs Conservative
• ↓ caffeine, EtOH
• Double voiding
Pathophysiology • Bladder training: hold on → ↑ time between voiding
• Benign nodular or diffuse hyperplasia of stromal and
epithelial cells Medical
• Affects inner (transitional) layer of prostate (cf. Ca) • Useful in mild disease and while awaiting TURP
st
§ → urethral compression • 1 : α-blockers
• DHT produced from testosterone in stromal cells by § Tamsulosin, doxazosin
5α-reducatase enzyme. § Relax prostate smooth muscle
• DHT-induced GFs → ↑ stromal cells and ↓ epithelial § SE: drowsiness, ↓BP, depression, EF, wt. ↑,
cell death. extra-pyramidal signs
nd
• 2 : 5α-reductase inhibitors
§ Finasteride
Presentation § Inhibit conversion of testosterone → DHT
§ Preferred if significantly enlarged prostate.
• Storage Symptoms
§ SE: excreted in semen (use condoms), ED
§ Nocturia
§ Frequency
§ Urgency Surgical Mx
§ Overflow incontinence • Indications
• Voiding Symptoms § Symptoms affect QoL
§ Hesitancy § Complications of BPH
§ Straining • TURP
§ Poor stream/flow + terminal dribbling § Cystoscopic resection of lateral and middle
§ Strangury (urinary tenesmus) lobes
§ Incomplete emptying: pis en deux § ≤14% become impotent
•
O
Bladder stones (2 to stasis) • Transurethral incision of prostate (TUIP)
•
O
UTI (2 to stasis) § < destruction → ↓ risk to sexual function
§ Similar benefits to TURP if small prostate
(<30g)
• Tranurethral ElectroVaporisation of Prostate
Examination § Electric current → tissue vaporisation
• PR • Laser prostatectomy
§ Smoothly enlarged prostate § ↓ ED and retrograde ejaculation
§ Definable median sulcus § Similar efficacy as TURP
• Bladder not usually palpable unless acute-on-chronic • Open retropubic prostatectomy
obstruction § Used for very large prostates (>100g)

Ix TURP Complications
• Blood: U+E, PSA (after PR)
• Urine: dip, MC+S
Immediate
• Imaging
• TUR syndrome
§ Transrectal US ± biopsy
§ Absorption of large quantity of fluids → ↓Na
• Urodynamics: pressure / flow cystometry
• Haemorrhage
• Voiding diary
Early
• Haemorrhage
• Infection
• Clot retention: requires bladder irrigation

Late
• Retrograde ejaculation: common
• ED: ~10%
• Incontinence: ≤10%
• Urethral stricture
• Recurrence

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 Prostate Cancer
Epidemiology Gleason Grade
• Commonest male Ca • Score two worst affected areas
rd
• 3 commonest cause of male Ca death • Sum is inversely proportional to prognosis
• Prevalence: 80% of men >80yrs
• Race: ↑ in Blacks TNM Stage

Pathology TIS Carcinoma in situ

• Adenocarcinoma T1 Incidental finding on TURP or ↑PSA
• Peripheral zone of prostate T2 Intracapsular tumour c̄ deformation of prostate
T3 Extra-prostatic extension
Presentation T4 Fixed to pelvis + invading neighbouring structures
• Usually asymptomatic N1-4 1 or more lymph nodes involved
• Urinary: nocturia, frequency, hesitancy, poor stream,
M1 Distant mets, e.g. spine
terminal dribbling, obstruction
• Systemic: wt. loss, fatigue
• Mets: bone pain Prognostic Factors
• Help determine whether to pursue radical Rx
Examination • Age
• Hard irregular prostate on PR • Pre-Rx PSA
• Loss of midline sulcus • Tumour stage
• Tumour grade
Spread
• Local: seminal vesicles, bladder, rectum Mx
• Lymph: para-aortic nodes • Difficult to know which tumours are indolent and will not
• Haem: sclerotic bony lesions → mortality before something else.
• Radical therapy assoc. c̄ significant morbidity.
Ix
• Bloods: PSA, U+E, acid and alk phos, Ca Conservative: Active Monitoring
• Imaging • Close monitoring c̄ DRE and PSA
§ XR chest and spine
§ Transrectal US + biopsy Radical Therapy
§ Bone scan
• Radical prostatectomy (+ goserelin if node +ve)
§ Staging MRI
§ Performed laparoscopically c̄ robot
- Contrast enhancing magnetic
§ Only improves survival vs. active monitoring if
nanoparticles ↑s detection of affected
<75yrs
nodes.
• Brachytherapy: implantation of palladium seeds
• SEs: ED, urinary incontinence, death (0.2-0.5%)
PSA
• Proteolytic enzyme used in liquefaction of ejaculate
Medical
• Not specific for prostate Ca
• Used for metastatic or node +ve disease
§ ↑ c̄ age, PR, TURP, and prostatitis
• LHRH analogues
• >4ng/ml: 40-90% sensitivity, 60-90% specificity
§ E.g. goserelin
§ Only 1-in-3 will have Ca
§ Inhibit pituitary gonadotrophins → ↓ testosterone
• Normal in 30% of small cancers
• Anti-androgens
§ E.g. cyproterone acetate, flutamide

Symptomatic
• TURP for obstruction
• Analgesia
• Radiotherapy for bone mets / cord compression

Screening c̄ PSA
• Population based screening not recommended in UK
• PSA not an accurate tumour marker
• ERSPC trial showed small mortality benefit, PLCO trial
showed no benefit.
• Must balance mortality benefit c̄ harm caused by over
diagnosis and over treatment of indolent cancers.

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Prostatitis Urinary Incontinence
Aetiology Male
• S. faecalis • Usually caused by prostatic enlargement
• E. coli § Urge incontinence or dribbling may result from partial
• Chlamydia retention.
§ Retention may → overflow (palpable bladder after
Presentation voiding)
• TURP and pelvic surgery may weaken external urethral
• Usually >35yrs
sphincter.
• UTI / dysuria
• Pain
§ Low backache Women
§ Pain on ejaculation • Stress Incontinence
• Haematospermia § Leakage from incompetent sphincter when IAP ↑
• Fever and rigors § Loss of small amounts of urine when coughing
• Retention § Pelvic floor weakness
• Malaise • Urge Incontinence / Overactive Bladder
§ Can’t hold urine for any length of time
§ May have precipitant: arriving home, running water,
Examination coffee
• Pyrexia § Dx: urodynamic studies
• Swollen / boggy / tender prostate on PR
• Examine testes to exclude epididymo-orchitis Mx
• Check
Ix § PR: faecal impaction
• Blood: FBC, U+E, CRP § Palpable bladder after voiding: retention c̄ overflow
• Urine: dip, MC+S § UTI
§ DM
Rx § CNS: MS, Parkinson’s stroke, spinal trauma
• Analgesia § Diuretics
• Levofloxacin 500mg/d for 28d • Stress Incontinence
§ Pelvic floor exercises
§ Ring pessary
§ Duloxetine
§ Surgery: tension-free vaginal tape
• Urge Incontinence
§ Bladder training
§ Wt. loss
§ Anti-AChM: tolterodine, imipramine

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Undescended Testes Testicular Torsion
Epidemiology Aetiology
O
• 3% @ birth • Usually 2 to some exertion or minor trauma
• 1% @ 1yr • Occurs because testicle doesn’t have a large “bare area”
• Unilateral 4x commoner cf. bilateral to attach to scrotal wall.
§ Should have genetic testing if bilateral § Tunica vaginalis invests whole of testicle
- Noonan’s, Prader-Willi § Free-hanging “clapper bell” testicle can twist on
• Commoner in prems: incidence up to 30% its mesentery.

Normal Descent Presentation

• Testes remain in abdomen (retroperitoneal) until 7mo
• Gubernaculum connects inferior pole of testis to
scrotum.
• Testis descends through inguinal canal to scrotum c̄
an out-pouching of peritoneum: processus vaginalis.
• Usually 10-25yrs
• Sudden onset severe pain in one testis
• May have lower abdominal pain (testis supplied by T10)
• Assoc. c̄ n/v
• May be Hx of previous testicular pain or torsion

Classification Examination
• Inflam of one testis: hot, swollen, extremely tender
Cryptorchidism • Testis rides high and lies transversely
• Complete absence of testis from scrotum
• Anorchism = absence of both testes Differential
• Epididymo-orchitis
Retractile Testis § Older pt.
• Normal development but excessive cremasteric reflex § UTI symptoms
• Testicle often found at external inguinal ring § More gradual onset
• Will descend: no Rx required • Torted Hydatid of Morgagni
§ Remnant of Mullerian duct
Maldescended Testis § Younger pt.
• Found anywhere along normal path of descent § Less pain
§ Tiny blue dot visible on scrotum
• Testis and scrotum are usually under-developed
• Tumour
• Often assoc. c̄ patent processus vaginalis
• Trauma
• Strangulated hernia
Ectopic Testis • Appendicitis
• Found outside line of descent
• Usually in sup. inguinal pouch (ant. to external
oblique aponeurosis)
Ix
• Abdominal, perineal, penile, femoral triangle • Doppler US may demonstrate absence of flow
§ Must not delay surgical exploration
Complications
• Infertility
Mx
• Surgical emergency
• 10x ↑ risk of malignancy (remains after surgery)
§ 4-6h window from onset of pain to salvage testis
• ↑ risk of trauma
• Inform senior
• ↑ risk of torsion
• NBM
• Assoc. c̄ hernias (90%) or urinary tract abnormalities
• IV access
§ Analgesia
Mx § Bloods: FBC, U+E, G+S, clotting
• Restores potential for spermatogenesis • Surgery
• Makes Ca easier to Dx § Consent for possible orchidectomy
§ Bilateral orchidopexy: suture testes to scrotum
Surgical: Orchidopexy by Dartos Pouch Procedure • If no torsion found and epididymo-orchitis Dx, take fluid
• Perform before 2yrs sample from scrotum for bacteriology and Rx c̄ Abx.
• Mobilisation of testis and cord
• Removal of patent processus
• Testicle brought through a hole made in the dartos
muscle to lie in a subcutaneous pouch.
• Dartos prevents retraction.

Hormonal
• β-HCG may be tried if testis is in inguinal canal.

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 Lumps in the Groin and Scrotum
Differential Epididymo-Orchitis
• Can’t get above: inguinoscrotal hernia
• Separate, cystic: epididymal cyst Aetiology
• Separate, solid: varicocele, sperm granuloma, • STI: Chlamydia, gonorrhoea
epididymitis • Ascending UTI: E. coli
• Testicular, cystic: hydrocele • Mumps
• Testicular, solid: tumour, orchitis, haematocele
Features
Epididymal Cyst • Sudden onset tender swelling
• Develop in adulthood • Dysuria
• Contain clear or milky (spermatocele) fluid • Sweats, fever
• Lie above and behind testis
• Remove if symptomatic Examination
• Tender, red, warm, swollen testis and epididymis
Varicocele § Elevating testicle may relieve pain
•Dilated veins of pampiniform plexus • Secondary hydrocele
•Presentation • Urethral discharge
§ Feel like bag of worms in the scrotum
§ May be visible dilated veins Ix
§ ↓ size on lying down • Blood: FBC, CRP
§ Pt. may c/o dull ache • Urine: dip, MC+S (fist catch may be best)
§ May → oligospermia (↓ fertility) • Urethral swab and STI screen
• Pathology • US: may be needed to exclude abscess
O
§ 1 : Left side commoner: drain into left renal vein
O
§ 2 : left renal tumour has tracked down renal
Complications
vein → testicular vein obstruction.
• May → infertility
• Mx
§ Conservative: scrotal support
§ Surgical: clipping the testicular vein (open or Mx
lap) • Bed rest
• Analgesia
Sperm Granuloma • Scrotal support
• Painful lump of extravasated sperm after vasectomy • Abx: doxycycline or cipro
• Drain abscess if present
Hydrocele
• Collection of serous fluid w/i tunica vaginalis
• Primary
§ assoc. c̄ patent processus vaginalis
§ Commoner, larger, tense, younger men
• Secondary
§ Tumour, trauma, infection
§ Smaller, less tense
• Ix
§ US testicle to exclude tumour
• Mx
§ May resolve spontaneously
§ Surgery
- Lord’s Repair: plication of the sac
- Jaboulay’s Repair: eversion of the sac
§ Aspiration
st
- Usually recur so not 1 line.
- Send fluid for cytology and MC+S

Haematocele
• Blood in the tunica vaginalis
• Hx of trauma
• May need drainage or excision

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 Testicular Tumours
Epidemiology Staging: Royal Marsden Classification
• Commonest male malignancies from 15-44yrs • Disease only in testis
• Whites > Blacks = 5:1 • Para-aortic nodes involved (below diaphragm)
• Supra- and infra-diaphragmatic LNs involved
Presentation • Extra-lymphatic spread: lungs, liver
• Painless testicular lump
§ Often noticed after trauma Ix
• Haematospermia • Tumour markers
O
• 2 hydrocele § Useful for monitoring
• Mets: SOB from lung mets § ↑AFP and ↑hCG in 90% of teratomas
• Abdo mass: para-aortic lymphadenopathy § ↑hCG in 15% of seminomas
• Hormones: gynaecomastia, virilisation § Normal AFP in pure seminomas
• Contralateral tumour in 5% • Scrotum US
• Staging
§ CXR
Risk Factors § CT
• Undescended testis
§ 10% occur in undescended testes NB. Percutaneous biopsy should not be performed as it may
• Infant hernia → seeding along needle tract
• Infertility
Mx
Pathology • If both testes are abnormal, semen can be
cryopreserved
Germ Cell: 95% of tumours
• Pure Seminomas: 40% Seminomas
§ Commonest single subtype • Stage 1-2: inguinal orchidectomy + radiotherapy
§ 30-40yrs § Groin incision allows cord clamping to prevent
§ ↑ βhCG in 15% seeding
§ ↑ placental ALP in some • Stage 3-4: as above + chemo (BEP)
§ Very radiosensitive § Bleomycin, Etoposide, cisPlatin
• Non-seminomas (inc. mixed): 60%
§ Mixed: commonest NSGCT
Non-seminomas / Teratomas
§ Teratoma
• Stage 1: inguinal orchidectomy + surveillance
- Arise from all 3 germ layers
• Stage 2: orchidectomy + chemo + para-aortic LN
- Common and benign in children
dissection
- Rare and malignant in adults: 15-30yrs
• Stage 3: orchidectomy + chemo
- Secrete βhCG and/or AFP
- Chemosensitive
§ Yolk Sac Close f/up to detect relapse
- Commonest testicular tumour in children • Typically w/i 18-24mo
§ Choriocarcinoma • Repeat CT scanning and tumour markers
- ↑↑ βhCG
Prognosis
Sex-cord Stromal • Stage 1: 98% 5ys
• Leydig Cell • Stage 2: 85% 5ys
§ Mostly benign • Stage 4: 60% 5ys
§ May secrete androgens or oestrogens
• Sertoli Cell
§ Mostly benign
§ May secrete oestrogens

Lymphoma / Leukaemia
• NHL: commonest malignant testicular mass >60yrs
• ALL: commonest malignant testicular mass <5yrs

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 The Penis
Balanitis Penile Cancer
• Acute inflammation of the foreskin and glans
• Cause: Strep, Staph infection, Candida (DM) Epidemiology
• RFs: DM, young children c̄ tight foreskin • Incidence: 1:100,000/yr in UK
• Rx: hygiene advice, Abx, circumcision • Geo: commoner in Far East and Africa

Aetiology
Phimosis • V. rare if circumcised
• Foreskin occludes the meatus • Risk factors
• Children § HPV (16, 18, 31) infection
O
§ Chronic irritation 2 to smegma
§ Pres: recurrent balanitis and ballooning
§ Mx: Gentle retraction, steroid creams,
circumcision Pathology
• Adults • Erythroplasia of Querat: penile CIS
§ Pres: dyspareunia, infection • SCC
§ Mx: circumcision
§ Assoc. c̄ balanitis xerotica obliterans: thickening Presentation
of foreskin and glans → phimosis + meatal • Chronic fungating ulcer
narrowing • Bloody / purulent discharge
• 50% have inguinal nodes at presentation

Paraphimosis Mx
• Tight foreskin is retracted and becomes irreplaceable. • Medical
• ↓ venous return → oedema and swelling of the glans § Early growths c̄ no urethral involvement
§ Can rarely → glans ischaemia § DXT and iridium wires
• Causes: catheterisation, masturbation, intercourse • Surgical
• Mx: § Amputation required if urethral involvement
§ Manual reduction: use ice and lignocaine jelly § Lymph node dissection
§ May require glans aspiration or dorsal slit

Hypo- / epi-spadias
• Developmental abnormality of the position of the urethral
opening
• Hypospadia: opens on ventral surface of penis
• Epispadia: opens on dorsal surface

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Bone and Fracture Physiology Fracture Classification
Composition Classification
• Traumatic #
Cells: osteoblasts, osteoclasts, osteocytes, OPCs § Direct: e.g. assault c̄ metal bar
§ Indirect: e.g. FOOSH → clavicle #
Matrix § Avulsion
• Organic = osteoid (40%) • Stress #
§ Collagen type I § Bone fatigue due to repetitive strain
§ Resists tension, twisting and bending § E.g. foot #s in marathon runners
• Inorganic (60%) • Pathological #
§ Calcium hydroxyapatite § Normal forces but diseased bone
§ Resists compressive forces - Local: tumours
- General: osteoporosis, Cushing’s, Paget’s

Classification Describing a fracture: PAID

• Radiographs must be orthogonal: request AP and lat. films.
• Need images of joint above and joint below #.
Woven Bone
• Disorganised bone that forms the embryonic
skeleton and fracture callus. • Demographics
§ Pt. details
§ Date radiograph taken
Lamellar Bone § Orientation and content of image
• Mature bone that can be of two types:
§ Cortical/compact: dense outer layer • Pattern
§ Cancellous/trabecular: porous central
• Transverse • Crush
layer
• Oblique • Greenstick
• Spiral • Avulsion
• Multifragmentary
Formation
• Anatomical Location
Intramembranous Ossification
• Direct ossification of mesenchymal bone models • Intra- / extra-articular
formed during embryonic development. • Dislocation or subluxation
• Skull bones, mandible and clavicle.
• Deformity (distal relative to proximal)
Endochondral Ossification § Translation
• Mesenchyme → cartilage → bone § Angulation or tilt
• Most bones ossify this way § Rotation
§ Impaction (→shortening)

Fracture Healing • Soft Tissues

§ Open or closed
§ Neurovascular status
Reactive Phase (injury – 48hrs)
§ Compartment syndrome
• Bleeding into # site → haematoma
• Inflammation → cytokine, GF and vasoactive
• ? Specific # classification/type
mediator release → recruitment of leukocytes and
§ Salter-Harris
fibroblasts → granulation tissue
§ Garden
§ Colles’, Smith’s, Galeazzi, Monteggia
Reparative Phase (2 days – 2 wks)
• Proliferation of osteoblasts and fibroblasts →
cartilage and woven bone production → callus
formation.
• Consolidation (endochondral ossification) of
woven bone → lamellar bone

Remodelling Phase (1wk – 7yrs)

• Remodelling of lamellar bone to cope c̄
mechanical forces applied to it (Wolff’s Law: “form
follows function”)

Healing Time
• Closed, paediatric, metaphyseal, upper limb: 3wks
• “Complicating factor” doubles healing time
§ Adult
§ Lower limb
§ Diaphyseal
§ Open

© Alasdair Scott, 2018 111

 Fracture Management: 4Rs
1: Resuscitation 3: Restriction

Principles Principles
• Follow ATLS guidelines • Interfragmentary strain hypothesis dictates that tissue
O
• Trauma series in 1 survey: C-spine, chest and pelvis formed @ # site depends on strain it experiences.
O
• # usually assessed in 2 survey • Fixation → ↓ strain → bone formation
• Assess neurovascular status and look for dislocations • Fixation also → ↓ pain, ↑ stability, ↑ ability to function.
• Consider reduction and splinting before imaging
§ ↓ pain Methods
§ ↓ bleeding • Non-rigid
§ ↓ risk of neurovascular injury § Slings
• X-ray once stable § Elastic supports
• Plaster
Open fractures require urgent attention: 6As § POP
• Analgesia: M+M § In first 24-48h use back-slab or split cast due to
• Assess: NV status, soft tissues, photograph risk of compartment syndrome
• Antisepsis: wound swab, copious irrigation, cover with • Functional bracing
betadine-soaked dressing. § Joints free to move but bone shafts supported
• Alignment: align # and splint in cast segments.
• Anti-tetanus: check status (booster lasts 10yrs) • Continuous traction
• Abx § e.g. collar-and-cuff
§ Fluclox 500mg IV/IM + benpen 600mg IV/IM • Ex-Fix
§ Or, augmentin 1.2g IV § Fragments held in position by pins/wires which
are then connected to an external frame.
Mx: debridement and fixation in theatre § Intervention is away from field of injury.
§ Useful in open #s, burns, tissue loss to allow
wound access and ↓ infection risk.
Gustillo Classification of Open #s
§ Risk of pin-site infections
• Wound <1cm in length
• Internal fixation
• Wound ≥1cm c̄ minimal soft tissue damage § Pins, plates, screws, IM nails
• Extensive soft tissue damage § Usually perfect anatomical alignment
§ ↑ stability
Clostridium perfringes § Aid early mobilisation
• Most dangerous complication of open #
• Wound infections and gas gangrene
• ± shock and renal failure 4: Rehabilitation
• Rx: debride, benpen + clindamycin
Principles
2: Reduction • Immobility → ↓ muscle and bone mass, joint stiffness
• Need to maximise mobility of uninjured limbs
Principles • Quick return to function ↓s later morbidity
• Displaced #s should be reduced
§ Unless no effect on outcome, e.g. ribs Methods
• Aim for anatomical reduction (esp. if articular surfaces • Physiotherapy: exercises to improve mobility
involved) • OT: splints, mobility aids, home modification
• Alignment is more important than opposition • Social services: meals on wheels, home help

Methods
• Manipulation / Closed reduction
§ Under local, regional or general anaesthetic
§ Traction to disimpact
§ Manipulation to align
• Traction
§ Not typically used now.
§ Employed to overcome contraction of large
muscles: e.g. femoral #s
§ Skeletal traction vs. skin traction
• Open reduction (and internal fixation)
§ Accurate reduction vs. risks of surgery
§ Intra-articular #s
§ Open #s
§ 2 #s in 1 limb
§ Failed conservative Rx
§ Bilat identical #s

112 © Alasdair Scott, 2018

 Fracture Complications
General Complications Specific Complications
Tissue Damage Immediate
• Haemorrhage and shock • Neurovascular damage
• Infection • Visceral damage
• Muscle damage → rhabdomyolysis Early
Anaesthesia • Compartment syn.
• Anaphylaxis • Infection (worse if assoc. c̄ metalwork)
• Damage to teeth • Fat embolism → ARDS
• Aspiration Late
Prolonged Bed Rest • Problems c̄ union
• Chest infection, UTI • AVN
• Pressure sores and muscle wasting • Growth disturbance
• DVT, PE • Post-traumatic osteoarthritis
• ↓ BMD • Complex regional pain syndromes
• Myositis ossificans

Neurological Complications Problems with union

• Severance is rare, usaully stretching over bone edge
• Seddon classification describes three types of injury Delayed Union: union takes longer than expected
Non-union: # fails to unite
Neuropraxia
• Temporary interruption of conduction w/o loss of axonal Causative Factors: 5 Is
continuity. • Ischaemia: poor blood supply or AVN
• Infection
Axonotmesis • ↑ interfragmentary strain
• Disruption of nerve axon → distal Wallerian degeneration. • Interposition of tissue between fragments
• Connective tissue framework of nerve preserved. • Intercurrent disease: e.g. malignancy or malnutrition
• Regeneration occurs and recovery is possible.
Non-union Classification
Neurotmesis • Hypertrophic
• Disruption of entire nerve fibre § Bone end is rounded, dense and sclerotic
• Surgery required and recovery not usually complete. • Atrophic
§ Bone looks osteopenic
Common Palsies
Injury Palsy Test/Result Management
• Optimise biology: infection, blood supply, bone
Ant. shoulder dislocation Axillary N. Numb chevron
Humeral surgical neck Weak abduction graft, BMPs
# humeral shaft Radial N. Waiter’s tip • Optimise mechanics: ORIF
Elbow dislocation Ulnar N. Claw hand
Hip dislocation Sciatic N. Foot drop Malunion: # healed in an imperfect position
# neck of fibula Fibular N. Foot drop • Poor appearance and/or function
Knee dislocation • E.g. Gunstock deformity

Compartment Syndrome
• Osteofacial membranes divide limbs into muscle
compartments
• Oedema following # → ↑ compartment pressure → ↓
venous drainage → ↑ compartment pressure
• Compartment pressure > capillary pressure → ischaemia
• Muscle infarction →
§ Rhabdomyolysis and ATN
§ Fibrosis → Volkman’s ischaemic contracture
Presentation
• Pain > clinical findings
• Pain on passive muscle stretching
• Warm, erythematous, swollen limb
• ↑ CRT and weak/absent peripheral pulses
Avascular Necrosis
• Death of bone due to deficient blood supply.
• Sites: femoral head, scaphoid, talus
• Consequence: bone becomes soft and deformed →
pain, stiffness and OA.
• X-ray: sclerosis and deformity.
Myositis Ossificans
• Heterotopic ossification of muscle @ sites of
haematoma formation
• → restricted, painful movement
• Commonly affects the elbow and quadriceps
• Can be excised surgically

Rx Pellegrini-Stieda disease
• Elevate limb • Form of MO
• Remove all bandages and split/remove cast • Calcification of the superior attachment of MCL @
• Fasciotomy the knee following traumatic injury.

© Alasdair Scott, 2018 113

Complex Regional Pain Syndrome Type 1
= Reflex Sympathetic Dystrophy, Sudek’s Atrophy

Definition Rx
• Complex disorder of pain, sensory abnormalities, • Usually self-limiting
abnormal blood flow, sweating and trophic changes in • Refer to pain team
superficial or deep tissues. • Amitryptilline, gabapentin
• No evidence of nerve injury. • Sympathetic nerve blocks can be tried.

Causes CRPS Type II (= Causalgia)

• Injury: #s, carpal tunnel release, ops for Dupuytren’s • Persistent pain following injury caused by nerve
• Zoster, MI, Idiopathic lesions.

Presentation
• Wks – months after injury
• NOT traumatised area that is affected: affects a
NEIGHBOURING area.
• Lancing pain, hyperalgesia or allodynia
• Vasomotor: hot and sweaty or cold and cyanosed
• Skin: swollen or atrophic and shiny.
• NM: weakness, hyper-reflexia, dystonia, contractures

Growth Disturbance
• In children, damage to the physis (growth plate) can result in abnormal bone growth.
• The Salter-Harris classification (1963) categorises growth plate injuries:

Salter Harris Classification

• Straight across
• Above
• Lower
• Through
• CRUSH
Normal Type 1 - 5% Type 2 - ?5%

|'

l|
||'|'

>
Type 3 - 10% Type 4 - 10% Type 5 - uncommon

• ↑ing risk of growth plate injury

• SH 1: e.g. SUFE. Normal growth c̄ good reduction.
• SH 4: union across physis may interfere c̄ bone growth
• SH 5: crush → physis injury → growth arrest

114 © Alasdair Scott, 2018

 Hip Fracture
Epidemiology Classification
• 80/100,000 • Intracapsular: subcapital, transcervical, basicervical
• 50% in >80yrs • Extracapsular: Intertrochanteric, subtrochanteric
• F>M = 3:1
Garden Classification of Intracapsular Fractures
Pathophysiology • Incomplete #, undisplaced
• Old = osteoporosis c̄ minor trauma (e.g. fall) • Complete #, undisplaced
• Young = major trauma • Complete #, partially displaced
• Complete #, completely displaced
Osteoporosis Risk Factors: Age + SHATTERED
• Steroids ) 3
• Hyper- para/thyroidism , J

• Alcohol and Cigarettes

• Thin (BMI<22)
•
El: I -I

Testosterone low 1 ._

• Early Menopause 5 1
.

• Renal / liver failure ‘ F

• Erosive / inflame bone disease (e.g. RA, Type 1 Type 2

myeloma)
• Dietary Ca low / malabsorption, DM
k LQ
Presentation
‘r . .
- .| .
I I | T
I . U I r‘
-' .'\ I -

•
I ._ - ‘
O/E: shortened and externally rotated
• Key Qs:
§ Mechanism
§ RFs for osteoporosis / pathological #
§ Premorbid mobility Type 3 Type 4

§ Premorbid independence
§ Comorbidities
§ MMSE Surgical Management

Initial Management Intracapsular

• Resuscitate: dehydration, hypothermia • 1,2: ORIF c̄ cancellous screws
• Analgesia: morphine, fascia iliaca block • 3,4:
• Assess neurovascular status of limb § <55: ORIF c̄ screws.
• Imaging: AP and lateral films - f/up in OPD and do arthroplasty if AVN
• Prep for theatre develops (in 30%)
§ Inform Anaesthetist and book theatre § 55-75: total hip replacement
§ Bloods: FBC, U+E, clotting, X-match (2u) § >75: hemiarthroplasty
§ CXR - Mobilises: cemented Thompson’s
§ DVT prophylaxis: TEDS, LMWH - Non-mobiliser: uncemented Austin Moore
§ ECG
§ Films: orthogonal X-rays Extracapsular
§ Get consent • ORIF c̄ DHS

Imaging Discharge
• Ask for AP and lateral film • Involve OT and physios
• Look @ Shenton’s lines • Discharge when mobilisation and social circumstances
• Intra- or extra-capsular? permit.
• Displaced or non-displaced
• Osteopaenic? Specific Complications
• AVN of fem head in displaced #s (30%)
Key Anatomy • Non / mal-union (10-30%)
• Capsule attaches proximally to acetabular margin • Infection
and distally to intertrochanteric line. • Osteoarthritis
• Blood supply to fem head:
§ Retinacular vessels, in capsule, distal → Prognosis
prox • 30% mortality @ 1yr
§ Intramedullary vessels
• 50% never regain pre-morbid functioning
§ Artery of ligamentum teres.
• >10% unable to return to premorbid residence
• If retinacular vessels damaged there is risk of
• Majority will have some residual pain or disability.
AVN of the femoral head → pain, stiffness and OA

© Alasdair Scott, 2018 115

Distal Forearm Fractures Scaphoid Fractures
Colles’ Fracture Clinical Features
• FOOSH
Clinical Features • Pain in anatomical snuffbox
• Fall onto an outstretched hand • Pain on telescoping the thumb
• Most common in elderly females c̄ osteoporosis
• Dinner fork deformity Specific Management
• Request scaphoid x-ray view
Radiographic Features • If clinical hx and exam suggest a scaphoid #, it should
• Extra-articular # of dist. radius (w/i 1.5” of joint) initially be treated even if the x-ray is normal.
• Dorsal displacement of distal fragment § # may become apparent after 10 days due to
localised decalcification.
• Dorsal angulation of distal fragment
• Place wrist in scaphoid plaster (beer glass position)
§ Normally 11 degrees volar tilt
• If initial x-ray is negative, pt. returns to # clinic after 10
• ↓ radial height (norm =11mm)
O days for re-xray.
• ↓ radial inclination (norm =22 )
§ # visible → plaster for 6 wks
• ± avulsion of ulna styloid
§ No visible # but clinically tender → plaster for 2
• ± impaction wks
§ # not visible and not clinically tender → no plaster
Specific Management
• Examine for neurovascular injuries as median nerve Specific Complications
and radial artery lie close. • Main risk is AVN of the scaphoid as blood supply runs
• If much displacement → reduction distal to proximal.
§ Under haematoma block, IV regional § → stiffness and pain at the wrist
anaesthesia (Bier’s block) or GA.
§ Disimpact and correct angulation.
§ Position: ulnar deviation + some wrist flexion
§ Apply dorsal backslab: provide 3-point Radial and Ulna Shaft Fractures
pressure
• Re X-Ray – satisfactory position?
Classification
§ No: ortho review and consider MUA ± K
• Monteggia
wires rd
§ # of proximal 3 of ulna shaft
§ Yes: home c̄ # clinic f/up w/i 48hrs for
§ Anterior dislocation of radial head at capitellum
completion of POP
§ May → palsy of deep branch of radial nerve →
• 6 wks in POP + physio weak finger extension but no sensory loss
• If comminuted, intra-articular or re-displaces:
§ Surgical fixation c̄ ex-fix, Kirschner-wires or
• Galleazzi
ORIF and plates. § # of radial shaft between mid and distal 3
rds

§ Dislocation of distal radio-ulna joint

Specific Complications
• Median N. injury Specific Management
• Frozen shoulder / adhesive capsulitis • Unstable fractures
• Tendon rupture: esp. EPL § Adults: ORIF
• Carpal tunnel syn. § Children: MUA + above elbow plaster
• Mal- /non-union • Fractures of forearm should be plastered in most stable
• Sudek’s atrophy / CRPS position:
§ Proximal #: supination
§ Distal #: pronation
§ Mid-shaft #: neutral
Other Distal Forearm Fractures

Smith’s / Reverse Colles’

• Fall onto back of flexed wrist
• Fracture of distal radius c̄ volar displacement and
angulation of distal fragment.
• Reduce to restore anatomy and POP for 6wks

Barton’s Fracture
• Oblique intra-articular # involving the dorsal aspect
of distal radius and dislocation of radio-carpal joint
• Reverse Barton’s involves the volar aspect of the
radius

116 © Alasdair Scott, 2018

 The Shoulder
Shoulder Dislocation Impingement Syndrome / Painful Arc

Classification Pathology
• Anterior • Entrapment of supraspinatus tendon and subacromial
§ 95% of shoulder dislocations. bursa between acromion and grater tuberosity of
§ Direct trauma or falling on hand humerus.
§ Humeral head dislocates antero-inferiorly • → subacromial bursitis and/or supraspinatous tendonitis
• Posterior
§ Caused by direct trauma or muscle Presentation
contraction (seen in epileptics). O
• Painful arc: 60-120
• Weakness and ↓ ROM
Associated Lesions • +ve Hawkin’s test

Bankart Lesion Ix
• Damage to anteroinferior glenoid labrum. • Plain radiographs: may see bony spurs
• US
Hill-Sachs Lesion • MRI arthrogram
• Cortical depression in the posterolateral part of the
humeral head following impaction against the Rx
glenoid rim during anterior dislocation. • Conservative
• Occurs in 35-40% of anterior dislocations. § Rest
§ Physiotherapy
Presentation • Medical
• Shoulder contour lost: appears square § NSAIDs
• Bulge in infraclavicular fossa: humeral head § Subacromial bursa steroid ± LA injection
• Arm supported in opposite hand • Surgical
• Severe pain § Arthroscopic acromioplasty

Differential of Painful Arc

Specific Management
• Impingement
• Assess for neurovascular deficit: esp. axillary N.
• Supraspinatous tear or partial tear
§ Sensation over “chevron” area before and
• AC joint OA
after reduction.
§ Occurs in 5%
• X-ray: AP and transcapular view Frozen Shoulder: Adhesive Capsulitis
• Reduction under sedation (e.g. propafol)
§ Hippocratic: Longitudinal traction c̄ arm in Presentation
O
30 abduction and counter traction @ the • Progressive ↓ active and passive ROM
O
axilla § ↓ ext. rotation <30
O
§ Kocher’s: external rotation of adducted arm, § ↓ abduction <90
anterior movement, internal rotation • Shoulder pain, esp. @ night (can’t lie on affected side)
• Rest arm in a sling for 3-4wks
• Physio Cause
• Unknown, may follow trauma in elderly
Complications • Commonly assoc. c̄ DM
• Recurrent dislocation
§ 90% of pts. <20yrs with traumatic dislocation
Rx
• Axillary N. injury
• Conservative: rest, physio
• Medical
§ NSAIDs
Recurrent Shoulder Instability § Subacromial bursa steroid ± LA injection

TUBS: Traumatic Unilateral dislocations with a Rotator Cuff Tear

O
Bankart lesion often require Surgery • 2 to degeneration or a sudden jolt or fall
• Mostly young patients: 15-30yrs • Partial tears → painful arc
• Surgery involves a Bankart repair • Complete tear
§ Shoulder tip pain
AMBRI: Atraumatic Multidirectional Bilateral § Full range of passive movement
shoulder dislocation is treated with Rehabilitation, but § Inability to abduct the arm
§ Active abduction possible following passive
may require Inferior capsular shift O
abduction to 90
§ Lowering the arm beneath this → sudden drop
- “drop arm” sign

Rx: open or arthroscopic repair

© Alasdair Scott, 2018 117

 Supracondylar Fractures of the Humerus
Presentation
• Common in children after FOOSH
• Elbow very swollen and held semi-flexed.
• Sharp edge of proximal humerus may injure brachial
artery which lies anterior to it.
Classification
Extension
• Commonest type
• Distal fragment displaces posteriorly
• Gartland further classified extension type:
§ Type 1: non-displaced
§ Type 2: angulated c̄ intact posterior cortex
§ Type 3: displaced c̄ no cortical contact
Flexion
• Less common
• Distal fragment displaces anteriorly
Specific Management
• Ensure there is no neurovascular damage
§ If radial pulse absent or damage to brachial
artery suspected, take urgently to theatre for
reduction ± on-table angiogram.
§ Median nerve is also vulnerable
Specific Complications
Neurovascular Injury
• Brachial artery
• Radial nerve
• Median nerve: esp. anterior interosseous branch
§ Supplies deep forearm flexors (FPL, lateral half
of FDP and pronator quadratus)
Compartment syndrome
• Monitor closely during the first 24h
• Pain on passive extension of the fingers (stretches
flexor compartment) is early sign.
• Mx: try extension of the elbow, surgical Rx may be
needed.
• Volkmann’s ischaemic contracture can result →
fibrosis of flexors → claw hand.
Gunstock Deformity
• Valgus, varus and rotational deformities in the coronal
plane do not remodel and → cubitus varus.
• Cubitus varus deformity is referred to as a “gunstock”
deformity.

• Restore the anatomy

§ No displacement → flex the arm as fully as
possible and apply a collar and cuff for 3wks –
triceps acts as sling to stabilise fragments.
§ Displacement → MUA + fixation with K-wires
+ collar and cuff with arm flexed for 3wks.

118 © Alasdair Scott, 2018

Femoral and Tibial Fractures Ankle Injuries
Specific Management Ligament Strains
• Resus and Mx life-threatening injuries first. • Typically twisting inversion injury
• X-Match § Strains anterior talofibular part of lateral
§ Tibial #: 2 units collateral ligament
§ Femoral #: 4 units • Medial deltoid ligament strains are rare.
• Assess neurovascular status: esp. distal pulses • May be assoc. c̄ malleolar avulsion #s
• If open
§ Abx and ATT
§ Take to theatre urgently for debridement,
Ankle Fracture
washout and stabilisation
• Fixation methods
§ Intramedullary nail Ottowa Ankle Rules
§ Ex-fix • X-ray ankle if pain in malleolar zone + in any of:
§ Plates and screws § Tenderness along distal 6cm of posterior tib /
§ MUA c̄ fixed traction for 3-4mo fib including posterior tip of the malleoli.
§ Inability to bear weight both immediately and in
ED
Specific Complications
• Hypovolaemic shock Weber Classification
• Neurovascular
• Relation of fibula # to joint line
§ SFA: swelling and check pulses
• A: below joint line
§ Sciatic nerve
• B: at joint line
• Compartment syndrome
• C: above joint line
• Respiratory complications
• Weber’s B and C represent possible injury to the
§ Fat embolism
syndesmotic ligaments between tib and fib →
§ ARDS
instability
§ Pneumonia

Mx
• Weber A
§ Boot or below-knee POP
• Non-displaced Weber B/C
§ Below-knee POP
• Displaced Weber B/C
§ Closed reduction and POP if anatomical
reduction achieved
§ ORIF if closed reduction fails

© Alasdair Scott, 2018 119

 Knee Injuries
History Arthroscopy
• Mechanism • Direct vision of inside of knee joint by arthroscope
• Swelling • Can examine knee under anaesthesia (↓ muscle tone)
§ Immediate = haemarthrosis = # or torn • Meniscal tears can be trimmed or repaired.
cruciates
§ Overnight = effusion = meniscus or other lgt
Mx of Ruptured ACL
• Pain / tenderness
§ Joint line = meniscal
§ Med/lateral margins = collateral lgts. Conservative
• Locking: meniscal tear → mechanical obstruction • Rest
• Giving way: instability following lgt. injury • Physio to strengthen quads and hamstrings
• Not enough stability for many sports

Surgical
Knee Haemarthrosis • Gold-standard is autograft repair
O
• 1 : spontaneous bleeding
• Usually semitendinosus ± gracilis (can use patella
§ Coagulopathy: warfarin, haemophilia
O tendon)
• 2 : trauma
• Tendon threaded through heads of tibia and femur
§ ACL injury: 80%
and held using screws.
§ Patella dislocation: 10%
§ Meniscal injury: 10%
- Outer third where its vascularised
§ Osteophyte #

Unhappy Triad of O’Donoghue

• ACL
• MCL
• Medial Meniscus

Mx of acutely injured knee

• Full examination of acutely swollen knee after injury is
difficult.
• Take x-ray to ensure no #s
§ Fluid level indicates a lipohaemarthrosis and
indicates either a # or torn cruciate.
• If no # → RICE + later re-examination for pathology
• If meniscal or cruciate injury suspected → MRI

120 © Alasdair Scott, 2018

 Osteoarthritis
Definition Pathophysiology
• Degenerative joint disorder in which there is • Softening of articular cartilage → fraying and fissuring
progressive loss of hyaline cartilage and new bone of smooth surface → underlying bone exposure.
formation at the joint surface and its margin. • Subchondral bone becomes sclerotic c̄ cysts.
• Proliferation and ossification of cartilage in unstressed
Aetiology / Risk Factors areas → osteophytes.
• Age (80% > 75yrs) • Capsular fibrosis → stiff joints.
• Obesity
• Joint abnormality Differential
• Septic
Classification • Crystal
• Primary: no underlying cause • Trauma
• Secondary: obesity, joint abnormality
X-ray Changes
Symptoms • Loss of joint space
• Affects: knees, hips, DIPs, PIPs, thumb CMC • Osteophytes
• Pain: worse c̄ movement, background rest/night pain, • Subchondral cysts
worse @ end of day.
• Stiffness: especially after rest, lasts ~30min (e.g. AM) • Subchondral sclerosis
• Deformity • Deformity
• ↓ ROM
Bloods
Signs • CRP may be mildly elevated
• Bouchards and Heberdens nodes
§ ‘Pouchards’ (prox), ‘Heberdips’ (dist.) • Ca, PO4 and ALP all normal
• Thumb CMC squaring
• Fixed flexion deformity Rx
History MDT: GP, physio, OT, dietician, orthopod
• Pain severity, night pain
• Walking distance Conservative
• Analgesic requirements • Lifestyle: ↓ wt., ↑ exercise
• ADLs and social circumstances • Physio: muscle strengthening
• Co-morbidities • OT: walking aids, supportive footwear, home mods
• Underlying causes: trauma, infection, congenital
Medical
• Analgesia
§ Paracetamol
§ NSAIDs: e.g. arthrotec (diclofenac +
misoprostol)
§ Tramadol
• Joint injection: local anaesthetic and steroids

Surgical
• Arthroscopic Washout
§ Mainly knees
§ Trim cartilage
§ Remove loose bodies.
• Realignment Osteotomy
§ Small area of bone cut out
§ Useful in younger (<50yrs) pts. c̄ medial
knee OA
§ High tibial valgus osteotomy redistributes
wt. to lateral part of joint.
• Arthroplasty: replacement (or excision)
• Arthrodesis: last resort for pain management
• Novel Techniques
§ Microfracture: stem cell release → fibro-
cartilage formation
§ Autologous chondrocyte implantation

© Alasdair Scott, 2018 121

 Back Pain
Mechanical Pain Spondylolisthesis
• Soft tissue injury → dysfunction of whole spine → muscle • Displacement of one lumbar vertebra on another
spasm → pain. § Usually forward
• May have inciting event: e.g. lifting § Usually L5 on S1
• Younger pts. c̄ no sinister features • May be palpable

Mx Causes
• Conservative • Congenital malformation
§ Max 2d bed rest • Spondylosis
§ Education: keep active, how to lift / stoop • Osteoarthritis
§ Physiotherapy
§ Psychosocial issues re. chronic pain and disability Presentation
§ Warmth: e.g. swimming in a warm pool • Onset of pain usually in adolescence or early
• Medical adulthood
§ Analgesia: paracetamol ± NSAIDs ± codeine § Worse on standing
§ Muscle relaxant: low-dose diazepam (short-term) • ± sciatica, hamstring tightness, abnormal gait

Disc Prolapse Dx: Plain radiography

• Herniation of nucleus pulposus through annulus fibrosus
Rx
Presentation • Corset
• L5 and S1 roots most commonly compressed by prolapse • Nerve release
of L4/5 and L5/S1 discs. • Spinal fusion
• May present as severe pain on sneezing, coughing or
twisting a few days after low back strain Spinal Stenosis
• Lumbago: low back pain
• Developmental predisposition ± facet joint
• Sciatica: shooting radicular pain down buttock and thigh osteoarthritis → generalized narrowing of lumbar
spinal canal.
Signs
• Limited spinal flexion and extension Presentation
• Free lateral flexion • Spinal claudication
• Pain on straight-leg raise: Lesague’s Sign § Aching or heavy buttock and lower limb pain
• Lateral herniation → radiculopathy on walking
• Central herniation → corda equina syndrome § Rapid onset
§ May c/o paraesthesiae/numbness
L4/5 → L5 Root Compression § Pain eased by leaning forward (e.g. on bike)
• Weak hallux extension ± foot drop • Pain on spine extension
§ In foot drop due to L5 radiculopathy, weak
inversion (tib. post.) helps distinguish from Ix: MRI
peroneal N. palsy.
• ↓ sensation on inner dorsum of foot Rx
• Corsets
L5/S1 → S1 Root Compression • NSAIDs
• Weak foot plantarflexion and eversion • Epidural steroid injection
• Loss of ankle-jerk • Canal decompression surgery
• Calf pain
• ↓ sensation over sole of foot and back of calf Neurosurgical Emergencies
Ix: MRI (emergency if cauda equina) Acute Cord Compression
• Bilateral pain: back and radicular
Rx • LMN signs at compression level
• Brief rest, analgesia and mobilisation effective in ≥90% • UMN signs and sensory level below compression
• Conservative: brief rest, mobilisation/physio • Sphincter disturbance
• Medical: analgesia, transforaminal steroid injection
• Surgical: discectomy or laminectomy may be needed in Acute Cauda Equina Compression
cauda-equina syndrome, continuing pain or muscle
• Alternating or bilateral radicular pain in the legs
weakness.
• Saddle anaesthesia
• Loss of anal tone
Lumbar Microdiscectomy
• Bladder ± bowel incontinence
• Commonest procedure for disc prolapse
• Microscopic resection of the protruding nucleus pulposus
Rx
• Posterior approach c̄ pt. in prone position.
• Large prolapse: laminectomy / discectomy
• May be performed endoscopically
• Tumours: radiotherapy and steroids
• Abscesses: decompression

122 © Alasdair Scott, 2018

Osteochondritis Traction Apophysitis
• Idiopathic condition in which bony centres of
children/adolescents become temporarily softened due Osgood-Shlatter’s
to osteonecrosis. • Tibial tuberosity apophysitis + patellar tendonitis
• Pressure → deformation • Children 10-14yrs, M>F=3:1
• Bone hardens in new, deformed position • Assoc. c̄ physical activity
• Symptoms: pain below knee, esp c̄ quads contraction
Radiography • X-ray: tuberosity enlargement ± fragmentation
• Initially: ↑ density / sclerosis • Rx: rest, consider POP
• Then: patchy appearance
Sinding-Larsen’s Disease
Scheuermann’s Disease • Traction tendinopathy with calcification of proximal
• Vertebral ring epiphyses attachment of patellar tendon
• Auto dom • Children 8-10yrs
• Vertebral tenderness and kyphosis
• X-ray: wedge-shaped thoracic vertebra Sever’s Disease
• Calcaneal apophysitis
Kohler’s Disease • 8-13yrs
• Navicular bone • Symptoms: pain behind heal + limping
• Children 3-5yrs • Rx: physio
• Pain in mid-tarsal region → limp

Kienbochs Disease
• Lunate bone Osteochondritis Dissecans
• Adults • Piece of bone and overlying cartilage dissects off into
• Pain over lunate, esp. on active movement joint space.
• Impaired grip • Commonly knee (med. fem. condyle), also elbow, hip
and ankle.
• Young adult / adolescent
Friedberg’s Disease • Symptoms: pain, swelling, locking, ↓ ROM
nd rd
• 2 /3 metatarsal heads • X-ray: loose bodies, lucent crater
• Around puberty • Mx: arthroscopic removal
• Forefoot pain worse c̄ pressure

Panner’s Disease
• Capitulum of humerus Avascular Necrosis
Perthes’ Disease Causes
• Hip • # or dislocation
• SCD, thalassaemia
• SLE
• Gaucher’s
• Drugs: steroids, NSAIDs

© Alasdair Scott, 2018 123

 The Limping Child
Aetiology Perthes’ Disease
O
• DDH • Osteochondritis of the femoral head 2 to AVN.
• Transient synovitis
• Septic arthritis Epidemiology
• Perthes’ • 4-10yrs
• Slipped Capital Femoral Epiphyses • M>F=5:1
• JIA / Still’s Disease
Presentation
• Insidious onset
DDH • Hip pain initially, then painless
• Congenital hip joint deformity in which the femoral • 10-20% bilateral
head is or can be completely / partially displaced.
Ix
Epidemiology • X-rays normal initially
• Incidence: 1/1000 • ↑ density of femoral head
• Sex: F>M § Becomes fragmented and irregular
§ Flattening and sclerosis
• Bone scan is useful
Predisposing Factors
• FH
Mx
• Breach presentation
• Detected early and < half femoral head affected
• Oligohydramnios
§ Bed rest and traction
• More severe
Presentation § Maintain hip in abduction c̄ plaster
• Screening
§ Femoral or pelvic osteotomy
• Asymmetric skin folds
• Limp / abnormal gait

Ix
Slipped Capital Femoral Epiphysis
• Postero-inferior displacement of femoral head
• US is v. specific
epiphysis
• 10-15yrs
Mx: maintain abduction • Two main groups
• Double nappies § Fat and sexually underdeveloped
• Pavlik harness § Tall and thin
• Plaster hip spica
• Open reduction: derotation varus osteotomy Presentation
• Slip may be acute, chronic or acute-on-chronic
• Acute
Transient Synovitis: Irritable Hip § Groin pain
• Commonest cause of acute hip pain in children § Shortened, externally rotated leg
§ All movements painful
Presentation • 20% bilateral
• 2-12yrs
• Sudden onset hip pain / limp Ix
• Often following or with viral infection • Confirm Dx by x-ray
• Not systemically unwell
Mx
Ix • Acute: reduce and pin epiphysis
• PMN and ESR/CRP are normal • Chronic: in situ pinning
• -ve blood cultures § Epiphyseal reduction risks AVN
• May need joint aspiration and culture
Complications
Mx • Chondrolysis: breakdown of articular cartilage
• Rest and analgesia § ↑ risk c̄ surgery
• Settles over 2-3d

124 © Alasdair Scott, 2018

Acute Osteomyelitis Septic Arthritis
Pathophysiology Pathophysiology
• Source: local or haematogenous. • Source: local or haematogenous.
• Organisms • Organisms
§ Staph § Staph: 60%
§ Strep § Streps
§ E. coli § Gonococcus
§ Pseudomonas § Gm-ve bacilli
§ Salmonella (in SCD) • RFs
• RFs § Joint disease (e.g. RA)
§ Vascular disease § CRF
§ Trauma § Immunosuppression (e.g. DM)
§ SCD § Prosthetic joints
§ Immunosuppression (e.g. DM)
§ Children Symptoms
- Rich blood supply to growth plate
• Acutely inflamed tender, swollen joint.
- \ usually affects metaphysis
• ↓ROM
• Systemically unwell
Symptoms and Signs
• Pain, tenderness, erythema, warmth, ↓ROM Investigations
• Effusion in neighbouring joints
• Joint aspiration for MCS
• Signs of systemic infection 3
§ ↑↑ WCC (e.g. >50,000/mm ) : mostly PMN
• ↑ESR/CRP, ↑WCC, Blood cultures
Investigations • X-ray
• ↑ESR/CRP, ↑WCC
• +ve blood cultures in 60% Management
• X-ray: • IV Abx: vanc + cefotaxime
§ Changes take 10-14d
• Consider joint washout under GA
§ Haziness + ↓ bone density
• Splint joint
§ Sub-periosteal reaction
• Physiotherapy after infection resolved
§ Sequestrum and involucrum
• MRI is sensitive and specific
Complications
Management • Osteomyelitis
• Arthritis
• IV Abx: Vanc + cefotaxime until MCS known
• Ankylosis: fusion
• Drain abscess and remove sequestra
• Analgesia
Differential
• Crystal arthropathy
• Reactive arthritis

© Alasdair Scott, 2018 125

 Bone Tumours
Bony Mets
• Commonest bone tumours
• Bronchus, thyroid, breast, kidney and prostate
• Usually radiolucent (except prostate which is sclerotic)
• Usually axial skeleton (contains red marrow)
• Present with pain or pathological #
§ Path # → internal fixation
§ Pain → radiotherapy

Tumour-like Non-neoplastic Conditions

Lesion Clinical Features Location X-ray

Fibrous Dysplasia 0-30 Long bones “Ground Glass” lytic lesion
F>M Ribs
↑ # risk Skull Shepherds crook deformity of
prox. femur
McCune Albright’s Usually mono-ostotic
- Polyostotic fibrous dysplasia
- Precocious puberty (females)
- Café-au-lait spots
Simple bone cyst <20 Prox metaphysis of Well-defined lytic lesion
Lump migrates down shaft from humerus or femur Cortex can fall into cyst →
original growth plate site. “fallen fragment”
Aneurysmal bone cyst <30yrs MRI shows multiple fluid levels
Pain
Fibrous cortical defect Children Dist femur
and non-ossifying Non-painful, benign Prox tibia
fibroma Spontaneously regress Often multiple

Benign Cartilaginous Neoplasms

Lesion Clinical Features Location X-ray

7??
Osteochondroma
(exostosis)

Enchondroma /
Chondromas
10-20
M>F = 3:1
Commonest benign bone tumour
May be related to previous trauma
10-40
M=F
Knee

Hands
Feet
Cartilage-capped bony
outgrowth

O sign
- Oval lucencies c̄
radiodense rim
Ollier Disease = multiple enchondromas
Endosteal scalloping
Maffucci Syn = Enchondromatosis + multiple soft-
tissue haemangiomas

Multiple enchondromas have risk of malignant

transformation
Chondroblastoma 10-20 Epiphysis Radiolucent with sclerotic
M>F = 2:1 Knee border

Malignant Cartilaginous Neoplasms

Lesion Clinical Features Location X-ray

7?
Chondrosarcoma >40
Pain + lump
Arise de novo or from chondromas
70% 5ys
Pelvis
Axial skeleton
Lytic lesion
Fluffy “popcorn calcification”

126 © Alasdair Scott, 2018

Benign Bone-forming Neoplasms

Lesion Clinical Features Location X-ray

Osteoma Lump Skull
Usually solitary Facial bones
Multiple in Gardner syn
Osteoid osteoma M>F = 2:1 Lower limb Lytic lesion c̄ central nidus and sclerotic rim
Teens and 20s Diaphyseal cortex
Severe nocturnal pain relieved
by aspirin
Hot on bone scan
Osteoblastoma Pain unresponsive to aspirin Spine
Giant Cell Tumour 20-40 Knee Soap bubble appearance
/ Osteoclastoma (After fusion of growth plate) Abut joint surface Solitary, expansile, lytic lesion
F>M

Malignant Bone-forming Neoplasms

Lesion Clinical Features Location X-ray

Osteosarcoma Adolescents Knee Periosteal Elevation:
M>F = 2:1 Metaphysis - Sunburst appearance
O
Commonest 1 bone tumour - Codman’s triangle
Pain, warm, bruit
o
May arise 2 to Paget’s or irradiation
Ewing’s Sarcoma <20 Long bone diaphysis Lytic tumour
Painful, warm, enlarging mass Onion-skin periosteal
Systemic: fever, ↑ESR, anaemia, ↑WCC reaction

© Alasdair Scott, 2018 127

Brachial Plexus Injuries Other Nerve Injuries
Anatomy Radial Nerve (C5-T1)
• C5-T1
• Roots leave vertebral column between scalenus Low Lesions: posterior interosseous nerve
anterior and medius. • Site: # around elbow or forearm
• Divisions occur under the clavicle, medial to coracoid § E.g. # head of radius
process. • Loss of extension of CMC joints (finger drop)
• Plexus has intimate relationship c̄ subclavian and • No sensory loss
brachial arteries. Median N. is formed anterior to
brachial artery. High Lesions
• Site: # shaft of humerus where N. is in radial groove.
C5 • Wrist drop
Lat • Loss of sensation to dorsum of thumb root (snuff box)
MC
•

><
Triceps functions normally
C6
Post Axillary Median Very High Lesions
C7
• Site: axilla – e.g. crutches or Sat night palsy

/
Radial
C8 • Paralysis of triceps and wrist drop
Med Ulnar

T1 Ulnar Nerve (C8-T1)

Roots (5) Divisions (6)
Trunks (3) Cords (3)
Site
• Elbow: cubital tunnel
• Wrist: in Guyon’s Canal
Causes
• Direct: e.g. shoulder girdle #, penetrating or iatrogenic
injury Effects
• Indirect: e.g. avulsion or traction injuries • Intrinsic hand muscle paralysis → claw hand
• Ulnar paradox: lesion at elbow has less clawing as
th th
FDP is paralysed, decreasing flexion of 4 /5 digits.
Leffert Classification • Weakness of finger ad/abduction (interossei)
• Open
• Sensory loss over little finger
• Closed
§ Supraclavicular
§ Infraclavicular Tests
• Radiation-induced • Can’t cross fingers for luck
• Obstetric • Froment’s Sign: flexion of thumb IPJ when trying to
§ Upper hold onto paper held between thumb and finger.
§ Lower § Indicates weak adductor policis.
§ Mixed

High (C5/6): Erb’s Palsy Median Nerve (C5-T1)

• Abductors and external rotators paralysed
• Waiter’s tip position
• Loss of sensation in C5/6 dermatomes
Low (C8/T1): Klumpke’s Paralysis
• Paralysis of small hand muscles
• Claw hand
• Loss of sensation in C8/T1 dermatomes
Injury Above the Antecubital Fossa
• Can’t flex index finger IPJs (e.g. on clasping hands)
• Can’t flex terminal thumb phalanx (FPL)
• Loss of sensation in median distribution
Injury at the Wrist
• Typically affects abductor pollicis brevis

Carpal Tunnel Syndrome

128 © Alasdair Scott, 2018

 Carpal Tunnel Syndrome
Anatomy Ix
• Carpal tunnel formed by flexor retinaculum and carpal • Not usually performed
bones. • Nerve conduction studies
• Contains • US
§ 4 tendons of FDS
§ 4 tendons of FDP
Non-surgical Mx
§ 1 tendon of FPL
• Mx of underlying cause
§ Median N.
• Wrist splints
• Median N. supplies LLOAF (aBductor pollicis brevis)
§ Neutral position
• Palmer cutaneous branch travels superficial to flexor
§ Esp. @ night
retinaculum → spares sensation over thenar area.
• Local steroid injections

Surgical Mx
Causes • Carpal tunnel decompression by division of the flexor
• F>M retinaculum
• Primary / idiopathic
• Secondary Complications
• Water: pregnancy, hypothyroidism
• Scar formation: high risk for hypertrophic or keloid
• Radial #
• Scar tenderness: up to 40%
• Inflammation: RA, gout
• Nerve injury
• Soft tissue swelling: lipomas, acromegaly, amyloidosis § Palmar cutaneous branch of the median nerve
• Toxic: DM, EtOH § Motor branch to the thenar muscles
• Failure to relieve symptoms

Symptoms
• Tingling / pain in thumb, index and middle fingers Other Locations of Median Nerve Entrapment
• Pain worse @ night or after repetitive actions • Pronator syndrome
• Relieved by shaking / flicking Entrapment between two heads of
• Clumsiness pronator teres
• Anterior interroseous syndrome
§ Compression of the anterior interosseous
Signs branch by the deep head of pronator teres
• ↓ sensation over lateral 3½ fingers § Muscle weakness only
• ↓ 2-point touch discrimination § Pronator quadratus
§ Early sign of irreversible damage § FPL
• Wasting of thenar eminence § Radial half of FDP
§ Late sign of irreversible damage
• Phalen’s flexing and Tinel’s tapping

© Alasdair Scott, 2018 129

 Minor Hand Conditions
Dupuytren’s Contracture Trigger Finger
• Progressive, painless fibrotic thickening of palmar • Tendon nodule which catches on proximal side of
fascia. tendon sheath → triggering on forced extension.
• → Fixed flexion deformity
The Patient • Usually ring and middle fingers
• M>F • Assoc. c̄ RA
• Middle age / elderly • Rx: steroid injection (high recurrence) or surgery
• Skin puckering and tethering
• Fixed flexion contracture of ring and little fingers
• Often bilateral and symmetrical Ganglion
• MCP and IP joint flexion
• Smooth, multilocular cystic swellings
• Mucoid degeneration of joint capsule or tendon sheath
Associations: BAD FIBERS • May be in communication c̄ joint capsules / tendons
• Bent penis: Peyronies (3%)
• AIDS
Presentation
• DM
• 90% located on dorsum of wrist.
• FH: AD
• Subdermal, fixed to deeper structures.
• Idiopathic: commonest § Limits planes of movement
• Booze: ALD • May cause pain or nerve pressure symptoms
• Epilepsy and epilepsy meds (phenytoin)
• Reidel’s thyroiditis and other fibromatoses
Management
§ Ledderhose disease
• 50% disappear spontaneously
- Fibrosis of plantar aponeurosis
• Aspiration ± steroid and hyaluronidase injection
- 5% c̄ dupuytren’s
• Surgical excision
§ Retroperitoneal fibrosis
• Smoking
Differential
• Lipoma
Management
• Fibroma
• Conservative: e.g. physio / exercises
• Sebaceous cyst
• Fasciectomy
§ e.g. when hand can’t be placed flat on the
table.
§ Z-shaped scars: prevent contracture
§ Can damage ulnar nerve
§ Usually recurs

Differential
• Skin contracture: old laceration or burn
• Tendon fibrosis, trigger finger
• Ulnar N. palsy

130 © Alasdair Scott, 2018

 Minor Leg and Foot Conditions
Meralgia Paraesthetica Hallux Valgus
• Entrapment of lat. cutaneous nerve of thigh • Great toe deviates laterally @ MTP joint
§ Between ASIS and inguinal ligament • Pressure of MTP against shoe → bunion
nd
• Pain ± paraesthesia on the lateral thigh • ↑ wt. bearing @ 2 metatarsal head
• No motor deficit § → pain: “Transfer metatarsalgia”
• ↑ risk c̄ obesity: compression by belts, underwear § → hammer toe
§ Relieved by sitting down
• Can occasionally be damaged in lap hernia repair Aetiology
• Pointed shoes
• Wearing high heals
Chondromalacia Patellae
• Predominantly young women Mx
• Patellar aching after prolonged sitting or climbing • Conservative: bunion pads, plastic wedge between
stairs great and second toes.
• Pain on patellofemoral compression: Clarke’s test • Surgical: metatarsal osteotomy
• Ix: no abnormality on X-ray
• Rx: vastus medialis strengthening
Lesser Toe Deformities

Baker’s Cyst 3 Hammer toe

• Popliteal swelling arising between the medial head of
gastrocnemius and semimembranosus muscle
• Herniation from joint synovium
O
• Usually 2 to OA \
• Rupture: acute calf pain and swelling
§ DVT differential
'\ Claw toe

l 4

Mallet toe

\
/F<‘—~—-
l/"

Morton’s Metatarsalgia / Neuroma

• Pain from pressure on an interdigital neuroma
between the metatarsals.
• Pain radiates to medial side of one toe and lateral side
of another.
• Rx: neuroma excision

© Alasdair Scott, 2018 131

Audiometry Otalgia
• Quantify loss and determine its nature.
Otitis Externa

Pure tone audiometry (PTA) Presentation

• Headphones deliver tones at different • Watery discharge
frequencies and strengths in a sound-proofed • Itch
room. • Pain and tragal tenderness
• Pt. indicates when sound appears and
disappears. Causes
• Mastoid vibrator → bone conduction threshold. • Moisture: e.g. swimming
• Threshold at different frequencies are plotted to • Trauma: e.g. fingernails
give an audiogram. • Absence of wax
• Hearing aid

Tympanometry Organisms
• Measures stiffness of ear drum • Mainly pseudomonas
- Evaluates middle ear function • Staph aureus
• Flat tympanogram: mid ear fluid or perforation
• Shifted tympanogram: +/- mid ear pressure Management
• Aural toilet c̄ drops
§ Betamethasone for non-infected eczematous OE
Evoked response audiometry § Betamethasone c̄ neomycin
• Auditory stimulus c̄ measurement of elicited § Hydrocortisone c̄ gentamicin
brain response by surface electrode. § Acidifying drops
• Used for neonatal screening (if otoacoustic
emission testing negative)
Malignant Otitis Externa
• Life-threatening infection which can → skull osteomyelitis
• 90% of pts. are diabetic (or other immune compromise)
• Presentation
§ Severe otalgia which is worse @ night
§ Copious otorrhoea
§ Granulation tissue in the canal
• Rx
§ Surgical debridement
§ Systemic Abx

Bullous Myringitis
• Painful haemorrhagic blisters on deep meatal skin and TM.
• Assoc. c̄ influenza infection

TMJ Dysfunction

Symptoms
• Earache (referred pain from auriculotemporal N.)
• Facial pain
• Joint clicking/popping
• Teeth-grinding (bruxism)
• Stress (assoc. c̄ depression)

Signs
• Joint tenderness exacerbated by lateral movements of an
open jaw.

Investigation
• MRI

Management
• NSAIDs
• Stabilising orthodontic occlusal prostheses

© Alasdair Scott, 2018 133

 Otitis Media
Classification Chronic Suppurative OM
• Acute: acute phase
• Glue ear / OME: effusion after symptom regression Presentation
• Chronic: effusion > 3mo if bilat or > 6mo if unilat • Painless discharge and hearing loss
• Chronic suppurative OM: Ear discharge c̄ hearing
loss and evidence of central drum perforation. o/e
• TM perforation
Organisms
• Viral Rx
• Pneumococcus • Aural toilet
• Haemophilus • Abx / Steroid ear drops
• Moraxella
Complications
• Cholesteatoma
Acute OM

Presentation Mastoiditis
• Usually children post viral URTI • Middle-ear inflam → destruction of mastoid air cells and
• Rapid onset ear pain, tugging @ ear. abscess formation.
• Irritability, anorexia, vomiting
• Purulent discharge if drum perforates Presentation
• Fever
o/e • Mastoid tenderness
• Bulging, red TM • Protruding auricle
• Fever
Imaging: CT
Rx
• Paracetamol: 15mg/kg Rx
• Amoxicillin: may use delayed prescription • IV Abx
• Myringotomy ± mastoidectomy
Complications
• Intratemporal
§ OME
§ Perforation of TM
§ Mastoiditis
§ Facial N. palsy
• Intracranial
§ Meningitis / encephalitis
§ Brain abscess
§ Sub- / epi-dural abscess
• Systemic
§ Bacteraemia
§ Septic arthritis
§ IE

OME

Presentation
• Inattention at school
• Poor speech development
• Hearing impairment

o/e
• Retracted dull TM
• Fluid level

Ix
• Audiometry: flat tympanogram

Rx
• Usually resolves spontaneously,
• Consider grommets if persistent hearing loss
§ SE: infections and tympanosclerosis

134 © Alasdair Scott, 2018

Cholesteatoma Tinnitus
• Sensation of sound w/o external sound stimulation
Definition
• Locally destructive expansion of stratified Causes
squamous epithelium within the middle ear. • Specific
§ Meniere’s
Classification § Acoustic neuroma
• Congenital § Otosclerosis
•
O
Acquired: 2 to attic perforation in chronic § Noise-induced
suppurative OM § Head injury
§ Hearing loss: e.g. presbyacusis
• General
Presentation § ↑BP
• Foul smelling white discharge § ↓Hb
• Headache, pain • Drugs
• CN Involvement § Aspirin
§ Vertigo § Aminoglycosides
§ Deafness § Loop diuretics
§ Facial paralysis § EtOH

o/e Hx
• Appears pearly white c̄ surrounding inflammation • Character: constant, pulsatile
• Unilateral: acoustic neuroma
Complications • FH: otosclerosis
• Deafness (ossicle destruction) • Alleviating/exacerbating factors: worse @ night?
• Meningitis • Associations
• Cerebral abscess § Vertigo: Meniere’s, acoustic neuroma
§ Deafness: Meniere’s, acoustic neuroma
Mx • Cause: head injury, noise, drugs, FH
• Surgery
Examination
• Otoscopy
• Tuning fork tests
• Pulse and BP

Ix
• Audiometry and tympanogram
• MRI if unilateral to exclude acoustic neuroma

Mx
• Treat any underlying causes
• Psych support: tinnitus retraining therapy
• Hypnotics @ night may help

© Alasdair Scott, 2018 135

 Vertigo
Definition Ménière’s Disease
• The illusion of movement
Pathology
• Dilatation of endolymph spaces of membranous
Causes labyrinth (endolymphatic oedema)

Peripheral / Vestibular Central Presentation

• Meniere’s • Acoustic neuroma • Attacks occur in clusters and last up to 12h.
• BPV • MS • Progressive SNHL
• Labyrinthitis • Vertebrobasilar • Vertigo and n/v
insufficiency / stroke • Tinnitus
Head injury • Aural fullness
• Inner ear syphilis
Ix
Drugs (central/ototoxic) • Audiometry shows low-freq SNHL which fluctuates
• Gentamicin
• Loop diuretics Rx
• Metronidazole • Medical
• Co-trimoxazole § Vertigo: cyclizine, betahistine
• Surgical
§ Gentamicin instillation via grommets
§ Saccus decompression
Hx
• Is it true vertigo or just light-headedness?
§ Which way are things moving?
• Timespan Vestibular Neuronitis / Viral Labyrinthitis
• Assoc. symptoms: n/v, hearing loss, tinnitus,
nystagmus Presentation
• Follows febrile illness (e.g. URTI)
• Sudden vomiting
• Severe vertigo exacerbated by head movement
Examination and Tests
• Hearing
• Cranial nerves Rx
• Cerebellum and gait • Cyclizine
• Romberg’s +ve = vestibular or proprioception • Improvement in days
• Hallpike manouvre
• Audiometry, calorimetry, LP, MRI
Benign Positional Vertigo: BPV

Pathology
• Displacement of otoliths in semicircular canals
• Common after head injury.

Presentation
• Sudden rotational vertigo for <30s
- Provoked by head turning
• Nystagmus

Causes
• Idiopathic
• Head injury
• Otosclerosis
• Post-viral

Dx
• Hallpike manoeuvre → upbeat-torsional nystagmus

Rx
• Self-limiting
• Epley manoeuvre
• Betahistine: histamine analogue

136 © Alasdair Scott, 2018

 Adult Hearing Loss
Conductive Acoustic Neuroma / Vestibular Schwannoma
• Impaired conduction anywhere between auricle and
round window. Pathology
• Benign, slow-growing tumour of superior vestibular N.
External canal obstruction • Acts as SOL → Cerebellopontine angle syndrome
• Wax § 80% of CPA tumours
• Pus • Assoc. c̄ NF2
• Foreign body
Presentation
TM perforation • Slow onset, unilat SNHL, tinnitus ± vertigo
• Trauma • Headache (↑ICP)
• Infection • CN palsies: 5,7 and 8
• Cerebellar signs
Ossicle defects
• Otosclerosis Ix
• Infection • MRI of cerebellopontine angle
• Trauma § MRI all pts. c̄ unilateral tinnitus / deafness
• PTA
Inadequate eustachian tube ventilation of middle
ear Differential
• Meningioma
• Cerebellar astrocytoma
Sensorineural • Mets
• Defects of cochlea, cohlear N. or brain.
Rx
Drugs • Gamma knife
• Aminoglycosides • Surgery (risk of hearing loss)
• Vancomycin

Post-infective Otosclerosis
• Meningitis • AD condition characterised by fixation of stapes at the
• Measles oval window.
• Mumps • F>M=2:1
• Herpes
Presentation
Misc. • Begins in early adult life
• Meniere’s • Bilateral conductive deafness + tinnitus
• Trauma • HL improved in noisy places: Willis’ paracousis
• MS • Worsened by pregnancy/ menstruation/ menopause
• CPA lesion (e.g. acoustic neuroma)
• ↓B12 Ix
• PTA shows dip (Caharts notch) @ 2kHz

Rx
• Hearing aid or stapes implant

Presbyacussis
• Age-related hearing loss

Presentation
• >65yrs
• Bilateral
• Slow onset
• ± tinnitus

Ix: PTA

Rx: hearing aid

© Alasdair Scott, 2018 137

Hearing Loss in Children Miscellaneous Ear Conditions
Congenital Causes Congenital Anomalies
st nd st
• 1 and 2 branchial arches form auricle while 1
Conductive branchial groove forms external auditory canal.
• Anomalies of pinna, external auditory canal, TM or • Malfusion → accessory tags/auricles and preauricular
ossicles. pits, fistulae or sinuses.
• Congenital cholesteatoma • Sinuses may get infected, mimicking a sebaceous cyst.
• Pierre-Robin

Pinna Haematoma
SNHL • Blunt trauma → subperichondrial haematoma.
• Autosomal Dominant • Can → ischaemic necrosis of cartilage and subsequent
§ Waardenburgs: SNHL, heterochromia + fibrosis to “cauliflower ears”.
telecanthus • Mx: aspiration + firm packing to auricle contour.
• Autosomal recessive
§ Alport’s: SNHL + haematuria
§ Jewell-Lange-Nielson: SNHL + long QT
• X-linked
Exostoses
§ Alport’s • Smooth, symmetrical bony narrowing of external canals.
• Infections: CMV, rubella, HSV, toxo, GBS
• Ototoxic drugs Path
• Bony hypertrophy due to cold exposure
• e.g. from swimming / surfing
Perinatal
• Anoxia
Symptoms
• Asympto unless narrowing occludes → conductive
• Cerebral palsy
deafness.
• Kernicterus
• Infection: meningitis
Rx: conservative or surgical widening

Acquired Causes
Wax: Cerumen Auris
• OM/OME rd
• Secreted in outer 3 of canal to prevent maceration
• Infection: meningitis, measles
• Wax accumulation can → conductive deafness.
• Head injury
• Mx
§ Suction under direct vision c̄ microscope
§ Syringing after 1wk softening with olive oil
Universal Neonatal Hearing Tests
• Detection and Mx of hearing loss before 6mo
improves language.
• Tests TM Perforation
§ Otoacoustic emissions
§ Audiological brainstem responses. Causes
• OM
• Foreign body
• Barotrauma
• Trauma

138 © Alasdair Scott, 2018

Allergic Rhinosinusitis Sinusitis
Classification Pathophysiology
• Seasonal: hay-fever (prev = 2%) • Viruses → mucosal oedema and ↓ mucosal ciliary
O
• Perennial actions → mucus retention ± 2 bacterial infection
• Acute: Pneumococcus, Haemophilus, Moraxella
Pathology • Chronic: S. aureus, anaerobes
• T1HS IgE-mediated inflam from allergen exposure
→ mediator release from mast cells. Causes
O
• Allergens: pollen, house dust mites (perennial) • Majority are bacterial infection 2 to viral
O
• 5% 2 to dental root infections
Symptoms • Diving / swimming in infected water
• Sneezing • Anatomical susceptibility: deviated septum, polyps
• Pruritus • Systemic Disease
• Rhinorrhoea § PCD / Kartagener’s
§ Immunodeficiency
Signs
• Swollen, pale and boggy turbinates Symptoms
• Nasal polyps • Pain
§ Maxillary (cheek/teeth)
§ Ethmoidal (between eyes)
Ix § ↑ on bending / straining
• Skin-prick testing to find allergens • Discharge: from nose → post-nasal drip c̄ foul taste
- Don’t perform if prone to eczema • Nasal obstruction / congestion
• RAST tests
• Anosmia or cacosmia (bad smell w/o external source)
• Systemic symptoms: e.g. fever
Mx
Imaging
Allergen Avoidance • Nasendoscopy ± CT
• Regularly washing bedding (inc. toys) on high
heat or use acaricides.
• Avoid going outside when pollen count high. Mx

st
1 Line
• Anti-histamines: cetirazine, desloratidine
• Or, beclometasone nasal spray
• Or, chromoglycate nasal spray (children)
Acute / Single Episode
• Bed-rest, decongestants, analgesia
• Nasal douching and topical steroids
• Abx (e.g. clarithro) of uncertain benefit

2
nd
Line: intranasal steroids + anti-histamines Chronic / recurrent
• Usually a structural or drainage problem.
rd
3 Line: Zafirlukast • Stop smoking + fluticasone nasal spray
• Functional Endoscopic Sinus Surgery
rd - If failed medical therapy
4 Line: Immunotherapy
• Aim to induce desensitisation to allergen
• OD SL grass-pollen tablets → ↑ QOL in hay-fever Complications (rare)
• Injection immunotherapy • Mucoceles → pyoceles
• Orbital cellulits / abscess
Adjuvants • Osteomyelits – e.g. Staph in frontal bone
• Intracranial infection
• Nasal decongestants: Pseudoephedrine,
§ Meningitis, encephalitis
Otrivine
§ Abscess
§ Cavernous sinus thrombosis

© Alasdair Scott, 2018 139

Nasal Polyps Fractured Nose
The Patient Anatomy
rd
• Male, > 40yrs • Upper 3 of nose has bony support
• Lower 2/3 and septum are cartilaginous.
Sites
• Middle turbinates Hx
• Middle meatus • Time of injury
• Ethmoids • LOC
• CSF rhinorrhoea
Symptoms • Epistaxis
• Watery, anterior rhinorrhoea • Previous nose injury
• Purulent post-nasal drip • Obstruction
• Nasal obstruction • Consider facial #, check for
• Sinusitis § Teeth malocclusion
• Headaches § Diplopia (orbital floor #)
• Snoring
Ix
Signs • Cartilaginous injury won’t show and radiographs don’t
• Mobile, pale, insensitive alter Mx.

Associations Mx
• Allergic / non-allergic rhinitis • Exclude septal haematoma
• CF • Re-examine after 1wk (↓ swelling)
• Aspirin hypersensitivity • Reduction under GA c̄ post-op splinting best w/i 2wks
• Asthma
Septal haematoma
Single Unilateral Polyp • Septal necrosis + nasal collapse if untreated
• May be sign of rare but sinister pathology § Cartilage blood supply comes from mucosa
§ Nasopharyngeal Ca • Boggy swelling and nasal obstruction
§ Glioma • Needs evacuation under GA c̄ packing ± suturing.
§ Lymphoma
§ Neuroblastoma
§ Sarcoma
• Do CT and get histology

Nasal Polyps in Children

• Rare <10yrs old
• Must consider neoplasms and CF

Mx
• Drugs
§ Betamethasone drops for 2/7
§ Short course of oral steroids
• Endoscopic polypectomy

140 © Alasdair Scott, 2018

 Epistaxis
Causes Posterior / Major Epistaxis
• 80% unknown • Posterior packing (+ anterior pack)
• Trauma: nose-picking / #s § Pass 18/18G Foley catheter through the nose
• Local infection: URTI into nasopharynx, inflate c̄ 10ml water and pull
• Pyogenic granuloma forward until it lodges.
§ Overgrowth of tissue on Little’s area due to § Admit pt. and leave pack for ~48hrs.
irritation or hormonal factors. • Gold standard is endoscopic visualisation and direct
• Osler-Weber-Rendu / HHT control: e.g. by cautery or ligation.
• Coagulopathy: Warfarin, NSAIDs, haemophilia, ↓plats,
vWD, ↑EtOH After the Bleed
• Neoplasm • Don’t pick nose
• Sit upright, out of the sun
Classification • Avoid bending, lifting or straining
• Anterior • Sneeze through mouth
• Posterior • No hot food or drink
• Avoid EtOH and tobacco
Initial Mx
• Wear PPE
• Assess for shock and manage accordingly
• If not shocked Osler-Weber-Rendu / HHT
§ Sit up, head tilted down • Autosomal dominant
§ Compress nasal cartilage for 15min. • 5 genetic subtypes
• If bleeding not controlled remove clots c̄ suction or by
blowing and try to visualise bleed by rhinoscopy Features
• Telangiectasias in mucosae
Anterior Epistaxis § Recurrent spontaneous epistaxis
• Usually septal haemorrhage: Little’s area / § GI bleed (usually painless)
Kisselbach’s plexus • Internal telangiectasias and AVMs
§ Ant. Ethmoidal A. § Lungs
§ Sphenopalatine A. § Liver
§ Facial A. § Brain
• Insert gauze soaked in vasoconstrictor + LA • Rarely
§ Xylometazoline + 2% lignocaine § Pulmonary HTN
§ 5min § Colon polyps: may → CRC
• Bleeds can be cauterised c̄ silver nitrate sticks
• Persistent bleeds should be packed with Mericel pack
§ Refer to ENT if this fails or if you can’t visualise
the bleeding point.
§ They may insert a posterior pack or take pt. to
theatre for endoscopic control.

© Alasdair Scott, 2018 141

 Tonsillitis
Symptoms Strep Throat Complications
• Sore throat
• Fever, malaise Peritonsillar Abscess (Quinsy)
• Typically occurs in adults
Signs • Symptoms
• Lymphadenopathy: esp. jugulodigastric node § Trismus
• Inflamed tonsils and oropharynx § Odonophagia: unable to swallow saliva
• Exudates § Halitosis
• Signs
§ Tonsillitis
Organisms § Unilateral tonsillar enlargement
• Viruses are most common (consider EBV) § Contralateral uvula displacement
• Group A Strep: pyogenes § Cervical lymphadenopathy
• Staphs • Rx
• Moraxella § Admit
§ IV Abx
Mx § I&D under LA or tonsillectomy under GA
• Swabbing superficial bacteria is irrelevant and can
→ overdiagnosis.
• Analgesia: Ibuprofen / Paracetamol ± Difflam Retropharyngeal Abscess
gargle • Rare
• Consider Abx only if ill: use Centor Criteria • Presentation
§ Pen V 250mg PO QDS (125mg TDS in § Unwell child c̄ stiff, extended neck who refuses to
children) or erythromycin for 5/7 eat or drink
• NOT AMOXICILLIN → MACPAP RASH IN EBV § Fails to improve c̄ IV Abx
§ Unilateral swelling of tonsil and neck
Centor Criteria • Ix
• Guideline for admin of Abx in acute sore throat / § Lat. neck x-rays show soft tissue swelling
tonsillitis / pharyngitis § CT from skull-base to diaphragm.
• Rx
1 Point for each of § IV Abx
• Hx of fever § I&D
• Tonsillar exudates
• Tender anterior cervical adenopathy
• No cough Lemierre’s Syndrome
• IJV thrombophlebitis c̄ septic embolization most
Mx commonly affecting the lungs.
• 0-1: no Abx (risk of strep infection <10%) • Organism: Fusobacterium necrophorum
• 2: consider rapid Ag test + Rx if +ve • Rx
• ≥3: Abx § IV Abx: pen G, clinda, metro

Tonsillectomy Scarlet Fever

• “Sandpaper”-like rash on chest, axillae or behind ears
12-48h after pharyngotonsillitis.
Indications
• Circumoral pallor
• Recurrent tonsillitis if all the below criteria are met
• Strawberry tongue
§ Caused by tonsillitis
• Rx
§ 5+ episodes/yr
§ Start Pen V/G and notify HPA.
§ Symptoms for >1yr
§ Episodes are disabling and prevent normal
functioning
• Airway obstruction: e.g. OSA in children Rheumatic Fever
• Quinsy • Carditis
• Suspicion of Ca: unilateral enlargement or • Arthritis
ulceration • Subcutaneous nodules
• Erythema marginatum
Methods • Sydenham’s chorea
• Cold steel
• Cautery
Post-streptococcal Glomerulonephritis
Complications • Malaise and smoky urine 1-2wks after a pharyngitis
• Reactive haemorrhage
• Tonsillar gag may damage teeth, TMJ or posterior
pharyngeal wall.
• Mortality is 1/30,000

142 © Alasdair Scott, 2018

The Larynx Paediatric Airway Issues
Functions Laryngomalacia
• Phonation • Immature and floppy aryepiglottic folds and glottis →
• Positive thoracic pressure: inc. auto-PEEP laryngeal collapse on inspiration
• Respiration
• Prevention of aspiration Presentation
• Stridor: commonest cause in children
Laryngitis § Presents w/i first wks of life.
• Usually viral and self-limiting • Noticeable @ certain times
•
O
2 bacterial infection may develop § Lying on back,
• Symptoms: pain, hoarseness and fever § Feeding
• o/e: redness and swelling of the vocal cords § Excited/upset
• Rx: Supportive, Pen V if necessary • Problems can occur c̄ concurrent laryngeal infections or
c̄ feeding.
Laryngeal Papilloma
•
O
Pedunculated vocal cord swellings 2 to HPV Mx
• Present c̄ hoarseness • Usually no Rx required but severe cases may warrant
surgery.
• Usually occur in children
• Rx: laser removal

Recurrent Laryngeal N. Palsy Epiglottitis

• Supplies all intrinsic laryngeal muscles except for
cricothyroideus. Symptoms
§ Ext. branch of sup laryngeal N. • Sudden onset
• Responsible for ab- and ad-uction of vocal folds • Continuous stridor
• Drooling
Symptoms • Toxic
• Hoarseness
• “Breathy” voice c̄ bovine cough Pathogens: haemophilus, Group A Strep
• Repeated coughing from aspiration (↓ supraglottic
sensation) Rx
• Exertional dyspnoea (narrow glottis) • Don’t examine throat
• Consult c̄ anaesthetists and ENT surgeons
Causes • O2 + nebulised adrenaline
• 30% are cancers: larynx, thyroid, oesophagus, • IV dexamethasone
hypopharynx, bronchus • Cefotaxime
• 25% iatrogenic: para- / thyroidectomy, carotid • Take to theatre to secure airway by intubation
endarterectomy
• Other: aortic aneurysm, bulbar / pseudobulbar
palsy Foreign Body
• Sudden onset stridor in a previously normal child.
Laryngeal SCC • Back slaps and abdominal thrusts.
• Incidence: 2000/yr in uk • Needle cricothyrotomy in children
• Associations: smoking, EtOH • Can only exclude foreign body in bronchus by
bronchoscopy
Presentation
• Male smoker
• Progressive hoarseness → stridor Subglottic Stenosis
• Dys-/odono-phagia • Subglottis is narrowest part of respiratory tract in
• Wt. loss children.
• Symptoms: stridor, FTT
Ix • Causes
• Laryngoscopy + biopsy (inc. nodes) § Prolonged intubation
• MRI staging § Congenital abnormalities
• Rx
Mx § Mild: conservative
• Based on stage § Severe: Tracheostomy or partial tracheal
• Radiotherapy resection
• Laryngectomy

After total laryngectomy

• Pts have permanent tracheostomy
§ Speech valve
§ Electrolarynx
§ Oesophageal speech (swallowed air)
• Regular f/up for recurrence

© Alasdair Scott, 2018 143

 Facial Nerve Palsy
Bell’s Palsy Other Causes of Facial Palsy
• Inflammatory oedema from entrapment of CNVII in
narrow facial canal May be suggested by
• Probably of viral origin (HSV1). • Bilateral symptoms (Lyme, GBS, leukaemia,
• 75% of facial palsy sarcoid)
• UMN signs: sparing of frontalis and orbicularis oculi
Features • Other CN palsies (but seen in 8% of Bell’s)
• Sudden onset: e.g. overnight • Limb weakness
• Complete, unilateral facial weakness in 24-72h • Rashes
§ Failure of eye closure (Bell’s Sign) →
dryness and conjunctivitis Intracranial Lesions
§ Drooling, speech difficulty • Vascular, MS, SOL
• Numbness or pain around ear § Motor cortex → UMN signs
• ↓ taste (ageusia) § Brainstem nuclei → LMN signs
• Hyperacusis: stapedius palsy • Cerebellopontine angle lesion
th th th
§ May be accompanied by 5 , 6 , and 8 CN
Ix palsies
• Serology: Borrelia or VZV Abs
• MRI: SOL, stroke, MS Intratemporal Lesions
• LP • Otitis media
• Cholesteatoma
Mx • Ramsay Hunt Syndrome
• Protect eye
§ Dark glasses Infratemporal
§ Artificial tears • Parotid tumours
§ Tape closed @ night • Trauma
• Give prednisolone w/i 72hrs
§ 60mg/d PO for 5/7 followed by tapering Systemic
• Valaciclovir if zoster suspected (otherwise antivirals • Peripheral neuropathy
don’t help). § Demyelinating: GBS
• Plastic surgery may help if no recovery § Axonal: DM, Lyme, HIV, Sarcoid
• Pseudopalsy: MG, botulism
Prognosis
• Incomplete paralysis usually recovers completely
w/i wks.
• With complete lesions, 80% get full recovery but
the remainder have delayed recovery or permanent
neurological / cosmetic abnormalities.

Complications: Aberrant Neural Connections

• Synkinesis: e.g. blinking causes up-turning of
mouth
• Crocodile tears: eating stimulates unilateral
lacrimation, not salivation

Ramsay Hunt Syndrome

• American neurologist James Ramsay Hunt in 1907
• Reactivation of VZV in geniculate ganglion of CNVII

Features
• Preceding ear pain or stiff neck
• Vesicular rash in auditory canal ± TM, pinna,
tongue, hard palate (no rash = zoster sine herpete)
• Ipsilateral facial weakness, ageusia, hyperacusis,
• May affect CN7 → vertigo, tinnitus, deafness

Mx
• If Dx suspected give valaciclovir and prednisolone
w/i first 72h

Prognosis
• Rxed w/i 72h: 75% recovery
• Otherwise: 1/3 full recovery, 1/3 partial, 1/3 poor

144 © Alasdair Scott, 2018

 Pupils
Afferent Defect Horner’s Syndrome
• Johann Horner (1831-1886), Swiss ophthalmologist
Features
• No direct response but intact consensual response Features: PEAS
• Cannot initiate consensual response in contralateral • Ptosis: partial (superior tarsal muscle)
eye. • Enophthalmos
• Dilatation on moving light from normal to abnormal eye • Anhydrosis
• Small pupil
Causes
• Total CN II lesion Causes
• Central
Relative Afferent Pupillary Defect § MS
§ Wallenberg’s Lateral Medullary Syndrome
• = Marcus-Gunn Pupil
• Pre-ganglionic (neck)
§ Pancoast’s tumour: T1 nerve root lesion
Features § Trauma: CVA insertion or CEA
• Minor constriction to direct light • Post-ganglionic
• Dilatation on moving light from normal to abnormal eye. § Cavernous sinus thrombosis
• RAPD = Marcus Gunn Pupil O
§ Usually 2 to spreading facial infection via the
ophthalmic veins
Causes § CN 3, 4, 5, 6 palsies
• Optic neuritis
• Optic atrophy
• Retinal disease Argyll Robertson Pupil
Efferent Defect Features
• Small, irregular pupils
Feature • Accommodate but doesn’t react to light
• Dilated pupil does not react to light • Atrophied and depigmented iris
• Initiates consensual response in contralateral pupil
• Ophthalmoplegia + ptosis Cause
• DM
Cause • Quaternary syphilis
rd
• 3 nerve palsy
§ The pupil is often spared in a vascular lesion
(e.g. DM) as pupillary fibres run in the periphery.

Differential of a fixed dilated pupil

• Mydriatics: e.g. tropicamide
• Iris trauma
• Acute glaucoma
• CN3 compression: tumour, coning

Holmes-Adie Pupil

Features
• Young woman c̄ sudden blurring of near vision
• Initially unilateral and then bilateral pupil dilatation
§ Dilated pupil has no response to light and
sluggish response to accommodation.
§ A “tonic” pupil

Ix
• Iris shows spontaneous wormy movements on slit-lamp
examination
§ Iris streaming

Cause
• Damage to postganglionic parasympathetic fibres
• Idiopathic: may have viral origin

Holmes-Adie Syndrome
• Tonic pupil + absent knee/ankle jerks + ↓ BP

146 © Alasdair Scott, 2018

Optic Atrophy / Optic Neuropathy
Features
• ↓ acuity
• ↓colour vision (esp. red)
• Central scotoma
• Pale optic disc
• RAPD

Causes: CAC VISION

• Commonest: MS and glaucoma

Congenital
• Leber’s hereditary optic neuropathy
§ Epi: mitochondrial, onset 20-30s
§ PC: attacks of acute visual loss, sequential in
each eye ± ataxia and cardiac defects
• HMSN / CMT
• Friedrich’s ataxia
• DIDMOAD
• Retinitis pigmentosa

Alcohol and Other Toxins

• Ethambutol
• Lead
• B12 deficiency

Compression
• Neoplasia: optic glioma, pituitary adenoma
• Glaucoma
• Paget’s

Vascular: DM, GCA or thromboembolic

Inflammatory: optic neuritis – MS, Devic’s, DM
Sarcoid / other granulomatous
Infection: herpes zoster, TB, syphilis
Oedema: papilloedema
Neoplastic infiltration: lymphoma, leukaemia

© Alasdair Scott, 2018 147

 Red Eye: History, Examination and Differential
Visual History Differential

Site Mechanical Inflammation Infection

Vision
• Blurred
Lids Ectropion Blepharitis Preseptal cellulitis
Entropion Chalazion Orbital cellulitis
• Distorted
FB / Trichiasis
• Diplopia
Conjunctiva Sub conj haem Allergic conjunct Conjunctivitis
• Field defect / Scotoma
• Floaters, Flashes
Sclera Perforation Chemical burn
Episcleritis
Scleritis
Sensation Cornea Foreign body Keratitis
• Irritation Abrasion
• Pain Ant Chamber Acute glaucoma Iritis / uveitis Endophthalmitis
• Itching
• Photophobia
• FB
Presentation Summary
Appearance Acute Anterior Conjunctivitis
• Red: ?distribution
Glaucoma Uveitis
• Lump
• Puffy lids
Pain +++ ++ +/-
Photophobia ++ +
Discharge Acuity ↓ ↓ normal
• Watering Cornea hazy/cloudy normal normal
• Sticky Pupil large small normal
• Stringy IOP ↑↑ normal normal

Key Examination Questions

• Inspect from anterior to posterior
• Is acuity affected?
• Is the globe painful?
• Pupil size and reactivity?
• Cornea: intact, cloudy? Use fluorescein

Signs of Serious Disease

• Photophobia
• Poor vision
• Corneal fluorescein staining
• Abnormal pupil

148 © Alasdair Scott, 2018

 Red Eyes: Specific Diseases
Acute Closed Angle Glaucoma Anterior Uveitis / Acute Iritis
• Blocked drainage of aqueous from anterior chamber
via the canal of Schlemm. Pathophysiology
• Pupil dilatation (e.g. @ night) worsens the blockage. • Uvea is pigmented part of eye and included: iris,
• Intraocular pressure rises from 15-20 → >60mmHg ciliary body and choroid.
• Iris + ciliary body = anterior uvea
Risk Factors • Iris inflammation involves ciliary body too.
• Hypermetropia
• Shallow ant. chamber Symptoms
• Female • Acute pain and photophobia
• FH • Blurred vision (aqueous precipitates)
• ↑age
• Drugs Examination
§ Anti-cholinergics • Small pupil initially, irregular later
§ Sympathomimetics • Circumcorneal injection
§ TCAs • Hypopyon: pus in anterior chamber
§ Anti-histamines • White (keratic) precipitates on back of cornea
• Talbots test: ↑pain on convergence
Symptoms
• Prodrome: rainbow haloes around lights at night-time. Associations (most have no systemic associations)
• Severe pain c̄ n/v
• Seronegative arthritis: AS, psoriatic, Reiter’s
• ↓ acuity and blurred vision • Still’s / JIA
• IBD
Examination • Sarcoidosis
• Cloudy cornea c̄ circumcorneal injection • Behcet’s
• Fixed, dilated, irregular pupil • Infections: TB, leprosy, syphilis, HSV, CMV, toxo
• ↑ IOP makes eye feel hard
Mx
Ix • Refer to ophthalmologist
• Tonometry: ↑↑ IOP (usually >40mmHg) • Prednisolone drops
• Cyclopentolate drops: dilates pupil and prevents
Acute Mx: Refer to Ophthalmologist adhesions between iris and lens (synechiae)
• Pilocarpine 2-4% drops stat: miosis opens blockage
• Topical β-B (e.g. timolol): ↓ aqueous formation
• Acetazolamide 500mg IV stat: ↓ aqueous formation
• Analgesia and antiemetics

Subsequent Mx
• Bilat YAG peripheral iridotomy once IOP ↓ medically

© Alasdair Scott, 2018 149

Episcleritis Corneal Abrasion
• Inflammation below conjunctiva in the episclera • Epithelial breech w/o keratitis
• Cause: trauma
Presentation
• Localised reddening: can be moved over sclera Symptoms
• Painless / mild discomfort • Pain
• Acuity preserved • Photophobia
• Blurred vision
Causes
• Usually idiopathic Ix
• May complicate RA or SLE • Slit lamp: fluorescein stains defect green

Rx: Topical or systemic NSAIDs Rx

• Chloramphenicol ointment for infection prophylaxis

Scleritis
• Vasculitis of the sclera Corneal Ulcer + Keratitis (corneal inflam)

Presentation Causes: bacteria, herpes, fungi, protozoa, RA

• Severe pain: worse on eye movement • Dendritic ulcer = Herpes simplex
• Generalised scleral inflammation • Acanthamoeba: protazoal infection affecting contact
§ Vessels won’t move over sclera lens wearers swimming in pools.
• Conjunctival oedema (chemosis)
Presentation
• Pain, photophobia
Causes • Conjunctival hyperaemia
• Wegener’s • ↓ acuity
• RA • White corneal opacity
• SLE
• Vasculitis Risk factors: contact lens wearers

Mx Ix: green c̄ fluorescein on slit lamp

• Refer to specialist
• most need or corticosteroids or immunosuppressants
Complications:
• Scleromalacia (thinning) → globe perforation
Rx: refer immediately to specialist who will
• Take smears and cultures
• Abx drops, oral/topical aciclovir
• Cycloplegics/mydriatics ease photophobia
• Steroids may worsen symptoms: professionals only

Conjunctivitis Complications
• Scarring and visual loss
Presentation
• Often bilateral c̄ purulent discharge
§ Bacterial: sticky (staph, strep, Haemophilus) Ophthalmic Shingles
§ Viral: watery • Zoster of CNV1
• Discomfort • 20% of all Shingles (only commoner in thoracic
• Conjunctival injection dermatomes)
§ Vessels may be moved over the sclera
• Acuity, pupil responses and cornea are unaffected. Presentation
• Pain in CNV1 dermatome precedes blistering rash
Causes • 40% → keratitis, iritis
• Viral: adenovirus • Hutchinson’s sign
• Bacterial: staphs, chlamydia, gonococcus § Nose-tip zoster due to involvement of
• Allergic nasociliary branch.
§ ↑ chance of globe involvement as nasociliarry
Rx nerve also supplies globe
• Bacterial: chloramphenicol 0.5% ointment • Ophthalmic involvement
• Allergic: anti-histamine drops: e.g. emedastine § Keratitis + corneal ulceration (fluorescein
stains)
§ ± iritis

150 © Alasdair Scott, 2018

 Sudden Loss of Vision
Key Questions Central Retinal Artery Occlusion
• Headache associated: GCA
• Eye movements hurt: optic neuritis Presentation
• Lights / flashes preceding visual loss: detached retina • Dramatic unilateral visual loss in seconds
• Like curtain descending: TIA, GCA • Afferent pupil defect (may precede retinal changes)
• Poorly controlled DM: vitreous bleed from new vessels • Pale retina c̄ cherry-red macula

Causes
Anterior Ischaemic Optic Neuropathy (AION) • GCA
• Optic nerve damaged if posterior ciliary arteries • Thromboembolism: clot, infective, tumour
blocked by inflammation or atheroma.
• Pale / swollen optic disc Rx
• If seen w/i 6h aim is to ↑ retinal blood flow by ↓ IOP
Causes § Ocular massage
• Arteritic AION: Giant Cell Arteritis § Surgical removal of aqueous
• Non-arteritic AION: HTN, DM, ↑ lipids, smoking § Anti-hypertensives (local and systemic)

Optic Neuritis Retinal Vein Occlusion

Symptoms Central
• Unilateral loss of acuity over hrs – days • Commoner than arterial occlusion
• ↓ colour discrimination (dyschromatopsia) • Causes: arteriosclerosis, ↑BP, DM, polycythaemia
• Eye movements may hurt • Pres: sudden unilat visual loss c̄ RAPD
• Fundus: Stormy Sunset Appearance
Signs § Tortuous dilated vessels
• ↓ acuity § Haemorrhages
• ↓ colour vision § Cotton wool spots
• Enlarged blind-spot • Complications
• Optic disc may be: normal, swollen, blurred § Glaucoma
• Afferent defect § Neovascularisation
• Prognosis: possible improvement for 6mo-1yr
Causes
• Multiple sclerosis (45-80% over 15yrs) Branch
• DM • Presentation: unilateral visual loss
• Drugs: ethambutol, chloramphenicol • Fundus: segmental fundal changes
• Vitamin deficiency • Comps: retinal ischaemia → VEGF release and
• Infection: zoster, Lyme disease neovascularisation (Rx: laser photocoagulation)

Rx
• High-dose methyl-pred IV for 72h Retinal Detachment
• Then oral pred for 11/7 • Holes/tears in retina allow fluid to separate sensory
retina from retinal pigmented epithelium
O
• May be 2 to cataract surgery, trauma, DM
Vitreous Haemorrhage
Presentation: 4 F’s
Source • Floaters: numerous, acute onset, “spiders-web”
• New vessels: DM • Flashes
• Retinal tears / detachment / trauma • Field loss
• Fall in acuity
• Painless
Presentation
• Small bleeds → small black dots / ring floaters
• Large bleed can obscure vision → no red reflex, retina Fundus: grey, opalescent retina, ballooning forwards
can’t be visualised
Rx
Ix • Urgent surgery
• May use B scan US to identify cause • Vitrectomy + gas tamponade c̄ laser coagulation to
secure the retina.
Mx
• VH undergoes spontaneous absorption Causes of transient visual loss
• Vitrectomy may be performed in dense VH • Vascular: TIA, migraine
• MS
• Subacute glaucoma
• Papilloedema

© Alasdair Scott, 2018 151

 Gradual Visual Loss
Causes Chronic Simple (Open-Angle) Glaucoma

Common Pathogenesis
• Diabetic retinopathy • Depends on susceptibility of pt’s. retina and optic
• ARMD nerve to ↑ IOP damage.
• Cataracts • IOP >21mmHg → ↓blood flow and damage to optic
• Open-angle Glaucoma nerve → optic disc atrophy (pale) + cupping

Rarer Presentation
• Genetic retinal disease: retinitis pigmentosa • Peripheral visual field defect: superior nasal first
• Hypertension • Central field is intact \ acuity maintained until late
• Optic atrophy § Presentation delayed until optic N. damage is
irreversible

Screen if High Risk

Age-Related Macular Degeneration (ARMD) • >35yrs
• Commonest cause of blindness >60yrs
• Afro-Caribbean
• 30% of >75yrs will have dry AMD
• FH
• Drugs: steroids
Risk Factors • Co-morbidities: DM, HTN, migraines
• Smoking • Myopia
• ↑ age
• Genetic factors Ix
• Tonometry: IOP ≥21mmHg
Presentation • Fundoscopy: cupping of optic disc
• Elderly pts. • Visual field assessment: peripheral loss
• Central visual loss
Mx
Dry ARMD: Geographic Atrophy • Life-long f/up
• Drusen: fluffy white spots around macula
• Degeneration of macula Eye-drops to ↓ IOP to baseline
• Slow visual decline over 1-2yrs st
• 1 line: β-blockers
§ Timolol, betaxolol
Wet ARMD: Subretinal Neovascularisation § ↓ aqueous production
• Aberrant vessels grow into retina from choroid and → § Caution in asthma, heart failure
haemorrhage • Prostaglandin analogues
• Rapid visual decline (sudden / days / wks) c̄ distortion § Latanoprost, travoprost
• Fundoscopy shows macular haemorrhage → scarring § ↑ uveoscleral outflow
• Amsler grid detects distortion • α-agonists
§ Brimonidine, apraclonidine
Ix § ↓ aqueous production and ↑ uveoscleral
• OCT: optical coherence tomography outflow
§ Gives high resolution images of the retina • Carbonic anhydrase inhibitors
§ Dorzolamide drops, acetazolamide PO
Mx for Wet AMRD • Miotics
• Photodynamic therapy § Pilocarpine
• Intravitreal VEGF inhibitors
§ Bevacizumab (Avastin) Non-medical Options
§ Ranibizumab (Lucentis) • Laser trabeculoplasty
• Antioxidant vitamins (C,E) + zinc may help early • Surgery (trabeculectomy) is used if drugs fail
ARMD § New channel allows aqueous to flow into
conjunctival bleb

Tobacco-Alcohol Amblyopia
• Due to toxic effects of cyanide radicals when
combined with thiamine deficiency. Commonest Causes of Blindness Worldwide
• Pres: Optic atrophy, loss of red/green discrimination, • Trachoma
scotomata • Cataracts
• Rx: vitamins may help • Glaucoma
• Keratomalacia: vitamin A deficiency
• Onchocerciasis
• Diabetic Retinopathy

152 © Alasdair Scott, 2018

 The Eye in DM
The Problem Fundoscopy Findings
• DM is leading cause of blindness up to 60yrs
• 30% have ocular problems @ presentation Background Retinopathy: Leakage
• BP < 130/80 and normoglycaemia → ↓ diabetic • Dots: microaneurysms
retinopathy • Blot haemorrhages
• Hard exudates: yellow lipid patches
Pathogenesis
Pre-proliferative Retinopathy: Ischaemia
Cataract • Cotton-wool spots (infarcts)
• DM accelerates cataract formation • Venous beading
• Lens absorbs glucose which is converted to sorbitol by • Dark Haemorrhages
aldose reductase. • Intra-retinal microvascular abnormalities

Retinopathy Proliferative Retinopathy

• Microangiopathy → occlusion • New vessels
• Occlusion → ischaemia → new vessel formation in • Pre-retinal or vitreous haemorrhage
retina • Retinal detachment
§ Bleed → vitreous haemorrhage
§ Carry fibrous tissue c̄ them → retinal Maculopathy
detachment • Caused by macular oedema
• Occlusion also → cotton wool spots (ischaemia) • ↓ acuity may be only sign
• Vascular leakage → oedema and lipid exudates • Hard exudates w/i one disc width of macula
• Rupture of microaneurysms → blot haemorrhage

Screening
• All diabetics should be screened annually
• Fundus photography
• Refer those c̄ maculopathy, NPDR and PDR to
ophthalmologist
§ 30% NPDR develop PDR in 1yr

Ix
• Fluorescein angiography

Mx
• Good BP and glycaemic control
• Rx concurrent disease: HTN, dyslipidaemia, renal
disease, smoking, anaemia
• Laser photocoagulation
§ Maculopathy: focal or grid
§ Proliferative disease: pan-retinal (macula
spared)

CN Palsies
• CNIII and VI palsies may occur
• In diabetic CNIII palsy the pupil may be spared as its
nerve fibres run peripherally and receive blood from pial
vessels.

© Alasdair Scott, 2018 153

Cataracts The Retina
• Outer pigmented layer in contact c̄ the choroid
Presentation • Inner sensory layer in contact c̄ vitreous
• Increasing myopia • At centre is fovea
• Blurred vision → gradual visual loss
• Dazzling in sunshine / bright lights Optic Disc
• Monocular diplopia
Colour
Causes • Should be pale pink
• ↑Age: 75% of >65s • Paler in optic atrophy
• DM
• Steroids Contour
• Congenital • Margins blurred in papilloedema and optic neuritis
§ Idiopathic
§ Infection: rubella
Cup
§ Metabolic: Wilson’s, galactosaemia
• Physiological cup lies centrally and should occupy 1/3
§ Myotonic dystrophy
of disc diameter
• Cup widening and deepening in glaucoma
Ix
• Visual acuity
• Dilated Fundoscopy
• Tonometry
Retinitis Pigmentosa
• Blood glucose to exclude DM • Most prevalent inherited degeneration of the macula
• Various modes of inheritance
§ Mostly AR
Mx § AD has best prognosis
§ X-linked has worst prognosis
Conservative • Affects ~ 1/2000
• Glasses
• Mydriatic drops and sunglasses may give some relief Presentation
• Night blindness
Surgery • ↓↓ visual fields → tunnel vision
• Consider if symptoms affect lifestyle or driving (<6/10) • Most are registrable blind (<3/60) by mid 30s
• Day-case surgery under LA
§ Phacoemulsion + lens implant Fundoscopy
• 1% risk of serious complications • Pale optic disc: optic atrophy
§ Anterior uveitis / iritis • Peripheral retina pigmentation: spares the macula
§ VH
§ Retinal detachment
§ Secondary glaucoma
Associations
§ Endophthalmitis (→ blindness in 0.1%) • Friedrich’s ataxia
• Post-op capsule thickening is common • Refsum’s disease
§ Easily Rx c̄ laser capsulotomy. • Kearns-Sayre Syndrome
• Post-op eye irritation is common and requires drops • Usher’s Syndrome

Retinoblastoma
• Commonest intraocular tumour in children
• 1:15,000 LBs

Inheritance
• Hereditary type differs from non-hereditary type
• AD mutation of RB gene (a TSG)
• Pts. typically have one mutant allele in every retinal
cell; if the other allele mutates → retinoblastoma.

Associations
• 5% occur c̄ pineal or other tumour
• ↑ risk of osteosarcoma and rhabdomyosarcoma

Signs
• Stabismus
• Leukocoria (white pupil) → no red reflex

Rx
• Depends on size
• Options include: chemo, radio, enucleation

154 © Alasdair Scott, 2018

The External Eye Orbital Swellings
Inflammatory Lid Swellings Orbital Cellulitis

Stye or hordeolum externum Pathophysiology

• An abscess / infection in a lash follicle which points • Infection spreads locally: e.g. from paranasal sinuses,
outwards. eyelid or external eye.
• Rx: local Abx – e.g. fusidic acid • Staphs, pneumococcus, GAS

Chalazion or hordeolum internum Presentation

• Abscess of the Meibomian glands which points • Usually a child c̄ inflammation of the orbit + lid
inwards onto conjunctiva. swelling
§ Sebaceous glands of eyelid • Pain and ↓ range of eye movement
• Exophthalmos
Blepharitis • Systemic signs: e.g. fever
• Chronic inflammation of eyelid • ± tenderness over the sinuses
• Causes: seborrhoeic dermatitis, staphs
• Features Rx
§ Red eyes • IV Abx: Cefuroxime (20mg/kg/8h IV)
§ Gritty / itchy sensation
§ Scales on lashes. Complications
§ Often assoc. c̄ rosacea • Local extension → meningitis and cavernous sinus
• Rx thrombosis.
§ Clean crusts of lashes c̄ warm soaks • Blindness due to optic N. pressure.
§ May need fusidic acid drops

Entropion Carotid-cavernous fistula

• Lid inversion → corneal irritation • May follow carotid aneurysm rupture c̄ reflux of blood
• Degeneration of lower lid fascia into cavernous sinus.
• Causes: spontaneous, trauma
Ectropion • Presentation
• Low lid eversion → watering and exposure keratitis § Engorgement of eye vessels
• Assoc. c̄ ageing and facial N. palsy § Lid and conjunctival oedema,
§ Pulsatile exophthalmos
§ Eye bruit
Ptosis • Rx: endovascular treatment
• True ptosis is intrinsic LPS weakness
• Bilateral
§ Congenital Exophthalmos / Proptosis
§ Senile
• Protrusion of one or both eyes
§ MG
§ Myotonic dystrophy
• Unilateral Common Causes
rd
§ 3 Nerve palsy • Graves’ Disease
§ Horner’s syndrome (partial) § 25-50%
§ Mechanical: xanthelasma, trauma § ↑ risk in smoker’s
§ Anti-TSH Abs → retro-orbital inflammation and
lymphocyte infiltration → swelling
• Orbital cellulitis
Lagophthalmos • Trauma
• Difficulty in lid closure over the globe which may →
exposure keratitis
Other Causes
• Causes: exophthalmos, facial palsy, injury
• Idiopathic orbital inflammatory disease
• Rx
• Vasculitis: Wegener’s
§ Lubricate eyes c̄ liquid paraffin ointment
• Neoplasm
§ Temporary tarsorrhaphy may be needed if
§ Lymphoma
corneal ulcers develop.
§ Optic glioma: assoc. c̄ NF-1
§ Capillary haemangioma
Pinguecula § Mets
• Yellow vascular nodules either side of the cornea • Carotid-cavernous fistula

Pterygium
• Similar to pinguecula but grows over the cornea → ↓
vision.
• Benign growth of conjunctiva
• Assoc. c̄ dusty, wind-blown life-styles, sun exposure

© Alasdair Scott, 2018 155

Refractive Errors Strabismus / Squint / Tropia
Refractive errors arise from disorders of the size and shape Esotropia = convergent squint
of the eye. • Commonest type in children
• May be idiopathic or due to hypermetropia

Myopia: Short-sightedness Exotropia = divergent squint

• Older children
The Problem • Often intermittent
• The eye is too long
• Distant objects are focussed too far forward.
Non-Paralytic Squint
Causes
• Genetic Diagnosis
• Excessive close work in the early decades • Corneal reflection: should fall centrally and
symmetrically on each cornea.
The Solution • Cover test: movement of uncovered eye to take up
• Concave lenses fixation demonstrates manifest squint.

Management: 3 O’s
Astigmatism • Optical: correct refractive errors
• Orthoptic: patching good eye encourages use of
The Problem squinting eye.
• Cornea or lens doesn’t have same degree of • Operations: e.g. resection and recession of rectus
curvature in horizontal and vertical planes. muscles – help alignment and cosmesis.
• Image of object is distorted longitudinally or vertically

The Solution Paralytic Squint

• Correcting lenses • Diplopia is most on looking in direction of pull of
paralysed muscle.
• Eye won’t fixate on covering.
• Cover each eye in turn: which ever eye sees the outer
Hypermetropia: long-sightedness image is malfunctioning

The Problem CNIII

• Eye is too short
• Ptosis (LPS)
• When eye is relaxed and not accommodating, objects
• Fixed dilated pupil (no parasympathetic)
are focussed behind the retina.
• Eye looking down and out
• Contraction of ciliary muscles to focus image →
• Causes
tiredness of gaze and possibly a convergent squint in
§ Medical: DM, MS, infarction
children.
§ Surgical: ↑ ICP, cavernous sinus thrombosis,
posterior communicating artery aneurysm
The Solution
• Convex lenses CNIV
• Diplopia especially on going down stairs
• Head tilt
Presbyopia • Test: can’t depress in adduction
• With age, lens becomes stiff and less easy to deform. • Causes
• Start at about 40yrs and is complete by 60yrs. § Peripheral: DM (30%), trauma (30%),
• Use convex lenses. compression
§ Central: MS, vascular, SOL

CNVI
• Eye is medially deviated and cannot abduct
• Diplopia in the horizontal plane.
• Causes
§ Peripheral: DM, compression, trauma
§ Central: MS, vascular, SOL
• Rx: botulinum toxin can eliminate need for surgery

156 © Alasdair Scott, 2018

Eye Trauma Floaters, Flashes and Haloes
• Record acuity of both eyes
• Take detailed Hx of event Floaters
• If unable to open injured eye, instil LA (e.g. tetracaine • Small dark spots in the visual field
1%) • Sudden showers of floaters in one eye may be due to
blood or retinal detachment

Foreign Bodies Causes

• X-ray orbit if metal FB suspected
• Fluorescein may show corneal abrasions
Mx
• Chloramphenicol drops 0.5% prevent infection
§ Usually coagulase-negative Staphylococcus
• Eye patch
• Cycloplegic drops may ↓ pain
§ Tropicamide, cyclopentolate
Intra-ocular Haemorrhage
• Blood in anterior chamber = hyphaema
• Small amounts clear spontaneously, but some may
need evacuation.
• Complicated by corneal staining and glaucoma (pain)
• Keep IOP↓ and monitor
Orbital Blowout Fracture
• Blunt injury → sudden ↑ in orbital pressure c̄
herniation of orbital contents into maxillary sinus.
• Retinal detachment
• VH
• Diabetic retinopathy / Hypertension
• Old retinal branch vein occlusion
• Syneresis (degenerative opacities in the vitreous)
Flashes (Photopsia)
• Either from intraocular or intracerebral pathology
• Headache, n/v: migraine
• Flashes and floater: retinal detachment
Haloes
• Usually just diffractive phenomena
• May be caused by hazy ocular media – cataract,
corneal oedema, acute glaucoma
• Haloes + eye pain = acute glaucoma
• Jagged haloes which change shape are usually
migrainous.

Presentation
• Ophthalmoplegia + Diplopia
§ Tethering of inferior rectus and inferior oblique
• Loss of sensation to lower lid skin
§ Infraorbital nerve injury
• Ipsilateral epistaxis
§ Damage to anterior ethmoidal artery
• ↓ acuity
• Irregular pupil that reacts slowly to light

Mx
• Fracture reduction and muscle release necessary.

Chemical Injury
• Alkaline solutions are particularly damaging
• Mx
§ Copious irrigation
§ Specialist referral

© Alasdair Scott, 2018 157

Allergic Eye Disease Tropical Eye Disease
Seasonal Allergic Conjunctivitis (SAC) Trachoma
• 50% of allergic eye disease
• Small papillae on tarsal conjunctivae Pathophysiology
• Rx • Caused by Chlamydia trachomatis (A,B,C)
§ Antazoline: antihistamine drops: • Spread by flies
§ Cromoglycate: inhibits mast cell degranulation • Inflammatory reaction under lids → scarring → lid
distortion → entropion → eyelashes scratch cornea →
Perennial Allergic Conjunctivitis (PAC) ulceration → blindness
• Symptoms all year c̄ seasonal exacerbations
• Small papillae on tarsal conjunctivae Rx
• Rx: olopatadine (antihistamine and mast-cell • Tetracycline 1% ointment ± PO
stabiliser)
Prevention
Giant Papillary Conjunctivitis • Good sanitation
• Iatrogenic FBs: contact lenses, prostheses, sutures • Face washing
• Giant papillae on tarsal conjunctivae
• Rx: removal of FB, mast cell stabilisers Onchocerciasis (River Blindness)

Pathophysiology
Management of Allergic Eye Disorders • Caused by microfilariae of nematode Onchocerca
volvulus
1. Remove the allergen responsible where possible • Spread by flies
• Fly bites → microfilariae infection → invade the eye →
2. General measures inflammation → fibrosis → corneal opacities and
§ Cold compress synechiae
§ Artificial tears
3. Oral antihistamines: loratadine 10mg/d PO Rx
• Ivermectin
4. Eye drops
§ Antihistamines: antazoline, azelastine
§ Mast cell stabilizers: cromoglycate, Xerophthalmia and Keratomalacia
lodoxamide
• Manifestations of vitamin A deficiency
§ Steroids: dexamethasone
- Beware of inducing glaucoma
§ NSAIDs: diclofenac Presentation
• Night blindness and dry conjunctivae (xerosis)
• Corneal ulceration and perforation

Rx
• Vitamin A / palmitate reverses early corneal changes

158 © Alasdair Scott, 2018

Eye Signs in Systemic Conditions Ophthalmic Pharmacology
Hypertensive Retinopathy Mydriatics
• Keith-Wagener Classification
• Tortuosity and silver wiring Anti-Muscarinics
• AV nipping • Tropicamide
• Flame haemorrhages and soft / cotton wool spots § Duration: 3h
• Papilloedema • Cyclopentolate
• Grades 3 and 4 = malignant hypertension § Duration: 24h
§ Preferred for paediatric use
Granulomatous Disorders • Pupil dilatation + loss of light reflex
• TB, sarcoid, toxo, leprosy, brucella • Cycloplegia (ciliary paralysis) → blurred vision
• Uveitis (ant/post) and choroidoretinitis
Sympathomimetics
• Para-hydroxyamphetamine, phenylephrine
Systemic Inflammatory Disease
• May be used c̄ tropicamide
• Conjunctivitis: SLE, reactive arthritis, IBD
• Scleritis / episcleritis: RA, vasculitis, SLE, IBD • Don’t affect the light reflex or accommodation
• Iritis : ank spond, IBD, sarcoid
• Retinopathy: dermatomyositis Indications
• Eye examination
• Prevention of synechiae in ant. uveitis / iritis
Keratoconjunctivitis Sicca / Sjogren’s
• ↓ tear production (Schirmer’s: <5mm in 5min)
• Dry eyes and dry mouth
Caution
•
O O
1 or 2 : SLE, RA, sarcoid • May → acute glaucoma if shallow anterior chamber
• Rx: artificial tears or saliva

Vascular Occlusion Miotics

• Emboli → amaurosis fugax: GCA, carotid
atheroemboli Effect
• Microemboli → Roth spots: infective endocarditis • Constrict the pupil
§ Boat-shaped haemorrhage c̄ pale centre
Pilocarpine
Metabolic • Muscarinic agonist
• Kayser-Fleischer Rings: Wilson’s
• Exophthalmos: Graves’ Use
• Corneal calcification: HPT • Acute closed-angle glaucoma

HIV/AIDS
• CMV retinitis: pizza-pie fundus + flames Other
• HIV retinopathy: cotton wool spots
Tetracaine
• Anaesthetic used to permit examination of a painful
eye

Lubricants
• Hypomellose
• Carbomer (viscotears)

Topical Anti-histamine
• Emedastine
• Antazoline

Chronic Open-Angle Glaucoma

st
• 1 line: β-blockers
§ Timolol, betaxolol
§ ↓ aqueous production
§ Caution in asthma, heart failure
• Prostaglandin Analogues
§ Latanoprost, travoprost
§ ↑ uveoscleral outflow
• α-agonists
§ Brimonidine, apraclonidine
§ ↓ aqueous production and ↑ uveoscleral
outflow
• Carbonic anhydrase inhibitors
§ Dorzolamide drops, acetazolamide PO
• Miotics: Pilocarpine

© Alasdair Scott, 2018 159

 Cardiac Electrophysiology

HEAD
avr-1 -150° "9,°° R
I 3VL ~30°
RIGHT :t180°----I |[)° LEFT
ST
pg-_ Segment
P Sngmnnt T

+120° ||| "+60°

aVF PR Interval
Q
+ O
90 s
QT Interval
FEET

H 71- /“Q
. - 1'~ ‘F1 w.\ l Sinoatrial node W \ Bachmanns
bundle
.. - _ __. _ ‘_
Atrioventricular His bumfle
node D/ Q l
,-' . —\_.

<5?/’
£1-se__§__
-*'. \
Lfll
.-.... .
Lefi postenor-I
bundle
K -» _- _ ,

\'. ' -W Purkinje

-,~"'?i'-_;.>-
, ‘J.
- .6_.\ J4ie,".-_
H‘ Right bundl 9 1
\\
T.--\_ Hf‘ _
\
;, _ fibres
I
I

View Leads Vessel

Inferior II, III, aVF RCA
Anterolateral I, aVL, V5 + V6 L circumflex
Anteroseptal V2-V4 LAD
Anterior V2-V6 Left main stem
Posterior V1, V2, V3 (recip) RCA

© Alasdair Scott, 2012 2

 ECG Analysis
1. Rate 6. PR interval (120-200ms)
 300 / no. of large squares  Start of P wave to start of QRS
 6 = 50  Long: heart block
 5 = 60  Short
 4 = 75  Accessory conduction: e.g. WPW
 3 = 100  Nodal rhythm
 2 = 150  HOCM
 1 = 300  Depressed: pericarditis

2. Rhythm 7. QTc (380-420ms)

 Look for P waves followed by QRS complexes  Start of QRS to end of T wave
 AF  Bazett’s formula: QTc = actual QT/√R-R
 No discernable P waves  Long (>420ms): TIMME
 Irregularly irregular QRS  Toxins
 Atrial flutter: saw-toothed baseline  Macrolides
 Nodal rhythm: regular QRS but no P waves  Anti-arhythmics (Ia/III): quinidine, amiod
 TCAs
 Histamine antagonists
3. Axis  Inherited: e.g. Romano-Ward, Jervell (c̄ SNHL)
 I and II +ve = normal axis (-30 – +90)  Ischaemia
 I +ve and II –ve (leaving) = LAD (-30 – -90)  Myocarditis
 I –ve and II +ve (reaching) = RAD (+90 – +180)  Mitral valve prolapse
 Electrolytes: ↓Mg, ↓K, ↓Ca, ↓ temp
 Short (<380ms)
RAD (> +90) LAD (<-30)  Digoxin
Anterolateral MI Inferior MI  β-B
RVH, PE LVH  Phenytoin
L post. hemiblock L ant. Hemiblock
WPW WPW
ASD secundum ASD primum 8. ST Segments
 Elevated (limbs: >1mm, chest: >2mm)
 Acute MI
4. P Waves  Prinzmetal’s angina
 Pericarditis: saddle-shaped
 Absent: AF, SAN block, nodal rhythm
 Aneurysm: ventricular
 Dissociated: complete heart block
 P mitrale: bifid P waves = LA hypertrophy
 Depressed (>0.5mm)
 HTN, AS, MR, MS
 Ischaemia: flat
 P pulmonale: peaked P waves= RA hypertrophy  Digoxin: down-sloping
 pulmonary HTN, COPD

5. QRS 9. T-waves
 Wide QRS (>120ms)  Normally inverted in aVR and V1
 Ventricular initiation  + V2-V3 in blacks
 Conduction defect
 Abnormal if inverted in: I, II and V4-6
 WPW
 Strain
 Pathological Q wave  Ischaemia
 >1mm wide and > 2mm deep  Ventricular hypertrophy
 Full Thickness MI  BBB
 RVH: Dominant R wave in V1 + deep S wave in V6  Digoxin
 LVH  Peaked in ↑K+
 R wave in V6 >25mm  Flattened in ↓K+
 R wave in V5/V6 + S wave in V1 > 35mm

10. Extras
 U waves
 Occur after T waves
 Seen in ↓ K
 J waves / Osborne wave
 Occur between QRS and ST segment
 Causes
 hypothermia < 32OC
 SAH
 Hypercalcaemia
© Alasdair Scott, 2012 3
 ECG Abnormalities

Conduction Defects

Abnormality Features ECG

1st Degree Heart Block PR > 200ms ___IIII

_ _

________ _

‘|“"‘1|Il“l1'.I-|_l.:‘- -|'._ |'"_| "1':_"__''_ ‘I-_r

l I1- i_'fjr“L|[_-l I_|‘|'|' III .1‘illI.|_II|:'|l-_‘|.' _.|

2nd Degree Heart Block

____
|Il_ -I LPI-l|_r '+l|I '|-_
ll*l1‘_
I‘_‘__ _I1
P

Progressive lengthening of ‘
+_
I__ _1_
_|_____
___I_
____ __‘ _____ ___I _I_ ._'_|'L.'_ _ |._| '_ _' _'_‘' i
‘_
___!‘IllI |]_ |____‘_I__|_| |I

_I J_
'___‘'I- ‘ll

_____ ____
_ ___ _I___ _______I _‘I'.

- Wenckebach /Mobitz I PR interval

One non-conducted P wave

Next conducted beat has i

____I

shorter PR interval
2nd Degree Heart Block Constant PR +1]‘|l_Ill’'|1‘l |I

- Mobitz II 141

Occasional non-conducted P
waves
in___
I++Il. ___| _
___ _U __Q II '
J_.‘_
__ _ ‘ T __ § ___P|

Often wide QRS _ i__ _ ____ __ _ T _ ___' __

- block is usually in bundle

branches of Purkinje fibres ___

2nd Degree Heart Block Two P waves per QRS

- 2:1 Block
Normal consistent PR _- _ _ __ _ __ _II __ _

intervals __'|‘.|__ _.I .

I
_I|L_

3rd Degree Heart Block P waves and QRS @ Ill__ __

different rates ___

- dissociation

Abnormally shaped QRS __ __

- ventricular origin (40bpm)

Abnormality Features ECG

_v 11
Aetiology
Right BBB MaRRoW Infarct – Inferior MI
Wide QRS _I Normal variant
I ...‘

RSR pattern in V1
‘
U_
__I I ldl _ _ _I I‘
I

“_|l_ _l
_L I. _ _ _ __;| I ‘| _l IU
I M‘
ppp
p Congenital – ASD, VSD, Fallot’s
Hypertrophy – RVH (PE, Cor
Pulmonale)

Ivp 15¢ __I|IH __ ___ ___ F_|| |_ pvi :i““

‘i__ _ql_ WBM
1
__I ‘Ii 6
Left BBB WiLLiaM Fibrosis
Wide QRS c̄ notched LVH – AS, HTN
top Infarct – Inf. MI
T wave inversion in lat Il_l l Coronary HD
leads __

Jll I I Hi!
I'l

5
Bifascicular Block RBBB + LAD RBBB + Left ant. hemiblock
Trifascicular Block RBBB + LAFB + 1st
degree AV block

© Alasdair Scott, 2012 4

Escape Rhythms: appear late (after anticipated beat)
Abnormality Features ECG
Atrial Escape SAN fails to depolarise I
I-l
I ‘l l
-I

TI II
:l.LI,
.l

Abnormal P wave
A1HIlE.!\‘!II4IH.EI.IlII1
I f I - r - '1 III
I
Normal QRS |I
l

Ill I r > > ‘

II
60-80bpm __ 3 _;%
-1 aeiflflfifififli
I ll _

, l__ I ii

Junctional Escape Usually no P waves

I
(occasionally after QRS) -W In I-F

ITIIII |.'l_.l I I
___ ,II:mIrlIIQIPa:iI@
I-|I_l|II|

Normal QRS

40-60bpm
_l_._
—::,r¢

SE
YIEEE ‘=5!
Ventricular Escape Usually result of complete
AV block  regular P PI.P._P. I
waves seen (top).
P P P ~ p P
May be SAN failure → no P
waves (below).

Wide QRS,
1 _ I ll‘ I I
20bpm ‘l__nl'I -5 I "ll I I ll
IE
-'in

I F
' I

1- 1, Z2 I
J
l I
I LI! I .'
Ir.
I. HI‘ _

“T
I” El
'I' -I ' . : '!
1 I _II; 7 fin; ~ ‘I _ ___ ___
IQ ~ P PI
1 -
._ III I

IEHI: :I____;
1
IT -II I::T_ L___ ___1___I
I1
lI
I
I

Extrasystoles: appear early (before anticipated beat)

Abnormality Features ECG
Atrial Extrasystole Abnormal P wave 1 .

I-I. I
I. . " III ¢-|_.I.-I; III -I . -I
-0-I1- 0-1
Normal QRS
- . l

5 I I i 1
1 I -a-II--'I+- ----Q --I -- +-

' -----Qn---II----Il-- L I

Nodal Extrasystole P wave buried in QRS or ___:_I___ 7 I___ I___ _____ I___ I___ I___ 1: ___ :__—:i_ ___ I___ I___ ___: ___I____::_—_

sometimes immediately I I ll II I
before/after QRS. IIIIIIII.lIII!IlIIIl—-!III!.I I!!fll!
- may be negative ElII!HIII!HIII!HI‘.ZIIi!Hi|.Bllflfil
=!IIII— IIIIII
IIIIIIIIIII
I1
l L I_II I I
Normal QRS
Ventricular No P wave.

ii’it
Extrasystole
Wide QRS and abnormal T
ll l

I I _'I! _-II-I‘-|ll-—ll' III-

II:
wave.
-1-I I- III-' --——\v '_ ‘-——

fig-F‘-IU I-I
- # I-II
i:I$......
1 III
IIIII I III
‘III.
IIn-I-_
3 _II'.- l I '1

© Alasdair Scott, 2012 5

Narrow Complex Tachycardias
Abnormality Features ECG
AV Nodal Re- P wave absent or immediately
entrant before/after QRS
Tachycardia
Normal QRS

i Um"
fim

i_
‘~
___

___’_ M
w
q_ fll-P‘_ ii

AVRT P waves usually visible

‘___
_|
__
__-_ Q_
I ._________

between QRS complexes H__ _M

_H_____
__
_ _*_p _
_ __ '
___ __
_ ‘UNMP '
__
_ __ _&
_ F
_
_ __
_ __F
_ ___
_

QRS may be narrow or wide n ‘H _

_ _
_ T
_H
_ _H __ __ _
_ _
M

M __ _ F _ _ _

Accessory conduction bundle

Atrial Tachycardia Abnormally shaped P waves _____
_ II,'_lI_|I

Normal QRS complexes.

IM_?
?If%Iu?n_m?fRn_-u-
“_ _L "_J_
WIWM;LA
_*_+__
1 “H_ Hf_n
_
u
r_ H
h_._ -_ _i+__;.| __rJ_ A?MMMuW?Nru% im%u
?% ___?Wi-E.
LL %“kw
lm_IH_1_
_ _’_I: _.
H_I
J_,_ _ _ t_ _I‘._ _H_HHH

_.h|_.'|"‘_t|_rl'_-_|h ‘_| _TUH

:_
IIU
‘H_|*
_I‘I_
'___
.II__

"F
H. URI___IA_L_
_ __w
_W“ml-_5H%
Ii1
Im
H_
Rate > 150bpm
U__
__
H
H||_|_m:|:
‘

\ ___*
U_ m
L___
_A"H a
H
U_____
_ ___ _H_Rt_ __ k-H‘___
May be assoc. c̄ AV block.
___‘k:
Atrial Flutter “Saw-toothed” baseline as H ‘H
___.” -In.__
atria contract @ 300bpm. ffi
n
_
M L: M
N‘ IMA
M lit!______
Il

1“
MM
HML
AVN can’t conduct > 200bpm J]
___
‘_

 AV block occurs.
- 2:1(150), 3:1(100), 4:1(75) _‘ _4__qJ|"_

Normal QRS
Atrial Fibrillation No P waves – irregular line &_
______%
___
___
I

AJ‘
_H
Irregularly irregular QRS _
__ fl__? _
I_

© Alasdair Scott, 2012 6

Broad Complex Tachycardias
Abnormality Features ECG
-- .l|_

VT No P waves I ‘I-I

Regular, wide QRS

IIIIIIII

| Illlll
No T waves ll~%I1
HEI-
A I

Torsades

VF Shapeless, rapid |-
'-
'1
-

.,j
I

I
..

oscillations and no
-I

|?% -
I

I
organised complexes. 1
,. 4

'!'
,_ I - :_ - _ .
_- ...._1-_.. -_|

c.I- .I-_I.-| H.-,_I q_I I. . Z ’ .

I_ -_ -_ _._I._ I
I.-I-I- I-_I q-I-I

Ventricular tachycardia vs. SVT with bundle branch block

VT more likely if:

 Hx: recent infarction
 Atrioventricular dissociation
 Broad QRS complexes (>140ms)
 Concordant QRS direction in V1-V6
 Fusion and capture beats

© Alasdair Scott, 2012 7

P Wave Abnormalities
Abnormality Features ECG Aetiology
P pulmonale Peaked P wave RAH
- pulmonary HTN
- tricuspid stenois

P mitrale Broad, bifid P wave LAH

- mitral stenois

QRS Abnormalities
Abnormality Features ECG Aetiology
RVH Tall R wave in V1 Cor pulmonale
tall H-wave in V1 deep S-wave in V6
Deep S wave in V6
RAD IIl‘I
Normal QRS width
-EE-
II El
May be T wave inversion in IIII
V1-V3
LVH S in V1 + R in V6 >35mm HTN
deep S-wave In V1 tall R-wave in V6
and/or R wave in V6 >25mm AS
Illlll COA
May be LAD
ifllll H(O)CM

May be T wave inversion in IIIHI

II, aVL, V5, V6 IIII

© Alasdair Scott, 2012 8

Miscellaneous
Abnormality Features ECG
WPW Accessory conducting bundle.

Short PR interval

Slurred upstroke of QRS called a

delta wave (V3/4).

Can establish re-entrant circuit →

SVT (antidromic AVRT)

AF + WPW → irregularly irregular

\
broad QRS complexes (below).

Brugada RBBB Va
Syndrome
Coved ST elevation in V1-V3
I, """"""""""" U
V1

-j"\/--]~\/~»
I - I I I .

Digoxin Reverse tick I

'l
I
I 0
I I I
6

- Down-sloping ST depression
4

- T wave inversion .__-.l.—

4 .-- I J-
. I .
I - I I

K-I-Q-—-IQ‘-Q--1-I h-a ti-|—.-I-Q 11-Q

...- '

PE SI QIII TIII (rare) --

n '
.
I
-i’.

I
'

I
Q --1 ?--I-II-|—-IQ—a;

-i
‘I |

- deep S wave in I (RAD) - Q-----Q-4--—-0--.4-._

O I -

- pathological Q in III
- T inversion in III
—I

ZII

Right vent strain -III.-__AkI._

- RAD (S wave in I)
I ; Q - ;_-‘..-, .lv .,

- Dominant R wave and T I I -

wave inversion in V1-V3 1 id-—Q -- . --‘#1:-1-I‘.-_.I. _ - '-

↑ K+ Tall tented T waves A I] F

Widened QRS
Absent P waves
Sine wave appearance

Fig shows serial ↓ in K+

I I I I I ' I I I I I

↓ K+ Small T waves ST depression A. ‘B.

ST depression
Prolonged QT interval
Prominent U waves

I
Flat T wave

© Alasdair Scott, 2012 9

 Bradycardias
(<60bpm)

Causes: DIVISIONS Classification

 Sinus bradycardia
Drugs  First degree heart block: PR > 200ms
 Antiarrhythmics (type 1a, amiodarone)  Second degree heart block
 β-blockers  Wenkebach / Mobitz I
 Ca2+-channel blockers (verapamil)  Mobitz II
 Digoxin  Complete heart block
 Junctional: narrow QRS @ ~50bpm
Ischaemia / Infarction  Ventricular: broad QRS @ ~40bpm
 Inferior MI
Rx
Vagal hypertonia  If asymptomatic and rate >40: no Rx needed
 Athletes
 Vasovagal syncope
 Carotid sinus syndrome Urgent / rate <40bpm
1. Rx underlying cause: e.g. drugs, MI
Infection
 Viral myocarditis 2. Medical
 Rheumatic fever  Atropine 0.6–1.2g (max 3g) IV
 Infective endocarditis  Isoprenaline IVI

Sick sinus syndrome 3. Pacing: External

 Structural damage or fibrosis of SAN, AVN or
conducting tissue
 PC Elective
 SVT alternating c̄ either sinus bradycardia ± 1. Permanent pacing
arrest or SA/AV block  Mobitz II
 Rx  Complete AV block
 Bradyarrhythmias: pace  Sick sinus
 Tachyarrhythmias: amiodarone  AF
 Drug-resistant tachyarrhythmias
Infiltration: restrictive / dilated cardiomyopathy
 Autoimmune
 Sarcoid
 Haemochromatosis
 Amyloid
 Muscular dystrophy

O
 hypOthyroidism
 hypOkalaemia (or ↑)
 hypOthermia

Neuro: ↑ ICP

Septal defect: primum ASD

Surgery or catheterisation

© Alasdair Scott, 2012 10

 Narrow Complex Tachycardias = SVT

Definition: Rate >100bmp, QRS width < 120ms

Differential
1. Sinus tachycardia
2. Atrial
 AF
 Atrial flutter
 Atrial tachycardia
3. AV nodal re-entry tachycardia
4. AV re-entry tachycardia
Principles of Mx
 If pt. compromised → sedate + DC cardioversion
 Otherwise ID rhythm and Rx accordingly
 Key to ID irregular rhythm = AF = different Rx
 Vagal manoeuvres (carotid sinus massage, valsalva) transiently ↑ AV block and
may unmask underlying atrial rhythm.
 If manoeuvres unsuccessful, give adenosine while recording rhythm strip
 → transient AV block, unmasking atrial rhythm
 → cardioverts AVNRT/AVRT to sinus rhythm
 If adenosine fails, choose from:
 Digoxin
 Atenolol
 Verapamil (not if on β-blocker)
 Amiodarone
 If unsuccessful → DC cardioversion

Mx

O2 + IV access

Regular rhythm?
YES
 Continuous ECG trace
 Vagal manoeuvres
 Adenosine 6mg IV bolus
 Then 12mg, then 12mg
NO
I Treat as AF
 Control rate c̄ β-B (e.g. metoprolol) or digoxin
 If onset <48h consider cardioversion c̄ amiodarone (as
below) or DC shock
 Consider anticoagulation c̄ heparin and/or warfarin
NB. Avoid adenosine, CCB, β-B in AF/Flutter with WPW or Hx
of WPW as may → VF. Use Amiodarone or flecainide

YES
I
Adverse Signs? Sedation
 BP <90
 HF
 ↓ consciousness
Synchronised cardioversion:
 HR >200 100→200→360J

I NO I
Choose from:
Amiodarone:
 Digoxin (500ug over 30min)
 Amiodarone  300mg over 20-60min
 Verapamil  Then 900mg over next 23h
 Atenolol

Adenosine Prophylaxis
 MOA: temporary AVN block  β-B
 SEs: Transient chest tightness, dyspnoea, flushing, headache  AVRT: flecainide
 Relative CIs: asthma, 2nd/3rd degree block  AVNRT: verapamil
 Interactions
 fx ↑d by dipyridimole
 fx ↓d by theophylline

© Alasdair Scott, 2012 11

 Broad Complex Tachycardias
Definition: Rate >100bmp, QRS width > 120ms VT Causes: I’M QVICK
 Infarction (esp. c̄ ventricular aneurysm)
Differential  Myocarditis
1. VT  QT interval↑
2. Torsades de points  Valve abnormality: mitral prolapse, AS
3. SVT c̄ BBB  Iatrogenic: digoxin, antiarrhythmics, catheter
 Cardiomyopathy (esp. dilated)
 K↓, Mg↓, O2↓, acidosis

Mx

Pulse? I CPR
NO
YES

O2 + IV access

YES
 BP <90
Adverse Signs? I Sedation

 HF
 Chest pain
 ↓ consciousness
Synchronised cardioversion:
 HR > 150 200→300→360

NO

Correct Electrolyte Problems Amiodarone:

 ↓K+: max 60mM KCL @ 20mmol/h  300mg over 20-60min
 ↓Mg2+: 4ml 50% MgSO4 in 30min  Then 900mg over next 23h

Assess Rhythm
Regular (i.e. VT):
 Amiodarone (see opposite)
 Or lignocaine 50mg over 2min

If irregular, Dx is usually:
 AF c̄ BBB
 Pre-excited AF: flec / amio Recurrent / Paroxysmal VT
 TDP: MgSO4 2g IV over 10 min
Medical:
 Amiodarone
Failure
 β-B
Synchronised Cardioversion
ICD

© Alasdair Scott, 2012 12

 Atrial Fibrillation
Pathology Persistent AF
 LA loses refractoriness before the end of atrial systole.  >7d, may recur even after cardioversion
 → recurrent, uncoordinated contraction @ 300-600bpm
 Atrial contraction responsible for ~25% of CO Try rhythm control first-line if:
 often triggers heart failure  Symptomatic or CCF
 Younger (<65)
Causes  Presenting first time c̄ lone AF
 Secondary to treated precipitant
Common Other
 IHD  Alcohol Rhythm Control
 Rheumatic heart disease  Pneumonia  TTE first: structural abnormalities
 Thyrotoxicosis  PE  Anticoagulate c̄ warfarin for ≥3wks
 Hypertension  Post-op  or use TOE to exclude intracardiac thrombus.
 Hypokalaemia  Pre-Rx ≥4wks c̄ sotalol or amiodarone if ↑ risk of failure
 RA  Electrical or pharmacological cardioversion
 ≥ 4 wks anticoagulation afterwards (target INR 2.5)
Symptoms
 Asympto Maintenance antiarrhythmic
 Chest pain  Not needed if successfully treated precipitant
 1 : β-B (e.g. bisoprolol, metoprolol).
st
 Palpitations
 Dyspnoea  2nd: amiodarone
 Faintness
Other options
 Radiofrequency ablation of AV node
Signs  Maze procedure
 Irregularly irregular pulse
 Pacing
 Pulse deficit: difference between pulse and HS
 Fast AF → loss of diastolic filling → no palpable
pulse Rate control (target <90bpm at rest):
 Signs of LVF  1st line: β-B or rate-limiting CCB (NOT both!)
 2nd line: add digoxin (don’t use as monotherapy)
 3rd line: consider amiodarone
Ix
 ECG Mx of Permanent AF
 FBC, U+E, TFTs, Trop
 Failed cardioversion / unlikely to succeed
 Consider TTE: structural abnormalities  AF >1yr, valve disease, poor LV function
 Pt. doesn’t want cardioversion
 → Rate control
Acute AF (≤48h)
 Haemo unstable → emergency cardioversion Mx of Atrial Flutter
 (IV amiodarone 2nd line)  Manage as for AF
 Anti-AF drugs may not work, but try
Control ventricular rate  Amiodarone to restore sinus
 1st line: diltiazem or verapamil or metoprolol  Amiodarone or sotalol to maintain it
 2nd line: digoxin or amiodarone  Cavotricuspid isthmus ablation (RA) is Rx of choice.

Start LMWH CHA2-DS2-VAS Score

 Determines necessity of anticoagulation in AF
Cardioversion: only if acute AF <48hrs  Warfarin CI in AF
 Electrical cardioversion or pharmacological  Bleeding diathesis, ↓plats, BP > 160/90, poor
 1st: Flecainide (if no structural heart disease) compliance
 2nd: Amiodarone  Dabigatran may be cost-effective alternative.
 Long-term anticoagulation not needed if sinus restored
no RFs (0 CHADSVAS) + low recurrence risk. CHA2-DS2 VAS
 CCF  Vascular disease
 HTN  Age: 65-74yrs
Paroxysmal AF  Age≥75 (2 points)  Sex: female
 Self-limiting, <7d, recurs  DM
 Anticoagulate: use CHADSVAS  Stroke or TIA (2 points)
 Rx “pill-in-pocket” : flecainide, propafenone
 Prevention: β-B, sotalol or amiodarone Score
 0: aspirin 300mg
 ≥1: Warfarin

© Alasdair Scott, 2012 13

 Acute Coronary Syndromes
Definition Investigations
 ACS = unstable angina + evolving MI
 Divided into: ECG
 ST elevation or new onset LBBB  STEMI Sequence
 NSTEMI  Normal
 ST elevation + hyperacute (tall) T waves
 Q waves: full-thickness infarct
Epidemiology  Normalisation of ST segments
 T wave inversion
 Incidence 5/1000 for STEMI
 (New onset LBBB also = STEMI)
 NSTEMI
 ST depression
Pathophysiology  T wave inversion
 Plaque rupture, thrombosis and inflammation.  No Q waves = subendocardial infarct
 Rarely due to coronary spasm
View Leads Vessel Complication
Inferior II, III, aVF RCA Bradyarrhythmias
Risk Factors Anterolateral I, aVL, V4-V6 LCx
Anteroseptal V2-V4 LAD
Modifiable Non-modifiable Anterior V2-V6 LMS LVF
 HTN  Age Posterior V1, V2, V3 (recip) RCA
 DM  Male
 Smoking  FH (MI < 55yrs) Bloods
 ↑cholesterol  Troponin T/I
 Obesity  Myofibrillar proteins linking actin and myosin
 Elevated from 3-12h
Symptoms   need 12h trop to exclude MI
 Peak 24h
 Acute central/left chest pain >20min
 Baseline from 5-14d
 Radiates to left jaw or arm
 FBC, U+E, glucose, lipids, clotting
 Nausea
 Sweating
CXR
 Dyspnoea
 Cardiomegaly
 Palpitations
 Pulmonary oedema
 Widened mediastinum: aortic rupture
May get silent MIs in elderly/diabetics
 Syncope
 Delirium
 Post-op oliguria / hypotension Diagnosis
 STEMI / LBBB: Typical symptoms + ST elevation (/LBBB)
 NSTEMI: Typical symptoms + no ST elevation + +ve trop
 UA: Typical symptoms + no ST elevation + -ve trop
Signs
 Anxiety
 Pallor Sweating
 Pulse ↑/↓ Treatment
 BP ↑/↓  STEMI: PCI or thrombolysis
 4th heart sound  NSTEMI / UA: medical + elective angio ± PCI/CABG
 Signs of LVF (basal creps, ↑ JVP, 3rd HS)
 PSM: papillary muscle dysfunction / rupture
Prognosis
 Varies c̄ pt. factors
Differential  STEMI: 30 day mortality ~15%
 Angina  NSTEMI: overall mortality 1-2%
 Peri / endo / myocarditis
 Dissection
 PE, pneumothorax, pneumonia
 Costochondritis
 GI: e.g. GORD, spasm
 Anxiety

© Alasdair Scott, 2012 14

 MI Complications
Death Passing PRAED st.
Aneurysm: ventricular
Death: VF, LVF, CVA
Presentation
 4-6wk
Pump Failure  LVF
 Angina
Pericarditis  Recurrent VT
Presentation  Systemic emboli.
 Occurs early
 Mild fever ECG: persistent ST elevation.
 Central chest pain / change in pain
 Relieved by sitting forward Rx
 Pericardial friction rub  Anticoagulate
 Consider Excision
ECG
 Saddle-shaped ST elevation
 ± PR depression Embolism
 Arise from LV mural thrombus
Rx  Consider warfarin for 3mo after large anterior MI
 NSAIDs: ibuprofen
 Echo to exclude effusion
Dressler’s Syndrome: pleuro-pericarditis
 Due to auto-antibodies vs. myocyte sarcolemma.
Rupture: myomalacia cordis
 Cardiac tamponade: Left ventricular free wall rupture Presentation
 Beck’s triad (↓BP, ↑JVP, muffled heart sounds)  2-6wks
 Pulsus paradoxus  Recurrent pericarditis
 Papillary muscle / chordae → MR  Pleural effusions
 PSM  Fever
 Pulmonary oedema.  Anaemia
 Septum  ↑ESR
 PSM
 ↑JVP Rx
 Heart failure  NSAIDs
 Steroids if severe

Arrhythmias
Tachycardias
 SVT
 Sinus tachycardia – give O2 + analgesia
 AF or flutter
 Compromised → DC cardioversion
 Otherwise → rate control: digoxin ± β-B
 Ventricular
 Frequent PVC common after acute MI: no Rx
 Sustained VT
 Compromised → DC cardioversion
 Else → amiodarone or lignocaine
 May need pacing
 VF
 Early (<48hrs): reperfusion (good prog)
 Late (>48hrs): extensive heart damage
 Rx: DC shock

Bradycardias
 Sinus bradycardia
 esp. in inferior MI.
 Rx: atropine 0.6 – 1.2mg
 AV block
 Pace Mobitz II
 Ventricular bradycardia
 Suggests SA and AV node damage
© Alasdair Scott, 2012 15
 STEMI Management

12 lead ECG Primary Percutaneous Coronary Intervention

 Rx of choice if <12h
 Angioplasty and stenting
 + GP IIb/IIIa antagonist (tirofiban) if high risk
O2 2-4L aim for SpO2 94-98%  Delayed PCI, DM, complex procedure

Complications:
 Bleeding
IV access  Emboli
Bloods for FBC, U+E, glucose, lipids  Arrhythmia

Thrombolysis
Brief assessment  CI beyond 24hrs from pain onset
 Hx of CVD and risk factors
 Thrombolysis CIs ECG Criteria:
 CV exam  ST elevation > 1mm in 2+ limbs or > 2mm in 2+ chest leads.
 New LBBB
 Posterior: Deep ST “depression” and tall “R” waves in V1-V3

Antiplatelet Contraindications: AGAINST

 Aspirin 300mg PO (then 75mg/d)  Aortic dissection
 Clopidogrel 300mg PO (then 75mg/d)  GI bleeding
 Allergic reaction previously
 Iatrogenic: recent surgery
 Neuro: cerebral neoplasm or CVA Hx
Analgesia  Severe HTN (200/120)
 Morphine 5-10mg IV  Trauma, inc. CPR
 Metoclopramide 10mg IV st
Agents: 1 : streptokinase, alteplase (rt-PA), tenecteplase

Complications:
Anti-ischaemia  Bleeding
 GTN 2 puffs or 1 tablet SL  Stroke
 β-B atenolol 5mg IV (CI: asthma, LVF)  Arrhythmia
 Allergic reaction

Pts. not receiving any form of reperfusion therapy should be given

LMWH: e.g. enoxaparin IV then SC
fondaparinux.

Continuing Therapy: address risk factors

Admit to CCU for monitoring
 Arrhythmias
 Continue meds except CCBs
Primary PCI or Thrombolysis
Advice
 ACEi: start w/i 24hrs of MI (e.g. lisinopril 2.5mg)
 β-blocker: e.g. bisoprolol 10mg OD (or, CCB)
 Cardiac rehabilitation (group exercise and info) / Heart Manual
 DVT prophylaxis until fully mobile
 Continue for 3mo if large anterior MI
 Statin: regardless of basal lipids (e.g. atorvastatin 80mg)
 Stop smoking
 Diet: oily fish, fruit, veg, ↓ sat fats
 Exercise: 30min OD
 Work: return in 2mo
 Sex: avoid for 1mo
 Driving :avoid for 1mo

NB. Continue clopidogrel for 1mo following STEMI

Continue aspirin indefinitely.

© Alasdair Scott, 2012 16

 NSTEMI and UA Management

12 lead ECG + Admit to CCU

IVIV

O2 2-4L aim for SpO2 94-98%

IV access
Bloods for FBC, U+E, glucose, lipids,
Troponin

Brief Assessment
 Hx of CVD and risk factors
 CV exam

Antiplatelet
 Aspirin 300mg PO (then 75mg/d)
 Clopidogrel 300mg PO

Anticoagulate
 Fondaparinux 2.5mg SC

Analgesia
 Morphine 5-10mg IV
 Metoclopramide 10mg IV

Anti-ischaemia
 GTN: 2 puffs or 1 tablet SL
 β-B: atenolol 50mg/24h PO (CI: asthma, LVF)
 IV GTN if pain continues

Assess Cardiovascular Risk: GRACE/TIMI

Intermediate- to High-Risk Low-Risk

 Persistent/recurrent ischaemia, ST depression, DM,
positive trop.
 GPIIb/IIIa antagonist (tirofiban)
 Angiography (±PCI) w/i 96hrs
 Clopidogrel 75mg/d for one year
 No further pain, flat or inverted T waves or normal
ECG, negative trop.
 May discharge if 12h trop is negative.
 Outpatient tests: angio, perfusion scan, stress echo

Continuing Therapy: address risk factors

 ACEi (e.g. lisinopril 2.5mg)
 β-blocker (e.g. bisoprolol 10mg OD) or (CCB)
 Cardiac rehabilitation (group exercise and info) / Heart Manual
 Stop antithrombotic therapy when pain free (but give 3-5d)
 Statin (e.g. atorvastatin 80mg)

General advice as above

NB. Continue clopidogrel for 1yr following NSTEMI
Continue aspirin indefinitely.
© Alasdair Scott, 2012 17
 Angina Pectoris
Pathophysiology Mx
 Atherosclerosis → myocardial ischaemia
Lifestyle
Aetiology  Stop smoking
 Mostly atheroma.  Wt. loss and ↑ exercise
 Anaemia  Healthy diet: oily fish, fruit, veg, ↓ sat fats
 AS
 Tachyarrhythmias Medical
 Arteritis  2O Prevention: prevent cardiovascular events
 Aspirin 75mg OD
 ACEi (esp. if angina + DM)
Risk Factors  Statins: simvastatin 40mg
Modifiable: Non-modifiable:  Antihypertensives
 HTN  Age
 DM  Male  Anti-anginals: prevents angina episodes
 Smoking  FH (MI < 55yrs) 1. GTN (spray or SL) + either
 ↑cholesterol  Genetic: e.g. hyperlipidaemia  1st: β-B (e.g. Atenolol 50-100mg OD)
 Obesity  2nd: CCB (e.g. Verapamil 80mg TDS)
2. If either β-B or CCB doesn’t control symptoms,
Symptoms try the other option.
 Central chest tightness or heaviness 3. Can try β-B + dihydropyridine CCB
 e.g. amlodipine MR 10mg/24h
 Brought on by exertion, relieved by rest
4. If symptoms still not controlled
 May radiate to one/both arms, neck, jaw or teeth
 ISMN 20-40mg BD (8h washout @ PM) or
 Other ppts: emotion, cold weather, heavy meals slow-release nitrate (Imdur 60mg OD)
 Ivabradine (esp. if can’t take β-B)
Classification  Nicorandil 10-30mg BD
 Stable: induced by effort  Ranolazine
 Unstable: occurs at rest / minimal exertion
 Decubitus: induced by lying down
 Prinzmetal’s / variant: occurs during rest Interventional: PCI
 Due to coronary spasm  Indications
 ST elevation during attack: resolves as pain  Poor response to medical Rx
subsides.  Refractory angina but not suitable for CABG
 Rx: CCB + long-acting nitrate  Complications
 Syndrome X: angina pain + ST elevation on exercise  Re-stenosis (20-30% @ 6mo)
test but no evidence of coronary atherosclerosis.  Emergency CABG (<2%)
 Probably represents small vessel disease  MI (<2%)
 Death (<0.5%)
Differential  Clopidogrel ↓s risk of re-stenosis
 Bare metal stent: 1mo
 AS
 Drug-eluting (e.g. sirolimus) stent: 1yr
 Aortic aneurysm
 GI: GORD, spasm
 Musculoskeletal Surgical: CAGB
 Indications
Ix  L main stem disease
 Bloods: FBC, U+E, lipids, glucose, ESR, TFTs  Triple vessel disease
 ECG: usually normal  Refractory angina
 May show ST ↓, flat/inverted T waves, past MI  Unsuccessful angioplasty
 Consider exercise ECG  Complications
 Stress echo  MI
 Perfusion scan  Stroke
 CT coronary Ca2+ score  Pericardial tamponade or haemothorax
 Angiography (gold standard)  Postperfusion syn.
 Post-op AF
 Nonunion of sternum
 Graft stenosis

© Alasdair Scott, 2012 18

 Heart Failure: Concepts and Causes
Definition: CO is inadequate for the body’s requirements despite adequate filling pressures.

Epidemiology RVF
 Prev: 2% @ 50yrs → 10% @ 80yrs
Causes
 LVF
Pathophysiology  Cor pulmonale
 Tricuspid and pulmonary valve disease
Reduced CO initially → compensation
 Starling effect dilates heart to enhance contractility Symptoms
 Remodelling → hypertrophy  Anorexia and nausea
 RAS and ANP/BNP release
 Sympathetic activation Signs
 ↑JVP + jugular venous distension
Progressive ↓ in CO → decompensation  Tender smooth hepatomegaly (may be pulsatile)
 Progressive dilatation → impaired contractility +  Pitting oedema
functional valve regurgitation  Ascites
 Hypertrophy → relative myocardial ischaemia
 RAS activation → Na and fluid retention → ↑ venous
+

pressure → oedema LVF

 Sympathetic excess → ↑ afterload → ↓ CO
Causes
 1st: IHD
Low Output: CO ↓ and fails to ↑ c̄ exertion  2nd: idiopathic dilated cardiomyopathy
 3rd: Systemic HTN
1. Pump failure  4th: Mitral and aortic valve disease
 Systolic failure → impaired contraction  Specific cardiomyopathies
 Ischaemia/MI (commonest cause)
 Dilated cardiomyopathy Symptoms
 Hypertension  Fatigue
 Myocarditis  Exertional dyspnoea
 Orthopnoea + PND
 Diastolic failure → impaired filling  Nocturnal cough (± pink, frothy sputum)
 Pericardial effusion / tamponade / constriction  Wt. loss and muscle wasting
 Cardiomyopathy: restrictive, hypertrophic
Signs
 Arrhythmias  Cold peripheries ± cyanosis
 Bradycardia, heart block  Often in AF
 Tachycardias  Cardiomegaly c̄ displaced apex
 Anti-arrhythmics (e.g. beta-blocker, verapamil)  S3 + tachycardia = gallop rhythm
 Wheeze (cardiac asthma)
2. Excessive pre-load  Bibasal creps
 AR, MR
 Fluid overload

3. Excessive afterload Acute vs. Chronic

 AS  Acute
 HTN  New onset or decompensation of chronic
 HOCM  Peripheral / pulmonary oedema
 ± evidence of peripheral hypoperfusion
 Chronic
 Develops / progresses slowly
High Output: ↑ needs → RVF initially, then LVF  Venous congestion common
 Anaemia, AVM  Arterial pressure maintained until v. late
 Thyrotoxicosis, Thiamine deficiency (beri beri)
 Pregnancy, Paget’s

© Alasdair Scott, 2012 19

 Chronic Heart Failure
Dx of CCF: Framingham Criteria General Mx
 2 major criteria or 1 major + 2 minor
1O/2O Cardiovascular Risk
Major Minor  Stop smoking
 PND  Bilat ankle oedema  ↓ salt intake
 +ve abdominojugular reflux  SOBOE  Optimise wt.: ↑ or ↓ - dietician
 Neck vein distension  ↑HR >120  Supervised group exercised based rehab programme
 S3  Nocturnal cough  Aspirin
 Basal creps  Hepatomegaly  Statins
 Cardiomegaly  Pleural effusion
 Acute pulmonary oedema  30% ↓ vital capacity Rx Precipitants / Causes
 ↑ CVP (>16cmH2O)  Underlying cause
 Wt. loss >4.5kg in 5d 2O to Rx  Valve disease
 Arrhythmias
 Ischaemia
Ix  Exacerbating factors
 Bloods: FBC, U+E, BNP, TFTs, glucose, lipids  Anaemia
 CXR: ABCDE  Infection
 Alveolar shadowing  ↑BP
 Kerley B lines
 Cardiomegaly (cardiothoracic ratio >50%) Specific Mx
 Upper lobe Diversion  ACEi, β-B and spiro → ↓ mortality
 Effusions
 Fluid in the fissures 1st line: ACEi/ARB + β-B + loop diuretic
 ECG  ACEi/ARB: e.g. lisinorpil or candesartan
 Ischaemia  Hydralazine + ISDN if not tolerated
 Hypertrophy
 AF  β-B: e.g. carvedilol or bisoprolol (licensed)
 Echo: the key investigation  Start low, go slow
 Global systolic and diastolic function  E.g. carvedilol 3.125mg/12h → 25-50mg/24h
 Ejection fraction normally ~60%  Wait ≥2wks between increments
 Focal / global hypokinesia  Switch stable pts taking a β-B for a comorbidity
 Hypertrophy to a β-B licensed for heart failure
 Valve lesions  β-B therapy may be particularly good in COPD
 Intracardiac shunts
 Loop diuretic: frusemide or bumetanide
B-type Natriuretic Peptide: BNP or NTproBNP nd
 Secreted from ventricles in response to 2 line: get specialist advice
 ↑ pressure → stretch  Spironolactone / eplerenone
 Tachycardia  Watch K carefully (on ACEi too…)
 Glucocorticoids
 Thyroid hormones  ACEi + ARB
 Actions
 ↑ GFR and ↓ renal Na reabsorption  Vasodilators: hydralazine + ISDN
 ↓ preload by relaxing smooth muscle  Additional Rx in Blacks
 BNP is a biomarker of heart failure rd
 ↑ BNP (>100) better than any other variable and 3 line
clinical judgement in diagnosing heart failure  Digoxin
 BNP correlates c̄ LV dysfunction  Cardiac resynchronisation therapy ± ICD
 i.e. ↑ most in decompensated heart failure
 ↑ BNP = ↑ mortality Other Considerations
 BNP <100 excludes heart failure (96% NPV)  Monitoring
 BNP also ↑ in RHF: cor pulmonale, PE  BP: may be v. low
 Renal function
 Plasma K
New York Heart Association Classification  Daily wt.
1. No limitation of activity  Use amlodipine for comorbid HTN or angina
2. Comfortable @ rest, dyspnoea on ordinary activity  Avoid verapamil, diltiazem and nifedipine (short-
3. Marked limitation of ordinary activity acting)
4. Dyspnoea @ rest, all activity → discomfort
Invasive Therapies
 Cardiac resynchronisation ± ICD
 Intra-aortic balloon counterpulsation
 LVAD
 Heart transplant (70% 5ys)
© Alasdair Scott, 2012 20
 Severe Pulmonary Oedema

Sit pt. up Causes

I
II
Cardiogenic
 MI
O2
 Arrhythmia
 15L/min via reservoir mask
 Fluid overload: renal, iatrogenic
 Target SpO2: 94-98%
Non-cardiogenic
 ARDS: sepsis, post-op, trauma
IV access + monitor ECG  Upper airway obstruction
 Bloods for FBC, U+E, troponin, BNP, ABG  Neurogenic: head injury
 Rx any arrhythmias (e.g. AF)

Symptoms
 Dyspnoea
Diamorphine 2.5-5mg IV  Orthopnoea
+ Metoclopramide 10mg IV  Pink frothy sputum

Frusemide 40-80mg IV Signs

 Distressed, sweaty, cyanosed
 ↑HR, ↑RR
 ↑JVP
GTN 2 puffs or 2 x 300ug tabs SL
 S3 / gallop rhythm
Unless SBP <90mmHg
I
I  Bibasal creps
 Pleural effusions
 Wheeze (cardiac asthma)
Hx, Ex, Ix
 CXR: ABCDE
 ECG: MI, arrhythmias, pulsus alternans Differential
 Consider echo  Asthma/COPD
 Pneumonia
 PE
If SBP >100mmHg, start nitrate IVI
 ISMN 2-10mg/h IVI
 Keep SBP >90 Monitoring Progress
 BP
 HR and RR
If worsening, consider:  JVP
 CPAP  Urine Output
 More frusemide or ↑ nitrate infusion  ABG
 Haemofiltration / dialysis

If SBP <100mmHg: Rx as cardiogenic shock

 i.e. consider inotropes

Continuing Therapy
 Daily weights
 DVT prophylaxis
 Repeat CXR
 Change to oral frusemide or bumetanide
 ACEi + β-B if heart failure
 Consider spironolactone
 Consider digoxin ± warfarin (esp. if in AF)

Morphine in Pulmonary Oedema

 Make pt. more comfortable
 Pulm venodilators → ↓ pre-load → optimise position on Starling Curve
© Alasdair Scott, 2012 21
 Cardiogenic Shock
O2 Definition
 15L/min via reservoir mask  Inadequate tissue perfusion primarily due to
 Target SpO2: 94-98% cardiac dysfunction.
++
+++
++

Causes: MI HEART
IV access + monitor ECG  MI
 Bloods for FBC, U+E, troponin, ABG  Hyperkalaemia (inc. electrolytes)
 Endocarditis (valve destruction)
 Aortic Dissection
Diamorphine 2.5-5mg IV (pain/anxiety)  Rhythm disturbance
+ metoclopramide 10mg IV  Tamponade

Obstructive
 Tension pneumo
Correct any:
 Massive PE
 Arrhythmias
 Electrolyte disturbance
 Acid-base abnormalities Presentation
 Unwell: pale, sweaty, cyanosed, distressed
 Cold clammy peripheries
Hx, Ex, Ix  ↑RR ± ↑HR
 CXR  Pulmonary oedema
 Echo
 Consider CT thorax (dissection/PE)

Monitoring
 CVP, BP, ABG, ECG, urine output.

Consider need for dobutamine

Rx underlying cause

Tamponade

Causes: Signs: Ix:

 Trauma  Beck’s triad: ↓BP, ↑JVP, muffled heart sounds  Echo: diagnostic
 Lung/breast Ca  Kussmaul’s sign: ↑JVP on inspiration  CXR: globular heart
 Pericarditis  Pulsus paradoxus (pulse fades on inspiration)
 MI
 Bacteria (e.g. TB)

Mx:
 ABCs
 Pericardiocentesis (preferably under echo guidance)

© Alasdair Scott, 2012 22

 Hypertension
Definitions Management
 Stage 1: Clinic BP > 140/90  Do ABPM to confirm Dx before Rx (unless severe HTN)
 Stage 2: Clinic BP > 160/100
 Severe: Clinic BP > 180/110 Lifestyle interventions
 Malignant: BP > 180/110 + papilloedema and/or retinal  ↑ exercise
haemorrhage  ↓ smoking, ↓ EtOH, ↓ salt, ↓ caffeine
 Isolated SHT: SBP ≥140, DBP <90
Indications for Pharmacological Rx
Aetiology: PREDICTION  <80yrs, stage 1 HTN (>140/90) and one of:
 Primary: 95%  Target organ damage (e.g. LVH, retinopathy)
 Renal: RAS, GN, APKD, PAN  10yr CV risk ≥20%
 Endo: ↑T4, Cushing’s, phaeo, acromegaly, Conn’s  Established CVD
 Drugs: cocaine, NSAIDs, OCP  DM
 ICP ↑  Renal disease
 CoA  Anyone with stage 2 HTN (>160/100)
 Toxaemia of Pregnancy (PET)  Severe / malignant HTN (specialist referral)
 Increased viscosity  Consider specialist opinion if <40yrs with stage 1 HTN
 Overload with fluid and no end organ damage.
 Neurogenic: diffuse axonal injury, spinal section
BP Targets
 Under 80yrs: <140/90 (<130/80 in DM)
Aetiological clues  Over 80yrs: <150/90
 ↑HR: Thyrotoxicosis
 RF-delay: CoA CV Risk Mx
 Renal bruits: RAS  Statins indicated for 1 prevention if 10yr CVD risk ≥20%
O

 Palpable kidneys: APKD  Aspirin may be indicated: evaluate risk of bleeding

 Paroxysmal headache, tachycardia, sweating,
palpitations, labile or postural hypotension: phaeo
Antihypertensive Rx
End-organ damage: CANER < 55 > 55 / Black
Cardiac
 IHD 1: A C (or D)
 LVH → CCF
 AR, MR

Aortic 2: A + C (/D)
 Aneurysm
 Dissection
3: A+C+D
Neuro
 CVA: ischaemic, haemorrhagic
 Encephalopathy (malignant HTN)
4: Resistant HTN
 A+C+D+ consider further diuretic (e.g.
Eyes: hypertensive retinopathy
spiro) or α-blocker or β-B.
 Keith-Wagener Classification:
1. Tortuosity and silver wiring  Seek expert opinion
2. AV nipping
3. Flame haemorrhages and cotton wool spots A: ACEi or ARB
4. Papilloedema  e.g. lisinopril 10mg OD (↑ to 30-40mg)
 Grades 3 and 4 = malignant hypertension  e.g. candesartan 8mg OD (max 32mg OD)
C: CCB: e.g. nifedipine MR30-60mg OD
Renal D: Thiazide-like diuretic: e.g. chlortalidone 25-50mg OD
 Proteinuria
 CRF In step 2, use ARB over ACEi in blacks.
Avoid thiazides + β-B if possible (↑ risk of DM).
Ix Only consider β-B if young and ACEi/ARB not tolerated.
 24h ABPM
 Urine: haematuria, Alb:Cr ratio
 Bloods: FBC, U+Es, eGFR, glucose, fasting lipids Malignant HTN
 12 lead ECG: LVH, old infarct  Controlled ↓ in BP over days to avoid stroke
 Calculate 10yr CV risk  Atenolol or long-acting CCB PO
 Encephalopathy/CCF: fruse + nitroprusside / labetalol IV
 Aim to ↓ BP to 110 diastolic over ~4h
 Nitroprusside requires intra-arterial BP monitoring
© Alasdair Scott, 2012 23
 Aortic Stenosis
Causes Ix
 Senile calcification (60yrs +): commonest
 Congenital: Bicuspid valve (40-60yrs), William’s syn. Bloods: FBC, U+E, lipids, glucose
 Rheumatic fever
ECG
 LVH
Symptoms  LV strain: tall R, ST depression, T inversion in V4-V6
 Triad: angina, dyspnoea, syncope (esp. c̄ exercise)  LBBB or complete AV block (septal calcification)
 LVF: PND, orthopnoea, frothy sputum  May need pacing
 Arrhythmias
CXR
 Systemic emboli if endocarditis
 Calcified AV (esp. on lateral films)
 Sudden death
 LVH
 Evidence of failure
 Post-stenotic aortic dilatation
Signs
 Slow rising pulse c̄ narrow PP Echo + Doppler: diagnostic
 Aortic thrill  Thickened, calcified, immobile valve cusps
 Apex: Forceful, non-displaced (pressure overload)  Severe AS (AHA / ACC 2006 guidelines):
 Heart Sounds  Pressure gradient >40mmHg
 Quiet A2  Jet velocity >4m/s (or ↑ by 0.3m/s in a yr)
 Early syst. ejection click if pliable (young) valve  Valve area <1cm
2

 S4 (forceful A contraction vs. hypertrophied V)

 Murmur Cardiac Catheterisation + Angiography
 ESM  Can assess valve gradient and LV function
 Right 2nd ICS  Assess coronaries in all pts. planned for surgery
 Sitting forward in end-expiration
 Radiates to carotids Exercise Stress Test
 Contraindicated if symptomatic AS
 May be useful to assess ex capacity in asympto pts.
Clinical Indicators of Severe AS
 Quiet / absent A2 Mx
 S4
 Narrow pulse pressure Medical
 Decompensation: LVF  Optimise RFs: statins, anti-hypertensives, DM
 Monitor: regular f/up c̄ echo
 Angina: β-B
Differential  Heart failure: ACEi and diuretics
 Coronary artery disease  Avoid nitrates
 MR
 Aortic sclerosis Surgical
 Valve thickening: no pressure gradient  Poor prog. if symptomatic
 Turbulence → murmur  Angina/syncope: 2-3yrs
 ESM c̄ no radiation and normal pulse  LVF: 1-2yrs
 HOCM  Indications for valve replacement
 ESM murmur which ↑ in intensity c̄ valsalva (AS↓)  Severe symptomatic AS
 Severe asymptomatic AS c̄ ↓ EF (<50%)
 Severe AS undergoing CABG or other valve op
Aortic Stenosis Aortic Sclerosis  Valve types
Valve narrowing due to fusion of Valve thickening  Mechanical valves last longer but need
the commissures anticoagulation: young pts.
Narrow PP, slow rising pulse  Bioprosthetic don’t require anticoagulation but
Forceful apex fail sooner (10-15yrs)
ESM radiating → carotids ESM c̄ no radiation
ECG: LVF Options for unfit patients
 Balloon Valvuloplasty
 Limited use in adults as complication rate is
high (>10%) and restenosis occurs in 6-12mo
 Transcatheter Aortic Valve Implantation (TAVI)
 Folded valve deployed in aortic root.
 ↑ perioperative stroke risk cf. replacement
 ↓ major bleeding
 Similar survival @ 1yr
 Little Long-term data

© Alasdair Scott, 2012 24

 Aortic Regurgitation

Causes Ix

Acute Bloods: FBC, U+E, lipids, glucose

 Infective endocarditis
 Type A aortic dissection ECG: LVH (R6+S1 > 35mm)

Chronic CXR
 Congenital: bicuspid aortic valve  Cardiomegaly
 Rheumatic heart disease  Dilated ascending aorta
 Connective tissue: Marfan’s, Ehler’s Danlos  Pulmonary oedema
 Autoimmune: Ank spond, RA
Echo
 Aortic valve structure and morphology (e.g. bicuspid)
Symptoms  Evidence of infective endocarditis (e.g. vegetations)
 LVF: Exertional dyspnoea, PND, orthopnoea
 Severity
 Arrhythmias (esp. AF) → palpitations  Jet width (>65% of outflow tract = severe)
 Forceful heart beats  Regurgitant jet volume
 Angina  Premature closing of the mitral valve
 LV function: ejection fraction, end-systolic dimension
Signs
 Collapsing pulse (Corrigan’s pulse) Cardiac Catheterisation
 Wide PP  Coronary artery disease
 Apex: displaced (volume overload)  Assess severity, LV function, root size
 Heart Sounds
 Soft / absent S2
 ± S3 Mx
 Murmur
 EDM Medical
 URSE + 3rd left IC parasternal
 Optimise RFs: statins, anti-hypertensives, DM
 Sitting forward in end-expiration
 Monitor: regular f/up c̄ echo
 ± ejection systolic flow murmur
 ± Austin-Flint murmur  ↓ systolic hypertension: ACEi, CCB
 Underlying cause  ↓ afterload → ↓ regurgitation
 High-arched palate
 Spondyloarthropathy Surgery: aortic valve replacement
 Embolic phenomena  Definitive therapy
 Indicated in severe AR if:
 Symptoms of heart failure
Eponyms
 Asympto c̄ LV dysfunction: ↓EF/↑ES dimension
Corrigan’s sign: carotid pulsation
De Musset’s: head nodding
Quincke’s: capillary pulsation in nail beds
Traube’s: pistol-shot sound over femorals
Austin-Flint murmur
 Rumbling MDM @ apex due to regurgitant jet
fluttering the ant. mitral valve cusp.
 = severe AR
 Duroziez’s
 Systolic murmur over the femoral artery c̄ proximal
compression.
 Diastolic murmur c̄ distal compression

Clinical Indicators of Severe AR

 Wide PP and collapsing pulse
 S3
 Long murmur
 Austin Flint murmur
 Decompensation: LVF

© Alasdair Scott, 2012 25

 Mitral Stenosis
Causes Ix
 Rheumatic fever
 Prosthetic valve Bloods: FBC, U+E, LFTs, glucose, lipids
 Congenital (rare)
ECG
 AF
 P mitrale (if in sinus)
Pathophysiology  RVH c̄ strain: ST depression and T wave inversion in V1-V2
 Valve narrowing → ↑ left atrial pressure → loud S1
and atrial hypertrophy → AF CXR
 → pulmonary oedema and PHT → loud P2, PR  LA enlargement
 → RVH → left parasternal heave  Pulmonary oedema: ABCDE
 → TR → large v waves  Mitral valve calcification
 → RHF → ↑JVP, oedema, ascites
Echo + Doppler
 Severe MS (AHA 2006 Criteria)
2
Symptoms  Valve orifice <1cm
 Dyspnoea  Pressure gradient >10mmHg
 Fatigue  Pulmonary artery systolic pressure >50mmHg
 Chest pain  Use TOE to look for left atrial thrombus if intervention
 AF → palpitations + emboli considered.
 Haemoptysis: rupture of bronchial veins
Cardiac Catheterisation
 Assess coronary arteries
Signs
 Symptoms manifest when orifice <2cm2 (norm 4-6)
 AF, low volume pulse Mx
 Malar flush (↓CO → backpressure + vasoconstriction)
 JVP may be raised late on Medical
 Prominent a waves: PTH  Optimise RFs: statins, anti-hypertensives, DM
 Large v waves: TR  Monitor: regular f/up c̄ echo
 Absent a waves: AF  Consider prophylaxis vs. rheumatic fever: e.g. Pen V
 Left parasternal heave (RVH 2O to PHT)  AF: rate control and anticoagulate
 Apex: Tapping (palpable S1), non-displaced  Diuretics provide symptom relief
 Heart sounds
 Loud S1 Surgical
 Loud P2 (if PHT)  Indicated in mod–severe MS (asympto and symptomatic)
 Early diastolic opening snap  Percutaneous balloon valvuloplasty
 Murmur  Rx of choice
 Rumbling MDM  Suitability depends on valve characteristics
 Apex  Pliable, minimally calcified
 Left lateral position in end expiration  CI if left atrial mural thrombus
 Radiates to the axilla  Surgical valvotomy / commissurotomy: valve repair
O
 ± Graham Steell murmur (EDM 2 to PR)  Valve replacement if repair not possible

Clinical Indicators of Severe MS

 Mitral facies
 Longer murmur
 Opening snap closer to 2nd heart sound
 High LA pressure forcing valve open early
 Decompensation: RVF

Complications
 Pulmonary HTN
 Emboli: TIA, CVA, PVD, ischaemic colitis
 Hoarseness: rec laryngeal N. palsy = Ortner’s Syn
 Dysphagia (oesophageal compression)
 Bronchial obstruction

© Alasdair Scott, 2012 26

 Mitral Regurgitation

Causes Ix
 Mitral valve prolapse
Bloods: FBC, U+E, glucose, lipids
 LV dilatation: AR, AS, HTN
 Annular calcification → contraction (elderly) ECG
 Post-MI: papillary muscle dysfunction/rupture  AF
 Rheumatic fever  P mitrale (unless in AF)
 Connective tissue: Marfan’s, Ehlers-Danlos  LVH

Symptoms CXR
 Dyspnoea, fatigue  LA and LV hypertrophy
 AF → palpitations + emboli  Mitral valve calcification
 Pulmonary congestion → HTN + oedema  Pulmonary oedema

Signs Echo
 AF  Doppler echo to assess MR severity: multiple criteria
 Left parasternal heave (RVH)  Jet width (vena contracta) >0.6cm
 Apex: displaced  Systolic pulmonary flow reversal
 Volume overload as ventricle has to pump  Regurgitant volume >60ml
forward SV and regurgitant volume  TOE to assess severity and suitability of repair cf.
 → eccentric hypertrophy replacement.
 Heart Sounds
 Soft S1 Cardiac Catheterisation
 S2 not heard separately from murmur  Confirm Dx
 Loud P2 (if PTH)  Assess CAD
 Murmur
 Blowing PSM Mx
 Apex Medical
 Left lateral position in end expiration  Optimise RFs: statins, anti-hypertensives, DM
 Radiates to the axilla  Monitor: regular f/up c̄ echo
 AF: rate control and anticoagulate
Clinical Indicators of Severe MR  Also anticoagulate if: Hx of embolism, prosthetic
 Larger LV valve, additional MS
 Decompensation: LVF  Drugs to ↓ afterload can help ↓ symptoms
 AF  ACEi or β-B (esp. carvedilol)
 Diuretics
Differential
 AS Surgical
 TR  Valve replacement or repair
 VSD  Indications
 Severe symptomatic MR
 Severe asympto MR c̄ diastolic dysfunction: ↓EF

Mitral Valve Prolapse (Barlow Syndrome)

 Commonest valve prob. (~5%)

Causes Complications
 Primary: myxomatous degeneration  MR
 Often young women  Cerebral emboli
 MI  Arrhythmias → sudden death
 Marfan’s, ED
 Turner’s Mx
 β-B may relieve palpitations and chest pain
Symptoms  Surgery if severe (commonest reason for MV surgery)
 Usually asymptomatic
 Autonomic dysfunction: Atypical chest pain,
palpitations, anxiety, panic attack
 MR: SOB, fatigue

Signs
 Mid-systolic click ± late-systolic murmur

© Alasdair Scott, 2012 27

 Right Heart Valve Disease
Tricuspid Regurgitation Pulmonary Regurgitation

Causes Causes
 Functional: RV dilatation  Any cause of pulmonary HTN
 Rheumatic fever  PR 2O to MS = Graham-Steell murmur
 Infective endocarditis
 Carcinoid syndrome Signs
 Murmur: Decrescendo EDM @ ULSE
Symptoms
 Fatigue
 Hepatic pain on exertion
 Ascites, oedema Pulmonary Stenosis
Causes
Signs  Usually congenital: e.g. Turner’s, Fallot’s
 ↑JVP c̄ giant V waves  Rheumatic fever
 RV heave  Carcinoid syndrome
 Murmur:
 PSM Symptoms
 LLSE in inspiration (Carvallo’s sign)  Dyspnoea, fatigue
 Pulsatile hepatomegaly  Ascites
 Jaundice  Oedema

Ix Signs
 LFTs  Dysmorphia
 Echo  Large A wave
 RV heave
Mx  Ejection click, soft P2
 Rx cause  Murmur
 Medical: diuretics, ACEi, digoxin  ESM
 Surgical: valve replacement  ULSE → L shoulder

Ix
Tricuspid Stenosis  ECG
 P pulmonale
Causes  RAD
 Rheumatic fever (with MV and AV disease)  RBBB
 CXR:
Symptoms  Prominent pulmonary arteries: post-stenotic
 Fatigue dilatation
 Ascites  Catheterisation: diagnostic
 Oedema  Mx: valvuloplasty or valvotomy

Signs
 Large A waves
 Opening snap
 Murmur:
 EDM
 LLSE in inspiration

Mx
 Medical: diuretics
 Surgical: repair, replacement

© Alasdair Scott, 2012 28

 Infective Endocarditis
Definition Ix
 Cardiac valves develop vegetations composed of bacteria
and platelet-fibrin thrombus. Bloods
 N.chromic, N.cytic anaemia
Risk Factors  ↑ESR, ↑CRP
Cardiac disease → Subacute Normal valves → Acute  +ve IgG RF (immune phenomenon)
 Prosthetic valves  Dental caries  Cultures x 3, >12h apart
 Degen. valvulopathy  Post-op wounds  Serology for unusual organisms
 VSD, PDA, CoA  IVDU (tricuspid valve)
 Rheumatic fever  Immunocomp. (inc. DM) Urine: Micro haematuria

Aetiology ECG: AV block

Culture +ve Culture –ve Non-infective
Echo
 S. viridans (>35%)  Haemophilus  SLE
 TTE detects vegetations > 2mm
 S. bovis  Actinobacillus  Marantic
 TOE is more sensitive (90-100% vs. 50-60%)
 S. aureus  Cardiobacterium
 S. epidermidis  Eikenella
 Enterococci  Kingella
 Pseudomonas  Coxiella Rx
 Chlamydia  Empiric
 Acute severe: Fuclox + gent IV
 Subacute: Benpen + gent IV
Clinical Features
Sepsis Embolic phenomena
 Streps: benpen + gent IV
 Fever, rigors  Abscesses in brain, heart,
 Enterococci: amoxicillin + gent IV
 Night sweats kidney, spleen, gut and
 Staphs: fluclox ± rifampicin IV
 Wt. loss lung (if right-sided)
 Janeway lesions  Fungi: flucytosine IV + fluconazole PO.
 Anaemia
 Amphotericin if flucytosine resistance or
 Splenomegaly
Aspergillus.
 Clubbing Immune complex deposition
 Micro haematuria due to
GN Consider surgery if
Cardiac
 Heart failure
 New/changing murmur  Vasculitis
 Emboli
(MR: 85%, AR: 55%)  Roth spots
 Valve obstruction
 AV block  Splinter haemorrhages
 Prosthetic valve
 LVF  Osler’s nodes

Roth spots: boat-shaped retinal haemorrhages c̄ pale centre Prophylaxis

Janeway lesions: painless palmer macules  Abx prophylaxis solely to prevent IE not recommended
Osler’s nodes: painful, purple papules on finger pulps

Dx: Duke Criteria Mortality

 30% c̄ staphs
Major  14% c̄ bowel flora
1. +ve blood culture
 6% c̄ sensitive streps
 Typical organism in 2 separate cultures, or
 Persistently +ve cultures, e.g. 3, >12h apart
2. Endocardium involved
 +ve echo (vegetation, abscess, valve dehiscence)
or
 New valvular regurgitation

Minor
1. Predisposition: cardiac lesion, IVDU
2. Fever >38
3. Emboli: septic infarcts, splinters, Janeway lesions
4. Immune phenomenon: GN, Osler nodes, Roth spots, RF
5. +ve blood culture not meeting major criteria

Dx if:
 2 major
 1 major + 3 minor
 All 5 minor

© Alasdair Scott, 2012 29

 Rheumatic Fever

Aetiology Ix
 Group A β-haemolytic strep. (pyogenes)
Bloods
Epidemiology  Strep Ag test or ASOT
 5-15yrs  FBC, ESR/CRP
 Rare in West. Common in developing world. ECG
 Only 2% of population susceptible Echo

Pathophysiology
 Ab cross-reactivity following S. pyogenes infection → Rx
T2 hypersensitivity reaction (molecular mimicry).  Bed rest until CRP normal for 2wks
 Abs. vs. M protein in cell wall.  Benpen 0.6-1.2mg IM for 10 days
 Cross react c̄ myosin, muscle glycogen and SM cells.  Analgesia for carditis/arthritis: aspirin / NSAIDs
 Path: Aschoff bodies and Anitschkow myocytes.  Add oral pred if: CCF, cardiomegaly, 3rd degree block
 Chorea: Haldol or diazepam
Dx: revised Jones Criteria
 Evidence of GAS infection plus:
 2 major criteria, or Prognosis
 1 major + 2 minor  Attacks last ~ 3mo.
 60% c̄ carditis develop chronic rheumatic heart
Evidence of GAS infection disease.
 +ve throat culture  Recurrence ppted by
 Rapid strep Ag test  Further strep infection
 ↑ ASOT or DNase B titre  Pregnancy
 Recent scarlet fever  OCP
 Valve disease: regurgitation → stenosis
Major Criteria  Mitral (70%)
 Pancarditis  Aortic (40%)
 Arthritis  Tricuspid (10%)
 Subcutaneous nodules  Pulmonary (2%)
 Erythema marginatum
 Sydenham’s chorea
Secondary Prophylaxis
Minor criteria  Prevent recurrence
 Fever  Pen V 250mg/12h PO
 ↑ESR or ↑CRP  Carditis + valve disease: until 40yrs old
 Arthralgia (not if arthritis is major)  Carditis w/o valve disease:10yrs
 Prolonged PR interval (not if carditis is a major)  No carditis: 5yrs
 Prev rheumatic fever

Symptomatology
Pancarditis (60%)
 Pericarditis: chest pain, friction rub
 Myocarditis: sinus tachy, AV block, HF, ↑CK, T
inversion
 Endocarditis: murmurs
 MR, AR, Carey Coombs’ (MDM)

Arthritis (75%)
 Migratory polyarthritis of large joints (esp. knees)

Subcutaneous nodules (2-20%)

 Small mobile, painless nodules on extensor surfaces
(esp. elbows)

Erythema marginatum (2-10%)

 Red, raised edges c̄ central clearing.
 Trunk, thighs and arms.

Sydenham’s Chorea (10%)

 Occurs late
 Grimacing, clumsy, hypotonia (stops in sleep)

© Alasdair Scott, 2012 30

 Pericardial Disease

Acute Pericarditis Pericardial Effusion

Causes
Causes
 Any cause of pericarditis
 Viral: coxsackie, flu, EBV, HIV
 Bacterial: pneumonia, rheumatic fever, TB, staphs
Clinical Features
 Fungi  Dyspnoea
 MI, Dressler’s  ↑JVP (prominent x descent)
 Drugs: penicillin, isoniazid, procainamide, hydralazine
 Bronchial breathing @ left base
 Other: uraemia, RA, SLE, sarcoid, radiotherapy  Ewart’s sign: large effusion compressing left lower
lobe
Clinical Features  Signs of cardiac tamponade may be present.
 Central / retrosternal chest pain
 Sharp Ix
 Pleuritic
 CXR: enlarged, globular heart
 Worse lying down
 ECG
 Relieved by sitting forward
 Low-voltage QRS complexes
 Radiates to left shoulder
 Alternating QRS amplitude (electrical alterans)
 Pericardial friction rub.
 Echo: echo-free zone around heart
 Fever
 Signs of effusion / tamponade Mx
 Treat cause
Ix
 Pericardiocentesis may be diagnostic or therapeutic
 ECG: saddle-shaped ST-elevation ± PR depression
 Culture, ZN stain, cytology
 Bloods: FBC, ESR, trop (may be ↑), cultures, virology

Mx
 Analgesia: ibuprofen 400mg/8h PO
 Rx cause
 Consider steroids / immunosuppression

Constrictive Pericarditis
 Heart encased in a rigid pericardium. Tamponade
 Accumulation of pericardial fluid → ↑ intra-pericardial
Causes pressure → poor ventricular filling → ↓ CO
 Often unknown
 May occur after any pericarditis Causes
 Any cause of pericarditis
Clinical features  Aortic dissection
 RHF c̄ ↑JVP (prominent x and y descents)  Warfarin
 Kussmaul’s sign: ↑JVP c̄ inspiration  Trauma
 Quiet heart sounds
Signs
 S3
 Beck’s Triad: ↓ BP, ↑ JVP, quiet heart sounds
 Hepatosplenomegaly
 Pulsus paradoxus: pulse fades on inspiration
 Ascites, oedema
 Kussmaul’s sign
Ix
Ix
 CXR: small heart + pericardial calcification
 ECG: low-voltage QRS ± electrical alternans
 Echo
 CXR: large, globular heart
 Cardiac Catheterisation
 Echo: diagnostic, echo-free zone around heart
Mx
Mx
 Surgical excision
 Urgent pericardiocentesis
 20ml syringe + long 18G cannula
O
 45 , just left of xiphisternum, aiming for tip of left
scapula.
 Aspirate continuously and watch ECG.
 Treat cause
 Send fluid for cytology, ZN stain and culture

© Alasdair Scott, 2012 31

 Myocardial Disease
Acute Myocarditis Cardiac Myxoma
 Rare, benign cardiac tumour
Causes  F>M=2:1
 Idiopathic (~50%)  May be familial: e.g. Carney Complex
 Viral: coxsackie B, flu, HIV  Cardiac and cutaneous myxoma, skin
 Bacterial: S. aureus, syphilis pigmentation, endocrinopathy (e.g. Cushing’s)
 Drugs: cyclophosphamide, Herceptin, CBZ, phenytoin  90% in left atrium (fossa ovalis)
 Autoimmune: Giant cell myocarditis assoc. c̄ SLE  Features:
 Clubbing, fever, ↓wt., ↑ESR
Symptoms  Signs similar to MS (MDM, systemic emboli, AF)
 Flu-like prodrome: fever, sore throat, myalgia but vary c̄ posture.
 Dyspnoea, fatigue  Dx: Echo
 Chest pain (may coexist c̄ Bornholm disease)  Rx: Excision
 Arrhythmia → palpitations
Restrictive Cardiomyopathy
Signs
 Soft S1 Causes: miSSHAPEN
 S4 gallop  Sarcoid
 Systemic sclerosis
Ix  Haemochromatosis
 ECG  Amyloidosis
 ST-elevation or depression  Primary: endomyocardial fibrosis
 T wave inversion  Eosinophilia (Loffler’s eosinophilic endocarditis)
 Transient AV block  Neoplasia: carcinoid (→ TR and PS)
 Bloods: +ve trop, ↑CK
Clinical Features: as constrictive pericarditis
Mx
 Supportive Dx: Catheterisation
 Rx cause
Rx: Rx cause

Hypertrophic Obstructive Cardiomyopathy

 LVOT obstruction from asymmetric septal hypertrophy Dilated Cardiomyopathy
 AD inheritance (but 50% sporadic)
 β-myosin heavy chain mutation commonest Causes: DILATE
 Ask re family Hx of sudden death  Dystophy: muscular, myotonic, glycogen storage disease
 Infection: complication of myocarditis
Symptoms  Late pregnancy: peri-, post-partum
 Angina  Autoimmune: SLE
 Dyspnoea  Toxins: EtOH, doxorubicin, cyclophosphamide, DXT
 Palpitations: AF, WPW, VT  Endocrine: thyrotoxicosis
 Exertional syncope or sudden death
Presentation
Signs  LVF and RVF
 Jerky pulse  Arrhythmias
 Double apex beat
 Harsh ESM @ LLSE c̄ systolic thrill Signs
 S4  JVP ↑↑
 Displaced apex
Ix  S3 gallop
 ECG  ↓BP
 LVH/LAD/L strain,  MR/TR
 Ventricular ectopics, VT, VF
 Echo: ASH Ix
 Exercise test ± holter monitor to quantify risk  CXR: cardiomegaly, pulmonary oedema
 ECG: T inversion, poor progression
Mx  Echo: globally dilated, hypokinetic heart + ↓EF
 Medical  Catheter + biopsy: myocardial fibre disarray
st nd
 -ve inotropes: 1 – β-B, 2 –verapamil
 Amiodarone: arrhythmias Mx
 Anticoagulate if AF or emboli  Bed rest
 Non-medical  Medical: diuretics, ACEi, digoxin, anticoagulation
 Septal myomectomy (surgical or chemical) if  Non-medical: biventricular pacing, ICD
severe symptoms  Surgical: heart Tx
 Consider ICD
© Alasdair Scott, 2012 32
 Congenital Heart Disease
Bicuspid Aortic Valve Ventricular Septal Defect
 No problems @ birth  Hole connects ventricles
 Most eventually develop stenosis ± regurgitation
 Pre-disposes to IE/SBE Causes
 Congenital
 Acquired: post-MI
Atrial Septal Defect
 Hole connects the atria Presentation
 Secundum defects high in the septum are commonest  Severe HF in infancy or incidental discovery in later life
 Often asympto until adulthood
 LV compliance ↓ c̄ age → ↑ L→R shunt Signs
 Small holes which are haemodynamically less significant
Symptoms → louder murmurs
 Dyspnoea  Harsh, pansystolic murmur @ left sternal edge
 Pulmonary HTN  Systolic thrill
 Left parasternal heave
 Arrhythmia
 Larger holes → PHT
 Chest pain
Complications
Signs
 Infective endocarditis
 AF
 PHT
 ↑ JVP
 Eisenmenger’s
 Pulmonary ESM
 PHT → TR or PR
ECG
 Small: normal
Complications
 Large: LVH + RVH
 Paradoxical emboli
 Eisenmenger’s syndrome
CXR
 ↑ RA pressure: R→L shunt → cyanosis
 Small: mild pulmonary plethora
Ix  Large: cardiomegaly + marked pulmonary plethora
 ECG:
Rx
 Secundum: RAD
 Surgical closure indicated if symptomatic VSD c̄ large
 CXR: pulmonary plethora
shunt.
 Echo: diagnostic

Rx
 Transcatheter closure Fallot’s Tetralogy
 Recommended in adults if high pulmonary to systemic  Commonest congenital cyanotic heart defect
blood flow ratio (≥1.5:1)  Abnormal separation of truncus arteriosus into aorta and
pulmonary arteries.

Coarctation of the Aorta Pathology

 Congenital narrowing of the aorta 1. VSD
 Usually occurs just distal to origin of left subclavian 2. Pulmonary stenosis
 M>F 3. RVH
4. Overriding aorta
Associations
Associations
 Bicuspid aortic valve
 Di George: CATCH-22
 Turner’s syndrome
Presentation
Signs
 Infants: hypercyanotic episodes, squatting, clubbing
 Radio-femoral delay + weak femoral pulse
 Adult
 Hypertension
 Often asympto
 Systolic murmur / bruit heard best over left scapula
 Unoperated: cyanosis, ESM of PS
 Repaired: dyspnoea, palpitations, RVF
Complications
 Heart failure ECG: RVH + RBBB
 IE CXR: Coeur en sabot
Echo: anatomy + degree of stenosis
Ix
 CXR: rib notching Rx: Surgical (usually before 1yr)
 ECG: LV strain  Closure of VSD
 CT angiogram  Correction of the pulmonary stenosis
Rx: balloon dilatation + stenting
© Alasdair Scott, 2012 33
 Inherited Connective Tissue Disorders
Marfan’s Syndrome Ehlers-Danlos Syndrome
Epidemiology Pathogenesis
 Autosomal dominant  Rare heterogeneous group of collagen disorders.
 Spontaneous mutation in 25%  6 subtypes c̄ varying severity
 M=F  Commonest types (1 and 2) are autosomal dominant
 Prevalence = 1/5000
Presentation
Pathophysiology  Hyperelastic skin
 Mutation in FBN1 gene on Chr 5  Hypermobile joints
 Encodes fibrillin-1 glycoprotein  Cardiac: MVP, AR, MR and aneurysms
 Fibrillin-1 is an essential component of elastin  Fragile blood vessels → easy bruising, GI bleeds
 Histology: “cystic medial necrosis”  Poor healing

Presentation Differential Diagnosis

 Cardiac  Cutis Laxa: loose skin + hypermobile joints
 Aortic aneurysm and dissection  Pseudoxanthoma elasticum: skin laxity
 Aortic root dilatation → regurgitation  Marfan’s
 MV prolapse ± regurgitation
 Ocular
 Lens dislocation: superotemporal
 MSK
 High-arched palate
 Arachnodactyly
 Arm-span > height
 Pectus excavatum
 Scoliosis
 Pes planus
 Joint hypermobility

Complications
 Ruptured aortic aneurysm
 Spontaneous pneumothorax
 Diaphragmatic hernia
 Hernias

Dx
 Two 2/3 organ systems must be involved

Differential Diagnosis
 MEN-2b
 Homocystinuria
 Ehlers-Danlos

Ix
 Slit-lamp examination: ectopia lentis
 CXR
 Widened mediastinum
 Scoliosis
 Pneumothorax
 ECG
 Arrhythmias: premature atrial and ventricular
ectopics
 Echo
 Aortic root dilatation → AR
 MVP and MR
 MRI: dural ectasia (dilation of neural canal)
 Genetic testing: FBN-1 mutation

Mx
 Refer to ortho, cardio and ophtho
 Life-style alteration: ↓ cardiointensive sports
 Beta-blockers slow dilatation of the aortic root
 Regular cardiac echo
 Surgery when aortic root ≥5cm wide
© Alasdair Scott, 2012 34
Clubbing Cyanosis
Features and Stages Definition
1. Bogginess / ↑ fluctuance of nail bed  Blue discoloration of mucosal membranes or skin
2. Loss of concave nail fold angle  Deoxygenated Hb >5g/dl
3. ↑ longitudinal and transverse curvature
4. Soft tissue expansion at distal phalanx (drumstick) Classification
 Peripheral: cold, blue nails
Causes  Central: blue tongue, lips

Respiratory Causes
 Carcinoma  Think of O2 cascade
 Bronchial
 Mesothelioma
Respiratory
 Chronic lung suppuration  Hypoventilation: COPD, MSK
 Empyema, abscess
 ↓ diffusion: pulm oedema, fibrosing alveolitis
 Bronchiectasis, CF
 V/Q mismatch: PE, AVM (e.g. HHT)
 Fibrosis
 Idiopathic pulmonary fibrosis / CFA
Cardiac
 TB
 Congenital: Fallot’s, TGA
Cardiac  ↓ CO: MS, systolic LVF
 Infective Endocarditis  Vascular: Raynaud’s, DVT
 Congenital cyanotic heart disease
RBCs
 Atrial myxoma
 Low affinity Hb, may be hereditary or acquired
GIT
 Cirrhosis
 Crohn’s, uC
 Coeliac
 Cancer: GI lymphoma

Other
 Familial
 Thyroid Acropachy
 Upper limb AVMs or aneurysms
 Unilateral clubbing

© Alasdair Scott, 2012 36

 Pneumonia
Epidemiology Ix
 Incidence: 1/100  Bloods: FBC, U+E, LFT, CRP, culture, ABG (if ↓SpO2)
 Mortality: 10% in hospital, 30% in ITU  Urine: Ag tests (Pneumococcal, Legionella)
 Sputum: MC&S
Anatomic Classification  Imaging: CXR
 infiltrates, cavities, effusion
Bronchopneumonia  Special
 Patchy consolidation of different lobes  Paired sera Abs for atypicals
 Mycoplasma, Chlamydia, Legionella
Lobar Pneumonia  Immunofluorescence (PCP)
 Fibrosuppurative consolidation of a single lobe  BAL
 Congestion → red → grey → resolution  Pleural tap
\w ‘F .1‘
Severity: CURB-65 (only if x-ray changes)
 Confusion (AMT ≤ 8) Score
 Urea >7mM  0-1 → home Rx
 Resp. rate >30/min  2 → hospital Rx
 BP <90/60  ≥3 → consider ITU
 ≥65

Mx
 Abx
 O2: PaO2≥8, SpO2 94-98%
 Fluids
Aetiological Classification
 Analgesia
Community Acquired Pneumonia  Chest physio
 Pneumococcus, mycoplasma, haemophilus  Consider ITU if shock, hypercapnoea, hypoxia
 S. aureus, Moraxella, Chlamydia, Legionella  F/up @ 6wks c̄ CXR
 Viruses: 15%  Check for underlying Ca

Hospital Acquired Pneumonia Empirical Abx

 >48hrs after hospital admission
 Gm-ve enterobacteria, S. aureus CAP

Aspiration Mild amoxicillin 500mg TDS PO for 5d or

 ↑ Risk: stroke, bulbar palsy, ↓GCS, GORD, achalasia clarithro 500mg BD PO for 7d
 Anaerobes
Mod amoxicillin 500mg TDS and clarithro 500mg BD PO/IV
Immunocompromised (clarithro alone if pen allergy) for 7d
 The usual suspects, plus
 PCP, TB, fungi, CMV/HSV Sev Co-amoxiclav 1.2g TDS IV / cefuroxime 1.5g TDS IV
and clarithro 500mg BD IV for 7-10d
Add fluclox if staph suspected.

Symptoms Atyp Chlamydia: tetracycline

 Fever, rigors PCP: Co-trimoxazole
 Malaise, anorexia Legionella: Clarithro + rifampicin
 Dyspnoea
 Cough, purulent sputum, haemoptysis HAP
 Pleuritic pain  Mild / <5d: Co-amoxiclav 625mg PO TDS for 7d
 Severe / >5d: Tazocin ± vanc ± gent for 7d

Aspiration
Signs
 Co-amoxiclav 625mg PO TDS for 7d
 ↑RR, ↑ HR
 Cyanosis
 Confusion
Pneumovax (23 valent)
 ≥65yrs
 Consolidation
 ↓ expansion  Chronic HLKP failure or conditions
 Dull percussion  DM
 Bronchial breathing  Immunosuppression: hyposplenism, chemo, HIV
 ↓ air entry  CI: P, B, fever
 Crackles
 Pleural rub NB. revaccinate every 6yrs
 ↑VR
© Alasdair Scott, 2012 37
Complications of Pneumonia Systemic Inflammatory Response
Syndrome
Respiratory failure
 Type 1: PaO2 <8kPa + PaCO2 <6kPa Inflammatory response to a variety of insults
 Type 2: PaO2 <8kPa + PaCO2 >6kPa
 Mx: O2 therapy, ventilation
manifest by ≥2 of:
 Temperature: >38°C or <36°C
 Heart rate: >90
Hypotension
 Respiratory rate: >20 or PaCO2 <4.6 KPa
 Cause: dehydration + septic vasodilatation
 WCC: >12x109/L or <4 x109/L or >10% bands
 Mx
 If SBP<90 → 250ml fluid challenge over 15min
 If no improvement: central line + IV fluids
 If refractory: ITU for inotropes Sepsis
 SIRS caused by infection
AF
 Usually resolves c̄ Rx
 Mx: Digoxin or β-B for rate control Severe Sepsis
 Sepsis c̄ at least 1 organ dysfunction or hypoperfusion
Pleural effusion
 Exudate
 Mx: tap and send for MC+S, cytology and chemistry Septic Shock
 Severe sepsis with refractory hypotension
Empyema
 Pus in the pleural cavity
 Anaerobes, Staph, Gm-ve MODS
 Assoc. c̄ recurrent aspiration  Impairment of ≥2 organ systems
 Pt. c̄ resolving pneumonia develops recurrent fever  Homeostasis cannot be maintained without therapeutic
 Tap: turbid, pH<7.2, ↓glucose, ↑LDH intervention.
 Mx: US guided chest drain + Abx

Lung Abscess
Causes
 Aspiration
 Bronchial obstruction: tumour, foreign body
 Septic emboli: sepsis, IVDU, RH endocarditis
 Pulmonary infarction
 Subphrenic / hepatic abscess

Features
 Swinging fever
 Cough, foul purulent sputum, haemoptysis
 Malaise, wt. loss
 Pleuritic pain
 Clubbing
 Empyema

Tests
 Blood: FBC, ESR, CRP, cultures
 Sputum: micro, culture, cytology
 CXR: cavity c̄ fluid level
 Consider CT and bronchoscopy

Mx
 Abx according to sensitivities
 Aspiration
 Surgical excision

Other Complications
 Sepsis
 Pericarditis / myocarditis
 Jaundice
 Usually cholestatic
 Causes: sepsis, drugs (fluclox, Augmentin),
Mycoplasma, Legionella
© Alasdair Scott, 2012 38
 Specific Pneumonias
Organism Risk Factors Pulmonary Features Extrapulmonary Mx
Pneumococcus Elderly Lobar consolidation Herpes labialis Amoxicillin
EtOH Benpen
Immunosuppressed Cephalosporins
CHF
Pulmonary disease
S. aureus Influenza infection Bilateral cavitating Fluclox
IVDU bronchopneumonia Vanc
Co-morbidities
Klebsiella Rare Cavitating pneumonia Cefotaxime
Elderly Esp. upper lobes
EtOH
DM
Pseudomonas Bronchiectasis Taz
CF
Mycoplasma Epidemics Dry cough Flu-like prodrome Dx: serology
- headache
Reticulo-nodular shadowing or - myalgia/arthralgia Clarithro
patchy consolidation Cipro
Cold agglutinins → AIHA
Cryoglobulin
Erythema multiforme
SJS
GBS
Hepatitis
Legionella Travel Dry cough Flu-like prodrome Lymphopenia
Air conditioning Dyspnoea ↓Na+
Anorexia Deranged LFTs
Bi-basal consolidation D&V
Hepatitis Dx: Urinary Ag or
Renal Failure serology
Confusion
SIADH → ↓Na Clarithro ± rifa
Chlam. Pharyngitis, otitis  pneumonia Sinus pain Dx: serology
pneumoniae Clarithro
Chlam. psittaci Parrots Dry cough Horder’s spots ~ rose spots Dx: serology
Patchy consolidation Splenomegaly
Epistaxis Clarithro
Hepatitis, nephritis
Meningo-encephalitis
PCP Immunocompromised Dry cough Dx: visualisation
Exertional dyspnoea from BAL,
Bilateral creps sputum, biopsy

CXR: normal or bilateral perihilar High dose co-

interstitial shadowing trimoxazole or
pentamidine

Prophylaxis if
CD4<200 or after
st
1 attack

© Alasdair Scott, 2012 39

 Bronchiectasis
Pathophysiology Complications
 Chronic infection of bronchi/bronchioles → permanent  Pneumonia
dilatation.  Pleural effusion
 Retained inflammatory secretions and microbes →  Pneumothorax
airway damage and recurrent infection  Pulmonary HTN
 Organisms:  Massive haemoptysis
 H. influenza  Cerebral abscess
 Pneumococcus  Amyloidosis
 S. aureus
 Pseudomonas
Ix
 Sputum MCS
Causes
 Blood: Se Ig, Aspergillus precipitins, RF, α1-AT level
 Idiopathic in 50%
 Test Ig response to pneumococcal vaccine
 CXR: thickened bronchial walls (tramlines and rings)
Congenital
 Spirometry: obstructive pattern
 CF (mainly upper lobe infiltration)
 HRCT chest
 Kartagener’s / PCD
 Dilated and thickened airways
 Young’s syn. (azoopermia + bronchiectasis)
 Saccular dilatations in clusters c̄ pools of mucus
Post-infectious  Bronchoscopy + mucosal biopsy
 Measles  Focal obstruction
 PCD
 Pertussis
 CF sweat test (pilocarpine iontopheresis)
 Pneumonia
 TB
 Bronchiolitis
Rx
Immunodeficiency  Chest physio: expectoration, drainage, pulm. rehab
 Hypogammaglobulinaemia  Abx for exacerbations: e.g. cipro for 7-10d
 X-linked agammaglobulinaemia: Bruton’s  Bronchodilators: nebulised β agonists
 CVID  Treat underlying cause
 IgG subclass deficiency  CF: DNAase
 IgA deficiency  ABPA: Steroids
 Immune deficiency: IVIg
Other  Surgery may be indicated in severe localised disease
 Bronchial obstruction: LNs, tumour, foreign body
 ABPA
 RA
 UC
 Yellow nail syndrome
 Yellow nail discoloration and dystrophy
 Lymphoedema
 Pleural effusions
 Bronchiectasis

Symptoms
 Persistent cough c̄ purulent sputum
 Haemoptysis (may be massive)
 Fever, wt. loss

Signs
 Clubbing
 Coarse inspiratory creps
 Wheeze
 Purulent sputum
 Cause
 Situs inversus (+ PCD = Kartagener’s syn.)
 Splenomegaly: immune deficiency

© Alasdair Scott, 2012 40

 Cystic Fibrosis
Pathogenesis Ix
 Auto recessive, 1:2000 live Caucasian births  Bloods: FBC, LFTs, clotting, ADEK levels, glucose TT
 Mutation in CFTR gene on Chr 7 (commonly ∆F508)  Sputum MCS
 → ↓ luminal Cl secretion and ↑ Na reabsorption →  CXR: bronchiectasis
viscous secretions.  Abdo US: fatty liver, cirrhosis, pancreatitis
 In sweat glands, ↓ Cl and Na reabsorption → salty sweat  Spirometry: obstructive defect
 Aspergillus serology / skin test (20% develop ABPA)

Clinical Features
Mx
Neonate
 FTT General
 Meconium ileus  MDT: physician, GP, physio, dietician, specialist nurse
 Rectal prolapse
Chest
Children / Young Adults  Physio: postural drainage, forced expiratory techniques
 Nose: nasal polyps, sinusitis  Abx: acute infections and prophylaxis
 Resp: cough, wheeze, infections, bronchiectasis,  Mucloytics: DNAse
haemoptysis, pneumothorax, cor pulmonale  Bronchodilators
 GI:  Vaccinate
 Pancreatic insufficiency: DM, steatorrhoea
 Distal Intestinal Obstruction Syndrome GI
 Gallstones  Pancreatic enzyme replacement: pancreatin (Creon)
O
 Cirrhosis (2 biliary)  ADEK supplements
 Other: male infertility, osteoporosis, vasculitis
 Insulin
 Ursodeoxycholic acid for impaired hepatic function
Signs
 Stimulates bile secretion
 Clubbing ± HPOA
 Cyanosis Advanced Lung Disease
 Bilateral coarse creps  O2
 Diuretics (Cor pulmonale)
 NIV
Common Respiratory Organisms  Heart/lung transplantation
 Early
 S. aureus Other
 H. influenza  Rx of complications: e.g. DM
 Late  Fertility and genetic counselling
 P. aeruginosa: 85%  DEXA osteoporosis screen
 B. cepacia: 4%

Dx
 Sweat test: Na and Cl > 60mM
 Genetic screening for common mutations
 Faecal elastase (tests pancreatic exocrine function)
 Immunoreactive trypsinogen (neonatal screening)

© Alasdair Scott, 2012 41

 Pulmonary Aspergillus Infections
Diseases
1. Asthma: T1HS reaction to spores
2. ABPA
3. Aspergilloma (mycetoma)
4. Invasive aspergillosis
5. Extrinsic allergic alveolitis

Allergic Bronchopulmonary Aspergillosis Invasive Aspergillosis

 T1 and T3 HS reaction to Aspergillus fumigatus  Aflatoxins → liver cirrhosis and HCC (especially A.
 Bronchoconstriction → bronchiectasis flavus)

Symptoms Risk factors

 Wheeze  Immunocompromise: HIV, leukaemia, Wegener’s
 Productive cough  Post-broad spectrum Abx
 Dyspnoea
Ix
Ix  CXR: consolidation, abscess
 CXR: bronchiectasis  Sputum MCS
 Aspergillus in sputum (black on silver stain)  BAL
 Aspergillus skin test or IgE RAST  +ve se precipitins
 +ve se precipitins  Serial galactomannan
 ↑ IgE and ↑ eosinophils
Rx
Rx  Voriconazole (better cf amphotericin)
 Pred 40mg/d + itraconazole for acute attacks
 Pred maintenance 5-10mg/d Prog
 Bronchodilators for asthma  30% mortality

Aspergilloma (mycetoma) Extrinsic Allergic Alveolitis

 Fungus ball within a pre-existing cavity  Sensitivity to Aspergillus clavatus → Malt worker’s lung
 e.g. TB or sarcoid

Features
 Usually asympto
 Can → haemoptysis (may be severe)
 Lethargy, ↓wt.

Ix
 CXR: round opacity w/i a cavity, usually apical
 Sputum culture
 +ve se precipitins
 Aspergillus skin test / RAST

Rx
 Consider excision for solitary lesions / severe
haemoptysis

© Alasdair Scott, 2012 42

 Lung Cancer: Presentation
Classification
 Clinically, the most important distinction is between small-cell and non-small-cell (SCC, adeno and large-cell)

Type Epidemiology Pathology Behaviour

SCC 35% Centrally located Locally invasive
M>F Histo: Evidence of squamous differentiation Metastasise late (via LN)
Smoking - Keratinisation PTHrP → ↑ Ca2+
Radon gas
Adeno 25% Peripherally located Extrathoracic mets common and early.
Females, Histo: Glandular differentiation - 80% present with mets
Non-smokers - Gland formation
Far East - Mucin production
Large-cell 10% Peripheral or central Poor prognosis
Histo: Large, poorly differentiated cells
Small-cell 20% Central location, near bronchi 80% present c̄ advanced disease
Smoking Histo: Small, poorly differentiated cells V. chemosensitive but v. poor prog
Ectopic hormone secretion

Other Lung Tumours: all rare Complications

 Adenoma: 90% are carcinoid tumours
 Hamartoma Local
 Mesothelioma  Recurrent laryngeal N. palsy
 Phrenic N. palsy
 SVC obstruction
 Horner’s (Pancoast’s tumour)
Epidemiology
 AF
 19% of all cancers
 27% of cancer deaths (commonest) Paraneoplastic
 Endo
+
 ADH → SIADH ( euvolaemic ↓Na )
Symptoms  ACTH → Cushing’s syndrome
 Cough and haemoptysis  Serotonin → carcinoid (flushing, diarrhoea)
 Dyspnoea  PTHrP → 1O HPT (↑Ca2+, bone pain) – SCC
 Chest pain  Rheum
 Recurrent or slow resolving pneumonia  Dermatomyositis / polymyositis
 Anorexia and ↓wt.  Neuro
 Hoarseness  Purkinje Cells (CDR2) → cerebellar degeneration
 Peripheral neuropathy
 Derm
 Acanthosis nigricans (hyperpigmented body folds)
Signs  Trousseau syndrome: thrombophlebitis migrans
Chest
Metastatic
 Consolidation
 Pathological #
 Collapse
 Hepatic failure
 Pleural effusion
 Confusion, fits, focal neuro
 Addison’s
General
 Cachexia
 Anaemia
 Clubbing and HPOA (painful wrist swelling)
 Supraclavicular and/or axillary LNs

Metastasis
 Bone tenderness
 Hepatomegaly
 Confusion, fits, focal neuro
 Addison’s

© Alasdair Scott, 2012 43

 Lung Cancer: Investigation and Management
Ix Mx
 MDT: pulmonologist, oncologist, radiologist,
Bloods: FBC, U+E, Ca2+, LFTs histopathologist, cardiothoracic surgeon, specialist
Cytology: sputum, pleural fluid nurses, palliative care, GP
 Assess risk of operative mortality: e.g. Thoracoscore
Imaging  Cardiorespiratory function
 CXR  Co-morbidities
 Coin lesion  Advise smoking cessation
 Hilar enlargement
 Consolidation, collapse NSCLC
 Effusion  Surgical Resection
 Bony secondaries  Rx of choice for peripheral lesions c̄ no metastatic
 Contrast-enhanced Volumetric CT spread = stage I/II (~25%)
 Staging: lower neck, chest, upper abdomen  Need good cardiorespiratory function
 Consider CT brain  Wedge resection, lobectomy or pneumonectomy
 PET-CT: exclude distant mets  ± adjuvant chemo
 Radionucleotide bone scan  Curative radiotherapy
 If cardiorespiratory reserve is poor
Biopsy  Chemo ± radio for more advanced disease
 Percutaneous FNA: peripheral lesions and LNs  Platinum-based regimens
 Bronchoscopy: biopsy and assess operability  MAbs targeting EGFR (e.g. cetuximab) or TKI
 Endoscopic bronchial US biopsy: mediastinal LNS (e.g. erlotinib)
 Mediastinoscopy
SCLC
Lung Function Tests  Typically disseminated @ presentation
 Assess treatment fitness  May respond to chemo but invariably relapse

CXR Coin Lesion Differential Palliation

 Foreign body  Radio: bronchial obstruction, haemoptysis, bone or CNS
 Abscess: Staph, TB, Klebsiella, Mycetoma mets
 Neoplasia (1O or 2O)  SVC obstruction: stenting + radio + dexamethasone
 Granuloma: RA, Wegener’s, TB, Sarcoid  Endobronchial therapy: stenting, brachytherapy
 Structural: AVM  Pleural drainage / pleurodesis
 Analgesia

TNM Staging for NSCLC

Prognosis
Tx Malignant cells in bronchial secretions  NSCLC: 50% 5ys w/o spread; 10% c̄ spread
Tis CIS  SCLC: 1-1.5yrs median survival treated; 3mo untreated
T0 None evident
T1 ≤3cm, in lobar or more distal airway
T2 >3cm and >2cm from carina or pleural involvement
T3 <2cm from carina or involves chest wall, diaphragm
T4 Involves mediastinum or malignant effusion is present

N0 None involved
N1 Peribronchial or ipsilateral hilum
N2 Ipsilateral mediastinum
N3 Contralateral hilum or mediastinum or supraclavicular

© Alasdair Scott, 2012 44

 ARDS
Pathogenesis Causes
 May result from direct pulmonary insult or be 2O to
severe systemic illness. Pulmonary
 Inflam mediators → ↑ capillary permeability and non-  Pneumonia
cardiogenic pulmonary oedema.  Aspiration
 Inhalation injury
 Contusion
Clinical Features
 Tachypnoea Systemic
 Cyanosis  Shock
 Bilateral fine creps  Sepsis
 SIRS  Trauma
 Haemorrhage and multiple transfusions
 Pancrea0titis
Ix  Acute liver failure
 Bloods: FBC, U+E, LFTs, clotting, amylase, CRP,  DIC
cultures, ABG.  Obs: eclampsia, amniotic embolism
 CXR: bilateral perihilar infiltrates  Drugs: aspirin, heroin

Dx Differential Dx of Pulmonary Oedema

 Acute onset
 CXR shows bilateral infiltrates Tranusdates
 No evidence of congestive cardiac failure  ↑ capillary hydrostatic pressure
 CCF
 PaO2:FiO2 <200
 Iatrogenic fluid overload
 Renal failure
 Relative ↑ in negative pressure pulmonary
Mx oedema
 Admit to ITU for organ support and Rx underlying cause  ↓ capillary oncotic pressure
 Liver failure
Ventilation  Nephrotic syndrome
 Indications  Malnutrition, malabsorption, protein-losing
 PaO2<8KPa despite 60% FiO2 enteropathy
 PaCO2>6KPa  ↑ interstitial pressure
 Method  ↓lymphatic drainage: e.g. Ca
 6ml/kg + PEEP (e.g. 10cm H2O)
 SEs Exudates
 VILI  ARDS
 VAP
 Weaning difficulty

Circulation
 Invasive BP monitoring
 Maintain CO and DO2 c̄ inotropes
 E.g. norad or dobutamine
 RF may require haemofiltration

Sepsis
 Abx

Other
 Nutritional support: enteral (best), TPN

Prognosis
 50-75% mortality

© Alasdair Scott, 2012 45

 Respiratory Failure
Type 1 Clinical Features
 PaO2 <8KPa and PaCO2 <6KPa
 V/Q mismatch and diffusion failure Hypoxia
 Acute
 Dyspnoea
Type 2  Agitation
 PaO2 <8KPa and PaCO2 >6KPa  Confusion
 Alveolar hypoventilation ± V/Q mismatch  Cyanosis
 Chronic
 Polycythaemia
 PHT
Causes  cor pulmonale

V/Q Mismatch (↑A-a gradient) Hypercapnoea

 Vascular  Headache
 PE  Flushing and peripheral vasodilatation
 PHT  Bounding pulse
 Pulmonary Shunt (R → L)  Flap
 Asthma (early)  Confusion → coma
 Pneumothorax
 Atelectasis
Mx: Rx underlying cause
Alveolar Hypoventilation
Type 1
 Obstructive
 Give O2 to maintain SpO2 94-98%
 COPD
 Asthma  Assisted ventilation if PaO2<8KPa despite 60% O2
 Bronchiectasis
 Bronchiolitis Type 2
 Intra- and Extra-thoracic (Ca, LN, epiglottitis…)  Controlled O2 therapy @ 24% O2 aiming for SpO2 88-92%
 Restrictive and a PaO2 >8kPa
 ↓ drive: CNS sedation, trauma, tumour  Check ABG after 20min
 NM disease: cervical cord lesion, polio, GBS,  If PaCO2 steady or lower can ↑ FiO2 if necessary
MG  If PaCO2 ↑>1.5KPa and pt. still hypoxic, consider
 Chest: flail, kyphoscoliosis, obesity NIV or respiratory stimulant (e.g. doxapram)
 Fluid and fibrosis

Diffusion Failure (↑A-a gradient)

Oxygen Therapy
 Fluid
 Pulmonary oedema Principles
 Pneumonia  Critically ill pts. should receive high conc O2 immediately.
 Infarction  O2 should be prescribed to achieve target SpO2
 Blood  94-98% for most patients
 Fibrosis  88-92% for those at risk or hypercapnic resp failure
 In pts. at risk of hypercapnic resp failure:
NB. Both fluid and fibrosis also → V/Q mismatch and  Start O2 therapy at 24% and do an ABG
alveolar hypoventilation due to ↓ compliance – Blue Venturi @ 2-4L/min
 Clinically: ↓ RR c̄ O2 may be useful sign
 If PCO2 ≤6kPa: ↑ target SpO2 to 94-98%
A-a Gradient  If PCO2 >6kPa: maintain target SpO2
 PAO2= (95 x FiO2) – (PaCO2/0.8)
 ↑ (PAO2-PaO2) suggests lung pathology Mechanisms
Nasal Prongs: 1-4L/min = 24-40% O2

Simple Face Mask

Non-rebreathing Mask
 Reservoir bag allows delivery of high concentrations of O2.
 60-90% at 10-15L

Venturi Mask
 Provide precise O2 concentration at high flow rates
 Yellow: 5%
 White: 8%
 Blue: 24%
 Red: 40%
 Green: 60%
© Alasdair Scott, 2012 46
 Chronic Asthma
Definition Differential
 Episodic, reversible airway obstruction due to  Pulmonary oedema (cardiac asthma)
bronchial hyper-reactivity to a variety of stimuli.  COPD

Epidemiology Ix
 Incidence 5-8% (↑ in children vs. adults)
 Peaks at 5yrs, most outgrow by adolescence Bloods
 FBC (eosinophila)
 ↑IgE
Pathophysiology
 Aspergillus serology
Acute (30min)
 Mast cell-Ag interaction → histamine release
CXR: hyperinflation
 Bronchoconstriction, mucus plugs, mucosal swelling
Spirometry
Chronic (12h)
 Obstructive pattern c̄ FEV1:FVC < 0.75
 TH2 cells release IL-3,4,5 → mast cell, eosinophil and
 ≥15% improvement in FEV1 c̄ β-agonist
B cell recruitment
 Airway remodelling
PEFR monitoring / diary
 Diurnal variation >20%
Causes  Morning dipping
Atopy Atopy: skin-prick, RAST
 T1 hypersensitivity to variety of antigens
 Dust mites, pollen, food, animals, fungus
Mx
Stress
 Cold air General Measures: TAME
 Viral URTI  Technique for inhaler use
 Exercise  Avoidance: allergens, smoke (ing), dust
 Emotion  Monitor: Peak flow diary (2-4x/d)
 Educate
Toxins  Liaise c̄ specialist nurse
 Smoking, pollution, factory  Need for Rx compliance
 Drugs: NSAIDS, β-B  Emergency action plan

Symptoms Drug Ladder

 Cough ± sputum (often at night)
 Wheeze 1 SABA PRN
 Dyspnoea  If use >1/d or nocte symptoms → step 2
 Diurnal variation c̄ morning dipping
2 Low-dose inhaled steroid: beclometasone 100-400ug bd
Hx
3 LABA: salmeterol 50ug bd
 Precipitants
 Good response: continue
 Diurnal variation
 Benefit but control still poor: ↑ steroid to 400ug bd
 Exercise tolerance
 No benefit: discontinue + ↑ steroid to 400ug bd
 Life effects: sleep, work
 Other atopy: hay fever, eczema If control is still poor consider trial of:
 Home and job environment  Leukotriene receptor antagonist (e.g. monetelukast)
 Esp. if exercise- / NSAID-induced asthma
Signs  SR Theophylline
 Tachypnoea, tachycardia
 Widespread polyphonic wheeze 4 Trials of
 Hyperinflated chest  ↑ inhaled steroid to up to 1000ug bd
 ↓ air entry  Leukotriene receptor antagonist
 Signs of steroid use  SR Theophylline
 MR β agonist PO

Associated Disease 5 Oral steroids: e.g. prednisolone 5-10mg od

 GORD  Use lowest dose necessary for symptom control
 Churg-Strauss  Maintain high-dose inhaled steroid
 ABPA  Refer to asthma clinic

© Alasdair Scott, 2012 47

 Acute Severe Asthma
Presentation Mx
 Acute breathlessness and wheeze
O2, Nebs and Steroids
Hx 1. Sit-up
 Precipitant: infection, travel, exercise? 2. 100% O2 via non-rebreathe mask (aim for 94-98%)
 Usual and recent Rx? 3. Nebulised salbutamol (5mg) and ipratropium (0.5mg)
 Previous attacks and severity: ICU? 4. Hydrocortisone 100mg IV or pred 50mg PO (or both)
 Best PEFR? 5. Write “no sedation” on drug chart

Ix
 PEFR If Life Threatening
 ABG  Inform ITU
 PaO2 usually normal or slightly ↓  MgSO4 2g IVI over 20min
 PaCO2 ↓  Nebulised salbutamol every 15min (monitor ECG)
 If PaCO2 ↑: send to ITU for ventilation
 FBC, U+E, CRP, blood cultures
If Improving
Assessment  Monitor: SpO2 @ 92-94%, PEFR
 Continue pred 50mg OD for 5 days
Severe: any one of
 Nebulised salbutamol every 4hrs
 PEFR <50%
 RR >25
 HR >110
 Can’t complete sentence in one breath IV Rx if No Improvement in 15-30min:
 Nebulised salbutamol every 15min (monitor ECG)
Life Threatening: any one of
 Continue ipratropium 0.5mg 4-6hrly
 PEFR <33%
 MgSO4 2g IVI over 20min
 SpO2 <92%, PCO2 >4.6kPa, PaO2 <8kPa
 Salbutamol IVI 3-20ug/min
 Cyanosis
 Consider aminophylline
 Hypotension
 Load: 5mg/kg IVI over 20min
 Exhaustion, confusion
 Unless already on theophylline
 Silent chest, poor respiratory effort  Continue: 0.5mg/kg/hr
 Tachy-/brady-/arrhythmias  Monitor levels
 ITU transfer for invasive ventilation
Differential
 Pneumothorax
 Acute exacerbation of COPD
 Pulmonary oedema Monitoring
 PEFR every 15-30min
 Pre- and post-β agonist
Admission Criteria
 SpO2: keep >92%
 Life-threatening attack
 ABG if initial PaCO2 normal or ↑
 Feature of severe attack persisting despite initial Rx
 May discharge if PEFR > 75% 1h after initial Rx

Discharge When
 Been stable on discharge meds for 24h
 PEFR > 75% c̄ diurnal variability < 20%

Discharge Plan
 TAME pt.
 PO steroids for 5d
 GP appointment w/i 1 wk.
 Resp clinic appointment w/i 1mo

© Alasdair Scott, 2012 48

 COPD
Definition Ix
 Airway obstruction: FEV1 <80%, FEV1:FVC <0.70  BMI
 Chronic bronchitis: cough and sputum production on  Bloods: FBC (polycythaemia), α1-AT level, ABG
most days for 3mo of 2 successive years.  CXR
 Emphysema: histological diagnosis of enlarged air  Hyperinflation (> 6 ribs anteriorly)
spaces distal to terminal bronchioles c̄ destruction of  Prominent pulmonary arteries
alveolar walls.  Peripheral oligaemia
 Bullae
Epidemiology  ECG:
 R atrial hypertrophy: P pulmonale
 Prevalence: 10-20% of >40s
 RVH, RAD
 Spirometry: FEV1 <80%, FEV1:FVC <0.70, ↑TLC, ↑RV
Causes  Echo: PHT
 Smoking
 Pollution
 α1ATD Chronic Rx
Assess Severity
Symptoms  Mild: FEV1 >80% (but FEV/FVC <0.7 and symptomatic)
 Cough + sputum  Mod: FEV1 50-79%
 Dyspnoea  Severe: FEV1 30-49%
 Wheeze  Very Severe: FEV1 < 30%
 Wt. loss
General Measures
Signs  Stop smoking
 Tachypnoea  Specialist nurse
 Prolonged expiratory phase  Nicotine replacement therapy
 Hyperinflation  Bupropion, varenicline (partial nicotinic agonist)
 ↓Cricosternal distance (normal = 3 fingers)  Support programme
 Loss of cardiac dullness  Pulmonary rehabilitation / exercise
 Displaced liver edge  Rx poor nutrition and obesity
 Wheeze  Screen and Mx comorbidities
 May have early-inspiratory crackles  e.g. cardiovasc, lung Ca, osteoporosis
 Cyanosis  Influenza and pneumococcal vaccine
 Cor pulmonale: ↑JVP, oedema, loud P2  Review 1-2x/yr
 Signs of steroid use  Air travel risky if FEV1<50%

Pink Puffers in emPhysema Mucolytics

 ↑ alveolar ventilation → breathless but not cyanosed
 Normal or near normal PaO2
 Normal or low PaCO2
 Progress → T1 respiratory failure
Blue Bloaters in chronic Bronchitis
 ↓ alveolar ventilation → cyanosed but not breathless
 ↓PaO2 and ↑ PaCO2: rely on hypoxic drive
 Progress → T2 respiratory failure and cor pulmonale
 Consider if chronic productive cough
 E.g. Carbocisteine (CI in PUD)
Breathlessness and/or exercise limitation
 SABA and/or SAMA (ipratropium) PRN
 SABA PRN may continue at all stages
Exacerbations or persistent breathlessness
 FEV1 ≥50%: LABA or LAMA (tiotropium) (stop SAMA)
 FEV1 <50%: LABA+ICS combo or LAMA

Persistent exacerbations or breathlessness

mMRC Dyspnoea Score  LABA+LAMA+ICS
1. Dyspnoea only on vigorous exertion
 Roflumilast / theophylline (PDIs) may be considered
2. SOB on hurrying or walking up stairs
 Consider home nebs
3. Walks slowly or has to stop for breath
4. Stops for breath after <100m / few min
LTOT
5. Too breathless to leave house or SOB on dressing
 Aim: PaO2 ≥8 for ≥15h / day (↑ survival by 50%)
 Clinically stable non-smokers c̄ PaO2 <7.3 (stable on
two occasions >3wks apart)
Complications
 PaO2 7.3 – 8 + PHT / cor pulmonale / polycythaemia /
 Acute exacerbations ± infection
nocturnal hypoxaemia
 Polycythaemia
 Terminally ill pts.
 Pneumothorax (ruptured bullae)
 Cor Pulmonale Surgery
 Lung carcinoma  Recurrent pneumothoraces
 Isolated bullous disease
 Lung volume reduction
© Alasdair Scott, 2012 49
 Acute Exacerbation of COPD
Causes Management
 Viral URTI (30%)
 Also bacterial infections
Controlled O2 Therapy
 Sit-up
Presentation  24% O2 via Venturi mask: SpO2 88-92%,
 Cough + sputum  Vary FiO2 and SpO2 target according to ABG
 Breathlessness  Aim for PaO2 >8 and ↑ in PCO2 of <1.5kPa
 Wheeze

Hx
 Smoking status Nebulised Bronchodilators
 Exercise capacity  Air driven c̄ nasal specs
 Current treatment  Salbutamol 5mg/4h
 Previous exacerbations  Ipratropium 0.5mg/6h

Ix
 PEFR
 Bloods: FBC, U+E, ABG, CRP, cultures Steroids (IV and PO)
 Sputum culture  Hydrocortisone 200mg IV
 CXR: infection, pneumothorax  Prednisolone 40mg PO for 7-14d
 ECG

Differential
Abx
 Pneumothorax
 If evidence of infection
 Pulmonary oedema
 Doxy 200mg PO STAT then 100mg OD PO for 5d
 PE
 Asthma
NIV if no response:
Discharge  Repeat nebs and consider aminophylline IV
 Spirometry  Consider NIV (BiPAP) if pH<7.35 and/or RR >30
 Establish optimal maintenance therapy  Consider invasive ventilation if pH<7.26
 GP and specialist f/up  Depends on pre-morbid state: exercise capacity,
 Prevention using home oral steroids and Abx home O2, comorbidity
 Pneumococcal and Flu vaccine
 Home assessment

© Alasdair Scott, 2012 50

 Pulmonary Embolism
Causes Management
 Usually arise from DVTs in proximal leg or iliac veins
 Rarely:
O2
 Right ventricle post MI
 Sit-up
 Septic emboli in right sided endocarditis
 100% O2 via non-rebreather mask
Risk Factors: SPASMODICAL
 Sex: F
 Pregnancy Analgesia
 Age: ↑  Morphine ± metoclopramide if distressed
 Surgery (classically 10d post-op straining at stool)
 Malignancy
 Oestrogen: OCP/HRT If critically ill c̄ massive PE consider thrombolysis
 DVT/PE previous Hx
 Immobility
 Alteplase 50mg bolus stat
 Colossal size  (Surgical or interventional embolectomy)
 Antiphospholipid Abs
 Lupus Anti-coagulant
LMWH Heparin
Presentation e.g. enoxaparin 1.5mg/kg/24h SC
 Symptoms and signs depend on size, number and
distribution of emboli

Symptoms SBP?
 Dyspnoea
 Pleuritic pain
 Haemoptysis
<90 >90
 Syncope
 500ml colloid  Start Warfarin
 Confirm Dx
Signs
 Fever
 Cyanosis
 Tachycardia, tachypnoea Inotropes if BP still ↓
 RHF: hypotension, ↑JVP, loud P2  Dobutamine: aim for SBP >90
 Evidence of cause: DVT  Consider addition of NORAD
 Consider thrombolysis (medical or surgical)
Ix
 Bloods: FBC, U+E, clotting, D-dimers
 ABG: normal or ↓PaO2 and ↓PaCO2, ↑pH On-going Mx
 CXR: normal or oligaemia, linear atelectasis
 ECG: sinus tachycardia, RBBB, right ventricular strain  TEDS stockings in hospital
(inverted T in V1-V4)  Graduated compression stockings for 2yrs if DVT:
 S1, Q3, T3 is rare prevent post-phlebitic syndrome (10-30%)
 Doppler US: thigh and pelvis (+ve in 60%)  Continue LMWH until INR >2 (at least 5d)
 CTPA + venous phase of legs and pelvis  Target INR = 2-3
 85-95% sensitivity  Duration
 V/Q scan no longer used  Remedial cause: 3mo
 No identifiable cause: 6mo
Dx  On-going cause: indefinite
1. Assess probability using Wells’ Score  VC filter if repeat DVT/PE despite anticoagulation
2. Low-probability → perform D-dimers
 Negative → excludes PE
 Positive → CTPA
3. High probability → CTPA

NB. –ve D-dimer has 95% NPV for PE

Prevention
 Risk assessment for all pts
 TEDS
 Prophylactic LMWH
 Avoid OCP/HRT if @ risk

© Alasdair Scott, 2012 51

 Pneumothorax
Definition Mx
 Accumulation of air in the pleural space c̄ 2O lung
collapse.
Tension PTX
 Resuscitate pt.
Classification  No CXR
 Closed: intact chest wall and air leaks from lung into  Large bore Venflon into 2nd ICS, mid-clavicular line
pleural cavity.
 Insert ICD
 Open: defect in the chest wall allows communication
between PTX and exterior: may be sucking.
 Tension: air enters pleural cavity through one-way valve Traumatic
and cannot escape → mediastinal compression.  Resuscitate pt.
 Analgesia: e.g. morphine
Causes  3-sided wet dressing if sucking
Spontaneous  Insert ICD

7
 1O: no underlying lung disease
 Young, thin men (ruptured subpleural bulla)
 Smokers
1O PTX
 2O: underlying lung disease
 COPD SOB and/or rim ≥2cm?
No
Consider d/c
 Marfan’s, Ehler’s Danlos II
 Pulmonary fibrosis, sarcoidosis Yes
Yes

Trauma Aspiration successful?

 Penetrating
 Blunt ± rib #s No

Iatrogenic Insert ICD

 Subclavian CVP line insertion
 Positive pressure ventilation
 Transbronchial biospy 2O PTX
 Liver biopsy Yes
SOB and > 50yrs and Insert ICD
rim ≥2cm?
Presentation II
No No
Symptoms
 Sudden onset Aspiration successful?
 Dyspnoea
 Pleuritic chest pain Yes
 Tension: respiratory distress, cardiac arrest
Admit for 24h
Signs
 Chest
 ↓ expansion
 Resonant percussion
 ↓ breath sounds
 ↓VR
 Tension: ↑JVP, mediastinal shift, ↑HR, ↓BP
 Crepitus: surgical emphysema

Ix
 ABG
 US
 CXR (expiratory film may be helpful)
 Translucency + collapse (2cm rim = 50% vol loss)
 Mediastinal shift (away from PTX)
 Surgical emphysema
 Cause: rib #s, pulmonary disease (e.g. bullae)

© Alasdair Scott, 2012 52

 Pleural Effusion
Classification Ix
 Effusion protein < 25g/L = transudate  Blood: FBC, U+E, LFT, TFT, Ca, ESR
 Effusion protein >35g/L = exudate  CXR
 Between 25-35g/L: apply Light’s Criteria  Blunt costophrenic angles
 Dense shadow c̄ meniscus
Light’s Criteria  Mediastinal shift away
 An exudate has one of:  Cause: coin lesion, cardiomegaly
 Effusion : serum protein ratio >0.5  US: facilitates tapping
 Effusion : serum LDH ratio >0.6  Volumetric CT
 Effusion LDH is 0.6 x ULN
Diagnostic Tap
 Percuss upper boarder and go 1-2 spaces below
Causes  Infiltrate down to pleura c̄ lignocaine.
 Aspirate c̄ 21G needle
Exudates: ↑ capillary permeability  Send for
 Infection: pneumonia, TB  Chemistry: protein, LDH, pH, glucose, amylase
 Neoplasm: bronchial, lymphoma, mesothelioma  Bacteriology: MCS, auramine stain, TB culture
 Inflammation: RA, SLE  Cytology
 Infarction  Immunology: SF, ANA, complement

Empyema, Ca, TB, RA and SLE

Transudates: ↑ capillary hydrostatic or ↓ oncotic pressure  ↑ protein
 CCF  ↓ glucose <3.3mM
 Renal failure  ↓ pH <7.2
 ↓ albumin: nephrosis, liver failure, enteropathy  ↑ LDH > 0.6 x serum / ULN
 Hypothyroidism
 Meig’s Syndrome Oesophageal Rupture
 Right pleural effusion  ↓ pH <7.2
 Ascites  ↑ amylase (also ↑ in pancreatitis)
 Ovarian fibroma
Pleural Biopsy
 If pleural fluid is inconclusive
Presentation  CT-guided c̄ Abrams needle

Symptoms
 Asymptomatic Mx
 Dyspnoea
 Rx underlying cause
 Pleuritic chest pain
 May use drainage if symptomatic (≤2L/24h)
 Repeated aspiration or ICD
Signs
 Chemical pleurodesis if recurrent malignant effusion
 Chest
 Persistent effusions may require surgery
 Tracheal deviation away from effusion
 ↓ expansion
 Stony dull percussion
 ↓ air entry
 Bronchial breathing just above effusion
 ↓VR
 Associated disease
 Ca: cachexia, clubbing, HPOA, LNs, radiation
burn, radiation tattoo
 Chronic liver disease
 Cardiac failure
 RA, SLE
 Hypothyroidism

© Alasdair Scott, 2012 53

 Sarcoidosis
Definition Ix
 Multisystem granulomatous disorder of unknown cause
Bloods
Epidemiology  ↑ESR
 Age: 20-40yrs  ↑Ca
 Sex: F>M  Lymphopenia
 Geo: Afro Caribbean  ↑ se ACE
 HLA-DRB1 and DQB1 alleles  ↑ Ig
 ↑LFTs
Clinical Features: GRAULOMAS CXR, CT, MRI
 In 20-40% the disease presents incidentally on CXR
 Acute sarcoidosis: EN, fever, polyarthralgia, BHL Tuberculin skin test
 Negative in 2/3
General
 Fever Lung function tests
 Anorexia and ↓ wt.  Restrictive pattern c̄ ↓FVC
 Fatigue  ↓ transfer factor
 Lymphadenopathy and HSM
Tissue Biopsy
Respiratory  Lung, LNs, skin nodules, liver
 Upper: otitis, sinusitis  Diagnostic: non-caseating granulomas
 Lower (seen in 90%)
 Dry cough, SOB, chest pain, ↓ lung function Ophthalmology assessment
 Stage 1: BHL
 Stage 2: BHL + peripheral infiltrates
 Stage 3: Peripheral infiltrates alone
 Stage 4: Progressive mid-zone fibrosis c̄ bullae
Rx
 Pts. c̄ asymptomatic BHL do not require Rx.
Arthralgia  Acute sarcoidosis
 Polyarthralgia  Usually resolves spontaneously
 Bed rest and NSAIDs
 Dactylitis
 Chronic sarcoidosis
Neurological  Steroids: pred 40mg/d for 4-6wks
 Additional immunosuppression: methotrexate,
 Peripheral and cranial polyneuropathy (esp. Bell’s palsy)
ciclosporin, cyclophosphamide
 Meningitis, transverse myelitis, SOL

Urine
 ↑Ca → Renal stones, nephrocalcinosis, DI
Prognosis
Low Hormones  60% c̄ thoracic sarcoidosis resolve over 2yrs
 Pituitary dysfunction: e.g. amenorrhoea  20% respond to steroids
 20% no improvement despite Rx
Ophthalmological
 Uveitis
 Keratoconjunctivitis BHL Differential
 Sicca / Mikulicz / Sjogrens syndrome  Sarcoidosis
 Infection: TB, mycoplasma
Myocardial  Malignancy: lymphoma, carcinoma
 Restrictive cardiomyopathy 2 to granulomas + fibrosis
O
 Interstitial disease: EAA, silicosis
 Pericardial effusion

Abdominal Granulomatous Disease Differential

 Hepatomegaly + cholestatic LFTs  Infections: TB, leprosy, syphilis, crypto, schisto
 Splenomegaly  AI: PBC
 Vasculitis: GCA, PAN, Wegener’s, Takayasu’s
Skin  Idiopathic: Crohns, Sarcoid
 EN: painful erythematous nodules on shins (paniculitis)  Interstitial lung: EAA, silicosis
 Lupus pernio: raised, dusky purple plaque on nose,
cheek, fingers

© Alasdair Scott, 2012 54

 Interstitial Lung Disease
Principal Features Cause by Location
 Dyspnoea
 Dry cough Upper Zone: A PENT
 Abnormal CXR / CT  Aspergillosis : ABPA
 Restrictive Spirometry  Pneumoconiosis: Coal, Silica
 Extrinsic allergic alveolitis
 Negative, sero-arthropathy
Causes  TB

Known Cause Lower Zone: STAIR

 Environmental: asbestosis, silicosis  Sarcoidosis (mid zone)
 Drugs: BANS ME  Toxins: BANS ME
 Bleomycin, Busulfan  Asbestosis
 Amiodarone  Idiopathic pulmonary fibrosis
 Nitrofurantoin  Rheum: RA, SLE, Scleroderma, Sjogren’s, PM/DM
 Sulfasalazine
 MEthotrexate, MEthysergide
 Hypersensitivity: EAA
 Infection: TB, viral, fungi

Assoc. c̄ systemic disease

 Sarcoidosis
 RA
 SLE, systemic sclerosis, Sjogren’s, MCTD
 UC, ankylosing spondylitis

Idiopathic
 Idiopathic pulmonary fibrosis (CFA)

© Alasdair Scott, 2012 55

Extrinsic Allergic Alveolitis Idiopathic Pulmonary Fibrosis (CFA)
 Acute allergen exposure in sensitised pts. → T3HS
 Chronic exposure → granuloma formation and Epidemiology
obliterative bronchiolitis (T4HS)  Commonest cause of interstitial lung disease
 Middle age
Causes  M>F = 2:1
 Bird fancier’s lung: proteins in bird droppings  Assoc. c̄ other AI disease in 30%
 Farmer’s / mushroom worker’s
 Malt worker’s lung: Aspergillus clavatus Presentation

Clinical Features Symptoms

 Dry cough
4-6h post-exposure  Dyspnoea
 Fever, rigors, malaise  Malaise, wt. loss
 Dry cough, dyspnoea  Arthralgia
 Crackles (no wheeze)  OSA

Chronic Signs
 Increasing dyspnoea  Cyanosis
 Wt. loss  Clubbing
 T1 respiratory failure  Crackles: fine, end-inspiratory
 Cor pulmonale
Complications
Ix  ↑ risk Ca lung
 Bloods  Type 2 respiratory failure and cor pulmonale
 Acute: neutrophilia, ↑ESR
 +ve se precipitins Ix
 CXR
 Upper zone reticulonodular opacification or Bloods
fibrosis → honeycomb lung  ↑CRP
 BHL (rare)  ↑Ig
 Spirometry  ANA+ (30%)
 Restrictive defect  RF+ (10%)
 ↓ transfer factor during acute attacks  ABG: ↓PaO2, ↑PaCO2
 BAL
 ↑ lymphocytes and mast cells CXR
 ↓ lung volume
Mx  Bilat lower zone retic-nod shadowing
 Avoid exposure  Honeycomb lung
 Steroids: acute / long-term
 Compensation may be payable HRCT
 Shows similar changes to CXR
 More sensitive

Industrial Lung Disease Spiro

 Restrictive defect
 ↓ transfer factor
Coal-workers Pneumoconiosis
 CWP → Progressive Massive Fibrosis Other
 Presents as progressive dyspnoea and chronic  Lung biopsy: Usual interstitial pneumonia
bronchitis  BAL: may indicate disease activity
 CXR: upper zone fibrotic masses  ↑ lymphocytes: good prognosis
 ↑ PMN or eosinophils: bad prognosis
Silicosis  DTPA scan: may reflect disease activity
 Quarrying, sand-blasting
 Upper zone reticular shadowing and egg shell Mx
calcification of hilar nodes → PMF  Supportive care
 Stop smoking
Asbestosis  Pulmonary rehabilitation
 Demolition and ship building  O2 therapy
 Basal fibrosis, pleural plaques  Palliation
 ↑ risk of mesothelioma  Rx symptoms of heart failure
 Chest pain, wt. loss, clubbing, recurrent effusions,  Lung Tx offers only cure
dyspnoea.
 CXR: pleural effusions, thickening Prognosis
 Dx by histology of pleural biopsy  50% 5yr survival
 <2yr survival
© Alasdair Scott, 2012 56
Pulmonary Hypertension Cor Pulmonale
Definition Definition
 PA pressure >25mmHg  RHF due to chronic PHT

Causes Symptoms
 Dyspnoea
Left Heart Disease  Fatigue
 Mitral stenosis  Syncope
 Mitral regurgitation
 Left ventricular failure
 L → R shunt Signs
1. ↑ JVP c̄ prominent a wave
Lung Parenchymal Disease 2. Left parasternal heave
 Mechanism 3. Loud P2 ± S3
 Chronic hypoxia → hypoxic vasoconstriction 4. Murmurs
 Perivascular parenchymal changes  PR: Graham Steell EDM
 COPD  TR: PSM
 Asthma: severe, chronic 5. Pulsatile hepatomegaly
 Interstitial lung disease 6. Fluid: Ascites + Peripheral oedema
 CF, bronchiectasis

Pulmonary Vascular Disease Ix

 Idiopathic pulmonary hypertension  Bloods: FBC, U+E, LFTs, ESR, ANA, RF
 Pulmonary vasculitis: scleroderma, SLE, Wegener’s  ABG: hypoxia ± hypercapnoea
 Sickle cell  CXR
 Pulmonary embolism: acute or chronic  Enlarged R atrium and ventricle
 Portal HTN: portopulmonary HTN  Prominent pulmonary arteries
 Peripheral oligaemia
Hypoventilation  ECG: P pulmonale + RVH
 OSA  Echo: RVH, TR, ↑ PA pressure
 Morbid obesity (Pickwickian syn.)  Spirometry
 Thoracic cage abnormality: kyphosis, scoliosis  Right heart catheterisation
 Neuromuscular: MND, MG, polio

Mx
Ix  Rx underlying condition
 ECG  ↓ pulmonary vascular resistance
 P pulmonale  LTOT
 RVH  CCB: e.g. nifedipine
 RAD  Sildenafil (PDE-5 inhibitor)
 Echo  Prostacycline analogues
 Velocity of tricuspid regurgitation jet  Bosentan (endothelin receptor antagonist)
 Right atrial or ventricular enlargement  Cardiac failure
 Ventricular dysfunction  ACEi + β-B (caution if asthma)
 Valve disease  Diuretics
 Right heart catheterisation: gold standard  Heart-Lung Tx
 Mean pulmonary artery pressure
 Pulmonary vascular resistance
 CO Prognosis
 Vasoreactivity testing to guide Rx  50% 5ys

© Alasdair Scott, 2012 57

Obstructive Sleep Apnoea Smoking Cessation
Definition Very Brief Advice
 Intermittent closure/collapse of pharyngeal airway →  Ask: enquire as to smoking status
apnoeic episodes during sleep.  Advise: best way to stop is with support and medication
 Act: provide details of where to get help
 E.g. NHS stop smoking helpline
Risk Factors
 Obesity
 Male Facilitating Quitting
 Smoker  Refer to specialist stop smoking service
 EtOH  Nicotine replacement
 Idiopathic pulmonary fibrosis  Gum
 Structural airway pathology: e.g. micrognathia  Patches
 NM disease: e.g. MND  Varenicline: selective partial nicotine receptor agonist
 Recommended by NICE
 23% abstinence @ 1yr vs. 10% for placebo
 Start while still smoking
Ix  Bupropion: also an option
 SpO2
 Polysomnography is diagnostic

Rx
 Wt. loss
 Avoid smoking and EtOH
 CPAP during sleep

Clinical Features
Nocturnal
 Snoring
 Choking, gasping, apnoeic episodes

Daytime
 Morning headache
 Somnolence
 ↓ memory and attention
 Irritability, depression

Complications
 Pulmonary hypertension
 Type 2 respiratory failure
 Cor pulmonale

Mx
 ↓ wt.
 Stop smoking
 CPAP @ night via a nasal mask
 Surgery to relieve pharyngeal obstruction
 Tonsillectomy
 Uvulopalatpharyngoplasty

© Alasdair Scott, 2012 58

DM: Classification, Dx and Causes DM: Conservative Mx
Definition MDT
 Multisystem disorder due to an absolute or relative lack of  GP, endocrinologist, surgeons, specialist nurses,
endogenous insulin → metabolic and vascular dieticians, chiropodists, fellow patients (education
complications. groups).

Monitoring: 4Cs
Classification
Control, glycaemic
T1DM  Record of complications: DKA, HONK, hypos
 Path: autoimmune destruction of β-cells → absolute  Capillary blood glucose
insulin deficiency.  Fasting: 4.5-6.5mM
 Age: usually starts before puberty  2h post-prandial: 4.5-9mM
 Presentation: polyuria, polydipsia, ↓wt., DKA  HbA1c
 Genetics: concordance only 30% in MZs  Reflects exposure over last 6-8wks
 Assoc.: HLA-D3 and –D4, other AI disease  Aim <45 - 50mM (7.5 - 8%)
 Abs: anti-islet, anti-GAD  BP, lipids

T2DM Complications
 Path: insulin resistance and β-cell dysfunction → relative  Macro
insulin deficiency  Pulses
 Age: usually older patients  BP
 Presentation: polyuria, polydipsia, complications  Cardiac auscultation
 Genetics: concordance 80% in MZs  Micro
 Assoc.: obesity, ↓exercise, calorie and EtOH excess  Fundoscopy
 ACR + U+Es
 Sensory testing plus foot inspection
Dx
Competency
 Symptomatic: Polyuria, polydipsia, ↓wt., lethargy
 With insulin injections
 ↑ plasma venous glucose detected once
 Checking injection sites
 Fasting ≥7mM
 BM monitoring
 Random ≥11.1mM
 Asymptomatic
Coping
 ↑ venous glucose on 2 separate occasions
 Psychosocial: e.g. ED, depression
 Or, 2h OGTT ≥ 11.1mM
 Occupation
 Domestic
Glucose Testing
 OGTT only needed if borderline fasting or random
Lifestyle Modification: DELAYS
glucose measurements.
Diet
Normal IFG IGT Diabetes  Same as that considered healthy for everyone
Fasting <6.1 6.1 – 6.9 ≥7.0  ↓ total calorie intake
75g OGTT <7.8 7.8 – 11 ≥11.1  ↓ refined CHO, ↑ complex CHO
 ↑ soluble fibre
 ↓ fat (especially saturated)
Secondary Causes of DM  ↓ Na
 Avoid binge drinking
 Drugs: steroids, anti-HIV, atypical neuroletics, thiazides
 Pancreatic: CF, chronic pancreatitis, HH, pancreatic Ca
Exercise
 Endo: Phaeo, Cushings, Acromegaly, T4
 Other: glycogen storage diseases Lipids
 Rx of hyperlipidaemia
 1 prevention c̄ statins if >40yrs (regardless of lipids)
O
Metabolic Syndrome
 Central obesity (↑ waist circumference) and two of: ABP
 ↑ Triglycerides  ↓ Na intake and EtOH
 ↓ HDL  Keep BP <130/80
 HTN
 ACEis best (β-B: mask hypos, thiazides: ↑ glucose)
 Hyperglycaemia: DM, IGT, IFG
Aspirin
 1 prevention if >50yrs or <50 c̄ other CVD RFs
O

Yearly / 6 monthly check-up: 4Cs

Smoking cessation
© Alasdair Scott, 2012 60
DM: Oral Hypoglycaemics
1. Lifestyle Modification: DELAYS
2. Start Metformin
(if HBA1c >target after lifestyle changes)
 SE: nausea, diarrhoea, abdo pain, lactic acidosis
 CI: GFR<30, tissue hypoxia (sepsis, MI), morning
before GA and iodinated contrast media
 500mg after evening meal, ↑ing to 2g max.
3. Metformin + Sulfonylurea
(if HBA1c >target)
 E.g. gliclazide MR 30mg c̄ breakfast
 SE: hypoglycaemia, wt. gain
 CI: omit on morning of surgery
Other Options
 Consider adding a rapid-acting insulin
secretagogue (e.g. nateglinide) to metformin
instead of a sulfonylurea.
 May be preferable if erratic lifestyle.
 Consider adding pioglitazone to metformin instead
of a sulfonylurea
4. Additional Therapy
1st line
 Add insulin → insulin + metformin + sulfonylurea
2nd line
 Add sitagliptin or pioglitazone if insulin
unacceptable
 Employment, social or recreational issues
 Obesity
 metformin + sulfonylurea + sitagliptin / pio
3rd line
 Add exenatide (SC) if insulin unacceptable or BMI>35
 metformin + sulfonylurea + exenatide
4th line
 Consider acarbose if unable to use other glucose-
lowering drugs
© Alasdair Scott, 2012
Insulin
Principles
 Ensure pt. education about
 Self-adjustment c̄ exercise and calories
 Titrate dose
 Family member can abort hypo c̄ sugary drinks
or GlucoGel
 Pre-prandial BM don’t tell you who much glucose is
needed
 Fasting BM before meal informs re long-acting insulin
dose.
 Finger-prick BM after meal informs re short-acting
insulin dose (for that last meal)
Common Regimes
BD Biphasic Regime
 BD insulin mixture 30min before breakfast and dinner
 Rapid-acting: e.g. actrapid
 Intermediate- / long-acting: e.g. insulatard
 T2 or T1 DM c̄ regular lifestyle: children, older pts.
 Assoc, c̄ fasting hyperglycaemia
Basal-Bolus Regime
 Bedtime long-acting (e.g. glargine) + short acting
before each meal (e.g. lispro)
 Adjust dose according to meal size
 ~50% of insulin given as long-acting
 T1DM allowing flexible lifestyle
 Best outcome
OD Long-Acting Before Bed
 Initial regime when switching from tablets in T2DM
Illness
 Insulin requirements usually ↑ (even if food intake ↓)
 Maintain calories (e.g. milk)
 Check BMs ≥4hrly and test for ketonuria
 ↑ insulin dose if glucose rising
Side-Effects
 Hypoglycaemia
 At risk: EtOH binge, β-B (mask symptoms),
elderly
 Need to admit sulfonylurea-induced hypo
 Lipohypertrophy
 Rotate injection site: abdomen, thighs
 Wt. gain in T2DM
 ↓ wt. gain if insulin given c̄ metformin
61
 Diabetic Complications
Summary Retinopathy, Maculopathy and Cataracts
 Hyperglycaemia: DKA, HONK
 Hypoglycaemia Pathogenesis
 Infection  Microvascular disease → retinal ischaemia → ↑VEGF
 Macrovascular: MI, CVA  ↑ VEGF → new vessel formation
 Microvascular
Presentation
 Retinopathy and maculopathy
Macrovascular  Cataracts
 MI: May be “silent” due to autonomic neuropathy
 Rubeosis iris: new vessels on iris → glaucoma
 PVD: claudication, foot ulcers
 CN palsies
 CVA
Diabetic Retinopathy and Maculopathy
Rx: Manage CV risk factors
 Commonest cause of blindness up to 60yrs
 BP (aim <130/80)
 Refer if pre-proliferative retinopathy / maculopathy
 Smoking
 Ix: fluorescein angiography
 Lipids
 Rx: laser photocoagulation
 HBA1c
Background Retinopathy
Prevention  Dots: microaneurysms
 Good glycaemic control (e.g. HbA1c <6%) prevents  Blot haemorrhages
both macro- and micro-vascular complications.  Hard exudates: yellow lipid patches
 Proved by DCCT, EDIC and UKPDS trials Pre-proliferative Retinopathy
 Regular screening: fundoscopy, ACR, foot check  Cotton-wool spots (retinal infarcts)
 Venous beading
Diabetic Feet  Haemorrhages
Proliferative Retinopathy
 New vessels
Ischaemia
 Pre-retinal or vitreous haemorrhage
 Critical toes
Maculopathy
 Absent pulses (do ABPI)  ↓ acuity may be only sign
 Ulcers: painful, punched-out, foot margins, pressure  Hard exudates w/i one disc width of macula
points

Neuropathy Neuropathy
 Loss of protective sensation
 Deformity: Charcot’s joints, pes cavus, claw toes Pathophysiology
 Injury or infection over pressure points  Metabolic: glycosylation, ROS, sorbitol accumulation
 Ulcers: painless, punched-out, metatarsal heads,  Ischaemia: loss of vasa nervorum
calcaneum
Symmetric sensory polyneuropathy
 Glove and stocking: length-dependent ( feet 1st)
Mx  Loss of all modalities
 Conservative  Absent ankle jerks
 Daily foot inspection (e.g. c̄ mirror)  Numbness, tingling, pain (worse @ night)
 Comfortable / therapeutic shoes  Rx
 Regular chiropody (remove callus)  Paracetamol
 Medical  Amitriptyline, Gabapentin, SSRI
 Rx infection: benpen + fluclox ± metronidazole  Capsaicin cream
 Surgical  Baclofen
 Abscess or deep infection
 Spreading cellulitis Mononeuropathy / Mononeuritis Multiplex
 Gangrene  E.g. CN3/6 palsies
 Suppurative arthritis
Femoral Neuropathy / Amyotrophy
 Painful asymmetric weakness and wasting of quads c̄
Nephropathy loss of knee jerks
 Dx: nerve conduction and electromyography
Pathophysiology
 Hyperglycaemia → nephron loss and Autonomic Neuropathy
glomerulosclerosis
 Postural hypotension – Rx: fludrocortisone
 Gastroparesis → early satiety, GORD, bloating
Features
 Diarrhoea: Rx c̄ codeine phosphate
 Microalbuminuria: urine albumin:Cr (ACR) ≥30mg/mM
 If present → ACEi / ARA  Urinary retention
 Refer if UCR >70  ED

© Alasdair Scott, 2012 62

 Diabetic Ketoacidosis
Pathogenesis Management
Ketogenesis
 ↓ insulin → ↑ stress hormones and ↑ glucagon Fluids
 → ↓ glucose utilisation + ↑ fat β-oxidation  0.9% NS infusion via large bore cannula
 ↑ fatty acids → ↑ ATP + generation of ketone bodies.  SBP<90 → 1L stat + more until SBP >90
 SBP>90 → 1L over 1h
Dehydration  Then: 1L over next 2h, 1L/2h, 1L/4h, 1L/4h, 1L/6h
 ↓ insulin → ↓ glucose utilisation + ↑ gluconeogenesis →  Switch to 10% dex 1L/8h when glucose <14mM
severe hyperglycaemia
 → osmotic diuresis → dehydration Start Potassium Replacement in 2nd Bag of Fluids
 Also, ↑ ketones → vomiting  >5.5mM → Nil
 3.5-5.5mM → 40mmol/L
Acidosis  <3.5mM → consult senior for review
 Dehydration → renal perfusion
 Hyperkalaemia
Insulin Infusion
Precipitants  Actrapid 0.1u/kg/h IVI (6u if no wt., max 15u)
 Infection / stress ± stopping insulin
 New T1DM

Presentation Assessment
 Abdo pain + vomiting  Hx + full examination
 Gradual drowsiness  Investigations: capillary, urine, blood, imaging
 Sighing “Kussmaul” hyperventilation
 Dehydration
 Ketotic breath Additional Measures
 Urinary catheter (aim: 0.5ml/kg/hr)
Dx  NGT if vomiting or ↓GCS
 Acidosis (↑AG): pH <7.3 (± HCO3 <15mM)  Thromboprophylaxis c̄ LMWH
 Hyperglycaemia: ≥11.1mM (or known DM)  Refer to Specialist Diabetes Team
 Ketonaemia: ≥3mM (≥2+ on dipstix)  Find and treat precipitating factors

Ix
 Urine: ketones and glucose, MCS
 Cap glucose and ketones
 VBG: acidosis + ↑K
 Bloods: U+E, FBC, glucose, cultures
 CXR: evidence of infection
Subtleties
 Hyponatraemia is the norm
 Osmolar compensation for hyperglycaemia
 ↑/↔ Na indicates severe dehydration
 Avoid rapid ↓ in insulin once glucose normalised
 Glucose decreases faster than ketones and
insulin is necessary to get rid of them.
 Amylase is often ↑ (up to 10x)
 Excretion of ketones → loss of potential bicarbonate →
hyperchloraemic metabolic acidosis after Rx
Complications
 Cerebral oedema: excess fluid administration
 Commonest cause of mortality
 Aspiration pneumonia
 Hypokalaemia
 Hypophosphataemia → resp and skeletal muscle
weakness
 Thromboembolism
Monitoring
 Hrly capillary glucose and ketones
 VBG @ 60min, 2h and then 2hrly
 Plasma electrolytes 4hrly
Aims
 ↓ ketones by ≥0.5mM/h or ↑HCO3 by ≥3mM/h
 ↓ plasma glucose by ≥3mM/h
 Maintain K in normal range
 Avoid hypoglycaemia
Resolution
 Ketones <0.3mM + venous pH>7.3 (HCO3 >18mM)
 Transfer to sliding scale if not eating
 Transfer to SC insulin when eating and drinking
Transfer to SC Insulin
 When biochemically resolved and eating
 Start long-acting insulin the night before
 Give short-acting insulin before breakfast
 Stop IVI 30min after short acting

Mx: in HDU
 Gastric aspiration Pt. Education
 Rehydrate  ID precipitating factors and provide action plan
 Insulin infusion  Provision of ketone meter c̄ education on use.
 Potassium replacement
© Alasdair Scott, 2012 63
Hyperosmolar Non-Ketotic Coma
The Patient
 Usually T2DM, often new presentation
 Usually older
 Long hx (e.g. 1wk)

Metabolic Derangement
 Marked dehydration and glucose >35mM
 No acidosis (no ketogenesis)
 Osmolality >340mosmol/kg

Complications
 Occlusive events are common: DVT, stroke
 Give LMWH

Mx
 Rehydrate c̄ 0.9% NS over 48h
 May need ~9L
 Wait 1h before starting insulin
 It may not be needed
 Start low to avoid rapid changes in osmolality
 E.g. 1-3u/hr
 Look for precipitant
 MI
 Infection
 Bowel infarct

© Alasdair Scott, 2012 64

 Hypoglycaemia
Hypoglycaemia: Whipple’s Triad Mx
 Low plasma glucose ≤3mM
 Symptoms consistent c̄ hypoglycaemia Alert and Orientated: Oral Carb
 Relief of symptoms by glucose administration  Rapid acting: lucozade
 Long acting: toast, sandwich
Symptoms
Drowsy / confused but swallow intact: Buccal Carb
Autonomic: 2.5-3 Neuroglycopenic: <2.5  Hypostop / Glucogel
 Sweating  Confusion  Consider IV access
 Anxiety  Drowsiness
 Hunger  Seizures Unconscious or Concerned re Swallow: IV dextrose
 Tremor  Personality change  100ml 20% glucose (50ml 50% dextrose: not used)
 Palpitations  Focal neurology (e.g. CN3)
Deteriorating / refractory / insulin-induced / no access
 Coma (<2.2)
 1mg glucagon IM/SC
 Won’t work in drunks + short duration of effect (20min)
Fasting Hypoglycaemia  Insulin release may → rebound hypoglycaemia

Causes: EXPLAIN
 Usually insulin or sulfonylurea Rx in a known diabetic
 Exercise, missed meal, OD
 Exogenous drugs
 Pituitary insufficiency
 Liver failure
 Addison’s
 Islet cell tumours (insulinomas)
 Immune (insulin receptor Abs: Hodgkin’s)
 Non-pancreatic neoplasms: e.g. fibrosarcomas

Ix
 72h fast c̄ monitoring
 Sympto: Glucose, insulin, C-peptide, ketones

Dx
 Hyperinsulinaemic hypoglycaemia
 Drugs
 ↑ C-pep: sulfonylurea
 Normal C-pep: insulin
 Insulinoma
 ↓ insulin, no ketones
 Non-pancreatic neoplasms
 Insulin receptor Abs
 ↓ insulin, ↑ ketones
 Alcohol binge c̄ no food
 Pituitary insufficiency
 Addison’s

Insulinoma
 Path: 95% benign β-cell tumour usually seen c̄ MEN1
 Pres: fasting- / exercise-induced hypoglycaemia
 Ix:
 Hypoglycaemia + ↑ insulin
 Exogenous insulin doesn’t suppress C-pep
 MRI, EUS pancreas
 Rx: excision

Post-Prandial Hypoglycaemia
 Dumping post-gastric bypass

© Alasdair Scott, 2012 65

 Thyrotoxicosis
Definition Other Causes
 The clinical effect of ↑T4, usually from gland
hyperfunction. Toxic Multinodular Goitre = Plummer’s Disease
 Autonomous nodule develops on background multinodular
Symptoms goitre.
 Diarrhoea  Elderly and iodine-deficient areas
 ↑ appetite but ↓ wt.  Iodine scan shows hot nodules
 Sweats, heat intolerance
Toxic Adenoma
 Palpitations
 Solitary hot nodule ± producing T3/T4
 Tremor
 Most nodules are non-functional
 Irritability
 Oligomenorrhoea ± infertility Thyrotoxic Phase of Thyroiditis
 Hashimoto’s
Signs  de Quervain’s
Hands  Subacute lymphocytic
 Fast / irregular pulse
 Warm, moist skin Drugs
 Fine tremor  Thyroxine
 Palmer erythema  Amiodarone
Face
 Thin hair
 Lid lag
Rx
 Lid retraction
Medical
 Symptomatic: β-B (e.g. propranolol 40mg/6h)
Neck
 Anti-thyroid: carbimazole (inhibits TPO)
 Goitre or nodules
 Titrate according to TFTs or block and replace
 In Graves’ Rx for 12-18mo then withdraw
Graves’ Specific
 ~50% relapse → surgery or radioiodine
 Ophthalmopathy
 SE: agranulocytosis
 Exophthalmos
 Ophthalmoplegia: esp. up-gaze palsy
 Eye discomfort and grittiness Radiological: Radio-iodine
 Photophobia and ↓ acuity  Most become hypothyroid
 Chemosis  CI: pregnancy, lactation
 Dermopathy: pre-tibial myxoedema
Surgical: Thyroidectomy
 Thyroid acropachy
 Recurrent laryngeal N. damage → hoarseness
 Hypoparathyroidism
Ix
 Hypothyroidism
 ↓TSH, ↑T4/↑T3
 Abs: TSH receptor, TPO
 ↑Ca, ↑LFTs
 Isotope scan Thyroid Storm
 ↑ in Graves’
 ↓ in thyroiditis Features
 Ophthalmopathy: acuity, fields, movements  ↑ temp
 Agitation, confusion, coma
Graves’ Disease  Tachycardia, AF
 Acute abdomen
Epidemiology  Heart failure
 60% of cases of thyrotoxicosis
 Prev: 0.5% Precipitants
 Sex: F>>M=9:1  Recent thyroid surgery or radio-iodine
 Age: 40-60yrs  Infection
 MI
Features  Trauma
 Diffuse goitre c̄ ↑iodine uptake
Rx
 Ophthalmopathy and dermopathy
1. Fluid resuscitation + NGT
 Triggers: stress, infection, child-birth
2. Bloods: TFTs + cultures if infection suspected
3. Propranolol PO/IV
Associations
4. Digoxin may be needed
 T1DM 5. Carbimazole then Lugol’s Iodine 4h later to inhibit thyroid
 Vitiligo 6. Hydrocortisone
 Addison’s 7. Rx cause
© Alasdair Scott, 2012 66
 Hypothyroidism
Epidemiology Ix
 Incidence: 4/1000/yr  ↑TSH, ↓T3/T4
 Sex: F>>M=6:1  ↑MCV ± normochromic anaemia
 Age: >40yrs  ↑ triglyceride + ↑ cholesterol
 Hyponatraemia (SIADH)
Symptoms  ↑ CK if assoc. myopathy
 Lethargy  Abs: TPO, TSH
 Cold intolerance
 ↓ appetite but ↑wt. Rx
 Constipation  Levothyroxine
 Menorrhagia  Titrate to normalise TSH
 ↓ mood  Enzyme inducers ↑ thyroxine metabolism
 Clinical improvement takes ~2wks
Signs  Check for other AI disease: e.g. Addison’s, PA
 Cold hands
 Bradycardic
 Slow-relaxing reflexes Myxoedema Coma
 Dry hair and skin
 Puffy face Features
 Goitre  Looks hypothyroid
 Myopathy, neuropathy  Hypothermia
 Ascites  Hypoglycaemia
 Myxoedema  Heart failure: bradycardia and ↓BP
 SC tissue swelling in severe hypothyroidism  Coma and seizures
 Typically around eyes and dorsum of hand
Precipitants
Causes  Radioiodine
 Thyroidectomy
Primary  Pituitary surgery
 Atrophic thyroiditis (commonest UK)  Infection, trauma, MI, stroke
 Hashimoto’s thyroiditis
 Subacute thyroiditis (e.g. post-partum) Mx
 Post De Quervain’s thyroiditis  Bloods: TFTs, FBC, U+E, glucose, cortisol
 Iodine deficiency (commonest worldwide)  Correct any hypoglycaemia
 Drugs: carbimazole, amiodarone, lithium  T3/T4 IV slowly (may ppt. myocardial ischaemia)
 Congenital: thyroid agenesis  Hydrocortisone 100mg IV
 Rx hypothermia and heart failure
Post-surgical
 Thyroidectomy
 Radioiodine

Secondary
 Hypopituitarism (v. rare cause)

Atrophic Thyroiditis
 Thyroid antibodies +ve: anti-TPO, anti-TSH
 Lymphocytic infiltrate → atrophy (no goitre)
 Associations
 Pernicious anaemia
 Vitiligo
 Endocrinopathies

Hashimoto’s Thyoiditis
 TPO +ve
 Atrophy + regeneration → goitre
 May go through initial thyrotoxicosis phase
 May be euthyroid or hypothyoid

© Alasdair Scott, 2012 67

 Benign Thyroid Disease
Disease Clinical Features Cause Management
Simple goitre Diffuse painless goitre Endemic Thyroxine
Mass effects: - iodine deficiency
- dysphagia Total or subtotal
- stridor Sporadic thyroidectomy if pressure
- SVC obstruction - autoimmune symptoms.
- goitrogens (e.g. sulphonylureas)
Usually euthyroid, may → hypothyroid - hereditary (dyshormogenic)
Multinodular Evolves from long-standing simple As for simple goitre Thyroxine
goitre goitre.
Mass effects.
Euthyroid (or subclinical hyperthyroid) Total or subtotal
thyroidectomy if pressure
symptoms.
Plummer’s Multinodular goitre Autonomous nodule develops on Anti-thyroid drugs
Toxic multinodular Thyrotoxicosis background multinodular goitre
goitre Uneven iodine uptake with hot nodule Total or subtotal
thyroidectomy.
Graves’ Diffuse goitre c̄ bruit Autoimmune (T2 hypersensitivity) Propranolol
Ophthalmopathy - anti-TSH Carbimazole
Dermopathy Radioiodine
Thyrotoxicosis Thyroidectomy
Assoc. c̄ other AI disease (T1DM, PA)
↑ uptake on radionucleotide scan
Hashimoto’s Diffuse painless goitre Autoimmune (T2 and T4) Levothyroxine
thyroiditis May have transient thyrotoxicosis, then - anti-TPO, -Tg
hypothyroidism
Assoc. c̄ other AI disease (T1DM, PA)
de Quervain’s Diffuse painful goitre Viral: Coxsackie common Self-limiting
Preceding viral URTI common
Thyrotoxicosis → hypo→eu Autoimmune
↓ iodine uptake
Subacute Diffuse painless goitre Autoimmune Self-limiting
lymphocytic May occur post-partum
Thyrotoxicosis → hypo→eu
Riedel’s thyoiditis Hard fixed thyroid mass Autoimmune fibrosis Conservative
Mass effects
Assoc. c̄ retroperitoneal fibrosis
Follicular Single thyroid nodule Hemithyroidectomy
adenoma ± thyrotoxicosis (majority are cold)
May get pressure symptoms
Thyroid cysts Solitary thyroid nodule Aspiration or excision
Asympto or pressure symptoms
Can → localised pain due to cyst bleed

© Alasdair Scott, 2012 68

 Malignant Thyroid Disease
Disease Frequency Age Cell Origin Spread Mx
Papillary 80% 20-40 Follicular cells Nodes and lung Total thyroidectomy
- JDG node = ± node excision
Assoc. c̄ Tg tumour marker lateral aberrant ± radioiodine
irradiation thyroid T4 to suppress TSH

>95% 10ys
Follicular 10% 40-60 Follicular cells Blood → bone Total thyroidectomy +
F>M = 3:1 and lungs T4 suppression +
Tg tumour marker Radioiodine

>95% 10ys
Medullary 5% Men: young Parafollicular C-cells Do phaeo screen pre-op
30% are familial Sporadic: 40-50 Thyroidectomy +
- e.g. MEN2 CEA and calcitonin Node clearance
markers Consider radiotherapy
Anaplastic Rare >60 Undifferentiated Rapid growth Usually palliative
F>M = 3:1 follicular cells
Aggressive: local, May try thyroidectomy +
LN and blood. radiotherapy

<1% 10ys
Lymphoma 5% Lymphocytes Chemo-radiotherapy
- MALToma in Hashi’s

Presentation: Complications of Thyroid Surgery

 Non-functional (cold)
 Painless neck mass Early
 Cervical mets  Reactionary haemorrhage → haematoma (<1%)
 Compression symptoms  Can → airway obstruction.
 Dysphagia  Call anaesthetist and remove wound clips
 Stridor  Evacuate haematoma and re-explore wound
 SVC obstruction  Laryngeal oedema
 Damage during intubation or surgical
Risk factors for malignancy in thyroid nodules manipulation
 Solitary  Can → airway obstruction
 Solid  Recurrent laryngeal nerve palsy (0.5%)
 Younger  Right RLN more common (oblique ascent)
 Damage to one → hoarse voice
 Male
 Damage to both → obstruction needing
 Cold
trachyostomy
 Risk factor: e.g. radiation exposure
 Hypoparathyroidism (2.5%)
2+
 → ↓ Ca → Chvostek’s and Trousseau’s
 Thyroid storm
Thyroid Surgery  Severe hyperthyroidism
 Rx: propranolol, antithyroid drugs, Lugol’s iodine
Indications and hydrocortisone sodium succinate
 Pressure symptoms
 Relapse hyperthyroidism (>1 failed drug Rx) Late
 Cosmesis  Hypothyroidism
 Carcinoma  Recurrent hyperthyroidism
 Keloid scar
Practicalities
 Render euthyroid pre-op c̄ antithyroid drugs
 Stop 10 days prior to surgery (they ↑ vascularity)
 Alternatively just give propronalol
 Check for phaeo pre-op in medullary carcinoma
 Laryngoscopy: check vocal cords pre- and post-op

Procedure
 Collar incision

© Alasdair Scott, 2012 69

Hyperparathyroidism Hypoparathyroidism
 ↓PTH due to gland failure
Physiology
 PTH secreted in response to ↓Ca2+ Presentation: ↓Ca → SPASMODIC
 ↑osteoclast activity  Spasms (carpopedal = Trousseau’s sign)
 ↑Ca2+ and ↓PO4 reabsorption in kidney  Perioral paraesthesia
 ↑ 1α-hydroxylation of 25OH-Vit D3  Anxious, irritable
 Seizures
Presentation: ↑ Ca2+  Muscle tone ↑→ colic, wheeze, dysphagia
 Stones  Orientation impaired (confusion)
 Renal stones  Dermatitis
 Polyuria and polydipsia (nephrogenic DI)  Impetigo herpetiformis (↓Ca + pustules in pregnancy)
 Nephrocalcinosis  Chovsteks, cardiomyopathy (↑ QTc → TdP)
 Bones
 Bone pain Causes
 Pathological #s  Autoimmune
 Moans: depression  Congenital: DiGeorge
 Groans  Cardiac abnormality: Fallot’s
 Abdo pain  Abnormal facies
 n/v and constipation  Thymic aplasia
 Pancreatitis  Cleft palate
 PUD (↑gastrin secretion)  Hypocalcaemia
 Other:  Chr 22
2+
 ↑ BP (check Ca in all with HTN)  Iatrogenic
 Surgery
Primary Hyperparathyroidism  Radiation

Causes Ix
 Solitary adenoma: 80%  ↓Ca, ↑PO4, ↓PTH, normal ALP
 Hyperplasia: 20%
 Pathyroid Ca: <0.5% Rx
 Ca supplements
Ix  Calcitriol
 ↑Ca2+ + ↑ or inappropriately normal PTH, ↑ALP, ↓PO4
 ECG: ↓QTc → bradycardia → 1st degree block Pseudohypoparathyroidism
 X-ray: osteitis fibrosa cystica → phalangeal erosions
 Failure of target organ response to PTH
 DEXA: osteoporosis  Symptoms of hypocalcaemia
 Short 4th and 5th metacarpals, short stature
Rx  Ix: ↓Ca, ↑PTH
 General  Rx: Ca + calcitriol
 ↑ fluid intake
 Avoid dietary Ca2+ and thiazides (↑ serum Ca)
Pseudopseudohypoparathyroidism
 Surgical: excision of adenoma
 Normal (maternal) receptor in kidney → normal
 Hypoparathyroidism
biochem
 Recurrent laryngeal N. palsy
 Abnormal (paternal) receptors in body →
pseudohypoparathyroidsm phenotype
Secondary Hyperparathyroidism
Causes
 Vitamin D deficiency
 Chronic renal failure

Ix
 ↑PTH, ↓Ca, ↑PO4, ↑ALP, ↓vit D

Rx
 Correct causes
 Phosphate binders
 With Ca: calcichew
 W/o Ca: sevelamer, lanthanum
 Vit D: calcitriol (active), cholecalciferol (innactive)
 Cinacalcet: ↑ parathyroid Ca-sensitivity

Tertiary Hyperparathyroidism
 Prolonged 2O HPT → autonomous PTH secretion
 ↑Ca2+, ↑PTH, ↓PO4, ↑ALP
© Alasdair Scott, 2012 70
 Cushing’s Syndrome

Definition ACTH-Independent
 Clinical state produced by chronic glucocorticoid excess  ↓ACTH due to –ve FB
 No suppression c̄ any dose of dex

Causes
Features
 Iatrogenic steroids: commonest cause
 Adrenal adenoma / Ca: carcinoma often → virilisation
Catabolic Effects
 Proximal myopathy  Adrenal nodular hyperplasia
 Carney complex: LAME Syndrome
 Striae
 McCune-Albright
 Bruising
 Osteoporosis

Glucocorticoid Effects ACTH-Dependent

 DM  ↑ ACTH
 Obesity
Causes
Mineralocorticoid Effects  Cushing’s disease
 HTN  Bilat adrenal hyperplasia from ACTH-secreting
 Hypokalaemia pituitary tumour (basophilic microadenoma)
 Cortisol suppression c̄ high-dose dex
 Ectopic-ACTH
 SCLC
Appearance  Carcinoid tumour
 Moon face  Skin pigmentation, metabolic alkalosis, wt. loss,
 Acne and hirsutism hyperglycaemia
 Interscapular and supraclavicular fat pads  No suppression c̄ any dose of dex
 Centripetal obesity
 Striae
 Thin limbs Ix
 Bruising  1st: 24h urinary free cortisol
 Thin skin  Late night serum or salivary cortisol
 Highest @ AM, lowest @ midnight
 Dexamethasone suppression tests
 ACTH (but ACTH degrades v. quick after venepuncture)
 Localisation: CT, MRI
 DEXA scan

Rx: Treat Causes

 Cushing’s disease: trans-sphenoidal excision
 Adrenal adenoma / Ca: adrenelectomy
 Ectopic ACTH: tumour excision, metyrapone (inhibits
cortisol synthesis)

Nelson’s Syndrome
 Rapid enlargement of a pituitary adenoma following
bilateral adrenelectomy for Cushing’s syndrome
 Not typically performed nowadays
 Presentation
 Mass effects: bitemporal hemianopia
 Hyperpigmentation

© Alasdair Scott, 2012 71

 Hyperaldosteronism
Primary Hyperaldosteronism Secondary Hyperaldosteronism
 Excess aldosterone, independent of RAS  Due to ↑ renin from ↓ renal perfusion

Features Causes
 Hypokalaemia: weakness, hypotonia, hyporeflexia,  RAS
cramps  Diuretics
 Paraesthesia  CCF
 ↑BP  Hepatic failure
 Nephrotic syndrome
Causes
 Bilateral adrenal hyperplasia (70%) Ix
 Adrenocortical adenoma (30%): Conn’s syndrome  Aldosterone:renin ratio: normal

Ix Bartter’s Syndrome
 U+E: ↑/↔ Na, ↓K, alkalosis  Autosommal recesive
 Care c̄ diuretics, hypotensives, laxatives, steroids  Blockage of NaCl reabsorption in loop of Henle (as if
 Aldosterone:renin ratio: ↑ c̄ primary taking frusemide)
 ECG: flat / inverted T waves, U waves, depressed ST  Congenital salt wasting → RAS activation →
segments, prolonged PR and QT intervals hypokalaemia and metabolic alkalosis
 Adrenal CT/MRI  Normal BP

Rx
 Conn’s: laparoscopic adrenelectomy
 Hyperplasia: spironolactone, eplerenone or amiloride

© Alasdair Scott, 2012 72

 Adrenal Insufficiency
Primary Adrenal Insufficiency: Addison’s Addisonian Crisis
 Destruction of adrenal cortex → glucocorticoid and
mineralocorticoid deficiency Presentation
 Shocked: ↑HR, postural drop, oliguria, confused
Causes  Hypoglycaemia
 Autoimmune destruction: 80% in the UK  Usually known Addisonian or chronic steroid user
 TB: commonest worldwide
 Metastasis: lung, breast, kidneys Precipitants
 Haemorrhage: Waterhouse-Friedrichson  Infection
 Congenital: CAH  Trauma
 Surgery
Symptoms  Stopping long-term steroids
 Wt. loss + anorexia
 n/v, abdo pain, diarrhoea/constipation Mx
 Lethargy, depression  Bloods: cortisol, ACTH, U+E, cultures
 Hyperpigmentation: buccal mucosa, palmer creases  Check CBG: glucose may be needed
 Postural hypotension → dizziness, faints  Hydrocortisone 100mg IV 6hrly
 Hypoglycaemia  IV crystalloid
 Vitiligo  Septic screen
 Addisonian crisis  Treat underlying cause

Ix
 Bloods
 ↓Na/↑K
 ↓glucose
 ↓Ca
 Anaemia
 Differential
 Short synACTHen test
 Cortisol before and after tetracosactide
 Exclude Addison’s if ↑ cortisol
 ↑ 9am ACTH (usually low)
 Other
 21-hydroxylase Abs: +ve in 80% of AI disease
 Plasma renin and aldosterone
 CXR: evidence of TB
 AXR: adrenal calcification

Rx
 Replace
 Hydrocortisone
 Fludrocortisone
 Advice
 Don’t stop steroids suddenly
 ↑ steroids during intercurrent illness, injury
 Wear a medic-alert bracelet
 F/up
 Watch for autoimmune disease

Secondary Adrenal Insufficiency

 i.e. hypothalamo or pituitary failure

Causes
 Chronic steroid use → suppression of HPA axis
 Pituitary apoplexy / Sheehan’s
 Pituitary microadenoma

Features
 Normal mineralocorticoid production
 No pigmentation (ACTH ↓)

© Alasdair Scott, 2012 73

Phaeochromocytoma Multiple Endocrine Neoplasia
Pathology Definition
 Catecholamine-producing tumours arising from  Functioning hormone tumours in multiple organs
sympathetic paraganglia  All autosomal dominant
 Usually found in adrenal medulla
 Extra-adrenal phaeo’s found by aortic bifurcation MEN1
 Pituitary adenoma: prolactin or GH
Rule of 10s  Parathyroid adenoma / hyperplasia
 10% malignant  Pancreatic tumours: gastrinoma or insulinoma
 10% extra-adrenal
 10% bilateral
 10% part of hereditary syndromes MEN2
 Thyroid medullary carcinoma
Associations  Adrenal phaeochromocytoma
 10% part of herediatary syndromes  A) Hyperthyroidism
 MEN2a and 2b  B) Marfanoid habitus
 Neurofibrimatosis
 Von Hippel-Lindau: RCC + cerebellar signs
Carney Complex / LAME Syndrome
Presentation  Lentigenes: spotty skin pigmentation
 Triad: episodic headache, sweating and tachycardia  Differential: Peutz-Jehgers
 Other adrenergic features  Atrial Myxoma
 ↑BP, palpitations  Endocrine tumours: pituitary, adrenal hyperplasia
 Headache, tremor, dizziness  Schwannomas
 Anxiety
 d/v, abdo pain Peutz-Jeghers
 Heat intolerance, flushes  Mucocutaneous freckles on lips, oral mucosa and palms /
 May have precipitant soles
 Straining, abdo palpation  GI hamartomas: obstruction, bleeds
 Exercise, stress  Pancreatic endocrine tumours
 β-B, IV contrast, TCAs, GA  ↑ risk of cancer: CRC, pancreas, liver, lungs, breast
Ix
 Plasma + urine metadrenaline
Von Hippel-Lindau
 Also vanillylmandelic acid  Renal cysts
 Abdo CT/MRI  Bilateral renal cell carcinoma
 MIBG (mete-iodobenzylguanidine) scan  Haemangioblastomas
 Often in cerebellum → cerebellar signs
 Phaeochromocytoma
Rx
 Pancreatic endocrine tumours
Medical
 If malignant Neurofibrimatosis
 Chemo or radiolabelled MIGB  Dermal neurofibromas
 Café-au-lait spots
Surgery: adrenelectomy  Lisch nodules
 α-blocker first, then β-blockade pre-op  Axillary freckling
 Avoids unopposed α-adrenergic stimulation  Phaeochromocytoma
 Phenoxybenzamine = α-blocker
 Monitor BP post-op for ↓↓BP

Autoimmune Polyendocrine
Hypertensive Crisis Syndromes
Features Type 1
 Pallor  Autosomal recessive
 Pulsating headache  Addison’s
 Feeling of impending doom  Candidiasis
 ↑↑BP  Hypoparathyroidism
 ↑ ST and cardiogenic shock
Type 2: Schmidt’s Syndrome
Rx
 Polygenic
 Phentolamine 2-5mg IV (α-blocker) or labetalol 50mg IV
 Addison’s
 Repeat to safe BP (e.g. 110 diastolic)
 Thyroid disease: hypothyroidism or Graves’
 Phenoxybenzaime 10mg/d PO when BP controlled
 T1DM
 Elective surgery after 4-6wks to allow full α-blockade
© and volume
Alasdair Scott, expansion
2012 74
Hypopituitarism Pituitary Tumours
 10% of intracranial tumours
Causes
 Hypothalamic Classification
 Kallmann’s (anosmia + GnRH deficiency)
 Tumour Size
 Inflam, infection, ischaemia  Microadenoma: <1cm
 Pituitary Stalk  Macroadenoma: >1cm
 Trauma
 Surgery Pathology
 Tumour (e.g. craniopharyngioma)  Many are non-secretory
 Pituitary  ~50% produce PRL
 Irradiation  Others produce GH or ACTH
 Tumour
 Ischaemia: apoplexy, Sheehan’s
 Infiltration: HH, amyloid
Features

Commonest causes of panhypopituitarism Mass Effects

 Surgery  Headache
 Tumour  Visual field defect: bitemporal hemianopia
 Irradiation  CN palsies: 3, 4, 5, 6 (pressure on cavernous sinus)
 Diabetes insipidus
 CSF rhinorrhoea
Features Hormone Effects
 PRL → galactorrhoea, ↓libido, amenorrhoea, ED
Hormone Deficiency  ↑PRL → ↓GnRH → ↓LH/FSH
 GH: central obesity, atherosclerosis, ↓CO, ↓stength  GH → acromegaly
 LH/FSH  ACTH → Cushing’s Disease
 M: ↓libido, ED, ↓hair
 F: ↓libido, amenorrhoea, breast atrophy
Ix
 TSH: hypothyroidism
 MRI
 ACTH: 2O adrenal failure
 Visual field tests
Cause  Hormones: PRL, IGF, ACTH, cortisol, TFTs, LF/FSH
 Mass effects  Suppression tests
 Other hormones: e.g. prolactin excess
Rx
Medical
Ix  Replace hormones
 Basal hormone tests  Treat hormone excess
 Dynamic pituitary function test
 Insulin → ↑ cortisol + ↑ GH Surgical: Trans-sphenoidal excision
 GnRH → ↑ LH/FSH  Pre-op hydrocortisone
 TRH → ↑T4 + ↑ PRL
 Post-op dynamic pituitary tests
 MRI brain
Radiotherapy: sterotactic

Rx
 Hormone replacement Pituitary Apoplexy
 Treat underlying cause  Rapid pituitary enlargement due to bleed into a tumour
 Mass effects
 Headache, meningism, ↓GCS
 Bitemporal hemianopia
 Cardiovascular collapse due to acute hypopituitarism
 Rx: urgent hydrocortisone 100mg IV

Craniopharyngeoma
 Originates from Rathke’s pouch
 Commonest childhood intracranial tumour
 → growth failure
 Calcification seen on CT/MRI

© Alasdair Scott, 2012 75

Hyperprolactinaemia Acromegaly
Causes Causes
 Excess pituitary production  Pituitary acidophil adenoma in 99%
 Pregnancy, breastfeeding  Hyperplasia from GHRH secreting carcinoid tumour
 Prolactinoma (PRL >5000)  GH stimulates bone and soft tissue growth through ↑IGF1
 Hypothyroidism (↑TRH)
 Disinhibition by compression of pituitary stalk Symptoms
 Pituitary adenoma  Acroparaesthesia
 Craniopharyngioma  Amenorrhoea, ↓libido
 Dopamine antagonsists (commonest cause)  Headache
 Antiemetics: metoclopramide  Snoring
 Antipsychotics: risperidone, haldol
 Sweating
 Arthralgia, back ache
 Carpal tunnel (50%)
Symptoms
 Amenorrhoea
Signs
 Infertility
 Galactorrhoea
Hands
 ↓ libido  Spade-like
 ED  Thenar wasting
 Mass effects from prolactinoma  Boggy sweaty palms (if active)
 ↑ skin fold thickness
 Carpal tunnel: ↓ sensation + thenar wasting
Ix
 Basal PRL: >5000 = prolactinoma Face
 Pregnancy test, TFTs  Prominent supraorbital ridges
 MRI  Scalp folds: cutis verticis gyrata
 Coarse face, wide nose and big ears
 Prognathism: look from side
Rx  Macroglossia
 1st line: Cabergoline or bromocroptine  Widely-spaced teeth
 Da agonist  Goitre
 ↓ PRL secretion and ↓ tumour size
 SE: nausea, postural hypotension, fibrosis (lung Other
and heart)  Puffy, oily, darkened skin skin
 2nd line: Trans-sphenoidal excision  Proximal weakness + arthropathy
 If visual or pressure symptoms don’t response to
 Pituitary mass effects: bitemporal hemianopia
medical Rx
Complications
 Endocrine
 Impaired glucose tolerance (40%)
 DM (15%)
 Cardiovascular
 ↑BP
 LVH
 Cardiomyopathy
 ↑ IHD and ↑ stroke
 Neoplasia
 ↑ risk of CRC

Ix
 ↑IGF1
 ↑ glucose, ↑Ca, ↑PO4
 Glucose tolerance test
 GH fails to suppress c̄ glucose in acromegaly
 Visual fields and acuity
 MRI brain

Rx
 1st line: trans-sphenoidal excision
 2nd line: somatostatin analogues – octreotide
 3rd line: GH antagonist – pegvisomant
 4th line: radiotherapy

© Alasdair Scott, 2012 76

Diabetes Insipidus Sexual Characteristics
Symptoms Hirsutism
 Polyuria
 Polydipsia Causes
 Dehydration  Familial
 Hypernatraemia: lethargy, thirst, confusion, coma  Idiopathic
 ↑ androgens:
 Ovary: PCOS
Causes  Adrenals: Cushing’s, adrenal Ca
 Drugs: steroids
Cranial
 Idiopathic: 50% PCOS
 Congenital: DIDMOAD / Wolfram Syndrome  Features
O
 Tumours  2 oligo-/amenorrhoea → infertility
 Obesity
 Trauma
 Acene, hirsutism
 Vascular: haemorrhage (Sheehan’s syn.)
 US: bilateral polycystic ovaries
 Infection: meningoencephalitis
 Hormones: ↑ testosterone, ↓ SHBG, ↑LH:FSH ratio
 Infiltration: sarcoidosis
 Mx
 Metformin
 COCP
Nephrogenic
 Clomifene for infertility
 Congenital
 Metabolic: ↓K, ↑Ca
 Drugs: Li, demecleocycline, vaptans
 Post-obstructive uropathy
Gynaecomastia
 Abnormal amount of breast tissue in men
 May occur in normal puberty
Ix Causes
 Bloods: U+E, Ca, glucose
 Cirrhosis
 Urine and plasma osmolality
 Hypogonadism
 Exclude DI if U:P osmolality >2
 Hyperthyroidism
 Oestrogen- or HCG-producing tumours: e.g. testicular
 Drugs: spiro, digoxin, oestrogen
Dx
 Water deprivation test c̄ desmopressin trial
ED
Differential Organic Causes
 DM  Smoking
 Diuretics or Lithium  EtOH
 Primary polydipsia  DM
 Endo: hypogonadism, hyperthyroidism, ↑Prl
 Neuro: MS, autonomic neuropathy, cord lesion
Rx  Pelvic surgery: bladder, prostate
 Penile abnormalities: Peyronie’s disease
Cranial
 Find cause: MRI brain
 Desmopressin PO

Nephrogenic
 Treat cause

© Alasdair Scott, 2012 77

Diarrhoea C. diff Diarrhoea
Definitions Pathogen
 Diarrhoea = ↑ stool water → ↑ stool frequency  Gm+ve spore-forming anaerobe
 Steatorrhoea = ↑ stool fat → pale, float, smelly  Release enterotoxins A and B
 Faecal urgency = suggests rectal pathology  Spores are v. robust and can survive for >40d

Clinical Features and Causes Epidemiology

 Commonest cause (25%) of Abx assoc. diarrhoea
Acute  100% of Abx assoc. pseudomembranous colitis
 Suspect gastroenteritis  Stool carriage in 3% of healthy adults and 15-30% of
 Travel, diet, contacts? hospital pts.

Chronic Risk Factors

 Diarrhoea alternating c̄ constipation: IBS  Abx: e.g. clindamycin, cefs, augmentin, quinolones
 Anorexia, ↓wt., nocturnal diarrhoea: organic cause  ↑ age
 In hospital: ↑ c̄ length of stay, ↑ c̄ C. diff +ve contact
Bloody  PPIs
 Vascular: ischaemic colitis
 Infective: campylobacter, shigella, salmonella, E. coli, Clinical Presentations
amoeba, pseudomembranous colitis
 Asymptomatic
 Inflammatory: UC, Crohn’s
 Mild diarrhoea
 Neoplastic: CRC, polyps
 Colitis w/o pseudomembranes
 Pseudomembranous colitis
Mucus
 Fulminant colitis
 IBS, CRC, polyps
 May occur up to 2mo after discontinuation of Abx
Pus
 IBD, diverticulitis, abscess Pseudomembranous Colitis
 Severe systemic symptoms: fever, dehydration
Assoc. c̄ medical disease  Abdominal pain, bloody diarrhoea, mucus PR
 ↑ T4  Pseudomembranes (yellow plaques) on flexi sig
 Autonomic neuropathy (e.g. DM)  Complications
 Carcinoid  Paralytic ileus
 Toxic dilatation → perforation
Assoc. c̄ drugs  Multi-organ failure
 Abx
 PPI, cimetidine Ix
 NSAIDs  Bloods: ↑↑CRP, ↑↑WCC, ↓albumin, dehydration
 Digoxin  CDT ELISA
 Stool culture

Ix Severe Disease: ≥1 of
 WCC >15
Bloods  Cr >50% above baseline
 FBC: ↑ WCC, anaemia  Temp >38.5
 U+E: ↓K+, dehydration  Clinical / radiological evidence of severe colitis
 ↑ESR: IBD, Ca
 ↑CRP: IBD, infection Rx
 Coeliac serology: anti-TTG or anti-endomysial Abs General
 Stop causative Abx
Stool  Avoid antidiarrhoeals and opiates
 MCS and C. diff toxin  Enteric precautions
Specific
 1st line: Metronidazole 400mg TDS PO x 10-14d
 2nd line: Vanc 125mg QDS PO x 10-14d
Rx
 Failed metro
 Treat cause
 Severe: Vanc 1st (may add metro IV)
 Oral or IV rehydration
 ↑ to 250mg QDS if no response (max 500mg)
 Codeine phosphate or loperamide after each loose  Urgent colectomy may be needed if
stool
 Toxic megacolon
 Anti-emetic if assoc. c̄ n/v: e.g. prochlorperazine
 ↑ LDH
 Abx (e.g. cipro) in infective diarrhoea → systemic illness  Deteriorating condition
 Recurrence (15-30%)
 Reinfection or residual spores
 Repeat course of metro x 10-14d
© Alasdair Scott, 2012
 Vanc if further relapse (25%) 79
Constipation IBS
Definition Definition
 Infrequent BMs (≤3/wk) or passing BMs less often than  Disorders of enhanced visceral perception → bowel
normal or c̄ difficulty, straining or pain. symptoms for which no organic cause can be found.

Causes: OPENED IT Dx: ROME Criteria

Obstruction  Abdo discomfort / pain for ≥ 12wks which has 2 of:
 Mechanical: adhesions, hernia, Ca, inflamatory  Relieved by defecation
strictures, pelvic mass  Change in stool frequency (D or C)
 Pseudo-obstruction: post-op ileus  Change in stool form: pellets, mucus
 + 2 of:
Pain  Urgency
 Anal fissure  Incomplete evacuation
 Proctalgia fugax  Abdo bloating / distension
 Mucous PR
Endocrine / Electrolytes  Worsening symptoms after food
 Endo: ↓T4  Exclusion criteria
 Electrolytes: ↓Ca, ↓K, uraemia  >40yrs
 Bloody stool
Neuro  Anorexia
 MS  Wt. loss
 Myelopathy  Diarrhoea at night
 Cauda equina syndrome
Ix
Elderly  Bloods: FBC, ESR, LFT, coeliac serology, TSH
Diet / Dehydration  Colonoscopy: if >60yrs or any features of organic
IBS disease
Toxins
 Opioids Rx
 Anti-mACh  Exclusion diets can be tried
 Bulking agents for constipation and diarrhoea (e.g.
Mx fybogel).
General  Antispasmodics for colic/bloating (e.g. mebeverine)
 Drink more  Amitriptyline may be helpful
 ↑ dietary fibre  CBT

Bulking: ↑ faecal mass → ↑ peristalsis

 CI: obstruction and faecal impaction
 Bran
 Ispaghula husk (Fybogel)
 Methylcellulose

Osmotic: retain fluid in the bowel

 Lactulose
 MgSO4 (rapid)

Stimulant: ↑ intestinal motility and secretion

 CI: obstruction, acute colitis
 SE: abdo cramps
 Bisacodyl PO or PR
 Senna
 Docusate sodium
 Sodium picosulphate (rapid)

Softeners
 Useful when managing painful anal conditions
 Liquid paraffin

Enemas
 Phosphate enema (osmotic)

Suppositories
 Glycerol (stimulant)

© Alasdair Scott, 2012 80

 Dysphagia
Definition Achalasia
 Difficulty swallowing  Pathophysiology
 Degeneration of myenteric plexus (Auerbach’s)
Causes  ↓ peristalsis
 LOS fails to relax
Inflammatory  Cause
 Tonsillitis, pharyngitis  1O / idiopathic: commonest
 2O: oesophageal Ca, Chagas’ disease (T. cruzii)
 Oesophagitis: GORD, candida
 Presentation
 Oral candidiasis
 Dysphagia: liquids and solids at same time
 Aphthous ulcers
 Regurgitation
 Substernal cramps
Mechanical Block
 Wt. loss
 Luminal
 Comps: Chronic achalasia → oesophageal SCC
 FB
 Ix
 Large food bolus
 Ba swallow: dilated tapering oesophagus (Bird’s
 Mural
beak)
 Benign stricture
 Manometry: failure of relaxation + ↓ peristalsis
 Web (e.g. Plummer-Vinson)  CXR: may show widended mediastinum
 Oesophagitis  OGD: exclude malignancy
 Trauma (e.g. OGD)  Rx:
 Malignant stricture  Med: CCBs, nitrates
 Pharynx, oesophagus, gastric  Int: endoscopic balloon dilatation, botulinum toxin
 Pharyngeal pouch injection
 Extra-mural  Surg: Heller’s cardiomyotomy (open or endo)
 Lung Ca
 Rolling hiatus hernia
 Mediastinal LNs (e.g. lymphoma) Pharyngeal Pouch: Zenker’s Diverticulum
 Retrosternal goitre
 Outpouching of oesophagus between upper boarder of
 Thoracic aortic aneurysm
cricopharyngeus muscle and lower boarder of inferior
constrictor of pharynx
Motility Disorders
 Weak area called Killian’s dehiscence.
 Local
 Defect usually occurs posteriorly but swelling usually
 Achalasia
bulges to left side of neck.
 Diffuse oesophageal spasm
 Food debris → pouch expansion → oesophageal
 Nutcracker oesophagus
compression → dysphagia.
 Bulbar / pseudobulbar palsy (CVA, MND)
 Pres: regurgitation, halitosis, gurgling sounds
 Systemic
 Systemic sclerosis / CREST  Rx: excision, endoscopic stapling
 MG

Presentation Diffuse Oesophageal Spasm

 Dysphagia for liquids and solids at start  Intermittent dysphagia ± chest pain
 Yes: motility disorder  Ba swallow shows corkscrew oesophagus
 No, solids > liquids: stricture
 Difficulty making swallowing movement: bulbar palsy
 Odonophagia: Ca, oesophageal ulcer, spasm Nutcracker Oesophagus
 Intermittent: oesophageal spasm  ↑ contraction pressure c̄ normal peristalsis
 Constant and worsening: malignant stricture
 Neck bulges or gurgles on drinking: pharyngeal pouch

Signs
 Cachexia
 Anaemia
 Virchow’s node (+ve = Troisier’s sign)
 Neurology
 Signs of systemic disease (e.g. scleroderma)

Ix
 Bloods: FBC, U+E
 CXR
 OGD
 Barium swallow ± video fluoroscopy
 Oesophageal manometrry

© Alasdair Scott, 2012 81

 Dyspepsia
Definition Eradication Therapy
 Non-specific group of symptoms  7 days Rx
 Epigastric pain  NB. PPIs and cimetidine → false –ve C13 breath tests
 Bloating and antigen tests  stop >2wks before.
 Heartburn
PAC 500
ALARM Symptoms  PPI: lansoprazole 30mg BD
 Anaemia  Amoxicillin 1g BD
 Loss of wt.  Clarithromycin 500mg BD
 Anorexia
PMC 250
 Recent onset progressive symptoms
 PPI: lansoprazole 30mg BD
 Melaena or haematemesis
 Metronidazole 400mg BD
 Swallowing difficulty
 Clarithromycin 250mg BD

Failure
Causes  95% success
 Inflammation: GORD, gastritis, PUD  Mostly due to poor compliance
 Ca: oesophageal, gastric  Add bismuth
 Functional: non-ulcer dyspepsia  Stools become tarry black

Mx of New Onset Dyspepsia

 OGD if >55 or ALARMS

 Try conservative measures for 4 wks

 Stop drugs: NSAIDs, CCBs (relax LOS)
 Lose wt., stop smoking, ↓ EtOH
 Avoid hot drinks and spicy food
 OTC
 Antacids: magnesium trisilicate
 Alginates: gaviscon advance

 Test for H. pylori if no improvement: breath or serology

 +ve → eradication therapy
 Consider OGD if no improvement
 -ve → PPI trial for 4wks
 Consider OGD if no improvement
 PPIs can be used intermittently to control
symptoms.

 Proven GORD
 Full dose PPI for 1-2mo
 Then, low-dose PPI PRN

 Proven PUD
 Full dose PPI for 1-2mo
 H. pylori eradication if positive
 Endoscopy to check for resolution if GU
 Then, low-dose PPI PRN

© Alasdair Scott, 2012 82

 Peptic Ulcer Disease
Classification
 Acute: usually due to drugs (NSAIDs, steroids) or “stress”
 Chronic: drugs, H. pylori, ↑Ca, Zollinger-Ellison

Features

Duodenal Ulcers Gastric Ulcers

Pathology  4x commoner cf. GU  Lesser curve of gastric antrum
 1st part of duodenum (cap)  Beware ulcers elsewhere (often malignant)
 M>F

Risk Factors  H. pylori (90%)  H. pylori (80%)

 Drugs: NSAIDs, steroids
 Smoking
 EtOH
 ↑ gastric emptying
 Blood group O
 Smoking
 Drugs
 Delayed gastric emptying
 Stress
 Cushing’s: intracranial disease
 Curling’s: burns, sepsis, trauma

Presentation  Epigastric pain:  Epigastric pain:

 Before meals and at night  Worse on eating
 Relieved by eating or milk  Relieved by antacids
 Wt. loss

Complications Surgery for PUD

Haemorrhage
 Haematemeis or melaena Concepts
 Fe deficiency anaemia  No acid → no ulcer
Perforation  Secretion stimulated by gastrin and vagus N.
 Peritonitis
Gastric Outflow Obstruction Vagotomy
 Vomiting, colic, distension  Truncal: ↓ acid secretion but prevents pyloric sphincter
Malignancy relaxation  must be combined c̄ pyloroplasty or
 ↑ risk c̄ H. pylori gastroenterostomy.
 Selective: vagus nerve only denervated where it
supplies lower oesophagus and stomach
Ix  Nerves of Laterjet (supply pylorus) left intact
 Bloods: FBC, urea (↑ in haemorrhage)
 C13 breath test Antrectomy c̄ vagotomy
 OGD (stop PPIs >2wks before)  Distal half of stomach removed + anastomosis:
 CLO / urease test for H. pylori  Directly to duodenum: Billroth 1
 Always take biopsies of ulcers to check for Ca  To small bowel loop c̄ duodenal stump oversewn:
 Gastrin levels if Zollinger-Ellison suspected Billroth 2 or Polya

Subtotal gastrectomy c̄ Roux-en-Y

Mx  Occasionally performed for Zollinger-Ellison
Conservative Complications
 Lose wt. Physical
 Stop smoking and ↓ EtOH  Stump leakage
 Avoid hot drinks and spicy food  Abdominal fullness
 Stop drugs: NSAIDs, steroids  Reflux or bilious vomiting (improves c̄ time)
 OTC antacids  Stricture
Medical Metabolic
 OTC antacids: Gaviscon, Mg trisilicate  Dumping syndrome
 H. pylori eradication: PAC500 or PMC250  Abdo distension, flushing, n/v
 Full-dose acid suppression for 1-2mo  Early: osmotic hypovolaemia
 PPIs: lansoprazole 30mg OD  Late: reactive hypoglycaemia
 H2RAs: ranitidine 300mg nocte  Blind loop syndrome → malabsorption, diarrhoea
 Low-dose acid suppression PRN  Overgrowth of bacteria in duodenal stump
 Anaemia: Fe + B12
 Osteoporosis
 Wt. loss: malabsorption of ↓ calories intake
© Alasdair Scott, 2012 83
 GORD
Pathophysiology Rx
 LOS dysfunction → reflux of gastric contents →
oesophagitis. Conservative
 Lose wt.
Risk Factors  Raise head of bed
 Hiatus hernia  Small regular meals ≥ 3h before bed
 Smoking  Stop smoking and ↓ EtOH
 EtOH  Avoid hot drinks and spicy food
 Obesity  Stop drugs: NSAIDs, steroids, CCBs, nitrates
 Pregnancy
 Drugs: anti-AChM, nitrates, CCB, TCAs Medical
 Iatrogenic: Heller’s myotomy  OTC antacids: Gaviscon, Mg trisilicate
 1: Full-dose PPI for 1-2mo
Symptoms  Lansoprazole 30mg OD
 2: No response → double dose PPI BD
Oesophageal  3: No response: add an H2RA
 Heartburn  Ranitidine 300mg nocte
 Related to meals  Control: low-dose acid suppression PRN
 Worse lying down / stooping
 Relieved by antacids Surgical: Nissen Fundoplication
 Belching  Indications: all 3 of:
 Acid brash, water brash  Severe symptoms
 Odonophagia  Refractory to medical therapy
 Confirmed reflux (pH monitoring)
Extra-oesophageal
 Nocturnal asthma Nissen Fundoplication
 Chronic cough  Aim: prevent reflux, repair diaphragm
 Laryngitis, sinusitis  Usually laparoscopic approach
 Mobilise gastric fundus and wrap around lower
Complications oesophagus
 Oesophagitis: heartburn  Close any diaphragmatic hiatus
 Ulceration: rarely → haematemesis, melaena, ↓Fe  Complications:
 Gas-bloat syn.: inability to belch / vomit
 Benign stricture: dysphagia
 Dysphagia if wrap too tight
 Barrett’s oesophagus
 Intestinal metaplasia of squamous epithelium
 Metaplasia → dysplasia → adenocarcinoma Hiatus Hernia
 Oesophageal adenocarcinoma
Classification
Differential Dx
 Oesophagitis Sliding (80%)
 Infection: CMV, candida  Gastro-oesophageal junction slides up into chest
 IBD  Often assoc. c̄ GORD
 Caustic substances / burns
 PUD Rolling (15%)
 Oesophageal Ca  Gastro-oesophageal junction remains in abdomen but a
bulge of stomach rolls into chest alongside the
Ix oesophagus
 Isolated symptoms don’t need Ix  LOS remains intact so GORD uncommon
 Bloods: FBC  Can → strangulation
 CXR: hiatus hernia may be seen
 OGD if: Mixed (5%)
 >55yrs
 Symptoms >4wks Ix
 Dysphagia  CXR: gas bubble and fluid level in chest
 Persistent symptoms despite Rx  Ba swallow: diagnostic
 Wt. loss  OGD: visualises the mucosa but can’t exclude hernia
 OGD allows grading by Los Angeles  24h pH + manometry: exclude dysmotility or achalsia
Classification
 Ba swallow: hiatus hernia, dysmotility Rx
 24h pH testing ± manometry
 Lose wt.
 pH <4 for >4hrs
 Rx reflux
 Surgery if intractable symptoms despite medical Rx.
 Should repair rolling hernia (even if asympto)
as it may strangulate.
© Alasdair Scott, 2012 84
Haematemesis Differential Rectal Bleeding Differential
VINTAGE DRIPING Arse
Varices Diverticulae

Inflammation Rectal
 Oesophago-gastro-duodenitis  Haemorrhoids
 PUD: DU is commonest cause
Infection
Neoplasia  Campylobacter, shigella, E. coli, C. diff, amoebic
 Oesophageal or gastric Ca dysentery

Trauma Polyps
 Mallory-Weiss Tear
 Mucosal tear due to vomiting Inflammation
 Boerhaave’s Syndrome  UC, Crohn’s
 Full-thickness tear
 2cm proximal to LOS Neoplasia

Angiodysplasia + other vascular anomalies Gastic-upper bowel bleeding

 Angiodysplasia
 HHT Angio
 Dieulafoy lesion: rupture of large arteriole in stomach or  Ischaemic colitis
other bowel  HHT
 Angiodysplasia
Generalised bleeding diathesis
 Warfarin, thrombolytics
 CRF

Epistaxis

© Alasdair Scott, 2012 85

 Upper GI Bleeding
Hx
 Previous bleeds Management
 Dyspepsia, known ulcers
 Liver disease or oesophageal varices Resuscitate
 Dysphagia, wt. loss  Head-down.
 Drugs and EtOH  100% O2, protect airway
 Co-morbidities  2 x 14G cannulae + IV crystalloid infusion up to 1L.
 Bloods: FBC, U+E (↑ urea), LFTs, clotting, x-match
o/e 6u, ABG, glucose
 Signs of CLD
 PR:melaena
 Shock? Blood if remains shocked
 Cool, clammy, CRT>2s  Group specific or O- until X-matched
 ↓BP (<100) or postural hypotension (>20 drop)
 ↓ urine output (<30ml/h)
 Tachycardia
 ↓GCS Variceal Bleed
 Terlipressin IV (splanchnic vasopressor)
Common Causes  Prophylactic Abx: e.g. ciprofloxacin 1g/24h
 PUD: 40% (DU commonly)
 Acute erosions / gastritis:20%
 Mallory-Weiss tear: 10% Maintenance
 Varices: 5%  Crystalloid IVI, transfuse if necessary (keep Hb≥10)
 Oesophagitis: 5%  Catheter + consider CVP (aim for >5cm H2O)
 Ca Stomach / oesophagus:<3%  Correct coagulopathy: vit K, FFP, platelets
 Thiamine if EtOH
Rockall Score: (Prof T Rockall, St. Mary’s)  Notify surgeons of severe bleeds
 Prediction of re-bleeding and mortality
 40% of re-bleeders die
 Initial score pre-endoscopy
Urgent Endoscopy
 Age
Haemostasis of vessel or ulcer:
 Shock: BP, pulse
 Adrenaline injection
 Comorbidities
 Thermal / laser coagulation
 Final score post-endoscopy
 Final Dx + evidence of recent haemorrhage  Fibrin glue
 Active bleeding  Endoclips
 Visible vessel
Variceal bleeding:
 Adherent clot
 2 of: banding, sclerotherapy, adrenaline,
 Initial score ≥3 or final >6 are indications for surgery
coagulation
 Balloon tamponade c̄ Sengstaken-Blakemore tube
Oesophageal Varices  Only used if exsanguinating haemorrhage or
 Portal HTN → dilated veins @ sites of porto-systemic failure of endoscopic therapy
anastomosis: L. gastric and inferior oesophageal veins  TIPSS if bleeding can’t be stopped endoscopically
 30-50% c̄ portal HTN will bleed from varices
 Overall mortality 25%: ↑ c̄ severity of liver disease.

Causes of portal HTN After endoscopy

 Pre-hepatic: portal vein thrombosis
 Hepatic: cirrhosis (80% in UK), schisto (commonest
worldwide), sarcoidosis.
 Post-hepatic: Budd-Chiari, RHF, constrict pericarditis
Bleed Prevention
 1 : β-B, repeat endoscopic banding
O
 Omeprazole IV + continuation PO (↓s re-bleeding)
 Keep NBM for 24h → clear fluids → light diet @ 48h
 Daily bloods: FBC, U+E, LFT, clotting
 H. pylori testing and eradication
 Stop NSAIDs, steroids et.c.

 2O: β-B, repeat banding, TIPSS Indications for Surgery

Transjugular Intrahepatic Porto-Systemic Shunt (TIPSS)
 IR creates artificial channel between hepatic vein and
portal vein → ↓ portal pressure.
 Colapinto needle creates tract through liver
parenchyma which is expand using a balloon and
maintained by placement of a stent.
 Used prophylactically or acutely if endoscopic therapy
fails to control variceal bleeding.
© Alasdair Scott, 2012
 Re-bleeding
 Bleeding despite transfusing 6u
 Uncontrollable bleeding at endoscopy
 Initial Rockall score ≥3, or final >6.
Open stomach, find bleeder and underrun vessel.
NB. Avoid 0.9% NS in uncompensated liver disease (worsens
ascites). Use blood or albumin for resus and 5% dex for
maintenance.
86
 Jaundice
Physiology
 Normal BR = 3-17uM
 Jaundice visible @ 50uM (3 x ULN)
 Hb → unconjugated to BR by splenic macrophages
 uBR → cBR by BR-UDP-glucuronyl transferase in liver
 Secreted in bile then cBR → urobilinogen (colourless)
 Some urobilinogen is reabsorbed, returned to liver and re-excreted into bile.
 Some reabsorbed urobilinogen is excreted into the urine
 The urobilinogen that remains in the GIT is converted to stercobilin (brown) and excreted.

Causes
Pre-Hepatic Hepatic Post-Hepatic
Unconjugated Conjugated
Excess BR production ↓ BR Uptake Hepatocellular Dysfunction Obstruction
 Haemolytic anaemia  Drugs: contrast, RMP  Congen: HH, Wilson’s, α1ATD  Stones
 Ineffective erythropoiesis  CCF  Infection: Hep A/B/C, CMV, EBV  Ca pancreas
 e.g. thalassaemia  Toxin: EtOH, drugs  Drugs
↓ BR Conjugation  AI: AIH  PBC
 Hypothyroidism  Neoplasia: HCC, mets  PSC
 Gilbert’s (AD)  Vasc: Budd-Chiari  Biliary atresia
 Crigler-Najjar (AR)  Choledochal cyst
↓ Hepatic BR Excretion  Cholangio Ca
Neonatal jaundice is both  Dubin-Johnson
↑ production + ↓ conjug.  Rotor’s

Drug-induced Jaundice Gilbert’s

 Auto dom partial UDP-GT deficiency
Mechanism Drug  2% of the population
Haemolysis  Antimalarials (e.g. dapsone)  Jaundice occurs during intercurrent illness
 Dx: ↑ uBR on fasting, normal LFTs
Hepatitis  Paracetamol OD
 RMP, INH, PZA Crigler-Najjar
 Valproate  Rare auto rec total UDP-GT deficiency
 Statins  Severe neonatal jaundice and kernicterus
 Halothane  Rx: liver Tx
 MOAIs

Cholestasis  Fluclox (may be wks after Rx)

 Co-amoxiclav
 OCP
 Sulfonylureas
 Chlopromazine, prochlorperazine

Ix
Pre-Hepatic Hepatic Post-Hepatic
Urine  No BR (acholuric)  ↑BR  ↑↑ BR
 ↑ urobilinogen  ↑ urobilinogen  No urobilinogen
 ↑Hb if intravascular haemolysis
LFTs  ↑ uBR  ↑ cBR (usually)  ↑↑ cBR
 ↑ AST  ↑AST:↑ALT  ↑ AST, ↑ ALT
 ↑ LDH  > 2 = EtOH  ↑↑ ALP
 < 1 = Viral  ↑ GGT
 ↑ GGT (EtOH, obstruction)
 ↑ ALP
 Function: ↓ albumin, ↑ PT
Other  FBC and film  FBC: anaemia  Abdo US: ducts >6mm
 Coombs Test  Anti- SMA, LKM, SLA, ANA  ERCP, MRCP
 Hb electrophoresis  α1AT, ferritin, caeruloplasmin  Anti- AMA, ANCA, ANA
 Liver biopsy

© Alasdair Scott, 2012 87

 Liver Failure
Causes Mx
 Cirrhosis (see below)  Manage in ITU
 Acute  Rx underlying cause: e.g. NAC in paracetamol OD
 Infection: Hep A/B, CMV, EBV, leptospirosis  Good nutrition: e.g. via NGT c̄ high carbs
 Toxin: EtOH, paracetamol, isoniazid, halothane  Thiamine supplements
 Vasc: Budd-Chiari  Prophylactic PPIs vs. stress ulcers
 Other: Wilson’s, AIH
 Obs: eclampsia, acute fatty liver of pregnancy Monitoring
 Fluids: urinary and central venous catheters
Signs  Bloods: daily FBC, U+E, LFT, INR
 Jaundice  Glucose: 1-4hrly + 10% dextrose IV 1L/12h
 Oedema + ascites
 Bruising Complications
 Encephalopathy  Bleeding: Vit K, platelets, FFP, blood
 Aterixis  Sepsis: tazocin (avoid gent: nephrotoxicity)
 Constructional apraxia (5-pointed star)  Ascites: fluid and salt restrict, spiro, fruse, tap, daily wt
 Fetor hepaticus  Hypoglycaemia: regular BMs, IV glucose if <2mM
 Signs of cirrhosis / chronic liver disease  Encephalopathy: avoid sedatives, lactulose ± enemas,
rifaximin
Ix  Seizures: lorazepam
Blood  Cerebral oedema: mannitol
 FBC: infection, GI bleed, ↓ MCV (EtOH)
 U+E Prescribing in Liver Failure
 ↓U, ↑Cr: hepatorenal syndrome  Avoid: opiates, oral hypoglycaemics, Na-containing IVI
 Urea synth in liver  poor test of renal function  Warfarin effects ↑
 LFT  Hepatotoxic drugs: paracetamol, methotrexate,
 AST:ALT > 2 = EtOH isoniazid, salicylates, tetracycline
 AST:ALT < 1 = Viral
 Albumin: ↓ in chronic liver failure Poor Prognostic Factors
 PT: ↑ in acute liver failure  Grade 3/4 hepatic encephalopathy
 Clotting: ↑INR  Age >40yrs
 Glucose  Albumin <30g/L
 ABG: metabolic acidosis  ↑INR
 Cause: Ferritin, α1AT, caeruloplasmin, Abs,  Drug-induced liver failure
paracetamol levels

Microbiology
 Hep, CMV, EBV serology Liver Transplant
 Blood and urine culture
Types
 Ascites MCS + SAAG
 Cadaveric: heart-beating or non-heart beating
Radiology  Live: right lobe
 CXR
Kings College Hospital Criteria in Acute Failure
 Abdo US + portal vein duplex
Paracetamol-induced Non-paracetamol
Hepatorenal Syndrome pH< 7.3 24h after ingestion PT > 100s
 Renal failure in pts. c̄ advanced CLF Or all of: Or 3 out of 5 of:
 Dx of exclusion PT > 100s Drug-induced
Cr > 300uM Age <10 or >40
Pathophysiology: “Underfill theory” Grade 3/4 encephalopathy >1wk from jaundice to
 Cirrhosis → splanchnic arterial vasodilatation → encephalopathy
effective circulatory volume → RAS activation → renal PT > 50s
arterial vasoconstriction. BR ≥ 300uM
 Persistent underfilling of renal circulation → failure

Classification
 Type 1: rapidly progressive deterioration (survival
<2wks)
 Type 2: steady deterioration (survival ~6mo)

Rx
 IV albumin + splanchnic vasoconstrictors (terlipressin)
 Haemodialysis as supportive Rx
 Liver Tx is Rx of choice
© Alasdair Scott, 2012 88
 Cirrhosis
Causes Ix
 Common Bloods
 Chronic EtOH  FBC: ↓WCC and ↓ plats indicate hypersplenism
 Chronic HCV (and HBV)  ↑LFTs
 NAFLD / NASH  ↑INR
 Other  ↓Albumin
 Genetic: Wilson’s, α1ATD, HH, CF
 AI: AH, PBC, PSC Find Cause
 Drugs: Methotrexate, amiodarone, methyldopa,  EtOH: ↑MCV, ↑GGT
INH  NASH: hyperlipidaemia, ↑ glucose
 Neoplasm: HCC, mets  Infection: Hep, CMV, EBV serology
 Vasc: Budd-Chiari, RHF, constrict. pericarditis  Genetic: Ferritin, α1AT, caeruloplasmin (↓ in Wilson’s)
 Autoimmune: Abs (there is lots of cross-over)
Signs  AIH: SMA, SLA, LKM, ANA
 PBC: AMA
Hands  PSC: ANCA, ANA
 Clubbing (± periostitis)  Ig: ↑IgG – AIH, ↑IgM – PBC
 Leuconychia (↓ albumin)  Ca: α-fetoprotein
 Terry’s nails (white proximally, red distally)
 Palmer erythema Abdo US + PV Duplex
 Dupuytron’s contracture  Small / large liver
 Focal lesions
Face  Reversed portal vein flow
 Pallor: ACD  Ascites
 Xanthelasma: PBC
 Parotid enlargement (esp. c̄ EtOH) Ascitic Tap + MCS
 PMN >250mm indicates SBP
3

Trunk
 Spider naevi (>5, fill from centre) Liver biopsy
 Gynaecomastia
 Loss of 2 sexual hair
O
Mx
General
Abdo  Good nutrition
 Striae  EtOH abstinence: baclofen helps ↓ cravings
 Hepatomegaly (may be small in late disease)  Colestyramine for pruritus
 Splenomegaly  Screening
 Dilated superficial veins (Caput medusa)  HCC: US and AFP
 Testicular atrophy  Oesophageal varices: endoscopy

Specific
Complications  HCV: Interferon-α
 PBC: Ursodeoxycholic acid
1. Decompensation → Hepatic Failure  Wilson’s: Penicillamine
 Jaundice (conjugated)
 Encephalopathy Complications
 Hypoalbuminaemia → oedema + ascites  Varices: OGD screening + banding
 Coagulopathy → bruising  HCC: US + AFP every 3-6mo
 Hypoglycaemia
Decompensation
2. SBP  Ascites: fluid and salt restrict, spiro, fruse, tap, daily wt
 Coagulopathy: Vit K, platelets, FFP, blood
3. Portal Hypertension: SAVE  Encephalopathy: avoid sedatives, lactulose ± enemas,
 Splenomegaly rifaximin
 Ascites  Sepsis / SBP: tazocin (avoid gent: nephrotoxicity)
 Varices  Hepatorenal syndrome: IV albumin + terlipressin
 Oesophageal varices (90% of cirrhotics)
 Caput medusa Child-Pugh Grading of Cirrhosis
 Worsens existing piles  Predicts risk of bleeding, mortality and need for Tx
 Encephalopathy  Graded A-C using severity of 5 factors
 Albumin
4. ↑ risk of HCC  Bilirubin
 Clotting
 Distension: Ascites
 Encephalopathy
 Score >8 = significant risk of variceal bleeding
© Alasdair Scott, 2012 89
 Portal Hypertension
Causes Sequelae: SAVE
 Pre-hepatic: portal vein thrombosis (e.g. pancreatitis)  Splenomegaly
 Hepatic: cirrhosis (80% in UK), schisto (commonest  Ascites
worldwide), sarcoidosis.  Varices
 Post-hepatic: Budd-Chiari, RHF, constrictive  Encephalopathy
pericarditis, TR

Portosystemic Anastomoses Ascites

Effect Portal Systemic Pathophysiology

F =
Oesophageal Left and short Inf. oesophageal  Back-pressure → fluid exudation
varices gastric veins veins  ↓ effective circulating volume → RAS activation
Caput medusae Peri-umbilical Superficial abdo wall  (In cirrhosis: ↓ albumin → ↓ plasma oncotic pressure
veins veins and aldosterone metabolism impaired)
Haemorrhoids Sup. rectal veins Inf. and mid. Rectal
(worsened veins Symptoms
 Distension → abdominal discomfort and anorexia
Prominent Abdominal Veins  Dyspnoea
 A lot more common than caput medusa  ↓ venous return
 Blood flow down below the umbilicus: portal HTN
 Blood flow up below the umbilicus: IVC obstruction Differential: Serum Ascites Albumin Gradient (SAGG)
 SAAG ≥1.1g/dL = Portal HTN (97% accuracy)
Encephalopathy  Pre-, hepatic and post
 Cirrhosis in 80%
Pathophysiology  SAAG <1.1g/dL = Other Causes
 ↓ hepatic metabolic function  Neoplasia: peritoneal or visceral (e.g. ovarian)
 Diversion of toxins from liver directly into systemic  Inflammation: e.g. pancreatitis
system.  Nephrotic Syndrome
 Infection: TB peritonitis
 Ammonia accumulates and pass to brain where
astrocytes clear it causing glutamate → glutamine
Ix
 ↑ glutamine → osmotic imbalance → cerebral oedema.
 Bloods: FBC, U+E, LFTs, INR, chronic hepatitis screen
Classification  US: confirm ascites, liver echogenicity, PV duplex
 1: Confused – irritable, mild confusion, sleep inversion  Ascitic tap
 2: Drowsy – ↑ disorientated, slurred speech, asterixis  MCS and AFB
 Cytology
 3: Stupor – rousable, incoherence
 Chemistry: albumin, LDH, glucose, protein
 4: Coma – unrousable, ± extensor plantars
 SAAG = serum albumin – ascites albumin
 Liver biopsy
Presentation
 Asterixis, ataxia
Rx
 Confusion
 Daily wt. aiming for ≤0.5kg/d reduction
 Dysarthria
 Fluid restrict <1.5L/d and low Na diet
 Constructional apraxia
 Spironolactone + frusemide (if response poor)
 Seizures  Therapeutic paracentesis c̄ albumin infusion (100ml
20% albumin /L drained)
Precipitants → HEPATICS
 Respiratory compromise
 Haemorrhage: e.g. varices
 Pain / discomfort
 Electrolytes: ↓K, ↓Na  Renal impairment
 Poisons: diuretics, sedatives, anaesthetics  Refractory: TIPSS
 Alcohol
 Tumour: HCC SBP
 Infection: SBP, pneumonia, UTI, HDV  Pt. c̄ ascites and peritonitic abdomen
 Constipation (commonest cause)  E. coli, Klebsiella, Streps
 Sugar (glucose) ↓: e.g. low calorie diet  Complicated by hepatorenal syn. in 30%

3
Ix: ascitic PMN > 250mm + MC+S
Ix
 Rx: Tazocin or cefotaxime until sensitivities known
 ↑ plasma NH4
 Prophylaxis: high recurrence  cipro long-term
Rx
 Nurse 20O head up Splenomegaly
 Correct any precipitants  Splenic congestion
 Avoid sedatives  Hypersplenism: ↓ WCC, ↓ plats
 Lactulose ± PO4 enemas to ↓ nitrogen-forming bowel
bacteria → 2-4 soft stools/d
 Consider rifaximin PO to kill intestinal microflora
© Alasdair Scott, 2012 90
Alcoholism Alcoholic Hepatitis
Effects Presentation
 Anorexia
Hepatic  D/V
 Fatty liver → hepatitits → cirrhosis  Tender hepatomegaly
 AST:ALT >2, ↑ GGT  Ascites
 Severe: Jaundice, bleeding, encephalopathy
GIT
 Gastritis, erosions Ix
 PUD  Bloods: ↑MCV, ↑GGT, AST:ALT>2
 Varices  Ascitic tap
 Pancreatitis  Abdo US + PV duplex
 Carcinoma
Rx
CNS
 Stop EtOH
 Poor memory / cognition
 Rx withdrawal
 Peripheral polyneuropathy (mainly sensory)
 High dose B vitamins: Pabrinex
 Wernicke’s encephalopathy
 Optimise nutrition
 Confusion
 Ophthalmoplegia (nystagmus, LR palsy)  Daily wt., LFT, U+E, INR
 Ataxia  Mx complications of failure
 Korsakoff’s: amnesia → confabulation
 Fits, falls Prognosis
 Maddrey score predicts mortality
Heart  Mild: 0-5% 30d mortality
 Arrhythmias: e.g. AF  Severe: 50% 30d mortality
 Dilated cardiomyopathy  1yr after admission: 40% mortality
 ↑BP

Blood
 ↑ MCV
 Folate deficiency → anaemia

Dx: CAGE
 Cut down?
 Annoyed by people’s criticisms
 Guilty about drinking
 Eye opener?

Withdrawal
 10-72h after last drink
 Consider in new ward pt (≤3d) c̄ acute confusion
 Signs
 ↑HR, ↓BP, tremor
 Confusion, fits, hallucinations: esp formication (DTs)
 Rx
 Tapering regimen of chlordiazepoxide PO /
lorazepam IM
 Thiamine

Mx
 Group therapy or self-help (e.g. AA)
 Baclofen: ↓ cravings
 Acamprosate: ↓ cravings
 Disulfiram: aversion therapy

© Alasdair Scott, 2012 91

Viral Hepatitis Non-alcoholic Fatty Liver Disease
(NAFLD)
Types  Cryptogenic cause of hepatitis and cirrhosis assoc. c̄
insulin resistance and the metabolic syndrome.
Type Spread Cause
 Non-alcoholic steatohepatitis (NASH) is most extreme
A FO Seafood, especially abroad form and → cirrhosis in 10%
B IV Blood, body fluids, babies (vertical)
C IV Mainly blood. Less vertical cf. HCV
Risk Factors
D IV Dependent on prior HBV infection
 Obesity
E FO Developing world
 HTN
 T2DM
Presentation  Hyperlipidaemia
Prodromal Phase
 Seen particularly in HAV and BV
Presentation
 Flu-like, malaise, arthralgia, nausea  Mostly asymptomatic
 Distaste for cigarettes in Hep A  Hepatomegaly and RUQ discomfort may be present.

Icteric Phase Metabolic Syndrome

 Acute jaundice in A>B>C (99, 75 and 25%)  Central obesity (↑ waist circumference) and two of:
 ↑ Triglycerides
 Hepatitis
 ↓ HDL
 Abdo pain
 HTN
 Hepatomegaly
 Hyperglycaemia: DM, IGT, IFG
 Cholestasis: dark urine, pale stools
 Extrahepatic features due to complexes (esp. Hep B)
 Urticaria or vasculitic rash Ix
 Cryoglobulinaemia  BMI
 PAN  Glucose, fasting lipids
 GN  ↑ transaminases: AST:ALT <1
 Arthritis  Liver biopsy

Chronic Phase Mx
 Mainly HCV and childhood HBV  Lose wt.
 Cirrhosis → ↑ risk of HCC  Control HTN, DM and lipids

Hep B
 Carrier: 10%
 HBsAg +ve > 6mo
 Chronic hepatitis: 10%
Budd-Chiari Syndrome
 Hepatic vein obstruction → ischaemia and hepatocyte
 Cirrhosis: 5%
damage → liver failure or insidious cirrhosis.
Hep C
 Carrier: 80% Causes
 HCV RNA+ve >6mo  Hypercoagulable states: myeloproliferative disorders
 Chronic hepatitis: 80% (PV = commonest cause), PNH, anti-phospholipid, OCP
 Cirrhosis: 20%  Local Tumour: HCC
 Congenital: membranous obstruction of IVC
Ix
 FBC, LFTs, clotting Presentation
 Hep A/B/C serology  RUQ pain: stretching of Glisson’s capsule
 Hepatomegaly
Rx  Ascites: SAAG ≥1.1g/dL
 Supportive  Jaundice (and other features of liver failure)
 No EtOH
 Avoid hepatotoxic drugs (e.g. aspirin) Ix
 Anti-viral  Bloods: FBC, clotting, LFTs
 Indicated in chronic disease  US + hepatic vein Doppler
 HBV: PEGinterferon  Ascitic tap: ↑↑ protein (>2.5g/dL) c̄ ↑SAAG (≥1.1g/dL)
 HCV: PEGinterferon + ribavarin  Other: JAK2 mutation analysis, RBC CD55 and CD59
 Seroconversion: HBV – 40%, HCV – 10%
Rx
 Anticoagulate unless there are varices
 Ascites: fluid and salt restrict, spiro, fruse, tap, daily wt
 Other options: thrombolysis, angioplasty, TIPSS
 Transplant if fulminant hepatic failure or cirrhosis
 Rx underlying cause
© Alasdair Scott, 2012 92
Hereditary Haemochromatosis α1-Antitrypsin Deficiency
Epidemiology Epidemiology
 Prevalence: 1/3000, 10% are carriers.  Prevalence: 1/4000, 10% are carriers
 Age of onset: 40-60yrs (women later due to menses)  Genetics: AR, Chr 14
 Genetics: AR, HFE gene (High FE) on Chr6 (C282Y)  Homozygotes have PiZZ phenootype

Pathophysiology
 Inherited, multisystem disorder resulting from abnormal
iron metabolism.
 ↑ intestinal Fe absorption (↑ enterocyte DMT + ↓
hepatocyte hepcidin) → deposition in multiple organs.
Pathophysiology
 α1AT is a serpin involved in control of the inflammatory
cascade by inhibiting neutrophil elastase.
 α1AT is synthesised in the liver and comprises 90% of
se α1-globulin on electrophoresis.

Clinical Features: iron MEALS Presentation

 Variable presentation
Myocardial  Neonatal and childhood hepatitis
 Dilated cardiomyopathy  15% of adults develop cirrhosis by 50yrs
 Arrhythmias  75% of adults have emphysema (esp. smokers)

Endocrine Ix
 Pancreas: DM  Blood: ↓ se α1AT levels
 Pituitary: hypogonadism → amenorrhoea, infertility  Liver biopsy: PAS+ve, diastase-resistant globules
 Parathyroid: hypocalcaemia, osteoporosis  CXR: emphysematous changes
 Spirometry: obstructive defect
Arthritis  Prenatal Dx: possible by CVS
 2 and 3 MCP joints, knees and shoulders
nd rd

Liver Mx
 Chronic liver disease → cirrhosis → HCC  Mostly supportive for pulmonary and hepatic
 Hepatomegaly complications.
 Quit smoking
Skin  Can consider α1AT therapy from pooled donors.
 Slate grey discolouration

Ix
 Bloods: ↑LFT, ↑ ferritin, ↑Fe, ↓TIBC, glucose, genotype
 X-ray: chondrocalcinosis
 ECG, ECHO
 Liver biopsy: Pearl’s stain to quantify Fe and severity
 MRI: can estimate iron loading

Rx
 Iron removal
 Venesection: aim for Hct <0.5
 Desferrioxamine is 2nd line
 General
 Monitor DM
 Low Fe diet
 Screening
 Se ferritin and genotype
 Screen 1st degree relatives
 Transplant in cirrhosis

Prognosis
 Venesection returns life expectancy to normal if non-
cirrhotic and non-diabetic.
 Cirrhotic patients have >10% chance of HCC

© Alasdair Scott, 2012 93

Wilson’s Disease Autoimmune Hepatitis
Epidemiology Pathophysiology
 Prevalence: 3/100,000  Inflammatory disease of unknown cause characterised
 Age: presents between childhood and 30 (never >56) by Abs directed vs. hepatocyte surface antigens
 Genetics: AR, ATP7B gene on Chr 13  Predominantly young and middle-aged women
 Classified according to Abs
 T1: Adult, SMA+ (80%), ANA+ (10%), ↑IgG
Pathophysiology  T2: Young, LKM+
 T3: Adult, SLA+
 Mutation of Cu transporting ATPase
 Impaired hepatocyte incorporation of Cu into
caeruloplasmin and excretion into bile. Presentation
 Cu accumulation in liver and, later, other organs  Teens and early 20s (25%)
 Constitutional: fatigue, fever, malaise
 Cushingoid: hirsute, acne, striae
 Hepatitis
Clinical Features: CLANKAH  HSM
 Fever
Cornea  Amenorrhoea
 Kayser-Fleischer rings (70%, may need slit-lamp)  Polyarthritis
 Pulmonary infiltration
Liver Disease  Pleurisy
 Children usually present c̄ acute hepatitis.  Post-/peri-menopausal
 Fulminant necrosis may occur  Present insidiously c̄ chronic liver disease
 → cirrhosis

Arthritis Associated Diseases

 Chondrocalcinosis  Autoimmune thyroiditis
 Osteoporosis  DM
 Pernicious anaemia
Neurology  PSC
 Parkinsonism: bradykinesia, tremor, chorea, tics  UC
 Spasticity, dysarthria, dysphagia  GN
 Ataxia  AIHA (Coombs +ve)
 Depression, dementia, psychosis

Kidney
Ix
 Fanconi’s syn. (T2 RTA) → osteomalacia
 ↑LFTs
Abortions  ↑IgG
 Auto Abs: SMA, LKM, SLA, ANA
Haemolytic anaemia  ↓WCC and ↓plats = hypersplenism
 Coombs’ negative  Liver biopsy

Ix Mx
 Bloods: ↓Cu, ↓ caeruloplasmin  Immunosuppression
 Prednisolone
NB. Caeruloplasmin is an acute-phase protein and may  Azathioprine as steroid-sparer
be high during infection. It may also be low protein-  Liver transplant (disease may recur)
deficient states: nephrotic syndrome, malabsorption

 ↑ 24h urinary Cu Prognosis

 Liver biopsy: ↑ hepatic Cu  Remission in 80% of patients
 MRI: basal-ganglia degeneration  10yr survival 80%

Rx
 Diet: avoid high Cu foods: liver, chocolate, nuts
 Penicillamine lifelong (Cu chelator)
 SE: nausea, rash, ↓WCC, ↓Hb, ↓plats, lupus,
haematuria
 Monitor FBC and urinary Cu excretion
 Liver Tx if severe liver disease
 Screen siblings

© Alasdair Scott, 2012 94

Primary Biliary Cirrhosis Primary Sclerosing Cholangitis
Epidemiology Pathophysiology
 Prev: ≤4/100,000  Inflammation, fibrosis and strictures and intra- and
 Sex: F>>M = 9:1 extra-hepatic ducts.
 Age: 50s  Chronic biliary obstruction → 2O biliary cirrhosis → liver
failure

Pathology
 Intrahepatic bile duct destruction by chronic Epidemiology
granulomatous inflammation → cirrhosis  Age: 30-50yrs
 Sex: M>F = 2:1

Presentation: PPBBCCS
 Often asympto and Dx incidentally (↑ALP)
Presentation
 May be asypmto and Dx incidentally (↑ALP)
 Jaundice occurs late
 Pruritus and fatigue
Symptoms
 Pigmentation of face
 Jaundice
 Bones: osteoporosis, osteomalacia (↓ vit D)
 Pruritus and fatigue
 Big organs: HSM
 Abdo pain
 Cirrhosis and coagulopathy (↓ vit K)
 Cholesterol ↑: xanthelasma, xanthomata Signs
 Steatorrhoea  Jaundice: dark urine, pale stools
 HSM

Associated Diseases Complications

 Thyroid disease  Bacterial cholangitis
 RA, Sjogrens, scleroderma  ↑ Cholangiocarcinoma
 Coeliac disease  ↑ CRC
 RTA
 Membranous GN
Associated Diseases
 UC
Ix  3% of those c̄ UC have PSC
 LFTs: ↑↑ALP, ↑↑GGT, ↑AST/ALT  80-100% of those c̄ PSC have UC/Crohn’s
 Late: ↑BR, ↑PT, ↓albumin  Crohn’s (much rarer)
 Abs: AMA+ (98%)  AIH
 ↑IgM  HIV
 ↑ cholesterol
 ± ↑ TSH
 US to exclude extra-hepatic cholestasis Ix
 Liver biopsy: non-caseating granulomatous inflam  LFTs: ↑ALP initially, then ↑BR
 Abs: pANCA (80%), ANA and SMA may be +ve
 ERCP/MRCP: “beaded” appearance of ducts
Rx  Biopsy: fibrous, obliterative cholangitis
 Symptomatic
 Pruritus: colestyramine, naltrexone
 Diarrhoea: codeine phosphate Rx
 Osteoporosis: bisphosphonates  No curative medical therapy: transplant needed
 Specific  Symptomatic
 ADEK vitamins  Pruritus: colestyramine, naltrexone
 Ursodeoxcholic acid: ↓LFTs but no effect on  Diarrhoea: codeine phosphate
mortality or need for transplant  Specific
 Liver transplant  ADEK vitamins
 End-stage disease or intractable pruritus  Ursodeoxycholic acid improves cholestasis only
 Recurrence occurs in ~20% but doesn’t usually  Abx for cholangitis
→ graft failure.  Endoscopic stenting for dominant strictures
 Screening
 Cholangiocarcinoma: US + Ca19-9
Prognosis  CRC: colonoscopy
 Once jaundice develops survival is <2yrs  Transplant
 Recurrence occurs in 30%

© Alasdair Scott, 2012 95

Liver Tumours Liver Transplant in CLD
Pathology Types
 90% of liver tumours are 2O metastases  Cadaveric: heart-beating or non-heart beating
 1O in men: stomach, lung, colon  Live: right lobe
 1O in women: breast, colon, stomach, uterus
 Less common: pancreas, leukaemia, lymphoma Indications
 90% of primary tumours are HCC.  Advanced cirrhosis
 Benign tumours: haemangiomas, adenomas, cysts  HCC

Symptoms Contraindications
 Benign tumours are usually asymptomatic  Extra-hepatic malignancy
 Systemic: fever, malaise, wt. loss, anorexia  Severe cardiorespiratory disease
 RUQ pain: stretching of Glisson’s capsule  Systemic sepsis
 Jaundice is often late, except in cholangiocarcinoma  HIV infection
 May rupture → intraperitoneal haemorrhage  Non-compliance c̄ drug therapy

Signs Post-op
 Hepatomegaly: smooth or hard and irregular  12-24h on ITU
 Signs of chronic liver disease  Immunosuppression
 Abdominal mass  Ciclosporin / Tacrolimus +
 Hepatic bruit (HCC)  Azathioprine / Mycophenolate Mofetil +
 Prednisolone
Ix
 Bloods: LFTs, hepatitis serology, AFP Complications
 Imaging:  Acute rejection (T-cell mediated)
 US or CT / MRI ± guided diagnostic biopsy  50% @ 5-10 days
 ERCP + biopsy in suspected cholangiocarcinoma  Pyrexia, tender hepatomegaly
 Biopsy (seeding may occur along tract)  ↑ or change immunosuppressants
 Find primary: e.g. colonoscopy, mammography  Sepsis
 Hepatic artery thrombosis
Liver Mets  CMV infection
 Rx and prognosis vary c̄ type and extent of 1O  Chronic rejection (6-9mo): shrinking bile ducts
 Small, solitary CRC mets may be resectable  Disease recurrence (e.g. HBV)
 Advanced disease  prognosis: < 6mo
Prognosis
HCC  Depends on disease aetiology
 Rare in West, common in China and sub-Saharan Africa  60-90% 5ys

Causes
 Viral hepatitis
 Cirrhosis: EtOH, HH, PBC
 Aflatoxins (produced by Aspergillus)

Mx
 Resection of solitary tumours improves prognosis (13 →
59%), but 50% have recurrence.
 Also: chemo, percutaneous ablation and embolization

Cholangiocarcinoma
 Biliary tree malignancy (10% of liver 1O tumours)

Causes
 Flukes (Clonorchis)
 PSC
 Congenital biliary cysts
 UC

Presentation
 Fever, malaise
 Abdominal pain, ascites, jaundice
 ↑BR, ↑↑ALP

Mx
 30% resectable
 © Palliative stenting:
Alasdair Scott, 2012 percutaneous or ERCP 96
 Inflammatory Bowel Disease: Pathology and Presentation
Epidemiology Pathology
UC Crohn’s UC Crohn’s
Prev 100-200 /100,000 50-100 /100,000 Macroscopic
Age 30s 20s Location Rectum + colon Mouth to anus
Sex F>M (just) ± backwash ileitis esp. terminal ileum
Aet Concordance = 10% Concordance = 70% Distribution Contiguous Skip lesions
Smoking protective Smoking ↑ risk Strictures No Yes
TH2-mediated TH1/TH17-mediated
Microscopic
Inflammation Mucosal Transmural
Crypt Abscesses
Ulceration Shallow, broad Deep, thin, serpiginous
→ cobblestone mucosa
Fibrosis None Marked
Granulomas None Present
Pseudoplyps Marked Minimal
Fistulae No Yes
Presentation

UC Crohn’s
Symptoms
Systemic Fever, malaise, anorexia, wt. loss in active disease
Abdominal  Diarrhoea  Diarrhoea (not usually bloody)
 Blood ± mucus PR  Abdominal pain
 Abdominal discomfort  Wt. loss
 Tenesmus, faecal urgency

Signs
Abdominal  Fever  Aphthous ulcers, glossitis
 Tender, distended abdomen  Abdominal tenderness
 RIF mass
 Perianal abscesses, fistulae, tags
 Anal / rectal strictures
Extra-abdominal Skin Joints
 Clubbing  Arthritis (non-deforming, asymm)
 Erythema nodosum  Sacroiliitis
 Pyoderma gang (esp. UC)  Ank spond
HPB
Eyes  PSC + cholangiocarcinoma (esp. UC)
 Iritis  Gallstones (esp. Crohn’s)
 Episcleritis  Fatty liver
 Conjunctivitis Other
 Amyloidosis
 Oxalate renal stones (esp. Crohns)

Complications  Toxic megacolon  Fistulae

 Diameter >6cm  Entero-enteric/colonic → diarrhoea
 Risk of perforation  Enterovesical → frequency, UTI
 Bleeding  Enterovaginal
 Malignancy  Perianal → “pepperpot” anus
 CRC in 15% c̄ pancolitis for 20yrs  Strictures → obstruction
 Cholangiocarcinoma  Abscesses
 Strictures → obstruction  Abdominal
 Venous thrombosis  Anorectal
 Malabsorption
 Fat → Steatorrhoea, gallstones
 B12 → megaloblastic anaemia
 Vit D → osteomalacia
 Protein → oedema
 Toxic megacolon and Ca may occur (< cf.
UC)
© Alasdair Scott, 2012 97
 Ulcerative Colitis: Management
Ix Inducing Remission in Mild / Mod Disease
 Bloods:  OPD-based
 FBC: ↓Hb, ↑WCC
 LFT: ↓albumin Oral Therapy
 ↑CRP/ESR  1st line: 5-ASAs
 Blood cultures  2nd line: prednisolone
 Stool
 MCS: exclude Campy, Shigella, Salmonella… Topical Therapy: mainly left-sided disease
 CDT: C. diff may complicate or mimic  Proctitis: suppositories
 Imaging  More proximal disease: enemas or foams
 AXR: megacolon (>6cm), wall thickening  5-ASAs ± steroids (prednisolone or budesonide)
 CXR: perforation
 CT Additional Therapy: steroid sparing
 Ba / gastrograffin enema  Azathioprine or mercaptopurine
 Lead-pipe: no haustra  Infliximab: steroid-dependent pts
 Thumbprinting: mucosal thickening
 Pseudopolyps: regenerating mucosal island Maintaining Remission
 Ileocolonoscopy + regional biopsy: Baron Score  1st line: 5-ASAs PO – sulfasalazine or mesalazine
 Topical Rx may be used in proctitis
Severity  2nd line: Azathioprine or 6-mercaptopurine
 Relapsed on ASA or are steroid-dependent
Truelove and Witts Criteria  Use 6-mercaptopurine if azathioprine intolerant
 3rd line: Infliximab / adalimumab
Mild Moderate Severe
Motions <4 4-6 >6
Emergency Surgery
PR bleed small moderate large
 20% require surgery at some stage
Temp Apyrexic 37.1-37.8 >37.8
 30% c̄ colitis require surgery w/i 5yrs
HR <70 70-90 >90
Hb >11 10.5-11 <10.5
Indications
ESR <30 >30
 Toxic megacolon
 Perforation
 Massive haemorrhage
Acute Severe UC  Failure to respond to medical Rx
 Resus: Admit, IV hydration, NBM
 Hydrocortisone: IV 100mg QDS + PR Procedures
 Transfuse if required  Total / subtotal colectomy c̄ end ileostomy ± mucus
 Thromboprophylaxis: LMWH fistula
 Monitoring  Followed after ~3mo by either
 Bloods: FBC, ESR, CRP, U+E  Completion proctectomy + Ileal-pouch anal
 Vitals + stool chart anastomosis (IPAA) or end ileostomy
 Twice daily examination  Ileorectal anastomosis (IRA)
 ± AXR  Panproctocolectomy + permanent end ileostomy
 Acute colitis op mortality: 7% (30% if perforated)
NB. RCTs show no benefit of Abx: not routinely recommended
 May use: megacolon, perforation, uncertain Dx
Elective Surgery
Acute Complications
 Perforation Indications
 Bleeding  Chronic symptoms despite medical therapy
 Toxic megacolon (>6cm)  Carcinoma or high-grade dysplasia
 VTE
Procedures
Improvement → oral therapy  Panproctocolectomy c̄ end ileostomy or IPAA
 Switch to oral pred + a 5-ASA  Total colectomy c̄ IRA
 Taper pred after full remission
Surgical Complications
No Improvement → rescue therapy  Abdominal
 On day 3: stool freq >8 or CRP >45  SBO
 Predicts 85% chance of needing a colectomy during  Anastomotic stricture
the admission  Pelvic abscess
 Discussion between pt, physician and surgeon  Stoma: retraction, stenosis, prolapse, dermatitis
 Medical: ciclosporin, infliximab or visilizumab (anti-T cell)  Pouch
 Surgical  Pouchitis (50%): metronidazole + cipro
 ↓ female fertility
 Faecal leakage
© Alasdair Scott, 2012 98
 Crohn’s Disease: Management
Ix Inducing Remission in Mild / Mod Disease
 Bloods: (top 3 are severity markers)  OPD treatment
 FBC: ↓Hb, ↑WCC
 LFT: ↓albumin Supportive
 ↑CRP/ESR  High fibre diet
 Haematinics: Fe, B12, Folate  Vitamin supplements
 Blood cultures
 Stool Oral Therapy
 MCS: exclude Campy, Shigella, Salmonella…  1st line
 CDT: C. diff may complicate or mimic  Ileocaecal: budesonide
 Imaging  Colitis: sulfasalazine
 AXR: obstruction, sacroileitis  2nd line: prednisolone (tapering)
 CXR: perforation  3rd line: methotrexate
 MRI  4th line: infliximab or adalimumab
 Assess pelvic disease and fistula
 Assess disease severity Perianal Disease
 Small bowel follow-through or enteroclysis  Occurs in ~50%
 Skip lesions  Ix: MRI + EUA
 Rose-thorn ulcers  Rx
 Cobblestoning: ulceration + mural oedema  Oral Abx: metronidazole
 String sign of Kantor: narrow terminal ileum  Immunosuppression ± infliximab
 Endoscopy  Local surgery ± seton insertion
 Ileocolonoscopy + regional biopsy: Ix of choice
 Wireless capsule endoscopy Maintaining Remission
 Small bowel enteroscopy  1st line: azathioprine or mercaptopurine
 2nd line: methotrexate
 3rd line: Infliximab / adalimumab
Severe Attack
Assessment
Surgery
 ↑temp, ↑HR, ↑ESR, ↑CRP, ↑WCC, ↓albumin  50-80% need ≥1 operation in their life
 Never curative
Management  Should be as conservative as possible
 Resus: Admit, NBM, IV hydration
 Hydrocortisone: IV + PR if rectal disease Indications
 Abx: metronidazole PO or IV  Emergency
 Failure to respond to medical Rx
 Thromboprophylaxis: LMWH
 Intestinal obstruction or perforation
 Dietician Review
 Massive haemorrhage
 Elemental diet
 Elective
 Liquid prep of amino acids, glucose and fatty
 Abscess or fistula
acids
 Perianal disease
 Consider parenteral nutrition
 Chronic ill health
 Monitoring  Carcinoma
 Vitals + stool chart
 Daily examination Procedures
 Limited resection: e.g. ileocaecal
Improvement → oral therapy
 Stricturoplasty
 Switch to oral pred (40mg/d)
 Defunction distal disease c̄ temporary loop ileostomy
No Improvement → rescue therapy
Complications
 Discussion between pt, physician and surgeon
 Stoma complications
 Medical: methotrexate ± infliximab
 Enterocutaneous fistulae
 Surgical
 Anastomotic leak or stricture

Short gut
 <1-2m small bowel
 Features
 Steatorrhoea
 ADEK and B12 malabsorption
 Bile acid depletion → gallstones
 Hyperoxaluria → renal stones
 Rx
 Dietician
 Supplements or TPN
 Loperamide
© Alasdair Scott, 2012 99
 Coeliac Disease
Epidemiology Ix
 Prev: 0.5 – 1%  Bloods: FBC, LFTs (↓alb), INR, Vit D and bone, red cell
 Age: Any, bimodal: infancy and 50-60yrs folate, serum B12
 Sex: F>M  Abs
 Geo: ↑ in Ireland and N. Africa  Anti-endomysial IgA (95% specificity)
 Anti-TTG IgA
Pathophysiology  Both above ↓ c̄ exclusion diet
 HLA-DQ2 (95%) and DQ8  Anti-gliadin IgG persist c̄ exclusion diet)
 CD8+ mediated response to gliadin in gluten  IgA ↑ in most but may have IgA deficiency
 Stools
Presentation: GLIAD  Stool cysts and antibody: exclude Giardia
 OGD and duodenal biopsy
GI Malabsorption: fatigue, weakness  Subtotal villous atrophy
 Carb  Crypt hyperplasia
 N/V/D  Intra-epithelial lymphocytes
 Abdo distension + colic
 Flatus Rx
 Wt. ↓  Lifelong gluten-free diet
 Fat  Avoid: barley, rye, oats, wheat
 Steatorrhoea  OK: Maize, soya, rice
 Hyperoxaluria → renal stones  Verify diet by endomysial Ab tests
 Protein  Pneumovax as hyposplenic
 Protein-losing enteropathy if severe  Dermatitis herpetiformis: dapsone
 Haematinics
 ↓ Folate and Fe → anaemia
 Vitamins
 Vit D and Ca → bone pain, osteoporosis Malabsorption
 Vit K → petechiae and ↑INR
 B2 (riboflavin) → angular stomatitis Presentation
 B1 and B6 → polyneuropathy  Diarrhoea / Steatorrhoea
 Wt. loss
Lymphoma and Carcinoma  Lethargy
 Enteropathy-associated T-cell lymphoma
 Adenocarcinoma of small bowel Causes
 Other Ca: breast, bladder, breast  Common in UK: Coeliac, Chronic pancreatitis, Crohn’s
 Rarer
Immune Associations  ↓Bile: PBC, ileal resection, colestyramine
 IgA deficiency  Pancreatic insufficiency: Ca, CF, chronic panc
 T1DM  Small bowel: resection, tropical sprue, metformin
 PBC  Bacterial overgrowth: spontaneous, post-op
blind loops, DM, PPIs
Anaemia  Infection: Giardia, Strongyloides, Crypto parvum
 ↑ or ↓ MCV  Hurry: post-gastrectomy dumping
 Hyposplenism: Howell-Jolly bodies, target cells
Ix
Dermatological  Coeliac tests
 Dermatitis herpetiformis: 15-20%  Stool microscopy
 Symmetrical vesicles, extensor surfaces  Faecal elastase
 Esp. elbows  Hydrogen breath test
 Very itchy  MRI / CT
 Responds to gluten-free diet or dapsone
 ERCP (chronic pancreatitis)
 Biopsy: granular deposition of IgA
 Small bowel endoscopy
 Aphthous ulcers

© Alasdair Scott, 2012 100

Pancreatic Cancer Chronic Pancreatitis
Risk Factors: SINED Causes: AGITS
 Smoking  Alcohol
 Inflammation: chronic pancreatitis  Genetic
 Nutrition: ↑fat diet  CF
 EtOH  HH
 DM  Hereditary pancreatitis
 Immune
Pathology  Lymphoplasmacytic sclerosing pancreatitis (↑IgG4)
 Mostly ductal adenocarcinoma  Triglycerides ↑
 Metastasis early, present late  Structural
 60% head  Obstruction by tumour
 25% body  Pancreas divisum
 15% tail
 Endocrine tumours are rare Presentation
 Epigastric pain
 Bores through to back
Presentation
 Relieved by sitting back or hot water bottle →
 Typically male >60yrs
erythema ab igne
 Head: painless obstructive jaundice  Exacerbated by fatty food or EtOH
 c̄ dark urine + pale stools
 Steatorrhoea
 Body / Tail: epigastric pain  Wt. loss
 Radiates to back, relieved sitting forward  DM
 Anorexia and wt. loss  Epigastric mass: pseudocyst
 Acute pancreatitis
 Sudden onset DM in the elderly
Ix
Signs  ↑ glucose
 ↓ faecal elastase
 Epigastric mass
 US: pseudocyst
 Jaundice
 AXR: speckled pancreatic calcifications
 Palpable gallbladder
 CT: pancreatic calcifications
 Thrombophlebitis migrans (Trousseau Sign)
 Splenomegaly: PV thrombosis → portal HTN
 Ascites Rx
 Drugs
 Analgesia: may need coeliac plexus block
Ix
 Creon
 Bloods: cholestatic LFTs, ↑Ca19-9, ↑Ca
 ADEK vitamins
 Imaging  DM Rx
 US: pancreatic mass, dilated ducts, hepatic mets,
 Diet
allows biopsy
 No EtOH
 EUS: better than CT/MRI for staging
 ↓ fat, ↑ carb
 ERCP
 Surgery
 Shows anatomy
 Ind: unremitting pain, wt. loss
 Allows stenting
 Pancreatectomy
Rx Complications
 Surgery
 Pseudocyst
 Fit, no mets, tumour ≤3cm (≤10% of pts)
 DM
 Whipple’s pancreatoduodenectomy
 Pancreatic Ca
 Post-op chemo delays progression
 5ys = 5-14%  Biliary obstruction
 Palliation  Splenic vein thrombosis → splenomegaly
 Endoscopic / percutaneous stenting of CBD
 Palliative bypass surgery
 Pain relief: may need coeliac plexus block

Prognosis
 Mean survival <6mo Whipple’s Procedure
 5ys = <2%

I___I!_fiG

© Alasdair Scott, 2012 101

Carcinoid Tumours Nutritional Deficiencies
Pathology Vitamin A → Xerophthalmia
 Diverse group of neuroendocrine tumours of  Dry conjunctivae, develop spots (Bitots spots)
enterochromaffin cell origin capable of producing 5HT  Corneas become cloudy then ulcerate
 May secrete: 5-HT, VIP, gastrin, glucagon, insulin, ACTH  Night blindness → total blindness
 Carcinoid syndrome suggest bypass of first-pass
metabolism and is strongly assoc. c̄ metastatic disease. Thiamine (B1) → Beri Beri
 10% part of MEN1  Wet: heart failure + oedema
 Sites  Dry: polyneuropathy
 Appendix: 45%  Wernicke’s: ophthalmoplegia, ataxia, confusion
 Ileum: 30%
 Colorectum: 20% Niacin / Nicotinic acid (B3) → Pellagra
 Stomach: 10%  Diarrhoea, Dermatitis, Dementia
 Elsewhere in GIT and bronchus
 Also: neuropathy, depression, ataxia
 Consider all as malignant
 Causes: dietary, isoniazid, carcinoid syndrome

Pyridoxine (B6)
Presentation  Peripheral sensory neuropathy
 Cause: PZA
Local
 Appendicitis
Cyanocobalamin (B12)
 Intussusception or obstruction
 Glossitis → sore tongue
 Abdominal pain
 Peripheral neuropathy
 Paraesthesia
Carcinoid Syndrome: FIVE HT
 Early loss of vibration and proprioception
 Flushing: paroxysmal, upper body ± wheals
 → ataxia
 Intestinal: diarrhoea
 SCDC
 Valve fibrosis: tricuspid regurg and pulmonary stenosis  Dorsal and corticospinal tracts
 whEEze: bronchoconstriction  Sensory loss and UMN weakness
 Hepatic involvement: bypassed 1 pass metabolism
st
 Overall mixed UMN and LMN signs c̄ sensory
 Tryptophan deficiency → pellagra (3Ds) disturbance
 Extensor plantars + absent knee and ankle
jerks
Ix
 ↑ urine 5-hydroxyindoleacetic acid Vitamin C → Scurvy
 ↑ plasma chromogranin A  Gingivitis
 CT/MRI: find primary  Bleeding: gums, nose, hair follicles (petechial)
 Muscle pain / weakness
 Oedema
Rx  Corkscrew hairs
 Symptoms: octreotide or loperamide
 Curative Vitamin D → Osteomalacia
 Resection: tumours are v. yellow  Bone pain
 Give octreotide to avoid carcinoid crisis  #s

Carcinoid Crisis Vitamin K

 Tumour outgrows blood supply or is handled too
much → massive mediator release
 Vasodilatation, hypotension, bronchoconstriction,
hyperglycaemia
 Rx: high-dose octreotide
 ↓ factors: 2, 7, 9, 10, C and S
 Bruising, petechiae
 Bleeding: e.g. epistaxis, menorrhagia

Prognosis
 Median survival is 5-8yrs (~3yrs if mets present)

© Alasdair Scott, 2012 102

 Renal Physiology
The Glomerulus Diuretics
 Epithelial pouch invaginated by capillary tuft
 Semi-permeable filter Carbonic Anhydrase Inhibitors (acetazolamide)
 Endothelium  MOA: inhibit carbonic anhydrase in PCT
 Basement membrane  Effect: ↓ HCO3 reabsorption → small ↑ Na loss
 Epithelium  Use: glaucoma
 Mesangial cells are specialised smooth muscle cells that  SE: drowsiness, renal stones, metabolic acidosis
support the glomerulus and regulate blood flow and GFR

Loop Diuretics (fursemide, bumetanide)

Filtration
 Receive 25% of CO (1200ml/min)
 20% of blood volume is filtered (~250ml/min)
 GBM is –vely charged → retention of anionic proteins
such as albumin which are small enough to pass.
 Filtration is key to excretion of waste and remains
constant over a range of pressures (80-200mmHg).
 Flow will depend on Na and water reabsorption.
Na Reabsorption
 Main factor determining extracellular volume
 ↓BP and ↓NaCl @ macula densa (DCT) → renin release
→ aldosterone release → more Na/K pumps.
Water Reabsorption
 Determines ECF osmolality
 ↑osmolality or ↓BP → ADH release
The Nephron
PCT: Reabsorption of filtrate
 70% of total Na+ reabsorption
 Reabsorption of amino acids, glucose, cations
 Bicarbonate reabsorbed using carbonic anhydrase
Thick Ascending Limb: Creation of osmolality gradient
 20% of Na reabsorption
 Na/K/2Cl triple symporter
 MOA: inhibit Na/K/2Cl symporter in thick ascending limb
 Effect: massive NaCl excretion, Ca and K excretion
 Use: Rx of oedema – CCF, nephrotic syndrome,
hypercalcaemia
 SE: hypokalaemic met alkalosis, ototoxic, Hypovolaemia
Thiazide Diuretics (bendroflumethazide)
 MOA: inhibit NaCl co-transporter in DCT
 Effect: moderate NaCl excretion, ↑ Ca reabsorption
 Use: HTN, ↓ renal stones, mild oedema
 SE: ↓K, hyperglycaemia, ↑ urate (CI in gout)
K-Sparing Diuretics (spironolactone, amiloride)
 MOA
 Spiro: aldosterone antagonist
 Amiloride: blocks DCT/CD luminal Na channel
 Effect: ↑ Na excretion, ↓K and H excretion
 Use: used c̄ loop or thiazide diuretics to control K loss,
spiro has long-term benefits in aldosteronsim (LF, HF)
 SE: ↑K, anti-androgenic (e.g. gynaecomastia)
Osmotic Diuretics (mannitol)
 MOA: freely filtered and poorly reabsorbed
 Effect: ↓ brain volume and ↓ ICP
 Use: glaucoma, ↑ICP, rhabdomyolysis
 SE: ↓Na, pulmonary oedema, n/v

DCT: pH and Ca reabsorption

 5% of Na reabsorption
 Apical NaCl co-transporter
 Ca reabsorption under control of PTH
2+

Medullary CD: pH and K regulation

 Na reabsorption coupled to K or H excretion
 Basolateral aldosterone-sensitive Na/K pump

Cortical CD: Regulation of water reabsorption

 Water reabsorption controlled by aquaporin-2 channels

Endocrine Function
 Secretion of renin by juxtaglomerular apparatus
 EPO synthesis
 1α-hydroxylation of vitamin D (controlled by PTH)

© Alasdair Scott, 2012 104

Urine Urea and Creatinine
Haematuria Creatinine
Renal  Creatinine is synthesised during muscle turnover.
 Congenital: PCK  Freely filtered and small proportion secreted by PCT
 Trauma  ↑ muscle → ↑ creatinine: age, sex, race
 Infection: pyelonephritis  Plasma Cr doesn’t ↑ above normal until 50% ↓ in GFR
 Neoplasm
 Immune: GN, TIN
Urea
Extra-renal  Produced from ammonia by liver in ornithine cycle
 Trauma: stones, catheter  ↑ c̄ protein meal (e.g. upper GI bleed, supplements)
 Infection: cystitis, prostatitis, urethritis  ↓ c̄ hepatic impairment
 Neoplasm: bladder, prostate  10-70% is reabsorbed: depends on urine flow.
 Bleeding diathesis  ↓ flow → ↑ urea reabsorption (e.g. in dehydration)
 Drugs: NSAIDs, frusemide, cipro, cephalosporins

NB. False +ve: myoglobin, porphyria

Interpretation
 Isolated ↑ urea = ↓ flow (i.e. hypoperfusion / dehydration)
 ↑ U and ↑ Cr = ↓ filtration (i.e. renal failure)
Proteinuria
 30mg/dL = 1+
 300mg/dL = 3+ Creatinine Clearance
 PCR < 20mg/mM is normal, >300 = nephrotic  Vol of blood that can be cleared of a substance in 1min
 CrC roughly approximates GFR as it is freely filtered and
Causes only a small proportion secreted (~10%)
 Commonest  Slightly overestimates GFR
 DM  Requires urine concentration from 24h collection
 Minimal change  Can use radiolabelled EDTA: very rarely done
 Membranous
 Amyloidosis
 SLE
 Other
eGFR
 HTN (inc. PET)  Modifiation of Diet in Renal Disease (MDRD) equation
 ATN  Serum Cr, sex, age, race
 TIN  Obviates need for urine collection
 UTI
 Fever, orthostatic Problems
 Validated for patients c̄ established renal failure:
NB. False –ve: Bence-Jones protein ?applicable to general population.
 Most elderly people are in ≥ stage 3 CRF by eGFR:
Microalbuminuria may not progress or impinge on their health.
 Albumin 30-300mg/24h  eGFR is too pessimistic in mild renal impairment
 Causes: DM, ↑BP, minimal change GN

Casts
 RBC: glomerular haematuria
 WBC: interstitial nephritis or pyelonephritis
 Tubular: ATN

Differential
Pathology Urine
Glomerular Haematuria, proteinuria, red cells / casts
Tubular White cell casts, small protein, leukocyturia
Pre-renal Nothing
CRF Depends on cause

© Alasdair Scott, 2012 105

Causes of Renal Disease Presentation of Renal Failure
Pre-renal Uraemia (need GFR <15ml/min)
 Shock
 Renal Vascular Symptoms Signs
 RAS
 Toxins: NSAIDs, ACEi Pruritus Pale, sallow skin
 Thrombosis n/v, anorexia, wt. loss Striae
 Hepatorenal syn. Lethargy Pericardial or plueral rub
Confusion Fits
Restless legs Coma
Renal Metallic taste
 Glomerulonephritis Paraesthesia: neuropathy
 Acute Tubular Necrosis Bleeding
 Interstitial disease Chest pain: serositis
Hiccoughs

Post-renal
 Diseases of renal papillae, pelvis, ureters, bladder or
Protein loss and Na+ retention
urethra.
Symptoms Signs
 SNIPPIN
 Stone
 Neoplasm Polyuria, polydipsia Oedema
 Inflammation: stricture Oliguria, anuria ↑ JVP
 Prostatic hypertrophy Breathlessness HTN (or ↓BP)
 Posterior urethral valves
 Infection: TB, schisto
 Neuro: post-op, neuropathy Acidosis
Symptoms Signs

Breathlessness Kussmaul respiration

Confusion

Hyperkalaemia
Symptoms Signs

Palpitations Peaked T waves

Chest pain Flattened P waves
Weakness ↑ PR interval
Widened QRS
Sine-wave pattern → VF

Anaemia
Symptoms Signs

Breathlessness Pallor
Lethargy Tachycardia
Faintness Flow mumurs (ESM @ apex)
Tinnitus

Vitamin D Deficiency
Symptoms Signs

Bone pain Osteomalacia

#s - Looser’s zones (pseudo#s)
- Cupped mataphyses

© Alasdair Scott, 2012 106

 Urinary Tract Infection
Definitions Risk Factors
 Bacteriuria: bacteria in urine, symptomatic or asympto  Female
 UTI: symptomatic c̄ +ve culture or dipstick  Sex
 Urethral Syndrome: symptomatic but no bacteriuria  Pregnancy
 Menopause
Classification  DM
 Uncomplicated: normal GU tract and function  Abnormal tract: stone, obstruction, catheter,
 Complicated: abnormal GU tract, outflow obstruction, ↓ malformation
renal function, impaired host defence, virulent organism
 Recurrent: further infection c̄ new organism
 Relapse: further infection c̄ same organism Organisms
 E. coli
 Staphylococcus saprophyticus
Presentation  Proteus (alkaline urine → struvite renal stones)
 Klebsiella
Pyelonephritis
 Fever, rigors
 Loin pain and tenderness Ix
 Vomiting  Dipstick
 Oliguria if ARF  MSU for MCS
 Bloods: FBC, U+E, blood cultures (if systemic signs)
Cystitis  US: children, men, recurrence, pyelonephritis
 Frequency and urgency
 Polyuria Positive Culture
 Haematuria  >104 CFU/ml pure growth
 Dysuria  >103 CFU/ml pure growth of E. coli or S. saprophyticus
 Suprapubic tenderness  >105 CFU/ml mixed growth c̄ one predominant organism
 Foul smelling urine

Prostatitis Rx
 Flu-like symptoms
 Low backache General
 Dysuria  Drink plenty, urinate often
 Tender swollen prostate on PR
Cystitis
 Rx for 3-6d
Sterile Pyuria  Trimethoprim 200mg BD
 TB  Nitrofurantoin 50mg QDS (not in RF)
 Treated UTI  Cefalexin 500mg BD (good in RF)
 Appendicitis  Co-amoxiclav 625mg TDS
 Calculi
 TIN Pyelonephritis
 Papillary necrosis  Cefotaxime 1g IV BD for 10d
 Polycystic Kidney  No response: Augmentin 1.2g IV TDS + gentamicin
 Chemical cystitis (e.g. cyclophosphamide)

Prevention
 Drink more
 Abx prophylaxis
 ? cranberry juice

© Alasdair Scott, 2012 107

Glomerulonephritis Asymptomatic Haematuria
Features Causes
1. IgA Nephropathy
 Group of disorders resulting from glomerular damage
2. Thin BM
 Can → proteinuria ± haematuria
3. Alport’s
 Can → AKI or ESRF

1. IgA Nephropathy / Berger’s Disease

Presentations  Commonest GN in developed world
 Asymptomatic haematuria
 Nephrotic syndrome Features
 Nephritic syndrome  Young male c̄ episodic macroscopic haematuria
occurring a few days after URTI.
 Rapid recovery between attacks
Causes  ↑IgA
 Idiopathic  Can occasionally → nephritic syndrome
 Immune: SLE, Goodpastures, vasculitis
 Infection: HBV, HCV, Strep, HIV Biopsy: IgA deposition in mesangium
 Drugs: penicillamine, gold
 Amyloid Rx: Steroids or cyclophosphamide if ↓renal function

Prognosis: 20% ESRF after 20yrs

Ix
Blood 2. Thin BM Disease
 Basic: FBC, U+E, ESR  Autosomal dominant
 Complement (C3 and C4)  Commonest cause of asymptomatic haematuria
 Abs: ANA, dsDNA, ANCA, GBM
 Serum protein electrophoresis and Ig Features
 Infection: ASOT, HBC and HCV serology  Persistent, asymptomatic microscopic haematuria
 V. small risk of ESRF
Urine
 Dipstick: proteinuria ± haematuria
 Spot PCR 3. Alport’s Syndrome
 MCS  85% X-linked inheritance
 Bence-Jones protein
Features
Imaging  Haematuria, proteinuria → progressive renal failure
 CXR: infiltrates (Goodpasture’s, Wegener’s)  Sensorineural deafness
 Renal US ± biopsy  Lens dislocation and cataracts
 Retinal “flecks”
 Females: haematuria only
General Mx
 Refer to nephrologist
 Rx HTN aggressively (≤130/80)
 Include and ACEi / ARA

© Alasdair Scott, 2012 108

Nephritic Syndrome / Acute GN
 Haematuria (macro / micro) + red cell casts
 Proteinuria → oedema (esp. periorbital)
 Hypertension
 Oliguria and progressive renal impairment
Nephrotic Syndrome
 Proteinuria: PCR >300mg/mM or >3g/24h
 Hypoalbuminaemia: <35g/L
 Oedema: periorbital, genital, ascites, peripheral
 Often intravascularly depleted c̄ ↓ JVP (cf. CCF)

Causes Complications
1. Proliferative / post-streptococcal  Infection: ↓ Ig, ↓ complement activity
2. Crescentic / RPGN  VTE: up to 40%
 Hyperlipidaemia: ↑ cholesterol and triglycerides

1. Proliferative / Post-streptococcal Ix
 As for GN, check lipids
Features
 Biopsy
 Young child develops malaise and nephritic syndrome c̄
 All adults
smoky urine 1-2wks after sore throat or skin infection.  Steroids 1st c̄ children: mostly minimal change
 ↑ ASOT
 ↓C3
Secondary to Systemic Disease
Biopsy: IgG and C3 deposition  DM: glomerulosclerosis
 SLE: membranous
Rx: Supportive  Amyloidosis

Prognosis 1. Minimal Change Glomerulonephritis

 95% of children recover fully  Commonest cause of nephrotic syndrome in children
 Minority develop RPGN  Assoc.: URTI
 Biopsy: normal light micro, fusion of podocytes on EM
 Rx: steroids
2. Crescentic / RPGN  Prog: 1% → ESRF
 Most aggressive GN which can → ESRF in days
2. Membranous Nephropathy
Type 1: Anti-GBM (Goodpasture’s) – 5%  20-30% of adult nephrotic syndrome
 Ab to NC domain of collagen 4  Associations
 Haematuria and haemoptysis  Ca: lung, colon, breast
 CXR shows infiltrates  AI: SLE, thyroid disease
 Rx: Plasmapheresis and immunosuppression  Infections: HBV
 Drugs: Penicillamine, gold
Type 2: Immune Complex Deposition – 45%  Biopsy: subepithelial immune complex deposits
 Complication of any immune complex deposition  Rx: immunosuppression if renal function declines
 Berger’s, post-strep, endocarditis, SLE  Prog: 40% spontaneous remission

Type 3: Pauci Immune – 50% 3. FSGS

 cANCA: Wegener’s
 pANCA: microscopic polyangiitis, Churg-Strauss
 Even if ANCA+ve, may still be idiopathic
 i.e. no features of systemic vasculitis
 Commoner in Afro-Caribs
 Idiopathic or Secondary: VUR, Berger’s, SCD, HIV
 Biopsy: focal scarring, IgM deposition
 Rx: steroids or cyclophosphamide/ciclosporin
 Prog: 30-50% → ESRF (may recur in transplants)

5. Membranoproliferative / Mesangiocapillary GN
 Rare
 May → nephrotic (60%) or nephritic (30%) syndrome
 Asooc. c̄ HBV, HCV, endocarditis
 Prog: 50% → ESRF

Mx
 Monitor U+E, BP, fluid balance, wt.
 Treat underlying cause
 Symptomatic / Complication Rx:
 Oedema: salt and fluid restrict + frusemide
 Proteinuria: ACEi / ARA ↓ proteinuria
 ↑ Lipids: Statin
 VTE: Tinzaparin
 Rx HTN

© Alasdair Scott, 2012 109

 Acute Kidney Injury
Definition RIFLE Classification
 Significant decline in renal function over hrs or days  3 grades of AKI and 2 outcomes
manifesting as an abrupt and sustained ↑ in Se U and Cr  Classification determined by worst criteria

Causes (Pre-renal and ATN account for ~80%) Classification GFR UO

Risk ↑Cr x1.5 <0.5ml/kg/h x 6h
Pre-renal: commonest cause ↓GFR >25%
 Shock or renovascular compromise (e.g. NSAIDs, ACEi) Injury ↑Cr x 2 <0.5ml/kg/h x 12h
↓GFR >50%
Renal Failure ↑Cr x 3 <0.3ml/kg/h x 24h, or
 ATN: ↓GFR >75% anuria x12h
 Ischaemia: shock, HTN, HUS, TTP Loss Persistent ARF = complete LOF >1mo
 Direct nephrotoxins: drugs, contrast, Hb ESKD complete LOF >3mo
 Acute TIN: drug hypersensitivity
 Nephritic syndrome
Rx
Post-renal: SNIPPIN
General
 Identify and Rx pre-renal or post-renal causes
Presentation  Urgent US
 Uraemia / Azotaemia  Rx exacerbating factors: e.g. sepsis
 Acidosis  Give PPIs
 Hyperkalaemia  Stop nephrotoxins: NSAIDs, ACEi, gent, vanc
 Fluid overload  Stop metformin if Cr > 150mM
 Oedema, inc. pulmonary
 ↑ BP (or ↓) Monitor
 S3 gallop  Catheterise and monitor UO
 ↑ JVP  Consider CVP
 Fluid balance
Clinical Assessment  Wt.
1. Acute or chronic?
 Can’t tell for sure: Rx as acute Hyperkalaemia
 Chronic features  ECG Features (in order)
 Hx of comorbidity: DM, HTN  Peaked T waves
 Long duration of symptoms  Flattened P waves
 Previously abnormal bloods (GP records)  ↑ PR interval
 Widened QRS
2. Volume depleted?  Sine-wave pattern → VF
 Postural hypotension  Mx
 ↓ JVP  10ml 10% calcium gluconate
 ↑ pulse  100ml 20% glucose + 10u insulin (Actrapid)
 Poor skin turgor, dry mucus membranes  Salbutamol 5mg nebulizer
 Calcium resonium 15g PO or 30g PR
3. GU tract obstruction?  Haemofiltration (usually needed if anuric)
 Suprapubic discomfort
 Palpable bladder Pulmonary Oedema
 Enlarged prostate  Sit up and give high-flow O2
 Catheter  Morphine 2.5mg IV (± metoclopramide 10mg IV)
 Complete anuria (rare in ARF)  Frusemide 120-250mg IV over 1h
 GTN spray ± ISMN IVI (unless SBP <100)
4. Rare cause?  If no response consider:
 Assoc. c̄ proteinuria ± haematuria  CPAP
 Vasculitis: rash, arthralgia, nosebleed  Haemofiltration / haemodialysis ± venesection

Bleeding
Ix
 ↑ urea impairs haemostasis
Bloods: FBC, U+E, LFT, glucose, clotting, Ca, ESR
 FFP + plats as needed
ABG: hypoxia (oedema), acidosis, ↑K+
 Transfuse to maintain Hb >10
GN screen: if cause unclear
Urine: dip, MCS, chemistry (U+E, PCR, osmolality, BJP) Indications for Acute Dialysis (AEIOU)
ECG: hyperkalaemia 1. Persistent hyperkalaemia (>7mM)
CXR: pulmonary oedema 2. Refractory pulmonary oedema
Renal US: Renal size, hydronephrosis 3. Symptomatic uraemia: encephalopathy, pericarditis
4. Severe metabolic acidosis (pH <7.2)
NB. in pre-renal failure, urine is concentrated and Na is 5. Poisoning (e.g. aspirin)
reabsorbed → ↑osmolality, Na <20mM
© Alasdair Scott, 2012 110
 Management of Acute Renal Failure
Common Causes
 Pre-renal: shock (e.g. sepsis, hypovolaemia), HRS Resuscitate and Assess Fluid Status
 Renal: ATN, TIN, GN  A: ↓GCS may need airway Mx
 Post-renal: Stone, neoplasm, catheter  B: pulmonary oedema – sit up, high flow O2
 C: Assess fluid status:
CV Tissues End-organ
Presentation Postural BP CRT Mental state
 Usually presents in the context of critical illness JVP Cold / warm hands Urine output
 Uraemia HR Skin turgor
 Hyperkalaemia Mucus membranes
 Acidosis
 Oedema and ↑BP

Ix
 Bloods: FBC, U+E, LFT, glucose, clotting, Ca, ESR
 ABG: hypoxia (oedema), acidosis, ↑K+
 GN screen: if cause unclear
 Urine: dip, MCS, chemistry (U+E, CRP, osmolality, BJP)
 ECG: hyperkalaemia
 CXR: pulmonary oedema
 Renal US: Renal size, hydronephrosis
Rx Life-Threatening Complications
 Hyperkalaemia
 Pulmonary oedema
 Consider need for rapid dialysis
Rx Shock or Dehydration
 Fluid challenge 250-500ml over 30min
 Repeat as necessary: aim for CVP of 5-10cm
 Once replete, continue @ 20ml+UO/h

Hyperkalaemia
 ECG Features (in order)
 Peaked T waves Monitor
 Flattened P waves  Cardiac monitor
 ↑ PR interval  Urinary catheter
 Widened QRS  Consider CVP
 Sine-wave pattern → VF  Start fluid balance chart
 Mx
 10ml 10% calcium gluconate
 50ml 50% glucose + 10u insulin (Actrapid)
 Salbutamol 5mg nebulizer Look for Evidence of Post-Renal Causes
 Calcium resonium 15g PO or 30g PR  Palpable ± tender bladder
 Haemofiltration (usually needed if anuric)  Enlarged prostate
 Catheter in situ
 Complete anuria
Pulmonary Oedema
 Sit up and give high-flow O2
 Morphine 2.5mg IV (± metoclopramide 10mg IV)
Hx and Ix
 Frusemide 120-250mg IV over 1h
Hx: Evidence of Acute vs. Chronic RF
 GTN spray ± ISMN IVI (unless SBP <90)
 Duration of symptoms
 If no response consider:
 Co-morbidities
 CPAP
 Haemofiltration / haemodialysis ± venesection  Previous blood results

Ix
 Bloods, ABG
Indications for Acute Dialysis (AEIOU)
 Urine dip + MCS + chem
1. Persistent hyperkalaemia (>7mM)
2. Refractory pulmonary oedema  ECG
3. Symptomatic uraemia: encephalopathy, pericarditis  CXR and Renal US
4. Severe metabolic acidosis (pH <7.2)
5. Poisoning (e.g. aspirin)
Rx Sepsis
 Blood cultures and empirical Abx

Further Mx
 Call urologists if obstructed despite catheter
 Care with nephrotoxic drugs: e.g. gentamicin

© Alasdair Scott, 2012 111

Interstitial Nephritidies Nephrotoxins
 Either directly toxic → ATN
Acute Interstitial / Tubulointerstitial Nephritis  Or cause hypersensitivity → TIN
 Immune-mediated hypersensitivity c̄ either drugs or
other Ag acting as haptans Exogenous
 NSAIDs
Causes  Antimicrobials: AVASTA
 Drug hypersensitivity in 70%  Aminoglycosides
 NSAIDs  Vancomycin
 Abx: Cephs, penicillins, rifampicin, sulphonamide  Aciclovir
 Diuretics: frusemide, thiazides  Sulphonamides
 Allopurinol  Tetracycline
 Cimetidine  Amphotericin
 Infections in 15%  ACEi
 Staphs, streps  Immunosuppressants
 Immune disorders  Ciclosporin
 SLE, Sjogren’s  Tacrolimus
 Contrast media
Presentation  Anaesthetics: enflurane
 Fever, arthralgia, rashes
 AKI → olig/anuria
 Uveitis Endogenous
 Haemoglobin, myoglobin
Ix
 Urate
 ↑IgE, eosinophilia
 Ig: e.g. light chains in myeloma
 Dip: haematuria, proteinuria, sterile pyuria

Rx
 Stop offending drug Rhabdomyolysis
 Prednisolone
Pathogenesis
Prognosis: Most recover renal function  Skeletal muscle breakdown → release of:
 K+, PO4, urate
Chronic TIN  Myoglobin, CK
 Fibrosis and tubular loss  ↑K and AKI
 Commonly caused by:
Causes
 Reflux and chronic pyelonephritis
 DM  Ischaemia: embolism, surgery
 SCD or trait  Trauma: immobilisation, crush, burns, seizures,
compartment syndrome
 Toxins: statins, fibrates, ecstasy, neuroleptics
Analgesic Nephropathy
 Prolonged heavy ingestion of compound analgesics
Clinical
 Often a Hx of chronic pain: headaches, muscle pain  Muscle pain, swelling
 Red/brown urine
Features
 AKI occurs 10-12h later
 Sterile pyuria ± mild proteinuria
 Slowly progressive CRF
Ix
 Sloughed papilla can → obstruction and renal colic  Dipstick: +ve Hb, -ve RBCs
 Blood: ↑CK, ↑K, ↑PO4, ↑urate
Ix: CT w/o contrast (papillary calcifications)
Rx: stop analgesics
Rx
 Rx hyperkalaemia
Acute Urate Crystal Nephropathy  IV rehydration: 300ml/h
 AKI due to urate precipitation  CVP monitoring if oliguric
 Usually after chemo-induced cell lysis  IV NaHCO3 may be used to alkalinize urine and stabilise
 Rx: hydration, urinary alkalinisation a less toxic form of myoglobin.

Nephrocalcinosis
 Diffuse renal parenchymal calcification
 Progressive renal impairment
 Causes
 Malignancy
 ↑PTH
 Myeloma
 Sarcoidosis
 Vit D intoxification
 RTA
© Alasdair Scott, 2012 112
 Chronic Renal Failure
Features Renal Osteodystrophy
 Kidney damage ≥3mo indicated by ↓ function
 Symptoms usually only occur by stage 4 (GFR<30) Features
 ESRF is stage 5 or need for RRT  Osteoporosis: ↓ bone density
 Osteomalacia: ↓ mineralisation of osteoid (matrix)
Classification  2O/3O HPT → osteitis fibrosa cystica
 Subperiosteal bone resorption
Stage GFR  Acral osteolysis: short stubby fingers
1 >90  Pepperpot skull
2 60-89  May get spinal osteosclerosis → Rugger Jersey spine
3a 45-59  Sclerotic vertebral end-plate c̄ lucent centre
3b 30-44
4 16-29 Mechanism
5 <15  ↓ 1α-hydroxylase → ↓ vit D activation → ↓ Ca → ↑ PTH
 Phosphate retention → ↓ Ca and ↑ PTH (directly)
Causes  ↑ PTH → activation of osteoclasts ± osteoblasts
 Common  Also acidosis → bone resorption
 DM
 HTN Mx
 Other
 RAS General
 GN  Rx reversible causes
 Polycystic disease  Stop nephrotoxic drugs
 Drugs: e.g. analgesic nephropathy
 Pyelonephritis: usually 2O to VUR Lifestyle
 SLE  Exercise
 Myeloma and amyloidosis  Healthy wt.
 Stop smoking
Hx  Na, fluid and PO4 restriction
 Past UTI
 HTN, DM CV Risk
 FH  Statins (irrespective of lipids)
 DH  Low-dose aspirin
 Symptoms  Rx DM

Ix Hypertension
Blood  Target <140/90 (<130/80 if DM)
 ↓Hb, U+E, ESR, glucose, ↓Ca/↑PO4, ↑ALP, ↑PTH  In DM kidney disease give ACEi/ARB (inc. if normal BP)
 Immune: ANA, dsDNA, ANCA, GBM, C3, C4, Ig, Hep
Oedema
 Film: burr cells
 Frusemide
Urine: dip, MCS, PCR, BJP
Bone Disease
Imaging  Phosphate binders: sevelamer, calcichew
 CXR: cardiomegaly, pleural/pericardial effusion, oedema  Vit D analogues: alfacalcidol (1 OH-Vit D3)
 AXR: calcification from stones  Ca supplements
 Renal US  Cinacalcet: Ca mimetic
 Usually small (<9cm)
 May be large: polycystic, amyloid Anaemia
 Bone X-rays: renal osteodystrophy (pseudofractures)  Exclude IDA and ACD
 CT KUB: e.g. cortical scarring from pyelonephritis  EPO to raise Hb to 11g/dL (higher = thrombosis risk)

Renal biopsy: if cause unclear and size normal Restless Legs

 Clonazepam
Complications: CRF HEALS
 Cardiovascular disease
 Renal osteodystrophy
 Fluid (oedema)
 HTN
 Electrolyte disturbances: K, H
 Anaemia
 Leg restlessness
 Sensory neuropathy

© Alasdair Scott, 2012 113

 Renal Transplant
Treatment of choice for ESRF Complications
Assessment Post-op
 Virology status: CMV, HCV, HBV, HIV, VZV, EBV  Bleeding
 CVD  Graft thrombosis
 TB  Infection
 ABO and HLA haplotype  Urinary leaks

Hyperacute rejection (minutes)

Contraindications  ABO incompatibility
 Active infection  Thrombosis and SIRS
 Cancer
 Severe HD or other co-morbidity Acute Rejection (<6mo)
 ↑ing Cr (± fever and graft pain)
 Cell-mediated response
Types of Graft  Responsive to immunosuppression
 Cadaveric: brainstem death c̄ CV support
 Non-heart beating donor: no active circulation Chronic Rejection (>6mo)
 Live-related  Interstitial fibrosis + tubular atrophy
 Optimal surgical timing  Gradual ↑ in Cr and proteinuria
 HLA-matched  Not responsive to immunosuppression
 Improved graft survival
 Live unrelated Ciclosporin / tacrolimus nephrotoxicity
 Acute: reversible afferent arteriole constriction → ↓GFR
 Chronic: tubular atrophy and fibrosis
Immunosuppression
 Pre-op: campath / alemtuzumab (anti-CD52) ↓ Immune Function
 Post-op: prednisolone short-term and tacro/ciclo long-  ↑ risk of infection: opportunists, fungi, warts
term  ↑ risk of malignancy: BCC, SCC, lymphoma (EBV)

Cardiovascular Disease
Prognosis  Hypertension and atherosclerosis
 t½ for cadaveric grafts: 15yrs

Differential of Rising Cr in Tx pt.

 Rejection
 Obstruction
 ATN
 Drug toxicity

© Alasdair Scott, 2012 114

 Renal Complications of Systemic Disease
Diabetic Nephropathy Myeloma
 Causes ~20% of ESRF
 Advanced / ESRF occurs in 40% of T1 and T2 DM Pathology
 Excess production of monoclonal Ab ± light chains
Pathology (excreted and detected in 60% as urinary BJP).
 Diabetic nephropathy describes conglomerate of lesions  Light chains block tubules and have direct toxic effects
occurring concurrently. → ATN.
 Hyperglycaemia → renal hyperperfusion → hypertrophy  Myeloma also assoc. c̄ ↑↑Ca2+
and ↑ renal size
 Hypertrophy and metabolic defects inc. ROS production Presentation
→ glomerulosclerosis and nephron loss  ARF / CRF
 Nephron loss → RAS activation → HTN  Amyloidosis

Clinically Rx
 Microalbuminuria (30-300mg/d or albumin:creatinine >3)  Ensure fluid intake of 3L/d to prevent further impairment
 Strong independent RF for CV disease  Dialysis may be required in ARF
 Progresses to proteinuria (albuminuria >300mg/d)
 Diabetic retinopathy usually co-exists and HTN is
common Rheumatological Disease
Screening RA
 T2DMs should be screened for microalbuminuria 6moly  NSAIDs → ATN
 Penicillamine and gold → membranous GN
Rx  AA amyloidosis occurs in 15%
 Good glycaemic control delays onset and progression
 BP target 130/80 SLE
 Start ACEi/ARB even if normotensive  Involves glomerulus in 40-60% → ARF/CRF
 Stop smoking  Proteinuria and ↑BP common
 Combined kidney pancreas Tx possible in selected pts  Rx
 Proteinuria: ACEi
 Aggressive GN: immunosuppression
Amyloidosis
 Renal involvement usually caused by AL/AA amyloid Diffuse Systemic Sclerosis
 Features:  Renal crisis: malignant HTN + ARF
 Proteinuria  Commonest cause of death
 Nephrotic syndrome  Rx: ACEi if ↑BP or renal crisis
 Progressive renal failure
 Dx
 Large kidneys on US
 Biopsy

Infection
 GN: post-strep, HCV, HBV, HIV, SBE/IE, visceral
abscess
 Vasculitis: HBV, HCV, post-strep
 TIN: bacterial pyelonephritis, CMV, HBV, toxo

Malignancy
 Direct
 Renal infiltration: leukaemia, lymphoma
 Obstruction: pelvic tumour
 Mets
 Nephrotoxicity
 Toxic chemo
 Analgesics
 Tumour lysis syndrome

Hyperparathyroidism
 → hypercalcaemia

Sarcoidosis
 ↑ Ca and TIN
© Alasdair Scott, 2012 115
Renal Vascular Disease Renal Tubular Disease
Hypertension Renal Tubular Acidosis
 HTN can be both the cause and effect of renal damage.  Impaired acid excretion → hyperchloraemic met acidosis
 Renal diseases are commonest causes of 2O HTN  Both → RAS activation → K+ wasting and hypokalaemia
 Activation of RAS
 Retention of Na and water due to ↓ excretion Type 1 (Distal)
 Inability to excrete H+, even when acidotic
Renovascular Disease: RAS  May complicate other renal disorders
 Causes
Cause  Hereditary: Marfan’s, Ehler’s Danlos
 Atherosclerosis in 80%  AI: Sjogren’s, SLE, thyroiditis
 Fibromuscular dysplasia  Drugs
 Thromboembolism  Features
 External mass compression  Rickets / osteomalacia (bone buffering)
 Renal stones and UTIs
Features  Nephrocalcinosis → ESRF
 Refractory hypertension  Dx
 Worsening renal function after ACEi/ARB  Failure to acidify urine (pH >5.5) despite acid load
 Flash pulmonary oedema (no LV impairment on echo)
Type 2 (Proximal)
Ix  Defect in HCO3 reabsorption in PCT
 US + doppler: small kidney + ↓ flow  Tubules can reabsorb some HCO3 so can acidify urine in
 CT/MR angio systemic acidosis when HCO3 ↓
 Renal angiography: gold standard  Usually assoc. c̄ Fanconi syndrome
 Dx
Rx  Urine will acidify c̄ acid load (pH <5.5)
 Rx medical CV risk factors
 Angioplasty and stenting Fanconi Syndrome
 Disturbance of PCT function → generalised impaired
reabsorption
Haemolytic Uraemic Syndrome (HUS)  amino acids, K+, HCO3, phosphate, glucose
 E. coli O157:H7: verotoxin → endothelial dysfunction  Causes
 Idiopathic
Features  Inherited: inborn errors, Wilson’s
 Young children eating undercooked meat (burgers)  Acquired: tubule damage (drugs, myeloma…)
 Bloody diarrhoea and abdominal pain precedes:  Features
 MAHA  Polyuria (osmotic diuresis)
 Thrombocytopenia  Hypophosphataemic rickets (Vit D resistant)
 Renal failure  Acidosis, ↓K

Ix Hereditary Hypokalaemic Tubulopathies

 Schistocytes, ↓ plats  Bartter’s Syndrome
 Blockage of NaCl reabsorption in loop of Henle
 ↓Hb
(as if taking frusemide)
 Normal clotting
 Congenital salt wasting → RAS activation →
hypokalaemia and metabolic alkalosis
Rx
 Normal BP
 Usually resolves spontaneously
 Gitelman Syndrome
 Dialysis or plasma exchange may be needed  Blockage of NaCl reabsorption in DCT (as if
taking thiazides)
 Congenital salt wasting → RAS activation →
Thrombotic Thrombocytopenia Purpura (TTP) hypokalaemia and metabolic alkalosis +
 Genetic or acquired deficiency of ADAMTS13 → giant hypocalciuria
vWF multimers  Normal BP

Features
 Adult females
 Pentad
 Fever
 CNS signs: confusion, seizures
 MAHA
 Thrombocytopenia
 Renal failure

Ix: As HUS

Rx: Plasmapheresis, immunosuppression, splenectomy

© Alasdair Scott, 2012 116
 Renal Cystic Diseases
Auto Dom Polycystic Kidney Disease
 Prev: 1:1000 Auto Rec Polycystic Kidney Disease
 Age: Adults 40-60yrs  Prev: 1:40,000
 Genetics  Infancy
 PKD1 on Chr16, polycystin 1 (80%): cell-cell and cell-  Renal cysts and congenital hepatic fibrosis
matrix membrane receptor
 PKD2 on Chr4, polycystin 2: Ca2+ channel that
interacts c̄ polycystin 1
Medullary Sponge Kidney
 Path
 Multiple cystic dilatations of the CDs in the medulla
 Large cysts arising from all parts of nephron
 Progressive decline in renal function  Typically presents in 20-30s
 70% ESRF by 70yrs  Commoner in females
 Often asymptomatic, but predisposes to
Presentation: MISSHAPES  Hypercalciuria and nephrolithiasis
 Mass: abdo mass and flank pain  Recurrent UTIs and pyelonephritis
 Infected cyst  Haematuria
 Stones  Renal function is usually normal
 SBP ↑
 Haematuria or haemorrhage into cyst
 Aneurysms: berry → SAH Tuberous Sclerosis (Bourneville’s Disease)
 Polyuria + nocturia  AD condition c̄ hamartomas in skin, brain, eye,
 Extra-renal cysts: liver kidney
 Systolic murmur: mitral valve prolapse  Skin: nasolabial adenoma sebaceum, ash-leaf
macules, peri-ungual fibromas
Rx  Neuro: ↓IQ, epilepsy
 General  Renal: cysts, angiomyolipomas
 ↑ water intake, ↓ Na, ↓ caffeine (may ↓ cyst formation)
 Monitor U+E and BP
 Genetic counselling Renal Enlargement Differential: PHONOS
 MRA screen for Berry aneurysms  Polycystic kidneys: ADPKD, ARPKD, TS
 Medical  Hypertrophy 2O to contralateral renal agenesis
 Rx HTN aggressively: <130/80 (ACEi best)  Obstruction (hydronephrosis)
 Rx infections  Neoplasia: RCC, myeloma, amyloidosis
 Surgical  Occlusion (renal vein thrombosis)
 Pain may be helped by laparoscopic cyst removal or  Systemic: early DM, amyloid
nephrectomy.
 ESRF in 70% by 70yrs
 Dialysis or transplant

© Alasdair Scott, 2012 117

 Anaemia Classification and Causes
Low Hb Haemolytic Anaemia
 Men: <13.5g/dl
 Women: <11.5g/dl ↑ red cell breakdown
1. Anaemia c̄ ↑ MCV + polychromasia = reticulocytosis
2. ↑ unconjugated bilirubin
Symptoms 3. ↑ urinary urobilinogen
4. ↑ se LDH
 Fatigue
5. Bile pigment stones
 Dyspnoea
 Faintness Intravascular
 Palpitations 1. Haemoglobinaemia
 Headache 2. Haemoglobinuria
 Tinnitus 3. ↓ se haptoglobins
4. ↑ urine haemosiderin
5. Methaemalbuminaemia
Signs
 Pallor Extravascular
 Hyperdynamic circulation 1. Splenomegaly
 Tachycardia
 Flow murmur: apical ESM Acquired
 Cardiac enlargement  Immune-mediated DAT+ve
 Ankle swelling c̄ heart failure  AIHA: warm, cold, PCH
 Drugs: penicillin, quinine, methyldopa
 Allo-immune: acute transfusion reaction, HDFN
 PNH
Microcytic
 Mechanical:
 Haem Defect
 MAHA: DIC, HUS, TTP
 IDA
 Heart valve
 ACD
 Infection: malaria
 Sideroblastic / lead poisoning
 Burns
 Globin Defect
 Thalassaemia
Hereditary
 Enzyme: G6PD and pyruvate kinase deficiency
 Membrane: HS, HE
Normocytic
 Haemoglobinopathy: SCD, thalassaemia
 Recent blood loss
 Bone marrow failure
 Renal failure
 Early ACD
 Pregnancy (↑ plasma volume)

Macrocytic
 Megaloblastic
 Vit B12 or folate deficiency
 Anti-folate drugs: phenytoin, methotrexate
 Cytotoxics: hydroxycarbamide
 Non-megaloblastic
 Reticulocytosis
 Alcohol or liver disease
 Hypothyroidism
 Myelodysplasia

© Alasdair Scott, 2012 119

 Microcytic Anaemia
IDA Thalassaemia

Signs Pathophysiology
 Koilonychia  Point mutations (β) / deletions (α) → unbalanced
 Angular stomatitis / cheilosis production of globin chains
 Post-cricoid Web: Plummer-Vinson  → precipitation of unmatched globin
 → membrane damage → haemolysis while still in BM
Causes and removal by the spleen

Mechanism Examples Epidemiology

↑ Loss Menorrhagia  Common in Mediterranean and Far East
GI bleeding
Hookworms β Thalassaemia Trait / Heterozygosity
 β / β (↓ production) or β / β (no production)
+ O

↓ Intake Poor diet  Mild anaemia which is usually harmless

 ↓ MCV (“too low for the anaemia”): e.g. <75
Malabsorption Coeliac  ↑ HbA2 (α2δ 2) and ↑HbF (α2γ2)
Crohn’s
β Thalassaemia Major
Ix  βO / βO or βO / β+ or β+ / β+
 Haematinics: ↓ferritin, ↑TIBC, ↓ transferrin saturation  Features develop from 3-6mo
 Film: Anisocytosis, poikilocytosis, pencil cells  Severe anaemia
 Upper and lower GI endoscopy  Jaundice
 FTT
Rx  Extramedullary erythropoiesis
 Ferrous sulphate 200mg PO TDS  Frontal bossing
 SE: GI upset  Maxillary overgrowth
 HSM
 Haemochromatosis after 10yrs (transfusions)
Sideroblastic Anaemia  Ix
 Ineffective erythropoiesis  ↓Hb, ↓MCV, ↑↑HbF, ↑HbA2 variable
 ↑ iron absorption  Film: Target cells and nucleated RBCs
 Iron loading in BM → ringed sideroblasts  Rx
 Haemosiderosis: endo, liver and cardiac damage  Life-long transfusions
 SC desferrioxamine Fe chelation
Causes  BM transplant may be curative
 Congenital
 Acquired α Thalassaemia
 Myelodysplastic / myeloproliferative disease  Trait
 Drugs: chemo, anti-TB, lead  --/αα or α-/α-
 Asymptomatic
Ix  Hypochromic microcytes
 Microcytic anaemia not responsive to oral iron  HbH Disease
 ↑Ferritin, ↑ se Fe, ↔TIBC  --/-α
 Moderate anaemia: may need transfusions
Rx  Haemolysis: HSM, jaundice
 Remove cause  Hb Barts
 Pyridoxine may help  --/--
 Hydrops fetalis → death in utero

© Alasdair Scott, 2012 120

 Macrocytic Anaemia
Ix B12 Deficiency
Film Vit B12
 B12/Folate  Source: meat, fish and dairy (vegans get deficient)
 Hypersegmented PMN  Stores: 4yrs
 Oval macrocytes  Absorption: terminal ileum bound to intrinsic factor
 EtOH/Liver (released from gastric parietal cells)
 Target cells  Role: DNA and myelin synthesis

Blood Causes
 LFT: mild ↑ bilirubin in B12/folate deficiency
 TFT Mechanism Examples
 Se B12 ↓ Intake Vegan
 Red cell folate: reflects body stores over 2-3mo
↓ intrinsic factor Pernicious anaemia
BM biopsy: if cause not revealed by above tests Post-gastrectomy
 Megaloblastic erythropoiesis
 Giant metamyelocytes Terminal ileum Crohn’s
Ileal resection
Bacterial overgrowth
Folate Deficiency
Features
 General
Folate
 Symptoms of anaemia
 Source: Green veg, nuts, liver
 Lemon tinge: pallor + mild jaundice
 Stores: 4mo
 Glossitis (beefy, red tongue)
 Absorption: proximal jejunum
 Neuro
 Paraesthesia
Causes  Peripheral neuropathy
 Optic atrophy
Mechanism Examples  SACD
↓ Intake Poor diet
Subacute Combined Degeneration of the Cord
↑ Demand Pregnancy  Usually only caused by pernicious anaemia
Haemolysis
 Combined symmetrical dorsal column loss and
Malignancy
corticospinal tract loss.
 → distal sensory loss: esp. joint position and vibration
Malabsorption Coeliac
 → ataxia c̄ wide-gait and +ve Romberg’s test
Crohn’s
 Mixed UMN and LMN signs
Drugs EtOH  Spastic paraparesis
Phenytoin  Brisk knee jerks
Methotrexate  Absent ankle jerks
 Upgoing plantars
Rx  Pain and temperature remain intact
 Assess for underlying cause
 Give B12 first unless B12 level known to be normal Ix
 May precipitate or worsen SACD  ↓ WCC and plats if severe
 Folate 5mg/d PO  Intrinsic factor Abs: specific but lower sensitivity
 Parietal cell Abs: 90% +ve in PA but ↓ specificity

Rx
 Malabsorption → parenteral B12 (hydroxocobalamin)
 Replenish: 1mg/48h IM
 Maintain: 1mg IM every 3mo
 Dietary → oral B12 (cyanocobalamin)
 Parenteral B12 reverses neuropathy but not SACD

Pernicious Anaemia
 Autoimmune atrophic gastritis caused by autoAbs vs.
parietal cells or IF → achlorhydria and ↓ IF.
 Usually >40yrs, ↑ incidence c̄ blood group A
 Associations:
 AI: thyroid disease, Vitiligo, Addison’s, ↓HPT
 Ca: 3x risk of gastric adenocarcinoma

© Alasdair Scott, 2012 121

 Haemolytic Anaemias
AIHA
Hereditary Spherocytosis
Warm  Commonest inherited haemolytic anaemia in N. Europe
 IgG-mediated, bind @ 37OC
 Extravascular haemolysis and spherocytes Pathophysiology
 Ix: DAT+ve  Autosomal dominant defect in RBC membrane
 Causes: idiopathic, SLE, RA, Evan’s  Spherocytes get trapped in spleen → extravascular
 Rx: immunosuppression, splenectomy haemolysis

Cold Features
 IgM-mediated, bind @ <4OC  Splenomegaly
 Often fix complement → intravascular haemolysis  Pigment gallstones
 May cause agglutination → acrocyanosis or Raynaud’s  Jaundice
 Ix: DAT+ve for complement alone
 Causes: idiopathic, mycoplasma Complications
 Rx: avoid cold, rituximab  Aplastic crisis
 Megaloblastic crisis
Paroxysmal Cold Haemoglobinuria
 Rare: assoc. c̄ measles, mumps, chickenpox Ix
 IgG “Donath-Landsteiner” Abs bind RBCs in the cold and  ↑ osmotic fragility
→ complement-mediated lysis on rewarming  Spherocytes
 DAT-ve
PNH
Rx
 Absence of RBC anchor molecule (GPI) → ↓ cell-surface
complement degradation proteins → IV lysis  Folate and splenectomy (after childhood)
 Affects stem cells and  may also → ↓plats + ↓PMN

Features Hereditary Eliptocytosis

 Visceral venous thrombosis (hepatic, mesenteric, CNS)  Autosomal dominant defect → elliptical RBCs
 IV haemolysis and haemoglobinuria  Most pts. are asymptomatic
 Rx: folate, rarely splenectomy
Ix
 Anaemia ± thrombocytopenia ± neutropenia
 FACS: ↓CD55 and ↓CD59 G6PD deficiency
Rx Pathophysiology
 Chronic disorder  long-term anticoagulation  X-linked disorder of pentose phosphate shunt
 Eculizumab (prevents complement MAC formation)  ↓ NADPH production → RBC oxidative damage
 Affects mainly Mediterranean and Mid / Far East
Haemolytic Uraemic Syndrome (HUS)
Features Haemolysis Triggers
 E. coli O157:H7 from undercooked meat  Broad (Fava) beans
 Bloody diarrhoea and abdominal pain precedes:  Mothballs (naphthalene)
 MAHA  Infection
 Thrombocytopenia  Drugs: antimalarials, henna, dapsone, sulphonamides
 Renal failure
Ix
Ix: schistocytes, ↓ plats, normal clotting  Film
Rx  Irregularly contracted cells
 Usually resolves spontaneously  Bite cells, ghost cells and blister cells
 Exchange transfusion or dialysis may be needed  Heinz bodies
 G6PD assay after 8wks (reticulocytes have high G6PD)
Thrombotic Thrombocytopenia Purpura (TTP)
 Genetic or acquired deficiency of ADAMTS13 Rx
Features  Treat underlying infection
 Adult females  Stop and avoid precipitants
 Pentad  Transfusion may be needed
 Fever
 CNS signs: confusion, seizures
 MAHA Pyruvate Kinase Deficiency
 Thrombocytopenia  Autosomal recessive defect in ATP synthesis
 Renal failure  → rigid red cells phagocytosed in the spleen
 Features: splenomegaly, anaemia ± jaundice
Ix: schistocytes, ↓ plats, normal clotting  Ix: PK enzyme assay
Rx: Plasmapheresis, immunosuppression, splenectomy  Rx: often not needed or transfusion ± splenectomy
© Alasdair Scott, 2012 122
 Sickle Cell Disease
Epidemiology Mx Chronic Disease
 Africa, Caribbean’s, Middle-East  Pen V BD + immunisations
 UK prevalence = 1/2000  Folate
 Hydroxycarbamide if frequent crises

Pathogenesis
 Point mutation in β globin gene: glu→val Mx Acute Crises
 SCA: HbSS
 Trait: HbAS General
 HbS insoluble when deoxygenated → sickling  Analgesia: opioids IV
 Sickle cells have ↓ life-span → haemolysis  Good hydration
 Sickle cells get trapped in microvasc → thrombosis  O2
 Keep warm

Ix Ix
 Hb 6-9, ↑ retics, ↑ bilirubin  FBC, U+E, reticulocytes, cultures
 Film: sickle cells and target cells  Urine dip
 Hb electrophoresis  CXR
 Dx at birth c̄ neonatal screening
Rx
 Blind Abx: e.g. ceftriaxone
Presentation  Transfusion: exchange if severe
 Clinical features manifest from 3-6mo due to ↓HbF
 Triggers
 Infection
 Cold
 Hypoxia
 Dehydration

 Splenomegaly: may → sequestration crisis

 Infarction: stroke, spleen, AVN, leg ulcers, BM
 Crises: pulmonary, mesenteric, pain
 Kidney disease
 Liver, Lung disease
 Erection
 Dactylitis

Complications
 Sequestration crisis
 Splenic pooling → shock + severe anaemia
 Splenic infarction: atrophy and hyposplenism
 ↑ infection: osteomyelitis
 Aplastic crisis: parvovirus B19 infection
 Gallstones

© Alasdair Scott, 2012 123

 Bleeding Diatheses
Coagulation Tests Platelet Disorders
APTT Thrombocytopenia
 Intrinsic: 12, 11, 9, 8  ↓ production
 Common: 10, 5, 2, 1  BM failure: aplastic, infiltration, drugs (EtOH, cyto)
 Increased  Megaloblastic anaemia
 Lupus anti-coagulant  ↑ destruction
 Haemophilia A or B  Immune: ITP, SLE, CLL, heparin, viruses
 vWD (carries factor 8)  Non-immune: DIC, TTP, HUS, PNH, anti-
 Unfractionated heparin phospholipid
 DIC  Splenic pooling
 Hepatic failure  Portal hypertension
 SCD
PT
 Extrinsic: 7 ITP
 Common: 10, 5, 2, 1  Children: commonly post-URTI, self-limiting
 Increased  Adults: F>M, long-term
 Warfarin / Vit K deficiency  Ix: Anti-platelet Abs present
 Hepatic failure  Rx: conservative, steroids, IVIg, splenectomy
 DIC
Functional Defects
Bleeding Time / PFA-100  Drugs: aspirin, clopidogrel
 2 : paraproteinaemias, uraemia
O
 Platelet function
 Increased  Hereditary
 ↓ plats number or function  Bernard-Soulier: GpIb deficiency
 vWD  Glanzmann’s: GpIIb/IIIa deficiency
 Aspirin
 DIC
Coagulation Disorders
Thrombin Time
 Fibrinogen function Acquired
 Increased  Severe liver disease
 Quantitative/ qualitative fibrinogen defect  Anticoagulants
 DIC, dysfibrinogenaemia  Vitamin K deficiency
 Heparin
Haemophilia A: F8 Deficiency
Clinical Features  X-linked, affects 1/5000 males
 Pres: haemoarthroses, bleeding after surgery/extraction
Vascular or Platelet Disorder  Ix: ↑APTT, normal PT, ↓F8 assay
 Bleeding into skin: petechiae, purpura, echymoses  Mx
 Bleeding mucus mems: epistaxis, menorrhagia, gums  Avoid NSAIDs and IM injections
 Immediate, prolonged bleeding from cuts  Minor bleeds: desmopressin + tranexamic acid
 Major bleeds: rhF8
Coagulation Disorder
 Deep bleeding: muscles, joints, tissues Haemophilia B: F9 deficiency
 Delayed but severe bleeding after injury  X-linked, 1/20,000 males

VWD
Vascular Disorders  Commonest inherited clotting disorder (mostly AD)
 vWF
Congenital  Stabilises F8
 HHT  Binds plats via GpIb to damaged endothelium
 Ehler’s Danlos (easy bruising)  Ix
 Pseudoxanthoma elasticum  If mild, APTT and bleeding time may be normal
 ↑ APTT, ↑ bleeding time, normal plat, ↓ vWF AG
Acquired  Rx: desmopressin + tranexamic acid
 Senile purpura
 Vitamin C deficiency DIC
 Infection: e.g. meningococcus  ↑PT, ↑APTT, ↑TT, ↓plats, ↓fibrinogen, ↑FDPs
 Steroids  Schistocytes
 Vasculitis: e.g. HSP  Thrombosis and bleeding
 Causes: sepsis, malignancy (esp. APML), trauma, obs
 Rx: FFP, plats, heparin

© Alasdair Scott, 2012 124

 Thrombophilia
Definition Thrombophilia Screen
 Coagulopathy predisposing to thrombosis, usually
venous. Indications
 Arterial thrombosis <50yrs (?APL)
Inherited  Venous thrombosis <40yrs c̄ no RFs
 Familial VTE
Factor V Leiden / APC resistance  Unexplained recurrent VTE
 Protein C deactivates F5 and F8  Unusual site: portal, mesenteric
 c̄ protein S and thrombomodulin cofactors  Recurrent foetal loss
 Degradation resistance present in 5% of population  Neonatal
 Most don’t develop thrombosis
 Heterozygotes: 5x ↑VTE Investigations
 Homozygotes: 50x ↑ VTE  FBC, clotting, fibrinogen concentration
 Factor V Leiden / APC resistance
Prothrombin Gene Mutation  Lupus anticoagulant and anti-cardiolipin Abs
 ↑ prothrombin levels  Assays for AT, protein C and S deficiencies
 ↑ thrombosis due to ↓ fibrinolysis by thrombin-activated  PCR for prothrombin gene mutation
fibrinolysis inhibitor
Rx
Protein C and S Deficiency
 Rx acute thrombosis as per normal
 Heterozygotes for either have ↑ risk of thrombosis.
 Anticoagulate to INR 2-3
 Skin necrosis occurs – esp. c̄ warfarin
 Consider lifelong warfarin
 Heterozygotes → neonatal purpura fulminans
 If recurrence occurs on warfarin ↑INR to 3-4
Antithrombin III Deficiency
 AT is heparin co-factor → thrombin inhibition Prevention
 Deficiency affects 1/500  Lifelong anticoagulation not needed if asymptomatic
 Heterozygotes have ↑↑ thrombosis risk  ↑ VTE risk c̄ OCP or HRT
 Homozygosity is incompatible c̄ life  Prophylaxis in high risk situations
 Surgery
 Pregnancy
Acquired
 Progesterones in OCPs
 Anti-phospholipid syndrome
 CLOTs: venous and arterial
 Coagulation defect: ↑APTT
 Livido reticularis
 Obstetric complications: recurrent 1st
trimester abortion
 Thrombocyotpenia

© Alasdair Scott, 2012 125

 Blood Transfusion
Products FFP
 Stored @ -18OC
Packed red cells  Should be X-matched
 Stored @ 4OC in SAGM  Uses: DIC, liver disease, TTP, massive haemorrhage
 Hct ~70%
 Use to correct anaemia or blood loss Human Albumin Solution
 1u → ↑Hb by 1-1.5g/dL  Protein replacement
 After abdominal paracentesis
Platelets  Liver failure, nephrotic syndrome
 Stored @ RT under agitation
 Not needed if count >20 or not actively bleeding
 Should be x-matched

Immediate Transfusion Reactions

Reaction Time Clinical Features Mechanism Rx
Haemolytic Minutes Agitation ABO incompatibility Stop transfusion
Fever → IV haemolysis Tell lab
Abdo/chest pain Keep IV line open c̄ NS
↓ BP → shock Treat DIC
DIC → haemorrhage

I7I
Bacterial
Contamination

Febrile non-
Haemolytic
Allergic
<24h

<24h

Immediate
Renal failure
↑↑ temp + rigors
↓BP → Shock

Fever, rigors, chills

Urticaria, itch
Bacterial proliferation
Esp. plats
(Transfuse blood w/i 5h)
Recipient anti-HLA Abs

Recipient IgA deficiency

Stop transfusion
Send unit to lab
Abx: Taz+gent
Slow transfusion
Paracetamol 1g
Slow
Angioedoema Anti-IgA IgE Chlorphenamine 10mg IV/IM
Anaphylaxis
TRALI <6h ARDS: SOB, cough Anti-WBC Abs in donor plasma Stop transfusion
Bilat infiltrates on CXR Rx ARDS
Fluid <6h CCF Slow transfusion
Overload O2 + frusemide 40mg IV
Massive 24h ↑K Whole blood vol in 24h Massive Transfusion Protocol
Transfusion ↓ Ca (citrate chelation) (5L = 10units) - 1:1:1 ratio of PRBC:FFP:PLT
↓ F5 and F8 - → ↑ survival
↓ plats
Hypothermia Warm the blood

Delayed Transfusion Reactions

Reaction Time Clinical Features Mechanism Rx
Delayed 1-7d Jaundice Recipient anti-Rh Abs
Haemolytic Anaemia / ↓ing Hb Extravasc haemolysis
Fever
± Haemoglobinuria
Fe Overload Chronic SCA or Thal Major Chronic transfusions Desferrioxamine SC
Haemochromatosis
Post-transfusion 7-10d Thrombocytopenia AlloAbs attack recipient + donor plats IvIg
Purpura Purpura Plat transfusions
GvHD 4-30d Diarrhoea Viable lymphocytes transfused into
Skin rash immunocompromised host
Liver Failure → ↑ LFTs
Pancytopaenia Irradiate blood for vulnerable hosts

© Alasdair Scott, 2012 126

Bone Marrow Failure Myelodysplastic Syndromes
Pancytopenia Pathophysiology
 Heterogeneous group of disorders → BM failure
Congenital  Clone of stem cells c̄ abnormal development
 Fanconi’s anaemia: aplastic anaemia  → functional defects
 → quantitative defects
Acquired  May be primary or secondary
 Idiopathic aplastic anaemia  Chemo or radiotherapy
 BM infiltration
 Haematological Characteristics
 Leukaemia  Cytopenias
 Lymphoma  Hypercellular BM
 Myelofibrosis  Defective cells: e.g. ringed sideroblasts
 Myelodysplasia
 30% → AML
 Megaloblastic anaemia
 Infection: HIV
 Radiation
Clinical Features
 Elderly
 Drugs
 Cytotoxic: cyclophos, azathioprine, methotrexate  BM failure: anaemia, infection, bleeding, bruising
 Abx: chloramphenicol, sulphonamides  Splenomegaly
 Diuretic: thiazides
 Anti-thyroid: carbimazole Ix
 Anti-psychotic: clozapine  Film: blasts, Pelger-Huet anomaly, dimorphic
 Anti-epileptic: phenytoin  BM: Hypercellular, blasts, ringed sideroblasts

Mx
Aplastic Anaemia  Supportive: transfusions, EPO, G-CSF
 Rare stem cell disorder  Immunosuppression
 Allogeneic BMT: may be curative
Key Features
 Pancytopenia
 Hypocellular marrow

Presentation: Pancytopenia
 Age: 15-24yrs and >60yrs
 Anaemia
 Infections
 Bleeding

Causes
 Inherited
 Fanconi’s anaemia: Ashkenazi, short, pigmented
 Dyskeratosis congenita: premature ageing
 Swachman-Diamond syn.: pancreatic exocrine
dysfunction
 Acquired
 Drugs
 Viruses: parvovirus, hepatitis
 Autoimmune: SLE

Ix
 BM: Hypocellular marrow

Mx
 Supportive: transfusion
 Immunosuppression: anti-thymocyte globulin
 Allogeneic BMT: may be curative

© Alasdair Scott, 2012 127

 Chronic Myeloproliferative Disorders
Classification Essential Thrombocythaemia
 RBC → Polycythaemia Vera
 WBC → CML Features
 Platelets → Essential thrombocythaemia  Thrombosis
 Megakaryocytes → Myelofibrosis  Arterial: strokes, TIA, peripheral emboli
 Venous: DVT, PE, Budd-Chiari
 Bleeding (abnormal plat function)
Polycythaemia Vera  E.g. mucus membranes
 Erythromelagia
Features
 Hyperviscosity
Ix
 Headaches
 Plats >600 (often >1000)
 Visual disturbances
 Tinnitus  BM: ↑ megakaryocytes
 Thrombosis  50% JAK2+ve
 Arterial: strokes, TIA, peripheral emboli
Rx
 Venous: DVT, PE, Budd-Chiari
 Plats 400-1000: aspirin alone
 Histamine Release
 Aquagenic pruritus  Thrombosis or plats >1000: hydroxycarbamide
 Erythromelalgia  Anagralide may be used
 Sudden, severe burning in hands and feet c̄  Inhibits platelet maturation
 ↓ plat count and function
redness of the skin
 Splenomegaly: 75%
Prognosis
 Hepatomegaly: 30%
 5% → AML/MF over 10yrs
 Gout

Ix
 99% JAK2+ve
Thrombocythaemia Differential
 Primary: ET
 ↑RBC, Hb and Hct
 Secondary:
 ↑WCC and ↑plats
 Bleeding
 BM: hypercellular c̄ erythroid marrow
 Infection
 ↓EPO  Chronic inflammation: RA, IBD
 ↑ red cell mass c̄ isotope studies  Trauma / surgery
 Hyposplenism / splenectomy
Rx
 Aim to keep Hct <0.45 to ↓thrombosis
 Aspirin 75mg OD Primary Myelofibrosis
 Venesection if young  Clonal proliferation of megakaryocytes → ↑ PDGF →
 Hydroxycarbamide if older / higher risk Myelofibrosis
 Extramedullary haematopoiesis: liver and spleen
Prognosis
 Thrombosis and haemorrhage are main complications Features
 30% → MF  Elderly
 5% → AML  Massive HSM
 Hypermetabolism: wt. loss, fever, night sweats
 BM failure: anaemia, infections, bleeding
Polycythaemia Differential
Ix
True Polycythaemia: ↑ total volume of red cells  Film: leukoerythroblastic c̄ teardrop poikilocytes
 Primary: PV  Cytopenias
 Secondary  BM: dry tap (need trephine biopsy)
 Hypoxia: altitude, COPD, smoking  50% JAK2+ve
 EPO: renal cysts/tumours
Rx
Pseudopolycythaemia: ↓ plasma volume  Supportive: blood products
 Acute  Splenectomy
 Dehydration  Allogeneic BMT may be curative in younger pts.
 Shock
 Burns
Prognosis
 Chronic  5yr median survival
 Diuretics
 Smoking

© Alasdair Scott, 2012 128

Acute Lymphoblastic Leukaemia Acute Myeloid Leukaemia
Epidemiology Epidemiology
 Children 2-5yrs (commonest childhood Ca)  ↑ risk c̄ age: mean 65-70
 Rare in adults  Commonest acute leukaemia of adults

Aetiology Aetiology
 Arrest of maturation and proliferation of lymphoblasts  Neoplastic proliferation of myeloblasts
 80% B lineage, 20% T lineage
Risk Factors
Risk Factors  Chromosomal abnormalities
 Genetic susceptibility (often Chr translocations)  Radiation
 Environmental trigger  Down’s
 Radiation (e.g. during pregnancy)  Chemotherapy: e.g. for lymphoma
 Down’s  Myelodysplastic and myeloproliferative syndromes

Features FAB Classification (based on cell type)

 M2: granulocyte maturation
BM Failure  M3: acute promyelocytic leukaemia – t(15;17)
 Anaemia  M4: acute myelomonocytic leukaemia
 Thrombocytopenia → bleeding  M7: megakaryoblastic leukaemia – trisomy 21
 Leukopenia → infection
Features
Infiltration
 Lymphadenopathy BM Failure
 Orchidomegaly  Cytopenias
 Thymic enlargement
 HSM Infiltration
 CNS: CN palsies, meningism  Gum infiltration → hypertrophy and bleeding (M4)
 Bone pain  HSM
 Skin involvement
Ix  Bone pain
 ↑WCC: lymphoblasts
 ↓RBC, ↓PMN, ↓plats Blood
 BM aspirate  DIC: APML (M3)
 ≥20% blasts  Hyperviscosity: ↑↑WCC may → thrombi
 Cytogenetic and molecular analysis
 CXR+CT: mediastinal + abdominal LNs Ix
 LP: CNS involvement  ↑ WCC blasts (occasionally normal)
 Anaemia and ↓ plats
Mx  BM aspirate
 ≥20% blasts
Supportive  Auer rods are diagnostic
 Blood products
 Allopurinol Dx
 Hickman line or Portacath  Made by immunological and molecular phenotyping
 Flow cytometry
Infections  Cytogenetic analysis affects Rx and guides prognosis
 Gentamicin + tazocin
 Prophylaxis: e.g. co-timoxazole, ciprofloxacin Mx
Chemotherapy (recruited into national trials) Supportive: as for ALL
1. Remission induction Infections: as for ALL
2. Consolidation + CNS Rx
3. Maintenance for 2-3yrs Chemotherapy
 V. intensive → long periods of neutropenia and ↓ plats
BMT
 ATRA for APML
 Best option for younger adults
BMT
 Allogeneic if poor prognosis
Prognosis  Destroy BM and leukemic cells c̄ chemotherapy
 85% survival in children and total body irradiation.
 Worse in adults  Repopulate marrow c̄ HLA-matched donor HSCs
 Autologous if intermediate prognosis
 HSCs taken from pt.

© Alasdair Scott, 2012 129

Chronic Lymphocytic Leukaemia Chronic Myeloid Leukaemia
Epidemiology Epidemiology
 Commonest leukaemia in Western World  15% of leukaemia
 M>F=2:1  Middle-aged: 60-60yrs
 Elderly: 70s
Aetiology
Aetiology  Myeloproliferative disorder: clonal proliferation of
 Clone of mature B cells (memory cells) myeloid cells.

Features Features
 Often asymptomatic incidental finding  Systemic: wt. loss, fever, night sweats, lethargy
 Symmetrical painless lymphadenopathy  Massive HSM → abdo discomfort
 HSM  Bruising / bleeding (platelet dysfunction)
 Anaemia  Gout
 B symptoms: wt. loss, fever, night sweats  Hyperviscosity

Complications Philadelphia Chromosome

 Autoimmune haemolysis  Reciprocal translocation: t(9;22)
 Evan’s = AIHA and ITP  Formation of BCR-ABL fusion gene
 Infection (↓Ig): bacterial, zoster  Constitutive tyrosine kinase activity
 Marrow failure / infiltration  Present in >80% of CML
 Discovered by Nowell and Hungerford in 1960
Ix
 ↑ WCC, lymphocytosis Ix
 Smear cells  ↑↑WBC
 ↓ se Ig  PMN and basophils
 +ve DAT  Myelocytes
 Rai or Binet staging  ± ↓Hb and ↓plat (accelerated or blast phase)
 ↑urate
Dx  BM cytogenetic analysis: Ph+ve
 Immunophenotyping to distinguish from NHL
Natural Hx
Natural Hx  Chronic phase: <5% blasts in blood or DM
 Some remain stable for years  Accelerated phase: 10-19% blasts
 Nodes usually enlarge slowly (± lymphatic obstruction)  Blast crisis: usually AML, ≥20% blasts
 Death often due to infection: e.g. encapsulates, fungi
 Richter Transformation: CLL → large B cell lymphoma Rx
 Imatinib: tyrosine kinase inhibitor
Rx  → >90% haematological response
 Indications  80% 5ys
 Symptomatic  Allogeneic SCT
 Ig genes un-mutated (bad prognostic indicator)  Indicated if blast crisis or TK-refractory
 17p deletions (bad prognostic indicator)
 Supportive care
 Chemotherapy
 Cylophosphamide
 Fludarabine
 Rituximab
 Radiotherapy
 Relieve LN or splenomegaly

Prognosis
 1/3 never progress
 1/3 progress c̄ time
 1/3 are actively progressing

© Alasdair Scott, 2012 130

Non-Hodgkin’s Lymphoma Hodgkin’s Lymphoma
(80%) (20%)

Features Epidemiology
 M>F=2:1 (esp. in paeds)
Lymphadenopathy: 75% @ presentation  Bimodal age incidence: 20-29yrs and >60yrs
 Painless  May be assoc. c̄ EBV
 Symmetric
 Multiple sites Features
 Spreads discontinuously
Lymphadenopathy
Extranodal  Painless
 Skin: esp. T cell lymphomas  Asymmetric
 CNS  Spreads contiguously to adjacent LNs
 Oropharynx and GIT  Cervical nodes in 70% (also axillary and inguinal)
 Splenomegaly  May be alcohol-induced LN pain
 Mediastinal LN may → mass effects
B Symptoms  SVC obstruction
 Fever  Bronchial obstruction
 Night sweats
 Wt. loss (>10% over 6mo) B Symptoms
 Fever
Blood  Night sweats
 Pancytopenia  Wt. loss (>10% over 6mo)
 Hyperviscosity
Other
Ix  Itch
 FBC, U+E, LFT, LDH  Pel Ebstein Fever: cyclical fever
 ↑ LDH = worse prognosis  Hepato- and/or spleno-megaly
 Film
 Normal or circulating lymphoma cells Ix
 ± pancytopenias  FBC, film, ESR, LFT, LDH, Ca
 Classification: LN and BM biopsy  ↑ESR or ↓Hb = worse prognosis
 Staging: CT/MRI chest, abdomen, pelvis  LN excision biopsy or FNA
 Stage c̄ Ann Arbor System  Reed-Sternberg Cells (owl’s eye nucleus)
 Staging: CT/MRI chest, abdomen, pelvis
Classification  BM biopsy if B symptoms or Stage 3/4 disease

B Cell (commonest) Staging: Ann Arbor System

 Low Grade: usually indolent but often incurable 1. Single LN region
 Follicular 2. ≥2 nodal area on same side of diaphragm
 Small cell lymphocytic (=CLL) 3. Nodes on both sides of diaphragm
 Marginal Zone (inc. MALTomas) 4. Spread byond nodes: e.g. liver, BM
 Lymphoplamsacytoid (e.g. Waldenstrom’s)  + B if constitutional symptoms
 High Grade: aggressive but may be curable
 Diffuse large B cell (commonest NHL)
 Burkitt’s Mx
 Chemo, radio or both
T Cell
 ABVD regimen
 Adult T cell lymphoma: Caribs and Japs – HTLV-1
 BMT for relapse
 Enteropathy-assoc. T cell lymphoma: chronic coeliac
 Cutaneous T cell lymphoma: e.g. Sezary syn.
 Anaplastic large cell
Prognosis
 Depends on stage and grade
Mx  1A: >95% 5ys
 Diagnosis and management in an MDT  4B: <40% 5ys
High Grade (e.g. DLBCL)
 R-CHOP regimen
 BMT for relapse
 ~30% 5ys

Low Grade (e.g. follicular)

 Rx when clinically indicated (e.g. chloambucil)
 >50% 5ys

© Alasdair Scott, 2012 131

 Multiple Myeloma
Epidemiology Dx
 M=F  Clonal BM plasma cells ≥10%
 Blacks>White=2:1  Presence of se and/or urinary monoclonal protein
 ~70yrs  End-organ Damage: CRAB (1 or more)
 Ca ↑ (>2.6mM)
Pathogenesis  Renal insufficiency
 Clonal proliferation of plasma cells → monoclonal ↑↑Ig  Anaemia (<10g/dL)
 Usually IgG or IgA  Bone lesions
 Clones may also produce free light chain (λ or κ): ~2/3
 Excreted by kidney → urinary BJP Mx
 Light chains only seen in plasma in renal failure
 Clones produce IL-6 which inhibits osteoblasts (↔ ALP) Supportive
and activates osteoclasts.  Bone pain: Analgesia (avoid NSAIDs) +
bisphosphonates
Symptoms  Anaemia: Transfusions and EPO
 Renal impairment: ensure good hydration ± dialysis
Osteolytic Bone Lesions  Infections: broad spectrum Abx ± IVIg if recurrent
 Backache and bone pain
 Pathological #s Complications
 Vertebral collapse  ↑Ca: aggressive hydration, frusemide, bisphosphonates
 Cord compression: MRI, dexamethasone + local radio
BM Infiltration  Hyperviscosity: Plasmapheresis (remove light chains)
 Anaemia, neutropenia or thrombocytopenia  AKI: rehydration ± dialysis

Recurrent Bacterial Infections Specific

 Neutropenia  Fit pts.
 Immunoparesis (= ↓Ig)  Induction chemo: lenalidomide + low-dose dex
 Chemotherapy  Then allogeneic BMT
 Unfit pts.
Renal Impairment  Chemo only: melphalan + pred + lenalidomide
 Light chains  Bortezomib for relapse
 ↑Ca
 AL-amyloid Prognosis
 Mean survival: 3-5yrs
Complications  Poor prognostic indicators
 Hypercalcaemia  ↑ β2-microglobulin
 Neurological: ↑Ca, compression, amyloid  ↓ albumin
 AKI
 Hyperviscosity
 AL-amyloid (15%)

Ix
NB. Do ESR and Se electrophoresis if >50 c̄ back pain

Bloods
 FBC: normocytic normochromic anaemia
 Film: rouleaux ± plasma cells ± cytopenias
 ↑↑ESR/PV, ↑U+Cr, ↑Ca, normal ALP
 Se electrophoresis and β2-microglobulin

Urine
 Stix: ↑ specific gravity (BJP doesn’t show)
 Electrophoresis: BJP

BM trephine biopsy

X-ray: Skeletal Survey

 Punched-out lytic lesions
 Pepper-pot skull
 Vertebral collapse
 Fractures

© Alasdair Scott, 2012 132

Other Paraproteinaemias Amyloidosis
Smouldering / Asymptomatic Myeloma Definition
 Se monoclonal protein and/or BM plasma cells ≥10%  Group of disorders characterised by extracellular
 No CRAB deposits of a protein in an abnormal fibrillar form that is
resistant to degradation.
MGUS
 Se monoclonal protein <30g/L
 Clonal BM plasma cells <10% AL Amyloidosis
 No CRAB  Clonal proliferation of plasma cells c̄ production of
amyloidogenic light chains.
Solitary Plasmacytoma  1O: occult plasma cell proliferation
 Solitary bone/soft tissue plasma cell lesion  2O: myeloma, Waldenstrom’s, MGUS, lymphoma
 Se or urinary monoclonal protein
 Normal BM and normal skeletal survey (except lesion) Features
 No CRAB  Renal: proteinuria and nephrotic syndrome
 Heart: restrictive cardiomyopathy, arrhythmias, echo
Waldenstrom’s Macroglobulinaemia  “Sparkling” appearance on echo
 Lymphoplasmacytoid lymphoma→ monoclonal IgM  Nerves: peripheral and autonomic neuropathy, carpal
band tunnel.
 Features:  GIT: macroglossia, malabsorption, perforation,
 Hyperviscosity: CNS and ocular symptoms haemorrhage, hepatomegaly, obstruction.
 Lymphadenopathy + splenomegaly  Vascular: periorbital purpura (characteristic)
 Ix: ↑ESR, IgM paraprotein

Primary AL Amyloid AA Amyloidosis

 Occult proliferation of plasma cells → production of  Amyloid derived from serum amyloid A
amyloidogenic monoclonal protein.  SAA is an acute phase protein
 Se or urinary light chains  Chronic inflammation
 RA
Paraproteinaemia in lymphoma or leukaemia  IBD
 E.g. seen in 5% of CLL  Chronic infection: TB, bronchiectasis

Features
 Renal: proteinuria and nephrotic syndrome
 Hepatosplenomegaly

Familial Amyloidosis
 Group of AD disorders caused by mutations in
transthyretin (produced by liver)
 Features: sensory or autonomic neuropathy

Others: non-Systemic Amyloidosis

 β-amyloid: Alzheimer’s
 β2 microglobulin: chronic dialysis
 Amylin: T2DM

Dx
 Biopsy of affected tissue
 Rectum or subcut fat is relatively non-invasive
 Apple-green birefringence c̄ Congo Red stain under
polarized light.

Rx
 AA amyloid may improve c̄ underlying condition
 AL amyloid may respond to therapy for myeloma
 Liver Tx may be curative for familial amyloidosis

Prognosis
 Median survival: 1-2yrs

© Alasdair Scott, 2012 133

 Complications of Haematological Malignancies
Neutropenic Sepsis DIC
 Widespread activation of coagulation from release of pro-
General Precautions coagulants into the circulation.
 Barrier nursing in a side room  Clotting factors and plats are consumed → bleeding
 Avoid IM injections (may → infected haematoma)  Fibrin strands → haemolysis
 Swabs + septic screen
 TPR 4hrly Causes
 Malignancy: e.g. APML
Antimicrobials  Sepsis
 Start broad spectrum Abx: check local guidelines  Trauma
 Consider G-CSF  Obstetric events: e.g. PET

Signs
Hyperviscosity Syndrome  Bruising
 Bleeding
Causes  Renal failure
 ↑↑RBC / Hct >0.5: e.g. PV
 ↑↑WCC > 100: e.g. leukaemia Ix
 ↑↑ plasma proteins: Myeloma, Waldenstrom’s  ↓plats, ↓Hb, ↑APTT, ↑PT, ↑FDPs, ↓fibrinogen (↑TT)

Features Rx
 CNS: headache, confusion, seizures, faints  Rx cause
 Visual: retinopathy → visual disturbance  Replace: cryoprecipitate, FFP
 Bleeding: mucus membranes, GI, GU  Consider heparin and APC
 Thrombosis

Ix Tumour Lysis Syndrome

 ↑ plasma viscosity (PV)  Massive cell destruction
 FBC, film, clotting  High count leukaemia or bulky lymphoma
 Se + urinary protein electrophoresis  ↑K, ↑urate → renal failure
 Prevention: ↑ fluid intake + allopurinol
Rx
 Polycythaemia: venesection
 Leukopheresis: leukaemia
 Avoid transfusing before lowering WCC
 Plasmapheresis: myeloma and Waldenstrom’s

© Alasdair Scott, 2012 134

 Spleen and Splenectomy
Anatomy Splenectomy → Hyposplenism
 Intraperitoneal structure lying in the LUQ
 Measures 1x3x5 inches Indications
 Weighs ~7oz  Trauma
 Lies anterior to ribs 9-11  Rupture (e.g. EBV infection)
 AIHA
Features  ITP
1. Dull to percussion  HS
2. Enlarges to RIF  Hypersplenism
3. Moves inferiorly on respiration
4. Can’t get above it Complications
5. Medial notch  Redistributive thrombocytosis → early VTE
 Gastric dilatation (ileus)
Function: part of the mononuclear phagocytic system  Left lower lobe atelectasis: v. common
 Phagocytosis of old RBCs, WBCs and opsonised bugs  ↑ susceptibility to infections
 Antibody production  Encapsulates: haemophilus, pneumo, meningo
 Haematopoiesis
 Sequestration of formed blood elements Film
 Howell-Jolly bodies
 Pappenheimer bodies
Causes of Massive Splenomegaly: >20cm  Target cells
 CML
 Myelofibrosis Mx
 Malaria  Immunisation: pneumovax, HiB, Men C, yrly flu
 LeishManiasis  Daily Abx: Pen V or erythromycin
 Gaucher’s (AR, glucocerebrosidase deficiency)  Warning: Alert Card and/or Bracelet

Other Causes of Hyposplenism

 SCD
All Causes of Splenomegaly  Coeliac disease
 Haematological  IBD
 Haemolysis: HS
 Myelproliferative disease: CML, MF, PV
 Leukaemia, lymphoma
 Infective
 EBV, CMV, hepatitis, HIV,
 TB, infective endocarditis
 Malaria, leishmanias, hydatid disease
 Portal HTN: cirrhosis, Budd-Chiari
 Connective tissue: RA, SLE, Sjogrens
 Other
 Sarcoid
 Amyloidosis
 Gaucher’s
O
 1 Ab deficiency (e.g. CVID)

Splenomegaly may → Hypersplenism

 Pancytopenia as cells are sequestrated

© Alasdair Scott, 2012 135

 The Lab
ESR The Peripheral Film
 How far RBCs fall through anti-coagulated blood in 1h
 Normal: ~20mm/h (M: age/2, F: (age+10)/2 Feature Causes
 ↑ se proteins cover RBCs → clumping → rouleaux → Acanthocytes Abetaliproteinaemia
faster settling → ↑ESR. Alcoholic lever disease
Basophilic stippling Lead poisoning
↑ ESR Thalassaemia
 Plasma factors Blasts Leukaemia
 ↑ fibrinogen: inflammation Burr cels Uraemia
 ↑ globulins: e.g. myeloma Dimorphic picture Myelodysplasia
 Red cell factors Howell-Jolly bodies Hyposplenism
 Anaemia → ↑ESR Heinz bodies G6PD deficiency
Irregularly contracted cells G6PD deficiency
∆∆ of ESR >100 Leukoerythroblastic film BM infiltration
 Myeloma Pappenheimer bodies Hyposplenism
 SLE Pencil cells IDA
 GCA Reticulocytes Haemolysis
 AAA Haemorrhage
 Ca prostate Spherocytes AIHA
Hereditary spherocytosis
Differential White Count Schistocytes MAHA
Prosthetic valve
Neutrophilia Target cells Hyposplenism
 Bacterial infection Thalassaemia
 Left shift Liver disease
 Toxic granulation Tear-drop cells / Dacrocytes BM infiltration
 Vacuolation
 Stress: trauma, surgery, burns, haemorrhage
 Steroids Cytochemistry
 Inflammation: MI, PAN  Myeloblasts
 Myeloproliferative disorders: e.g. CML  Sudan black B
 MPO
Neutropenia  Hairy cell leukaemia: tartrate resistant acid phosphatase
 Viral infection  Leukocyte alkaline phosphatase
 Drugs: chemo, cytotoxics, carbimazole, sulphonamides  ↑: PV, ET, MF
 Severe sepsis  ↓: CML, PNH
 Hypersplenism: e.g. Felty’s

Lymphocytosis
 Viral infections: EBV, CMV
 Chronic infections: TB, Brucella, Hepatitis, Toxo
 Leukaemia, lymphoma: esp. CLL

Lymphopenia
 Drugs: steroids, chemo
 HIV

Monocytosis
 Chronic infection: TB, Brucella, Typhoid
 AML

Eosinophilia
 Parasitic infection
 Drug reactions: e.g. c̄ EM
 Allergies: asthma, atopy, Churg-Strauss
 Skin disease: eczema, psoriasis, pemphigus

Basophilia
 Parasitic infection
 IgE-mediated hypersensitivity: urticarial, asthma
 CML

© Alasdair Scott, 2012 136

 Tuberculosis
Pathophysiology Dx

Primary TB Latent TB
 Childhood or naïve TB infection  Tuberculin Skin Test
 Organism multiplies @ pleural surface → Ghon Focus  If +ve → IGRA
 Macros take TB to LNs
 Nodes + lung lesion = Ghon complex Active TB
 Mostly asympto: may → fever and effusion  CXR
 Cell mediated immunity / DTH controls infection in 95%  Mainly upper lobes.
 Fibrosis of Ghon complex → calcified nodule  Consolidation, cavitation, fibrosis, calcification
(Ranke complex)  If suggestive CXR take ≥3 sputum samples (one AM)
 Rarely may → 1 progressive TB (immunocomp)
O  May use BAL if can’t induce sputum
 Microscopy for AFB: Ziehl-Neelsen stain
Primary Progressive TB  Culture: Lowenstein-Jensen media (Gold stand)
 Resembles acute bacterial pneumonia
 Mid and lower zone consolidation, effusions, hilar LNs PCR
 Lymphohaematogenous spread → extra-pulmonary and  Can Dx rifampicin resistance
milliary TB  May be used for sterile specimens

Latent TB Tuberculin Skin Test

 Infected but no clinical or x-ray signs of active TB  Intradermal injection of purified protein derivative
 Non-infectious  Induration measured @ 48-72h
 May persist for years  Had BCG: +ve = ≥15mm
 No BCG: +ve = ≥6mm
 Weakened host resistance → reactivation
 False +ve: BCG, other mycobacteria, prev exposure
Secondary TB  False –ve: HIV, sarcoid, lymphoma
 Usually reactivation of latent TB due to ↓ host immunity
Interferon Gamma Release Assays (IGRAs)
 May be due to reinfection
 Pt. lymphocytes incubated c̄ M. tb specific antigens →
 Typically develops in the upper lobes
IFN-γ production if previous exposure.
 Hypersensitivity → tissue destruction → cavitation and
formation of caseating granulomas.  Will not be positive if just BCG (uses M. bovis)
 E.g. Quantiferon Gold and T-spot-TB
Clinical Features
Pulmonary Mx
 Cough, sputum  Rx w/o culture if clinical picture is consistent c̄ TB
 Malaise  Continue even if culture results are negative
 Fever, night sweats, wt. loss  Stress importance of compliance
 Haemoptysis  Check FBC, liver and renal function
 Pleurisy  CrCl 10-50ml/min → ↓ R dose by 50%, avoid E
 Pleural effusion  Test visual acuity and colour vision
 Aspergilloma / mycetoma may form in TB cavities  Give pyridoxine throughout Rx

Meningitis Initial Phase (RHZE): 2mos

 Headache, drowsiness  RMP: hepatitis, orange urine, enzyme induction
 Fever  INH: hepatitis, peripheral sensory neuropathy, ↓PMN
 Vomiting  PZA: hepatitis, arthralgia (CI: gout, porphyria)
 Meningism  EMB: optic neuritis
 Worsening over 1-3wks
 CNS signs Continuation Phase (RH): 4mos
 Papilloedema  RMP
 CN palsies  INH

Lymph Nodes TB Meningitis

 Cervical lymphadenitis: scrofula  RHZE: 2mos
 Painless neck mass: no signs of infection (cold)  RH: 10mos
 ± dexamethasone
GU TB
 Frequency, dysuria, loin/back pain, haematuria Chemoprophylaxis in latent TB
 Sterile pyuria  RH for 3mo or H alone for 6mo

Bone TB: vertebral collapse and Pott’s vertebra

Skin: Lupus vulgaris (jelly-like nodules)
Peritoneal TB: abdominal pain, GI upset, ascites
Adrenal: Addison’s disease
© Alasdair Scott, 2012 138
Other Mycobacterial Disease Influenza
Leprosy / Hansen’s Disease Pathophysiology
 Spread: droplet
Pathogenesis  Incubation: 1-4d
 Transmitted via nasal secretions  Infectivity: 1d before symptoms start to 7d after
 Not very infectious  Immunity: only strains which have already attacked pt
 M. leprae
Presentation
Classification  Fever
 Tuberculoid  Headache
 Less severe  Malaise
 TH1-mediated control of bacteria → paucibacillary
 Myalgia
 Anaesthetic hypopigmented macules
 n/v
 Lepromatous
 Conjunctivitis
 Weak TH1/2 → multibacillary
 Skin nodules
 Nerve damage (esp. ulnar and peroneal) Complications
 Bronchitis
Clinical Features  Pneumonia: esp. Staph
 Hypopigmented, insensate plaques  Sinusitis
 Trophic ulcers  Encephalitis
 Thickened nerves  Pericarditis
 Keratitis  Reyes: rash, vomiting, ↑LFTs in children give aspirin

Rx Ix
 Tuberculoid: 6mo Rx  Bloods: paired sera (takes 14d), lymphopenia,
 Rifampicin monthly thrombocytopenia
 Clofazamine daily  Culture: 1wk from nasal swabs
 Lepromatous: 2yrs  PCR: takes 36h, 94% sensitivity, 100% specificity
 Rifampicin monthly
 Clofazamine + dapsone daily Rx
 Bed rest + paracetamol
 If severe
MAI  Mx in ITU
 Complicates HIV infection  Cipro and co-amoxiclav: prevent Staph and Strep
 Widely disseminated: lungs / GIT  Oseltamivir
 Fever, night sweats, wt. loss  Neuraminidase inhibitor active vs. flu A and B
 Diarrhoea  May be indicated if >1yr c̄ symptoms of <48hr
 Hepatomegaly  Zanamivir
 Inhaled NA inhibitor active vs. influenza A and B
 >5yrs c̄ symptoms <48h
Buruli Ulcer
 M. ulcerans Prevention
 Australia and the Tropics  Good hygiene
 Transmitted by insects  Trivalent Vaccine
 Nodule → ulcer  >65yrs
 DM, COPD, heart, renal, liver failure
 Immunosuppression: splenectomy, steroids
 Medical staff
Fish Tank Granuloma  Oseltamivir
 M. marinum  Prophylactic use if influenza A/B is circulating
 Skin lesion appearing ~3wks after exposure and >1yr old and <48hr since exposure.

© Alasdair Scott, 2012 139

 HIV
Immunology Ix
 HIV binds via gp120 to CD4  HIV diagnostic tests
 TH cells, monocytes, macrophages, neural cells  Drug resistance studies
 CD4+ cells migrate to lymphoid tissue where virus  e.g. genotyping for reverse transcriptase or
replicates → infection of new CD4+ cells protease mutations
 Depletion and impaired function of CD4+ cells →  Mantoux test
immune dysfunction.  Serology: toxo, CMV, HBV, HCV, syphilis

Monitoring
Virology  CD4 count
 RNA retrovirus  Viral load (HIV RNA)
 After entry, viral reverse transcriptase makes DNA  FBC, U+E, LFTs, lipids, glucose
copy of viral RNA genome.
 Viral integrase enzyme integrates this c̄ host DNA
 Core viral proteins synthesised by host and then HAART
cleaved by viral protease into mature subunits.
 Completed virions released by budding Indications
 CD4 ≤350
 AIDS-defining illness
Natural Hx  Pregnancy
 HIVAN
Acute Infection: usually asympto  Co-infected c̄ HBV when Rx is indicated for HBV

Seroconversion Regimens
 Transient illness 2-6wks after exposure  1 NNRTI + 2 NRTIs
 Fever, malaise, myalgia, pharyngitis, macpap rash  NNRTI = Efavirenz
 Rarely meningoencephalitis  NRTI = emtricitabine + tenofovir (Truvada)
 Atripla = efavirenz + emtricitabine + tenofovir
Asymptomatic Infection  PI + 2 NRTIs
 But 30% will have PGL  PI = lopinavir (+ low dose ritonavir = Kaletra)
 Nodes >1cm in diameter
 ≥2 extra-inguinal sites Aim
 ≥3mo  Undetectable VL after 4mo
 If VL remains high despite good compliance
AIDS-related Complex (ARC)  Change to a new drug combination
 AIDS prodrome  Request resistance studies
 Constitutional symptoms: fever, night sweats, wt. loss
 Minor opportunistic infections
 Oral candida Prophylaxis
 Oral hairy leukoplakia (EBV)  CD4 <200: PCP – co-trimoxazole
 Recurrent HSV  CD4 <100: Toxo – co-trimoxazole
 Seborrhoeic dermatitis  CD4 <50: MAC – azithromycin
AIDS
 Defining illness
 CD4 usually <200 HIV Exposure
 Seroconversion post-needle-stick = ~0.3% (1/300)
Other Effects of HIV  Report to occupational health
 Osteoporosis  Immunise against hep B (active + passive)
 Dementia  Test blood from both parties: HIV, HBC, HCV
 Neuropathy  Repeat recipient testing @ 3 and 6mo
 Nephropathy
PEP
 Start PEP in high-risk exposure from HIV+ or unknown
source.
Dx
 Start ASAP as possible.
 ELISA: detect serum (or salivary) anti-HIV Abs
 Continue for at least 28d
 Western Blot: for confirmation
 E.g. Truvada + Kaletra
 If recent exposure, may be window period
 Usually 1-3wks
 Can be 3-6mo
 PCR: can detect HIV virions in the window period
 Rapid Antibody Tests: false positives are a problem
and results should be confirmed by Western Blot

© Alasdair Scott, 2012 140

 Complications of HIV Infection
Major AIDS-Defining Illnesses Candidiasis
 Candidiasis: oesophageal or pulmonary  Oral: nystatin suspension
 Cryptococcal meningitis  Oesophageal
 Cryptosporidiosis  Dysphagia
 CMV retinitis  Retrosternal pain
 Kapsosi sarcoma  Rx: itraconazole PO
 Lymphoma: Burkitt’s, 1O brain
 MAC
 PCP Cryptococcal Meningitis
 PML  Presentation
 Toxo  Chronic Hx
 TB  Headache
 Confusion
 Papilloedema
 CN lesions
TB and HIV
 Ix
 ↑ reactivation of latent TB
 India ink CSF stain
 ↑ risk of disseminated TB
 ↑↑ CSF pressure
 Higher bacterial loads but ↑ false –ve smears  CrAg in blood and CSF
 Fewer bacteria in sputum
 Rx
 False –ve skin tests (T cell anergy)  Amphotericin B + flucytosine for 2wks then
 Absence of characteristic granulomas fluconazole for for 6mo / until CD4 >200
 ↑ toxicity combining anti-TB and anti-HIV drugs
 IRIS: HAART → ↑ CD4 → paradoxical worsening of TB
symptoms PML
 Progressive multifocal leukoencephalopathy
 Demyelinating inflammation of brain white matter
PCP caused by JC virus.
 P. jiroveci: fungus  Presentation
 Presentation  Weakness
 Dry cough  Paralysis
 Exertional dyspnoea  Visual loss
 Fever  Cognitive decline
 CXR: bilat perihilar interstitial shadowing  Ix: JC viral PCR
 Dx: visualisation from sputum, BAL or lung biopsy  Rx: HAART, mefloquine may halt progression
 Rx
 High-dose co-trimoxazole IV
 Or, pentamidine IVI Kaposi’s Sarcoma
 Prednisolone if severe hypoxaemia
 Neoplasm derived from capillary endothelial cells or
 Prophylaxis fibrous tissue.
st
 Co-trimoxazole if CD4 <200 or after 1 attack
 Caused by HHV8 infection
 Presentation
 Purple papules
CMV  May have visceral involvement
 Mainly → retinitis  Rx
 ↓ acuity  HAART
 Eye pain, photophobia  Radiotherapy or chemotherapy
 “Pizza” sign on Fundoscopy
 Rx: ganciclovir eye implant
 Also: pneumonitis, colitis, hepatitis
Other Presentations
 Pulmonary
 MAI
Toxoplasmosis  Fungi: aspergillus, crypto, histoplasma
 Presentation  CMV
 Posterior uveitis  ↑ LFT and Hepatomegaly
 Encephalitis  Drugs
 Focal neurology  Viral hepatitis
 Dx  AIDS sclerosing cholangitis
 Toxoplasma serology  MAI
 Toxo isoloation from LN or CNS biopsy  Chronic Diarrhoea
 CT/MRI: ring-shaped contrast enhancing lesions  Bacteria: Salmonella, shigella, campylobacter
 Rx: pyrimethamine + sulfadiazine + folate  Protozoa: cryptosporidium, microsporidium
 Prophylaxis  Virus: CMV
 Co-trimoxazole if CD4 <100  Neuro
O
 Lifelong 2 prophylaxis  CMV encephalitis
© Alasdair Scott, 2012
 Lymphoma 141
 Viral Hepatitis
Types Hep C
 Mainly spread by blood.
Type Spread Cause  Thousands of UK cases due to transfusion (eligible for
A FO Seafood, especially abroad compensation from Skipton fund)
B IV Blood, body fluids, babies (vertical)
C IV Mainly blood. Less vertical cf. HCV Presentation
D IV Dependent on prior HBV infection  Initial infection is usually asymptomatic
E FO Developing world  25% have acute icteric phase
 85% → chronic infection
Hepatitis A  20-30% → cirrhosis w/i 20yrs
 ↑↑ risk of HCC
Presentation
 Prodromal Phase Ix
 Fever, malaise, arthralgia, nausea, anorexia  Anti-HCV Abs
 Distaste for cigarettes  HCV-PCR
 Icteric Phase  Liver biopsy if PCR+ve to assess liver damage and
 Jaundice, HSM, lymphadenopathy, cholestasis need for Rx
 HCV genotype
Ix
 ↑↑ALT, ↑AST (AST:ALT <2) Rx
 IgM+ ~25d after exposure  Indications
 = recent infection  Chronic haepatitis
 IgG+ for life  ↑ ALT
 Fibrosis
Rx  PEGinterferon α2b + ribavirin
 Supportive  ↓ efficacy if
 Avoid alcohol  Genotype 1, 4, 5 or 6
 IFN-α for fulminant hepatitis (rare)  ↑ VL
 Older
Prevention  Black
 Passive Ig → <3mo protection (e.g. travellers)  Male
 Can give active vaccine
Hep D
Hep B  Incomplete RNA virus that can only exist c̄ HBV
 ↑ risk of acute hepatic failure and cirrhosis
Incubation: 1-6mo  Ix: anti-HDV Ab

Presentation Hep E
 Prodromal phase and icteric phase as for Hep A  Similar to HAV
 Extra-hepatic features due to immune complexes  Common in Indochina
 Urticaria or vasculitic rash
 Cryoglobulinaemia Differential
 PAN
 GN Acute
 Arthritis  Infection: CMV, EBV, leptospirosis
 Toxin: EtOH, paracetamol, isoniazid, halothane
Ix  Vasc: Budd-Chiari
 HBsAg +ve = current infection  Obs: eclampsia, acute fatty liver of pregnancy
 +ve >6mo = chronic disease  Other: Wilson’s, AIH
 HBeAg +ve = high infectivity
 Anti-HBc IgM = recent infection Cirrhosis
 Anti-HBc IgG = past infection  Common:
 Anti-HBs = cleared infection or vaccinated  Chronic EtOH
 HBV PCR: monitoring response to Rx  NAFLD / NASH
 Other:
Rx  Genetic: Wilson’s, α1ATD, HH, CF
 Supportive  AI: AH, PBC, PSC
 Avoid EtOH  Drugs: Methotrexate, amiodarone, isoniazid
 Chronic disease: PEGinterferon α2b  Neoplasm: HCC, mets
 Vasc: Budd-Chiari, RHF, constrict. pericarditis
Complications
 Fulminant hepatic failure (rare)
 Chronic hepatitis (5-10%) → cirrhosis in 5%
 HCC

© Alasdair Scott, 2012 144

 Syphilis
Stages Dx

Stage Features: due to endarteritis obliterans Cardiolipin antibody

Primary  Mac→pap→indurated, painless ulcer = Chancre  E.g. VDRL, RPR
 Regional LNs  Not treponeme specific
 Heals in 1-3wks  False +ve: pregnancy, pneumonia, SLE,
malaria, TB
Secondary  6wks -6mo wks after chancre  +ve in 1O and 2O syphilis (wanes in late disease)
 Systemic bacteraemia → fever, malaise  Reflects disease activity: -ve after Rx
 Skin rash:
Treponeme-specific Ab
 Symmetrical, non-itchy, mac pap / pustular
 Palms, soles, face, trunk  +ve in 1O and 2O syphilis
 Buccal snail-track ulcers  Remains +ve despite Rx
 Warty lesions: condylomata lata  THPA and FTA

Treponemes
Tertiary  2-20yrs latency
 Seen by dark ground microscopy of chancre fluid
 Gummas
 Granulomas in skin, mucosa, bones, joints  Seen in lesions of 2O syphilis
 May not be seen in late syphilis
Quaternary  Syphilitic aortitis
 Aortic aneurysm
 Aortic regurg Rx
 Neurosyphilis  2-3 doses (1wk apart of benzathine penicillin
 Paralytic dementia  Or, doxycycline for 28d
 Meningovascular: CN palsies, stroke
 Tabes dorsalis Jarisch-Herxheimer Reaction
 Degeneration of sensory neurones,  Fever, ↑HR, vasodilatation hrs after first Rx
esp. legs  ? sudden release of endotoxin
 Ataxia and +ve Romberg’s  Rx: steroids
 Areflexia
 Plantars ↑↑
 Charcot’s joints
 Argyll-Robertson pupil
 accommodates, doesn’t react

© Alasdair Scott, 2012 148

 Malaria
Species
Species Inc Liver stage? Rhythm Rx
Falciparum 7-10d No Tertian: 48h Artemether-lumefantrine (Riamet)
(Quinine + Doxy)
Vivax 10-17d Chronic liver stage Tertian: 48h Chloroquine then primaquine
Ovale 10-17d Chronic liver stage Tertian: 48h Chloroquine then primaquine
Malariae 18-40d No Quartan: 72h Chloroquine then primaquine

Biology Mx
 Plasmodium sporozoites injected by females Anopheles
mosquito. Uncomplicated Ovale, Vivax, Malariae
 Sporozoites migrate to liver, infect hepatocytes and multiply  Chloroquine base
asymptomatically (incubation period) → merozoites  Then primaquine
 Merozoites released from liver and infect RBCs
 Multiply in RBCs Uncomplicated Falciparum
 Haemolysis  Artemether-lumefantrine
 RBC sequestration → splenomegaly
 Cytokine release Severe Falciparum Malaria
 Need ITU Mx
 IV antimalarials
Falciparum Malaria Prophylaxis
 90% present w/i 1mo
 No resistance: proguanil + chloroquine
 Resistance: mefloquine or malarone
Flu-like Prodrome
 Malarone: atovaquone + proguanil
 Headache, malaise, myalgia, anorexia

Fever Paroxysms
 Shivering ≤1h Antimalarial SEs
 Hot stage for 2-6h: ~41OC  Chloroquine: retinopathy
 Flushed, dry skin, headache, n/v  Fansidar: SJS, ↑LFTs, blood dyscrasias
 Sweating for 2-4h as temp falls  Primaquine: haemolysis if G6PD deficient
 Malarone: abdo pain, nausea, headache
Signs  Mefloquine: dysphoria, neuropsychiatric signs
 Anaemia
 Jaundice
 HSM
 No rash, no LNs

Complications
 Cerebral malaria: confusion, coma, fits
 Lactic acidosis → Kussmaul respiration
 Hypoglycaemia
 Acute renal failure: 2 to ATN
O

 ARDS

Dx
 Serial thick and thin blood films
 Parasitaemia level
 FBC: anaemia, thrombocytopenia
 Clotting: DIC
 Glucose
 ABG: lactic acidosis
 U+E: renal failure
 Urinalysis: haemoglobionuria

© Alasdair Scott, 2012 150

 Other Tropical Diseases
Disease Aetiolgy Presentation Diagnosis
African Tsetse fly Tender subcut nodule @ site of infection Thick and thin films
Trypanosomiasis - flagellated protozoa
Haemolymphatic Stage
“Sleeping sickness” - rash, fever, rigors, headaches Serology
- LNs and HSM
- Posterior cervical nodes (Winterbottom’s sign)

Miningoencephalitic Stage
- Wks – Mos after original infection
- Convulsions, agitation confusion
- Apathy, depression, hypersomnolence, coma

T. gambiense: West Africa

T. rhodesiense: East Africa
- More severe than gambiense
American Reduviids T. cruzi
Trypanosomiasis - Latin America
- Erythematous nodule, fever, LN, HSM
“Chagas Disease” - Swelling of eyelid (Romana’s Sign)
- Cardiac disease
Leishmaniasis Sandflies Cutaneous (L. major, L. tropica) Visceral:
- Skin ulcer @ bite → depigmented scar - Leucopaenia
South + Central Am Diffuse cutaneous - ↑Ig
Asia - Widespread nodules (fail to ulcerate) - Pancytopaenia
Africa - Serology
Mucocutaneous (L. braziliensis)
- South America Visualisation of
- Ulcer in mucous mems of mouth and nose Leishman-Donovan
bodies
Visceral = Kala Azar (L. donovani)
- Dry, warty hyperpigmented skin lesions
(dark face and hands)
- Prolonged fever
- Massive splenomegaly, LNs, abdo pain
Filariasis Asia Lymphatic Filariasis Nemotodes
Africa - Wuchereria bancrofti
South America - elephantiasis Microfilariae in the blood
Eosinophilia
Dengue Fever Aedes mosquito Flushes: facem neck, chest Serology
Central macpap rash Tourniquet test
RNA virus South + Central Am Headache, arthralgia
Asia HSM
Africa Jaundice
Haemorrhage: petechial, GI, gums or nose, GU

NB. can exclude if fever starts >2wks after

leaving endemic area.
Schistosomiasis Tremotode (Fluke) Itchy papular rash at site of penetration
Snail vector - Swimmer’s Itch

Mansoni
- abdo pain D&V
- later hepatic fibrosis and portal HTN
- HSM

Haematobium
- frequency, dysuria, haematuria
- may → hydronephrosis and renal failure
- ↑ risk of bladder SCC

© Alasdair Scott, 2012 151

Miscellaneous Infections Approach to Nosocomial Fever
Tetanus Epidemiology
 2-30% of hospital inpatients
Pathophysiology  Mostly due to bacterial infection
 Clostridium tetani spores live in faeces, dust, soil and
medical instruments. Definition
 Mucosal breach admits spores  Oral temp ≥38 that occurs ≥48hrs after admission and
 Spores germinate and produce exotoxin is recorded on at least two separate occasions over 48h
 Exotoxin prevents the release of inhibitory transmitters
 GABA and glycine Causes
 → generalised muscle over-activity  Infection (commonest cause)
 Inflammation
Presentation  Ischaemia
 Prodrome: fever, malaise, headache  Malignancy
 Trismus  Drug-induced
 Risus sardonicus
 Opisthotonus Mx
 Spasms: may → respiratory arrest
 Careful Hx and examination
 Autonomic dysfunction: arrhythmias, fluctuating BP
 Study drug list
 Basic Ix
Rx
 Urinalysis
 Mx on ITU: may need intubation
 FBC, LFT, ESR, cultures
 Human tetanus Ig  Swabs
 Metronidazole, benpen  CXR
Prevention
 Active immunisation c̄ tetanus toxoid
 Clean minor wounds
 Uncertain Hx / <3 doses: give vaccine
 ≥3 doses: only vaccinate if >10yrs since last dose
 Heavily contaminated wounds
 Uncertain Hx / <3 doses: vaccine + TIg
 ≥3 doses: vaccinate if ≥5yrs since last dose

Actinomycosis
 Actinomyces israelii
 Subcut infections: esp. on jaw
 Forms sinuses which discharge pus containing sulphur
granules.
 Rx: ampicillin for 30d, then pen V for 100d

© Alasdair Scott, 2012 152

 Basic Neuroscience
The Spinal Cord Cerebellum and Basal Ganglia
 Output to cortex via thalamus
Gross Anatomy  Regulation of posture, locomotion, coordination and
 Foramen magnum to L1 movement.
 Terminates as conus medullaris
 Spinal nerves continue inferiorly as corda equina
 Denticulate ligament and filum terminale are pial Internal Capsule
extensions that suspend cord in subarachnoid space.  White matter carrying axonal fibres from motor cortex to
pyramids of medulla.
 Infarction → contralateral hemiparesis
Blood Supply
 3 longitudinal vessels
 2 posterior spinal arteries: dorsal 1/3
 1 anterior spinal artery: ventral 2/3 Brain Lobar Function
 Reinforced by segmental feeder arteries
 E.g. artery of Adamkiewicz Frontal
 Longitudinal veins drain into extradural vertebral plexus  Cognition and memory
 Executive function
 Motor cortex
Organisation  Dominant hemisphere: motor speech (Broca’s area)

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 Mode: pain and temperature — Tongue
 Cell body: DRG §_..@.
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 Decussation: in cord, at entry level nira-abdominal ‘
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Lateral Corticospinal Tract

er
 Mode: motor (body)
 Cell body: 1 motor cortex
O
Brain Blood Supply
 Decussation: pyramidal decussation in ventral medulla

Thalamic Nucelei
 VPL: somoatosensory body
 VPM: somatosensory head
 LGN: visual
 MGN: auditory

© Alasdair Scott, 2012 155

NMJ Vestibulo-occular Pathways
Transmission Vestibulo-occular reflex (VOR)
1. Presynaptic voltage-gated Ca2+ channels open → Ca2+  Axons from vestibular neurones project via MLF to
influx abducens and occulomotor nuclei
2. ACh vesicles released from presynaptic terminal  Head turns L → eyes turn R
3. ACh diffuses across cleft  Absent Doll’s Eye sign = brainstem death
4. ACh binds to nicotinic receptors on post-synaptic
terminal Caloric Tests
5. → Na+ influx → depolarisation → Ca2+ release from SR  Warm → ↑ firing of vestibular N. → eyes turn to
→ muscle contraction contralateral side c̄ nystagmus to ipsilateral side
6. ACh is degraded by acetylcholinesterase and choline is  Remember fast direction of nystagmus – COWS
taken up into presynaptic terminal.  Cold: Opposite
 Warm: Same
Blockade  Absence of eye movements = brainstem damage on
1. Block presynaptic choline uptake: hemicholinium
side being tested.
2. Block ACh vesicle fusion: botulinum, LEMS
3. Block nicotinic ACh receptors
 Non-depolarising: atracurium, vecuronium Visual Field Defects
 Depolarising: suxamethonium VISUEII flifld dB*|B*I.‘;l$ '-.-'1sua1fields\ R

Dopamine Pathways
 Mesocorticolimbic: SCZ
1vi GP
x‘ ‘1 i Relina
 Nigrostriatal: Parkinsonism
 Tuberoinfundibular: Hyperprolactinaemia
O 3

P ‘ i our chiasm
Sympathetic NS
 Cell bodies from T1-L2
U9 4
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 GVE preganglionic fibres synapse @ either: C9 5 LOw'HfiDfB‘Sh

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 Paravertebral ganglia Fl Upper fibres in anterior Optic

 Prevertebral ganglia
 Chromaffin cells of adrenal medulla
O 6 parietal lobe

Omipiial cortex
radiation

 Preganglionic fibres are myelinated and release ACh @

nicotinic receptors Pupillary Light Reflex
 Postganglionic fibres are unmyelinated and release NA
@ adrenergic receptors /ii’,-..__ ___ Si}?!-rIClI3r
 Except @ sweat glands where they release ACh "" pupulljg

for muscarinic receptors.

Parasympathetic NS
\ ' J
 Cranial: CN 3, 7, 9, 10 -) OP";
Cmnn,»
Gang! |(}r1
 Ciliary: ciliary muscle and sphincter pupillae Nurve
 Pterygopalatine: mucus mems of nose and
palate, lacrimal gland
 Submandibular: submandibular and sublingual Opt-1'. C h|.u1.r1-
I
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glands
 Otic: parotid gland
 Vagus supplies thoracic and abdo viscera
 Sacral: pelvic splanchnic nerves (S2-4) innervate pelvic I
,,, M-rihram
I
viscera I

 Preganglionic fibres release ACh @ nicotinic receptors I

I

 Postganglionic fibres release ACh @ muscarinic ‘I

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receptors " Fran.-clai

J,/‘I Nucleus

CN Nuclei
Accnson
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 Midbrain: 3, 4, (5) l"H.lC|l.‘U‘l

 Pons: 5, 6, 7, 8
 Medulla: (5), 9, 10, 11, 12

NB. All nuclei except 4, innervate ipsilateral side. Fibres from

trochlear nucleus decussate in medulla and supply
contralateral SOB muscle.

© Alasdair Scott, 2012 157

 Motor and Sensory Patterns
Patterns of Motor Deficits
Cortical Lesions
 Hyperreflexia proximally in arm or leg
 Unexpected patterns: e.g. all movements in hand/foot

Internal Capsule and Corticospinal Lesions

 Contralateral hemiparesis c̄ pyramidal distribution.
 Lesion c̄ epilepsy, ↓ cognition or homonymous hemianopia = in a cerebral hemisphere
 Lesion c̄ contralateral CN palsy = brainstem lesion on the side of the palsy (E.g. Miilard-Gubler syn.)

Cord Lesions
 Quadriparesis / paraparesis
 Motor and reflex level: LMN signs at level of lesion and UMN signs below

Peripheral Neuropathies
 Usually distal weakness
 In GBS weakness is proximal (root involvement)
 Single nerve = mononeuropathy: trauma or entrapment
 Several nerves = mononeuritis multiplex: vasculitis or DM

UMN Lesions LMN Lesions

 Motor cells in pre-central gyrus to anterior horn cells  Anterior horn cells to peripheral nerves
in the cord  Wasting
 Pyramidal weakness: extensors in UL, flexors in LL  Fasciculation
 No wasting  Flaccidity: ↓ tone
 Spasticity: ↑ tone ± clonus  Hyporeflexia
 Hyperreflexia  Down-going plantars
 Up-going plantars

Primary Muscle Lesions

 Symmetrical loss
 Reflexes lost later vs. neuropathies
 No sensory loss
 Fatiguability in myasthenia

Patterns of Sensory Deficits

 Pain and temp travel in small fibres in peripheral
nerves and in anterolateral spinothalamic tracts
 Touch, joint position and vibrations travel in large
fibres peripherally and in dorsal columns centrally

Distal Sensory Loss

 Suggests a neuropathy

Sensory Level
 Hallmark of cord lesion
 Hemi-cord lesion → Brown-Sequard syndrome
 Ipsilateral loss of proprioception / vibration and
UMN weakness with contralateral loss of pain

Dissociated Sensory Loss

 Selective loss of pain and temp c̄ conservation of
proprioception and vibration
 Occurs in cervical cord lesions: e.g. syringomyelia

© Alasdair Scott, 2012 158

 Specific Neurological Patterns and Causes
Cerebellar Syndrome Vertebrobasilar Circulation
Effects: DANISH  Supplies cerebellum, brainstem and occipital lobes
 Dysdiadochokinesia  Combination of symptoms
 Dysmetria: past-pointing  Visual: hemianopia, cortical blindness
 Ataxia: limb / trunkal  Cerebellar: DANISH
 Nystagmus: horizontal = ipsilateral hemisphere  CN lesions
 Intention tremor  Hemi- / quadriplegia
 Speech: slurred, staccato, scanning dysarthria  Uni- / bi-lateral sensory symptoms
 Hypotonia

Common Causes: PASTRIES Lateral Medullary Syn. / Wallenberg’s Syn.

 Paraneoplastic: e.g. from bronchial Ca  Occlusion of one vertebral A. or PICA
 Alcohol: thiamine and B12 deficiency  Features: DANVAH
 Sclerosis  Dysphagia
 Tumor: e.g. CPA lesion  Ataxia (ipsilateral)
 Rare: MSA, Friedrich’s, Ataxia Telangiectasia  Nystagmus (ipsilateral)
 Iatrogenic: phenytoin  Vertigo
 Endo: hypothyroidism  Anaesthesia
 Stroke: vertebrobasilar  Ipsilat facial numbness + absent corneal
reflex
 Contralateral pain loss
 Horner’s syndrome (ipsilateral)
Anterior Cerebral Artery
 Supplies frontal and medial part of cerebrum
 Contralateral motor / sensory loss in the legs > arms Millard-Gubler Syndrome: crossed hemiplegia
 Face is spared  Pontine lesions (e.g. infarct)
 Abulia (pathological laziness)  Effects: 6th and 7th CN palsy + contralateral hemiplegia

Middle Cerebral Artery Locked-in Syndrome

 Supplies lateral / external part of hemisphere  Pt. is aware and cognitively intact but completely
 Contralateral motor / sensory loss in face and arms > paralysed except for the eye muscles.
legs.  Causes
 Contralateral homonymous hemianopia due to  Ventral pons infarction: basilar artery
involvement of optic radiation  Central potine myelinolysis: rapid correction of
 Cognitive changes hyponatraemia
 Dominant (L): aphasia
 Non-dominant (R): neglect, apraxia Cerebellopontine Angle Syndrome
 Causes: acoustic neuroma, meningioma, cerebellar
astrocytoma, metastasis (e.g. breast)
Posterior Cerebral Artery  Effects: ipsilat CN 5, 6, 7, 8 palsies + cerebellar signs
 Supplies occipital lobe  Absent corneal reflex
 Contralateral homonymous hemianopia c̄ macula  LMN facial palsy
sparing.  LR palsy
 Sensorineural deafness, vertigo, tinnitus
 DANISH

Subclavian Steal Syndrome

 Subclavian A. stenosis proximal to origin of vertebral A.
may → blood being stolen from this vertebral artery by
retrograde flow.
 Syncope / presyncope or focal neurology on using the
arm.
 BP difference of >20mmHg between arms

Anterior Spinal Artery / Beck’s Syndrome

 Infarction of spinal cord in distribution of anterior spinal
artery: ventral 2/3 or cord.
 Causes: Aortic aneurysm dissection or repair
 Effects
 Para- / quadri-paresis
 Impaired pain and temperature sensation
 Preserved touch and proprioception
© Alasdair Scott, 2012 159
Muscle Weakness Differential Hand Wasting Differential
1. Cerebrum / Brainstem 1. Cord
 Vascular: infarct, haemorrhage  Anterior Horn: MND, polio
 Inflammation: MS  Syringomyelia
 SOL
 Infection: encephalitis, abscess 2. Roots (C8 T1)
 Compression: spondylosis, neurofibroma
2. Cord
 Vascular: anterior spinal artery infarction 3. Brachial Plexus
 Inflammation: MS  Compression
 Injury  Cervical rib
 Tumour: Pancoast’s, breast
3. Anterior Horn  Avulsion: Klumpke’s palsy
 MND, polio
4. Neuropathy
4. Roots / Plexus  Generalised: CMT
 Spondylosis  Mononeuritis multiplex: DM
 Cauda equina syndrome  Compressive mononeuropathy
 Carcinoma  Median: thenar wasting
 Ulnar: hypothenar and interossei wasting
5. Motor Nerves
 Mononeuropathy: e.g. compression 5. Muscle
 Polyneuropathy: e.g. GBS, CMT  Disuse: RA
 Compartment syndrome: Volkman’s ischaemic
6. NMJ contracture
 GB, LEMS, botulism  Distal myopathy: myotonic dystophy
 Cachexia
7. Muscle
 Toxins: steroids
 Poly- / Dermato-myositis
 Inherited: DMD, BMD, FSH

Gait Disturbance Differential

Motor Sensory

Basal Ganglia: festinating / shuffling Vestibular (Romberg’s +ve)

 PD  Meniere’s
 Parkinsonism: MSA, PSP, Lewy body dementia, CBD  Viral labyrinthitis
 Brainstem lesion
UMN Bilateral: spastic, scissoring
 Cord: compression, trauma, hereditary spastic Cerebellar: ataxic
paraparesis, syringomyelia, transverse myelitis  EtOH
 Bihemispheric: CP, MS  Infarct

UMN Unilateral: spastic circumducting Proprioceptive Loss (Romberg’s +ve)

 Hemisphere lesion: CVS, MS, SOL  Dorsal columns: B12 deficiency
 Hemicord: MS, tumour  Peripheral neuropathy: DM, EtOH, uraemia

LMN Bilateral: bilat foot drop Visual Loss

 Polyneuropathy: CMT, GBS
 Corda equina
Other
LMN Unilateral: foot drop → high stepping gait  Myopathy, MG/LEMS
 Ant horn: polio  Medical: postural hypotension, Stokes-Adams, arthritis
 Radicular: L5 root lesion
 Sciatic / common peroneal nerve: trauma, DM

Mixed UMN and LMN: MAST

 MND
 Ataxia: Friedrich’s
 SACD
 Taboparesis
© Alasdair Scott, 2012 160
 Blackouts
Causes: CRASH Presentation and Investigation
Cardiac: Stokes-Adams Attacks Cardiogenic Syncope
 Brady: heart block, sick sinus, long-QT  Trigger: exertion, drug, unknown
 Tachy: SVT, VT  Before: palpitations, chest pain, dyspnoea
 Structural  During: pale, slow/absent pulse, clonic jerks may occur
 Weak heart: LVF, tamponade  After: rapid recovery
 Block: AS, HOCM, PE  Ix: ECG, 24hr ECG, Echo

Reflexes
1. Vagal overactivity Reflex: Vasovagal
 Vasovagal syncope  Trigger: prolonged standing, heat, fatigue, stress
 Situational: cough, effort, micturition  Before
 Carotid sinus syncope  Gradual onset: secs→mins
 Nausea, pallor, sweating, tunnel vision, tinnitus
2. Sympathetic underactivity = Post. Hypotension  Cannot occur lying down
 Salt deficiency: hypovolaemia, Addison’s  During
 Toxins  Pale, grey, clammy, brady
 Cardiac: ACEi, diuretics, nitrates, α-B  Clonic jerks and incontinence can occur, but no
 Neuro: TCAs, benzos, antipsychotics, L- tongue biting
DOPA  After: rapid recovery
 Autonomic Neuropathy: DM, Parkinson’s, GBS  Ix: Tilt-table testing
 Dialysis
 Unwell: chronic bed-rest
 Pooling, venous: varicose veins, prolonged Reflex: Postural Hypotension
standing  Trigger: Standing up
 Before, During and After as for vagal above
Arterial  Ix: Tilt-table testing
 Vertebrobasilar insufficiency: migraine, TIA, CVA,
subclavian steal
 Shock Arterial
 Hypertension: phaeochromocytoma  Trigger: Arm elevation (subclavian steal), migraine
 Before, During and After as for vasovagal ± brainstem
Systemic Sx (diplopia, nausea, dysarthria)
 Metabolic: ↓glucose  Ix: MRA, duplex vertebrobasilar circulation
 Resp: hypoxia, hypercapnoea (e.g. anxiety)
 Blood: anaemia, hyperviscosity
Systemic
Head  Hypoglycaemia: tremor, hunger, sweating, light-
 Epilepsy headedness → LOC
 Drop attacks

Head: Epileptic
 Trigger: flashing lights, fatigue, fasting
Examination  Before: e.g. aura in complex partial seizures – feeling
 Postural hypotension: difference of >20/10 after strange, epigastric rising, deja/jamias vu, smells, lights,
standing for 3min vs. lying down automatisms
 Cardiovascular  During: Tongue biting, incontinence, stiffness→jerking,
 Neurological eyes open, cyanosis, ↓SpO2
 After: headache, confusion, sleeps, Todd’s palsy
 Ix: EEG, ↑ se prolactin at 10-20min
Ix
 ECG ± 24hr ECG Head: Drop Attacks
 U+E, FBC, Glucose  Trigger: nil
 Tilt table  Before: no warning
 EEG, sleep EEG  During: sudden weakness of legs causes older woman
 Echo, CT, MRI brain to fall to the ground.
 After: no post-ictal phase

© Alasdair Scott, 2012 161

Vertigo and Dizziness Causes of Hearing Loss
Vertigo Conductive: WIDENING
 The illusion of movement: usually rotatory  Wax or foreign body
 Of patient or surroundings  Infection: otitis media, OME
 Worse on movement  Drum perforation
 Extra: ossicle discontinuity – otosclerosis, trauma
Not Vertigo  Neoplasia: carcinoma
 Faintness  INjury: e.g. barotrauma
 Light-headedness  Granulomatous: Wegener’s, Sarcoid
 Lost awareness

Dizziness Sensorineural: DIVINITY

 c̄ Impaired consciousness = blackout
 w/o impaired consciousness Developmental
 Vertigo: vestibular  Genetic: Alport’s, Waardenburgs
 Imbalance: vestibular, cerebellar, extrapyramidal  Congenital: TORCH
 Perinatal: anoxia

Causes of Vertigo: IMBALANCE Degenerative

 Presbyacusis
 Infection / Injury
 Labyrinthitis: post-viral severe vertigo, n/v Infection
 Ramsay Hunt  VZV, measles, mumps, influenza
 Trauma: to petrous temporal bone  Meningitis
 Meniere’s
 Recurrent vertigo (~20min) ± n/v Vascular
 Fluctuating SNHL  Ischaemia: Internal Auditory Artery (AICA) → sudden
 Tinnitus hearing loss and vertigo
 Aural fullness  Stroke
 Benign Positional Vertigo
 Sudden vertigo provoked by head rotation Inflammation
 Aminoglycosides / frusemide  Vasculitis
 Lymph, Peri-, fistula  Sarcoidosis
 Path: Connection between inner and middle ears
 Causes: Congenital, trauma Neoplasia
 PC: vertigo, SNHL  CPA tumours: acoustic neuroma (commonest cause
 o/e: Tullio’s phen – nystagmus evoked by loud of unilateral SNHL)
sound
 Arterial Injury
 Migraine  Noise
 TIA / stroke  Head trauma
 Nerve
 Acoustic neuroma / vestibular schwannoma Toxins
 Central lesions  Gentamicin
 Demyelination, tumour, infarct (e.g. LMS)  Frusemide
 Epilepsy  Aspirin
 Complex partial
lYmph
 Endolymphatic Hydrops = Meniere’s
 Perilymphatic fistula: ruptured round window

© Alasdair Scott, 2012 162

 Abnormal Involuntary Movements – Dyskinesias
Tremor Dystonia
 Regular, rhythmic oscillation  Prolonged muscle contracture → unusual joint posture
 NB. Asterixis = rhythmic myoclonus or repetitive movements

Types and Causes: RAPID Idiopathic generalised dystonia

 Often autosomal dominant
Resting  Childhood onset: starts in one leg and spreads on that
 Features side of the body over 5-10yrs
 4-6Hz, pill-rolling
 Abolished on voluntary movement Idiopathic focal dystonia
 ↑ c̄ distraction (e.g. counting backwards)  Commonest form of dystonia
 Causes: Parkinsonism  Confined to one part of the body
 Rx: Da agonists, antimuscarinic (e.g. procyclidine)  Worsened by stress
 Types:
Action / Postural  Spasmodic torticollis
 Features  Blepharospasm
 6-12Hz  Oromandibular
 Absent at rest  Writer’s / Muscician’s cramp
 Worse c̄ outstretched hands or movement
 Equally bad at all stages of movement Acute Dystonia
 Causes: BEATS  Torticollis, trismus and/or occulogyric crisis
 Benign essential tremor  Typically a drug reaction:
 Endocrine: thyroxicosis, ↓glucose, phaeo  Neuroleptics
 Alcohol withdrawal (or caffeine, opioids…)  Metoclopramide
 Toxins: β-agonists, theophylline, valproate, PHE  L-DOPA
 Sympathetic: physiological tremor may be  Rx: procyclidine (antimuscarinic)
enhanced: e.g. in anxiety
Chorea
Intention  Non-rhythmic, purposeless, jerky, flitting movements
 Features  E.g. facial grimacing, flexing / extending the fingers
 >6Hz, irregular, large amplitude  Causes
 Worse at end of movement  Huntington’s
 E.g. past-pointing  Sydenham’s
 Causes: cerebellar damage  Wilson’s
 L-DOPA
Dystonic
 Features: variable
Athetosis
 Causes: mostly idiopathic, as for dystonia
 Slow, sinuous, writhing movements
 Causes
Benign Essential Tremor  Cerebral palsy
 Autosomal dominant  Kernitcterus
 Occur c̄ action and worse c̄ anxiety, emotion, caffeine
 Arms, neck, voice Hemiballismus
 Doesn’t occur during sleep  Large amplitude, flinging hemichorea
 Better c̄ EtOH  Contralateral to a vascular lesion in the subthalamic
nucleus: often elderly diabetics
 Recovers spontaneously over months
Myoclonus
 Sudden, involuntary jerks Tardive Syndromes
 Delayed onset following chronic exposure to Da
Causes agonists (e.g. antipsychotics, antiemetics, L-DOPA)
 Metabolic = Asterixis (L, R, ↑CO2)
 Neurodegenerative diseases (LSDs) Classification
 CJD  Dyskinesia: orobuccolingual, truncal or choreifirm
 Myoclonic epilepsies (e.g. infantile spasms) movements
 Dystonia: sustained, stereotyped muscle spasms of
Benign Essential Myoclonus twitching or turning
 Auto dom  Akathisia: unpleasant sense of inner restlessness ±
 Childhood onset frequent generalised myoclonus w/o repetitive movements (e.g. pacing)
progression.
 May respond to valproate Rx
 Change (e.g. to atypical) or slowly withdraw drug
 Dyskinesia: Da antagonist (tetrabenazine)
 Akathisia: β-B

© Alasdair Scott, 2012 163

Dementia Delirium / ACS
Definition Definition
 Chronically impaired cognition that affects multiple  Globally impaired cognition and impaired consciousness
domains: memory, attention, language
 No impairment of consciousness Features
 Acquired and progressive (cf. LD)  Disorientation to person, time and place
 Reversal of sleep-wake cycle (hyperactive at night)
Alzheimer’s Disease  Labile mood
 Epi: 50%  Illusions, delusions and hallucinations
 Path: neurofibrillary tangles and β amyloid plaques  Cognitive impairment: mem, language, concentration…
 PC: progressive, global cognitive decline
 RFs: ApoE4 allele, presenillin 1/2 mutations, Down’s Causes: DELIRIUMS
 Ix: MRI – medial temporal lobe atrophy
 Rx: cholinesterase inhibitors (donepezil, rivastigmine) if  Drugs: opioids, sedatives, L-DOPA
MMSE is 10-20  Eyes, ears and other sensory deficits
 Low O2 states: MI, stroke, PE
Vascular Dementia  Infection
 Epi: 20%  Retention: stool or urine
 Path: multiple infarcts  Ictal
 PC: sudden onset, stepwise deterioration, patchy  Under- hydration / -nutrition
deficits, vascular RFs  Metabolic: DM, post-op, sodium, uraemia
 Ix: MRI – extensive infarcts or small vessel disease  Subdural haemorrhage or other intracranial pathology
 Rx: manage predisposing factors
Ix
Lewy Body Dementia  Bloods: FBC, U+E, LFTs, glucose, ABG
 Epi: 20%  Urine dip
 Path: Lewy Bodies in occipito-parital cortex  Septic screen
 PC: Fluctuating cognitive dysfunction, visual  ECG, LP
hallucinations, parkinsonism
 Rx: cholinesterase inhibitors Mx
 ID and Rx underlying cause
Frontotemporal Dementia (Pick’s)  Surround c̄ familiar people
 Epi: <5%  Nurse in moderately lit, quiet room
 Path: Pick Bodies  Find glasses, hearing aids…
 PC: disinhibition, personality change, early memory  Avoid sedatives if possible, but if disruptive:
preservation, progressive aphasia  Haldol 0.5-2mg PO/IM
 Ix: MRI – frontal or temporal atrophy  Chlorpromazine 50-100mg PO/IM (avoid in elderly)

Ameliorable Causes
Infection
 Viral: HIV, HSV, PML
 Helminth: cysticercosis, toxo

Vascular
 Chronic subdural haematoma

Inflammation
 SLE
 Sarcoid

Neoplasia

Nutritional
 Thiamine deficiency
 B12 and folate deficiency
 Pellagra (B3 / niacin deficiency)

Hypothyroid
Hypoadrenalism
Hypercalcaemia
Hydrocephalus (normal pressure)

© Alasdair Scott, 2012 164

 Headache: Differential and Investigation
Acute: VICIOUS Chronic: MCD TINGS

Vascular Migraine
 Haemorrhage: SAH, intracranial, intracerebral
 Infarction: esp. posterior circulation Cluster headaches
 Venous: Sinus / cortical thrombosis
Drugs
Infection/Inflammation  Analgesics
 Meningitis  Caffeine
 Encephalitis  Vasodilators: Ca2+ antagonists, nitrates
 Abscess
Tension headaches
Compression
 Obstructive hydrocephalus: tumour ICP ↑/↓
 Pituitary enlargement: apoplexy  ↑: tumour, aneurysm, AVM, benign intracranial HTN
 ↓: spontaneous intracranial hypotension
ICP
 Spontaneous intracranial hypotension Neuralgia (trigeminal)
 Acute dural CSF leak
 Worse on standing initially. Giant cell arteritis
Ophthalmic
Systemic
 Acute glaucoma
 HTN
 Organ failure: e.g. uraemia
Unknown
 Situational: cough, exertion, coitus

Systemic
 HTN: Phaeo, PET
 Infection: sinusitis, tonsillitis, atypical pneumonia
 Toxins: CO

Investigations:

Bloods
Urine
Micro
 Blood cultures
 Serology: enterovirus (common cause of viral meningitis), HSV, HIV, syphilis, crypto
 CSF

Radiology
 Non-contrast CT
 SAH: blood in sulci, cisterns (white). 90% sensitivity in first 24h
 MRI
 MRA: aneurysm
 MRV: sinus thrombosis

Special: CSF
 Opening pressure (norm = 5-20cm H2O):
 ↑: SAH, meningitis
 ↓: spontaneous intracranial hypotension
 Xanthochromia: yellow appearance of CSF due to bilirubin. Detect by spectrophotometry.

EA fi= E
Findings Bacterial TB Viral
Appearance Turbid Fibrin web Clear
Cells PMN Lympho / mononuc Lympho / mononuc
Count 100-1000 10-1000 50-1000
Glucose ↓ (< ½ plasma) ↓ (< ½ plasma) > ½ plasma
Protein (g/L) ↑↑ (>1.5) ↑↑↑ (1-5) mild ↑ (<1)

© Alasdair Scott, 2012 165

 Headaches: Key Features
SAH Hemicrania
 Sudden onset, worst ever, occipital headache.  Paroxysmal hemicranias: cluster-like headache lasting
 Meningism, focal signs, ↓consciousness 5-45min, 5-30x/day
 SUNCT: short-lasting unilateral neuralgia with
Venous Sinus Thrombosis conjunctival injection and tearing, attacks last 15-60s,
 Sagittal: headache, vomiting, seizures, ↓vision, recur 5-30x/hr
papilloedema  Hemicrania continua: continuous cluster-like headache
 Transverse: headache ± mastoid pain, focal CNS  Rx: all respond well to indomethacin
signs, seizures, papilloedema
Trigeminal neuralgia
Cortical Vein Thrombosis  Paroxysms of unilateral intense stabbing pain in
 Thunderclap headache trigeminal distribution (usually V2/3)
 Stroke-like focal symptoms over days  Triggers: washing area, shaving, eating, talking
 Focal seizures are common  Male > 50yrs
 2O in 14%: compression of CNV, MS, Zoster, Chiari
Meningitis malformation
 Fever, photophobia, neck stiffness, kernig’s +ve  Ix: exclude 2O cause by MRI.
 Purpuric rash  Rx Med: CBZ, lamotrigine, gabapentin
 ↓consciousness  Rx Surg: microvascular decompression

Encephalitis Analgesia Overuse

 Fever, odd behaviour, fits, focal neuro, ↓consciousness
Acute Glaucoma
 Constant unilat eye pain, radiating to forehead
 ↓ acuity, haloes, n/v
 Red eye, cloudy cornea.
 Dilated, non-responsive pupil
 Episodic headache becomes daily chronic headache
 Use OTC analgesia on 6 days/month max
ICP
 ↑: worse in AM, stooping, visual probs (papilloedema),
obese women
 ↓: worse sitting or standing

Tension Headache TMJ Dysfunction

 Bilateral / vertex-bitemporal, non-pulsatile, band-like
Migraine
 Prodrome → aura → headache
 Unilat, throbbing
 n/v, phono/photophobia
Cluster
 Rapid onset very severe pain around/behind one eye.
 Red, watery eye, nasal congestion
 Miosis, ptosis
 Attacks last 15min–3hrs, 1-2x/day, mostly nocturnal
 Clusters last 4-12wks, remission lasts 3mo-3yrs. Can
be chronic vs. episodic.
 Rx: 100% O2 via non-rebreathe mask, Sumitriptan
 Prevention: verapamil, topiramate, Li
 Preauricular pain on chewing
 Assoc. c̄ crepitus
 Earache, headache
Giant Cell Arteritis (>60yrs, ESR>60, pred 60mg)
 Unilateral temple/scalp pain and tenderness.
 Thickened, pulseless temporal artery
 Jaw claudication, amaurosis fugax, sudden blindness
 Assoc. c̄ PMR in 50%
 Ix: ESR↑↑↑, plats↑, ALP↑, Hb↓, temporal artery biopsy
 Rx
 High dose pred (60mg/d PO) for 5-7d
 Guided by symptoms and ESR.
 Give PPI+bisphosphonate.
 Prog: 2yr course then complete remission

© Alasdair Scott, 2012 166

 Migraine
Epidemiology Diagnostic Criteria
 8% prev  Typical aura + headache, or
 F:M = 2:1  ≥ 5 headaches lasting 4-72h with either n/v or
photo/phonophobia + ≥2 of:
Risk Factors  Unilat
 Obesity  Pulsating
 PFO  Interferes with normal life
 Worsened by routine activity
Pathophysiology
 Vascular: cerebrovascular constriction → aura,
dilatation → headache. Differential
 Brain: spreading cortical depression  Cluster / tension headache
 Inflammation: activation of CN V nerve terminals in  Cervical spondylosis
meninges and cerebral vessels.  HTN
 Intracranial pathology
Triggers  Epilepsy
 CHOCOLATE
 CHeese
 OCP Treatment
 Caffeine
 alcohOL Acute episode
 Anxiety  1st: Paracetamol + metoclopramide / domperidone
 Travel  2nd: NSAID (e.g. ketoprofen) + M/D
 Exercise  3rd: Rizatriptan
 CI: IHD, uncontrolled HTN, SSRIs
Symptoms  4th: ergotamine

Headache Prophylaxis
 Aura lasting 15-30min then unilat, throbbing  Avoid triggers
headache  1st: Propanolol, topiramate
 Phono/photophobia  2nd: Valproate, pizotifen (↑ wt.), gabapentin
 n/v
 Allodynia
 Often premenstrual

Prodrome (50%): precede migraine by hrs - days

 Yawning
 Food cravings
 Changes in sleep, appetite or mood

Aura (20%): precedes migraine by mins and may persist

 Visual: distortion, lines, dots, zig-zags, scotoma,
hemianopia
 Sensory: paraesthesia (fingers → face)
 Motor: dysarthria, ataxia, ophthalmoplegia,
hemiparesis (hemiplegic migraine)
 Speech: dysphasia, paraphasia

Classification
 Migraine c̄ aura (classical migraine)
 Migraine w/o aura (common migraine) 

© Alasdair Scott, 2012 167

 Subarachnoid Haemorrhage
Epidemiology Investigations
 9/100,000
 35-65yrs CT
 Detects >90% of SAH w/i first 48hrs
Causes
 Rupture of saccular aneurysms (80%) LP
 AVMs (15%)  If CT-ve and no CIs >12h after start of headache
 Xanthochromia due to breakdown of bilirubin
Risk Factors
 Smoking Mx
 HTN  Frequent neuro obs: pupils, GCS, BP
 EtOH  Maintain CPP: keep SBP >160
 Bleeding diathesis  Nimodipine for 3wks → ↓ cerebral vasospasm
 Mycotic aneurysms (SBE)  Endovascular coiling (preferable to surgical clipping)
 FH (close relatives have 3-5x risk)

Berry Aneurysms Complications

Sites Rebleeding: 20%

 Junction of post. communicating c̄ IC  Commonest cause of mortality
 Junction of ant. communicating c̄ ACA
Cerebral Ischaemia
 Bifurcation of MCA
 Due to vasospasm
Associations  Commonest cause of morbidity
 Adult polycystic kidneys
Hydrocephalus
 CoA
 Due to blockage of arachnoid granulations
 Ehlers Danlos
 May require ventricular or lumbar drain
Symptoms Hyponatraemia
 Sudden, severe occipital headache  Common
 Collapse  Don’t Mx c̄ fluid restriction
 Meningism: neck stiffness, n/v, photophobia
 Seizures
 Drowsiness → coma
Mx of Unruptured Aneurysms
 Young pts. c̄ aneurysms >7mm in diameter may
Signs benefit from surgery.
 Kernig’s
 Retinal or subhyaloid haemorrhage
 Focal neuro
 @ presentation suggests aneurysm location
 Later deficits suggests complications

Sentinel Headache
 ~6% of pts. experience sentinel headache from small
warning bleed.

Differential
 In 1O care, 25% of those c̄ thunderclap headache
have SAH
 50-60% no cause found
 Rest: meningitis, intracerebral bleeds, cortical vein
thrombosis

© Alasdair Scott, 2012 168

 Stroke: Causes and Investigation
Definition
 Rapid onset, focal neurological deficit due to a vascular lesion lasting >24h

Pathogenesis Ix
 Infarction due ischaemia (80%) or intracerebral  ID risk factors for further strokes
haemorrhage (20%).
HTN
Ischaemia (80%)  Retinopathy
 Atheroma  Nephropathy
 Large (e.g. MCA)  Big heart on CXR
 Small vessel perforators (lacunar)  (Don’t treat acutely)
 Embolism
 Cardiac (30% of strokes):AF, endocarditis, MI Cardiac emboli
 Atherothromboembolism: e.g. from carotids  ECG ± 24hr tape: AF
 Echo: mural thrombus, hypokinesis, valve lesions, ASD,
Haemorrhage (20%) VSD (paradoxical emboli)
 ↑BP
 Trauma Carotid artery stenosis
 Aneurysm rupture  Doppler US ± angio
 Anticoagulation  Endarterectomy beneficial if ≥ 70% symptomatic
 Thrombolysis stenosis

Rarer Causes Bleeding / thrombotic tendency

 Watershed stroke: sudden ↓ in BP (e.g. in sepsis)  Thrombophilia screen
 Carotid artery dissection  Thrombocytopenia
 Vasculitis: PAN, HIV
 Cerebral vasospasm 2 to SAH
O
Hyperviscosity
 Venous sinus thrombosis  Polycythaemia
 Anti-phospholipid syndrome, thrombophilia  SCD
 Myeloma

Risk Factors Metabolic

 HYPERTENSION  ↑↓ glucose
 Smoking, DM, ↑ lipids, FH  ↑ lipids
 Cardiac: AF, valve disease  Hyperhomocystinaemia
 Peripheral vascular disease
Vasculitis
 Previous history
 ↑ESR
 Ethnicity: ↑ in Blacks and Asians
 ANA
 ↑ PCV/Hct
 OCP

Cardiac Causes of Stroke

 AF: 4.5% /yr
 External cardioversion: 1-3%
 Prosthetic valves
 Acute MI: esp. large anterior
 Paradoxical systemic emboli
 Cardiac surgery
 Valve vegetations

© Alasdair Scott, 2012 169

 Stroke: Presentation
Oxford / Bamford Classification Brainstem Infarcts
 Based on clinical localisation of infarct  Complex signs depending on relationship of infarct to
 S=syndrome: prior to imaging CN nuclei, long tracts and brainstem connections
 I=infarct: after imaging when atheroembolic infarct
confirmed Features

Feature Structure
Total Anterior Circulation Stroke (TACS) Hemi- / quadr-paresis Corticospinal tracts
 Highest mortality (60% @ 1yr) + poor independence Conjugate gaze palsy Oculomotor system
 Large infarct in carotid / MCA, ACA territory
 All 3 of: Horner’s syndrome Sympathetic fibres
1. Hemiparesis (contralateral) and/or sensory deficit Facial weakness (LMN) CN7 nucleus
(≥2 of face, arm and leg)
2. Homonymous hemianopia (contralateral) Nystagmus, vertigo CN8 nucleus
3. Higher cortical dysfunction Dysphagia, dysarthria CN9 and CN10 nuclei
 Dominant (L usually): dysphasia
Dysarthria, ataxia Cerebellar connections
 Non-dominant: hemispatial neglect
↓ GCS Reticular activating syndrome

Partial Anterior Circulation Stroke (PACS)

 Carotid / MCA and ACA territory Lateral Medullary Syndrome / Wallenberg’s Syndrome
 2/3 of TACS criteria, usually:  PICA or vertebral artery
1. Hemiparesis (contralateral) and/or sensory deficit
 Features: DANVAH
(≥2 of face, arm and leg)
 Dysphagia
2. Higher cortical dysfunction
 Ataxia (ipsilateral)
 Dominant: dysphasia  Nystagmus (ipsilateral)
 Non-dom: neglect, constructional apraxia  Vertigo
 Deficit is less dense and/or incomplete  Anaesthesia
 Ipsilat facial numbness + absent corneal
reflex
Posterior Circulation Stroke (POCS)  Contralateral pain loss
 Infarct in vertebrobasilar territory  Horner’s syndrome (ipsilateral)
 Any of
1. Cerebellar syndrome
2. Brainstem syndrome Millard-Gubler Syndrome
3. Contralateral homonymous hemianopia  Pontine infarct
 6 and 7 CN nuclei + corticospinal tracts
th th

 Diplopia
Lacunar Stroke (LACS)  LMN facial palsy + loss of corneal reflex
 Small infarcts around basal ganglia, internal capsule,  Contralateral hemiplegia
thalamus and pons.
 Absence of
 Higher cortical dysfunction
Locked-in Syndrome
 Homonymous hemianopia
 Pt. is aware and cognitively intact but completely
 Drowsiness
paralysed except for the eye muscles.
 Brainstem signs
 Causes
 5 syndromes
 Ventral pons infarction: basilar artery
 Pure motor: post. limb of internal capsule
 Central potine myelinolysis: rapid correction of
 Commonest
hyponatraemia
 Pure sensory: post. thalamus (VPL)
 Mixed sensorimotor: internal capsule
 Dysarthria / clumsy hand
 Ataxic hemiparesis: ant. limb of internal capsule Stroke Differential
 Weakness + dysmetria  Head injury ± haemorrhage
 ↑↓ glucose
 SOL
Other Signs  Hemiplegic migraine
 Ischaemic pointers  Todd’s palsy
 Carotid bruit  Infections: encephalitis, abscesses, Toxo, HIV, HTLV
 AF  Drugs: e.g. opiate overdose
 Past TIA
 IHD
 Haemorrhagic pointers
 Meningism
 Severe headache
 Coma
© Alasdair Scott, 2012 170
 Stroke: Management
Acute Management Primary Prevention
 Control RFs: HTN, ↑ lipids, DM, smoking, cardiac
Resuscitate disease
 Ensure patent airway: consider NGT  Consider life-long anticoagulation in AF (use CHADS2)
 NBM until swallowing assessed by SALT  Carotid endarterectomy if symptomatic 70% stenosis
 Don’t overhydrate: risk of cerebral oedema  Exercise

Monitor
 Glucose: 4-11mM: sliding scale if DM Secondary Prevention
 BP: <185/110 (for thrombolysis)  Risk factor control as above
 Rx of HTN can → ↓ cerebral perfusion  Start a statin after 48h
 Neuro obs  Aspirin / clopi 300mg for 2wks after stroke then either
 Clopidogrel 75mg OD (preferred option)
Imaging  Aspirin 75mg OD + dipyridamole MR 200mg BD
 Urgent CT/MRI  Warfarin instead of aspirin/clopidogrel if
 Diffusion-weighted MRI is most sensitive for acute infarct  Cardioembolic stroke or chronic AF
 CT will exclude primary haemorrhage  Start from 2wks post-stroke (INR 2-3)
 Don’t use aspirin and warfarin together.
Medical  Carotid endarterectomy if good recovery + ipsilat
 Consider thrombolysis if 18-80yrs and <4.5hrs since stenosis ≥70%
onset of symptoms
 Alteplase (rh-tPA)
 → ↓ death and dependency (OR 0.64) Rehabilitation: MENDS
 CT 24h post-thrombolysis to look for haemorrhage  MDT: physio, SALT, dietician, OT, specialist nurses,
 Aspirin 300mg PO/PR once haemorrhagic stroke neurologist, family
excluded ± PPI  Eating
 Clopidogrel if aspirin sensitive  Screen swallowing: refer to specialist
 NG/PEG if unable to take oral nutrition
Surgery  Screen for malnutrition (MUST tool)
 Neurosurgical opinion if intracranial haemorrhage  Supplements if necessary
 May coil bleeding aneurysms  Neurorehab: physio and speech therapy
 Decompressive hemicraniectomy for some forms of MCA  Botulinum can help spasticity
infarction.  DVT Prophylaxis
 Sores: must be avoided @ all costs
Stroke Unit
 Specialist nursing and physio
 Early mobilisation
Occupational Therapy
 DVT prophylaxis
 Impairment: e.g. paralysed arm
Secondary Prevention  Disability: e.g. inability to write
 Handicap: e.g. can’t work as accountant
Rehabilitation  OT aims to minimise disability and abolish handicap

Prognosis @ 1yr
 10% recurrence
 PACS
 20% mortality
 1/3 of survivors independent
 2/3 of survivors dependent
 TACS is much worse
 60% mortality
 5% independence

© Alasdair Scott, 2012 171

 Transient Ischaemic Attack
Definition Mx: ACAS
 Sudden onset focal neurology lasting <24h due to  Time to intervention is crucial.
temporary occlusion of part of the cerebral circulation.  Intervention w/i 72hrs → 2% strokes @ 90d
 ~15% of 1st strokes are preceded by TIAs.  Intervention w/i 3wks → 10% strokes @ 90d
 Avoid driving for 1mo
Signs
 Symptoms usually brief
 Global events (e.g. syncope, dizziness) are not typical 1. Antiplatelet Therapy / Anticoagulate
 Signs mimic those of CVA in the same arterial territory  Aspirin/clopi 300mg/d for 2wks then 75mg/d
 Add dipyridamole MR to aspirin
 Warfarin if cardiac emboli: AF, MI, MS
Signs of Causes
 After 2wks
 Carotid bruits
 ↑BP 2. Cardiac Risk Factor Control
 Heart murmur  BP, lipids, DM, smoking
 AF  Exercise
 Diet: ↓ salt
Causes
 Atherothromboembolism from carotids is main cause 3. Assess risk of subsequent stroke
 Cardioembolism: post-MI, AF, valve disease  ABCD2 score
 Hyperviscosity: polycythaemia, SCD, myeloma
4. Specialist referral to TIA clinic
Differential  ABCD2 ≥4: w/i 24hrs
 Vascular: CVA, migraine, GCA  ABCD2 <4: w/i 1wk
 Epilepsy
 Hyperventilation Carotid Endarterectomy
 Beneficial if ≥70% symptomatic stenosis
 Hypoglycaemia
 50-70% stenosis may benefit if operative risk is <3%
 Surgery should be performed w/i 2wks.
Ix
 Endovascular stenting is an alternative, but safety and
 Aim to find cause and define vascular risk
long-term benefits (in-stent restenosis is common) are
 Bloods: FBC, U&E, ESR glucose, lipids still under Ix.
 CXR  Major complications are stroke and death.
 ECG
 Echo
 Carotid doppler ± angiography
 Consider brain imaging
Prognosis
 Diffusion weighted MRI is best  Combined risk of stroke and MI is ~9%/yr
 3x ↑ in mortality cf TIA-free populations

ABCD2 Score
 Predicts stroke risk following TIA
 Score ≥6 = 8% risk w/i 2d, 35% risk w/i 1wk
 Score ≥4 = pt. assessment by specialist w/i 24hrs
 All pts with suspected TIA should be seen by specialist
w/i 7d.

1. Age ≥ 60
2. BP ≥ 140/90
3. Clinical features
a. Unilateral weakness (2 points)
b. Speech disturbance w/o weakness
4. Duration
a. ≥ 1h (2 points)
b. 10-59min
5. DM

NB. 7 points max.

© Alasdair Scott, 2012 172

Subdural Haemorrhage
 Bleeding from bridging veins between cortex and
sinuses
 Haematoma between dura and arachnoid
 Often due to minor trauma that occurred a long time
previously – especially deceleration injuries
Extradural Haemorrhage
 Often due to # temporal or parietal bone → laceration of
middle meningeal artery and vein.
 Blood between bone and dura
 Suspect if after head injury GCS falls, is slow to
improve or there is a lucid interval.

Risk Factors Presentation

 Elderly: brain atrophy
 Falls: epileptics, alcoholics Lucid Interval
 Anticoagulation  Deterioration of GCS after head injury that caused no
LOC, or following initial improvement in GCS.
 Lucid interval may be hrs or days
Symptoms
 Headache ↑ ICP
 Fluctuating GCS, sleepiness  Headache
 Gradual physical or mental slowing  Vomiting
 Unsteadiness  Confusion → coma
 Fits

rd
Ipsilateral blown pupil (3 nerve palsy)
Signs  ± hemiparesis c̄ upgoing plantars and ↑ reflexes
 ↑ ICP (can → tentorial herniation)
 Localising signs occur late Brainstem Compression
 Deep irregular breathing (brainstem compression)
 Cushing response (↑BP, ↓HR) is late
Imaging: CT / MRI  Death by cardiorespiratory arrest
 Crescentic haematoma over one hemisphere
 Clot goes from white → grey c̄ time
 Mid-line shift
Imaging: CT
 Lens-shaped haematoma
 Skull # (↑↑ risk of extradural haemorrhage)
Mx
 1st line: irrigation/evacuation via burr-hole craniostomy
 2nd line: craniotomy
Mx
 Neuroprotective ventilation (O2>100, CO2 35-40)
 Address causes of trauma
 Consider mannitol (1g/kg IV via central line)
 Craniectomy for clot evacuation and vessel ligation
Differential
 Stroke
 Dementia
 SOL

© Alasdair Scott, 2012 173

 Intracranial Venous Thrombosis
Dural Venous sinus Thrombosis Common Causes
 Symptoms come one gradually over days-wks  Pregnancy / puerperium
 Sinus thrombosis may extend to cortical veins  OCP
 Head injury
Sagittal Sinus  Dehydration
 45% of IVT  Intracranial / extracranial malignancy
 Often co-exists if other sinuses are thrombosed  Thrombophilia
 Headache, vomiting, seizures, ↓ vision, papilloedema

Transverse Sinus Ix
 35% of IVT
 Exclude SAH and meningitis
 Headache ± mastoid pain, focal neuro, seizures,
 CT/MRI venography: absence of a sinus
papilloedema
 LP: ↑ pressure, may show RBCs and xanthochromia
Sigmoid Sinus
 Cerebellar signs, lower CN palsies
Mx
Inferior Petrosal Sinus  LMWH → warfarin (INR 2-3)
 5 and 6 CN palsies (Gradenigo’s syn.)
th th
 Fibrinolytics (e.g. streptokinase) can be used via
selective catheterisation.
Cavernous sinus  Thrombophilia screen
 Spread from facial pustules or folliculitis
 Headache, chemosis, eyelid oedema, proptosis, painful
ophthalmoplegia, fever

Cortical Vein Thrombosis

 Often → venous infarcts c̄ stroke-like focal symptoms
that evolve over days
 Thunderclap headache
 Focal-seizures

Differential
 SAH
 Meningitis
 Encephalitis
 Intracranial abscess
 Arterial stroke

© Alasdair Scott, 2012 174

 Meningitis
Features Abx Therapy
 Community: benpen 1.2g IV/IM
Meningitic  <50: ceftriaxone 2g IVI/IM BD
 Headache  >50: ceftriaxone + ampicillin 2g IVI /4h
 Neck stiffness  If viral suspected: aciclovir
 Kernig’s: Straightening leg c̄ hip @ 90O
 Brudzinski’s: lifting head → lifting of legs Organisms
 Photophobia  Viruses: enteroviruses (Coxsackie, echovirus), HSV2
 n/v  Meningococcus
 Pneumococcus
Neurological
 Listeria
 ↓ GCS → coma
 Haemophilus
 Seizures (20%)
 TB
 Focal neuro (20%): e.g. CN palsies
 Cryptococcus
Septic
 Fever Ix
 ↓BP, ↑HR  Bloods: FBC, U+Es, clotting, glucose, ABG
 ↑CRT  Blood cultures
 Purpuric rash  LP: MCS, glucose, virology/PCR, lactate
 DIC

Acute Management

ABC
 O2 15L – SpO2 94-98%
 IVI fluid resus c̄ crystalloid

Mainly Septicaemic Mainly Meningitic

 Don’t attempt LP  If no shock or CIs do LP
 Ceftriaxone 2g IVI  Dexamethasone 0.15mg/kg IV QDS
 Consider ITU if shocked  Ceftriaxone 2g IVI post-LP

Continuing Management
 Ceftriaxone 2g BD IVI
 Meningococcus: 7d IV then review
 Pneumococcus: 14d IV then review
 Maintenance fluids
 UO 30ml/h
 SBP >80mmHg
 If response is poor, consider intubation ± inotropic support
 Rifampicin prophylaxis for household contacts.

CIs to LP: Try LP Unless ContraINdicated CSF Findings

 Thrombocytopenia
Findings Bacterial TB Viral
 Lateness (delay in antibiotic admin)
Appearance Turbid Fibrin web Clear
 Pressure (signs of raised ICP)
Cells PMN Lympho / Lympho /
 Unstable (Cardio + resp systems) mononuc mononuc
 Coagulation disorder Count 100-1000 10-1000 50-1000
 Infection at LP site Glucose ↓ (< ½ ↓ (< ½ > ½ plasma
 Neurology (focal neurological signs) plasma) plasma)
Protein (g/L) ↑↑ (>1.5) ↑↑↑ (1-5) mild ↑ (<1)

© Alasdair Scott, 2012 175

Encephalitis Cerebral Abscess
Presentation Pre-disposing Factors
 Infectious prodrome: fever, rash, LNs, cold sores,  Infection: ear, sinus, dental or periodontal
conjunctivitis, meningeal signs.  Skull #
 Bizarre behaviour or personality change  Congenital heart disease
 Confusion  Endocarditis
 ↓ GCS → coma  Bronchiectasis
 Fever  Immunosuppression
 Headache
 Focal neuro Organisms
 Seizures  Frontal sinus/teeth: strep. Milleri, oropharyngeal
 Hx of travel or animal bite anaerobes
 Ear: Bacteroides, other anaerobes
Causes
Signs
Usually viral  Seizures
 HSV1/2  Fever
 CMV, EBV, VZV  Localizing signs
 Arboviruses  Signs of ↑ ICP
 HIV  Signs of infection elsewhere

Non-viral Ix
 Any bacterial meningitis  CT/MRI: ring-enhancing lesion
 TB  ↑WCC, ↑ESR
 Malaria
 Lyme disease
Rx
 Neurosurgical referral
Ix  Abx: e.g. ceftriaxone
 Bloods: cultures, viral PCR, malaria film  Treat ↑ ICP
 Contrast CT: focal bilat temporal involvement
suggests HSV
 LP: ↑ CSF protein, lymphocytes, PCR
 EEG: shows diffuse abnormalities, may confirm Dx

Mx
 Aciclovir STAT: 10mg/kg/8h IVI over 1h for 14/7
 Supportive measures in HDU/ITU
 Phenytoin for seizures

Prognosis
 70% mortality if untreated

w/o fever, consider encephalopathy

 ↓ glucose
 Hepatic
 DKA
 Drugs
 SLE
 Uraemia
 Hypoxic brain injury
 Beri-Beri

© Alasdair Scott, 2012 176

 Epilepsy: Features
Definition Presenting Features
 Recurrent tendency to spontaneous, intermittent,
abnormal electrical activity in part of the brain, manifest Simple Partial
as seizures.  Focal motor, sensory, autonomic or psychic symptoms

Causes Complex Partial: 5As

 2/3 are idiopathic (often familial)  Aura
 Congenital  Autonomic: change in skin colour, temperature, palps
 NF  Awareness lost: motor arrest, motionless stare
 Tuberous Sclerosis  Automatisms: lip-smacking, fumbling, chewing,
 TORCH swallowing
 Perinatal anoxia  Amnesia
 Acquired
 Vascular: CVA NB. Usually arise from temporal lobe
 Cortical scarring: trauma, infection
 SOL Absences (Petit mal)
 Other: SLE, PAN, MS, sarcoidosis  ABrupt onset and offset
 Non-epileptic / provoked seizures  Short: <10s
 Withdrawal: EtOH, opiates, benzos  Eyes: glazed, blank-stare
 Metabolic: glucose, Na, Ca, urea, NH3  Normal: intelligence, examination, brain-scan
 ↑ICP: trauma, haemorrhage, cortical venous  Clonus or automatisms may occur
thrombosis  EEG: 3Hz spike and wave
 Infection: meningitis, encephalitis, cycticerosis,  Stimulated by hyperventilation and photics
HIV
 Eclampsia Tonic-Clonic (Grand mal)
 Pseudoseizures  LOC
 Tonic: limbs stiffen
NB. 3-10% of provoked seizures tend to recur, cf 30-50% of  Clonic: rhythmic jerking of limbs
unprovoked seizures  ± cyanosis, incontinence, tongue biting (lateral)
 Post-ictal confusion and drowsiness
Seizure Terms
 Prodrome Myoclonic Seizure
 pt. or others may notice change in mood or  Sudden jerk of limb, face or trunk.
behaviour lasting hrs – days.
 Not part of seizure. Atonic (akinetic seizures)
 Aura  Sudden loss of muscle tone → fall
 A simple partial seizure (usually temporal) which  No LOC
may precede other manifestations.
 Experienced as a strange feeling: West Syndrome / Infantile Spasms
 Epigastric rising  Clusters of head nodding and arm jerks
 Deja/jamias vu,  EEG shows hypsarrhythmia
 Automatisms,
 Smells, lights, sounds
 Partial (focal) Localising Features
 Features referable to part of one hemisphere
 Primary Generalised Temporal
 No warning / aura
 Automatisms: lip smacking, chewing, fumbling
 Discharge throughout cortex w/o localising
 Deja/jamias vu
features
 Delusional behaviour
 Simple: awareness unimpaired
 Abdominal: rising, n/v
 Complex: awareness impaired
 Emotional disturbance: terror, panic, anger, elation
 Secondary Generalised
 Tastes, smells
 Focal seizure → generalised
 E.g. aura → tonic-clonic
Frontal
 Motor features: arrest, Jacksonian march, Todd’s palsy
Diagnostic Pointers Parietal
 Aura  Sensory disturbance: tingling, numbness
 Specific trigger: e.g. flashing lights
 Lateral tongue biting (> incontinence) Occipital
 Typical movements: e.g. tonic-clonic  Visual phenomena: spots, lines, flashes
 Cyanosis
 Post-ictal phase

© Alasdair Scott, 2012 177

 Epilepsy: Investigation and Management
General Principles Side Effects of Common AEDs
 After any seizure advise against driving, swimming,
et.c. until a Dx is established Enzyme Effects
 Don’t diagnose epilepsy from a single seizure  Inducers: CBZ, PHE and barbiturates
 Diagnosis should be made by a specialist  Inhibitors: Valproate
 After Dx, cannot drive until seizure-free for >1yr
 10yrs for HGV (no meds) Lamotrigine
 Skin rash → SJS: occurs w/i 1st 8wks
 Rash may be assoc. c̄ hypersensitivity → fever, ↑LFTs,
Investigations DIC.
 Basic bloods: FBC, U+Es, glucose  Diplopia, blurred vision
 Se ↑ Prl 10min after fit (relative to baseline)  Levels affected by enzyme inhibitors/inducers
 Se AED levels
 Urine toxicology Valproate
 ECG  Appetite ↑ → ↑wt.
 Liver failure: monitor LFTs over 1 6mo
st

EEG  Pancreatitis
 Use to support Dx (cannot exclude or prove)  Reversible hair loss
 Helps classification and prognosis  Oedema
 Use c̄ hyperventilation and photic stimulation  Ataxia
 Teratogenicity, Tremor, Thrombocytopaenia
Neuroimaging: typically MRI  Encephalopathy: due to ↑ ammonia
 Not routine for idiopathic generalised epilepsy
 Indications Carbamazepine
 Developed epilepsy as an adult  Leukopenia
 Any evidence of focal onset  Skin reactions
st
 Seizures continue despite 1 -line Rx  Diplopia, blurred vision
 SIADH → hyponatraemia

Drug Therapy Phenytoin

 Gingival hypertrophy
Seizure Type 1st line 2nd line  Hirsutism
Tonic-Clonic Valproate Lamotrigine  Cerebellar syn.
Absences Valproate Lamotrigine  Ataxia
Ethosuximide  Nystagmus
Tonic, atonic or Valproate Levetiracetam  Dysarthria
myoclonic  Peripheral sensory neuropathy
Focal ± 2O gen Lamotrigine CBZ  Diplopia
 Tremor

In Women / Pregnancy
 Avoid valproate: take lamotrigine (or CBZ)
 5mg folic acid daily if child-bearing age
 CBZ and PHE are enzyme inducers and ↓ the
effectiveness of the OCP

Other Options
 Neurosurgical resection can be an option if a single
epileptogenic focus is identified
 Vagal nerve stimulation can ↓ seizure frequency and
severity in ~33%

© Alasdair Scott, 2012 178

 Status Epilepticus
Definition Drugs
 Seizure lasting >30min, or
 Repeated seizures w/o intervening consciousness Lorazepam
 2-4mg IV bolus over 30s
Ix  2nd dose if no response w/i 2min
 BM  Alternatives:
 Bloods: glucose, ABG, U+E, FBC, Ca2+  Diazepam 10mg IV/PR (20mg max)
 ECG, EEG  Midazolam 10mg buccal
 Consider: AED levels, tox screen, LP, βhCG, CT
Phenytoin
 18mg/kg IVI @ 50mg/min
 100mg/6-8h maintenance
 Monitor ECG and BP
 CI: bradycardia or heart block

Diazepam Infusion
 100mg in 500ml 5% dex @ 40ml/hr (3mg/kg/24h)

Dexamethasone
 10mg IV if vasculitis / cerebral oedema (tumour)
possible

Acute Management

ABC
 Oral / nasal airway, intubate
 Suction
 100% O2
 Capillary blood glucose

IV Access + Bloods
 U+E, LFT, FBC, Glucose, Ca2+
 AED levels
 Tox screen

Reverse Potential Causes

 Thiamine 250mg IV if EtOH
 100ml 20% glucose unless glucose
known to be normal

Slow IV Bolus Phase

 Lorazepam 2-4mg IV
 2nd dose if no response w/i 2min

IV Infusion Phase
 Phenytoin 18mg/kg IVI (then 100mg/6-8h)
 Or, diazepam 100mg in 500ml 5% dex IVI

RSI Phase
Never spend >20min c̄ someone in status w/o
getting an anaesthetist

© Alasdair Scott, 2012 179

 Head Injury
Initial Mx Intubate
 GCS ≤ 8
1O Survey  PaO2 <9KPa on air / <13KPa on O2 or PCO2 >6KPa
 A: ? intubation, immobilise C-spine  Spontaneous hyperventilation: PCO2 <4KPa
 B: 100% O2, RR  Respiratory irregularity
 C: IV access, BP, HR
 D: GCS, pupils CT head guidelines: BANGS LOC
 Treat seizures  Break: open, depressed or base of skull
 Lorazepam 2-4mg IV  Amnesia >30min retrograde
 Phenytoin18mg/kg IVI then 100mg/6-8h  Neuro deficit or seizure
 E: expose pt. and look for other obvious injuries  GCS: <13 @ any time or <15 2h after injury
 Sickness: vomited > once
2O Survey  LOC or any amnesia and any of:
 Look for:  Dangerous mechanism: RTA, great height
 Lacerations  Age ≥ 65
 Obvious facial/skull deformity  Coagulopathy (inc. warfarin)
 CSF leak from nose or ears
 Battle’s sign, Racoon eyes
 Blood behind TM
Risk of Intracranial Haematoma in Adults:
 C-spine tenderness ± deformity  Fully conscious, no skull # = <1:1000
 Head-to-toe examination for other injuries  Confused, no skull # = 1:100
 Log role  Fully conscious, skull # = 1:30
 Confused, skull # = 1:4
Hx if possible
 How and when? GCS
 GCS and other vitals immediately after injury
 Headache, fits, vomiting, amnesia, EtOH Eyes: 4
4 – Spontaneous eye opening
Ix 3 – Open to voice
 Bloods: FBC, U+E, glucose, clotting, EtOH level, ABG 2 – Open to pain
 ? CT head + c-spine 1 – No opening

Verbal: 5
Rx 5 – Orientated conversation
 Neurosurgical opinion if signs of ↑ICP, CT evidence of 4 – Confused conversation
intracranial bleed significant skull # 3 – Inappropriate speech
 Admit if: 2 – Incomprehensible sounds
 Abnormalities on imaging 1 – No speech
 Difficult to assess: EtOH, post-ictal
 Not returned to GCS 15 after imaging Motor: 6
 CNS signs: vomiting, severe headache 6 – Obeys commands
 Neuro-obs half-hrly until GCS 15 5 – Localises pain
 GCS 4 – Withdraws to pain
 Pupils 3 – Decorticate posturing to pain (flexor)
 HR, BP 2 – Decerebrate posturing to pain (extensor)
 RR, SpO2 1 – No movement
 Temperature

Discharge Advice
 Stay with someone for first 48hrs
 Give advice card advising return on:
 Confusion, drowsiness, unconsciousness
 Visual problems
 Weakness
 Deafness
 V. painful headache that won’t go away
 Vomiting
 Fits

© Alasdair Scott, 2012 180

Space Occupying Lesions Idiopathic Intracranial Hypertension
Presentation Presentation
 Typically obese females
↑ ICP  As if SOL
 Headache: worse on waking, lying down, bending  ↑ ICP
forward, coughing, straining.  Headache
 Vomiting  Papilloedema
 Papilloedema  Visual
 ↓GCS  Blurred vision
 6th CN palsy
Seizures  Enlarged blind spot
 Exclude SOL in adult-onset seizures, especially c̄
localising aura or post-ictal weakness (Todd’s) Cause
 Usually idiopathic
Evolving Focal Neurology  May be 2O to venous sinus thrombosis or drugs
 May be false-localising (esp. CN6 palsy)
Mx
Subtle Personality Change  Wt. loss
 Acetazolamide
 Loop diuretics
Causes  Prednisolone
 Vascular: chronic subdural haematoma, AVM,  Lumbar-peritoneal shunt may be necessary if drugs fail
aneurysm and visual loss deteriorates.
 Infection: abscess, cyst (cysticercosis)
 Neoplasm: primary or secondary Prognosis
 Granuloma: TB, sarcoid  Usually self-limiting
 Permanent visual loss in 10%.
Tumours
 30% metastatic: breast, lung, melanoma
 Astrocytoma
 Glioblastoma multiforme
 Ependyoma
 Meningiomas
 Cerebellar haemangioblastoma
 CNS lymphoma

Differential
 Vasc: stroke, venous sinus thrombosis
 Traumatic head injury
 Infection: encephalitis
 Inflam: vasculitis, MS
 Metabolic disturbance
 Idiopathic intracranial hypertension

Ix
 CT or MRI (better for post. cranial fossa)
 Consider biopsy

© Alasdair Scott, 2012 181

 Raised ICP
Types of Cerebral Oedema
1. Vasogenic (↑ cap permeability): trauma, tumour, ischaemia, infection
2. Cytotoxic: e.g. from hypoxia
3. Interstitial: e.g. obstructive hydrocephalus, ↓Na+

Causes Acute Management

 Haemorrhage
ABC
 Tumours
 Infection: meningitis, encephalitis, abscess
 Hydrocephalus
 Status Treat seizures and correct hypotension
 Cerebral oedema

Signs and Symptoms Elevate bed to 40O

 Headache
 n/v
 Seizures Neuroprotective Ventilation
 Drowsiness → coma  PaO2: >13KPa (100mmHg)
 Cushing’s reflex: ↑BP, ↓HR, irregular breathing  PCO2: 4.5kPa
 6th CN palsy (may be false localising)  Good sedation ± NM blockade
 Cheyne-Stokes respiration
 Pupils: constriction → dilatation
 Papilloedema, loss of venous pulsation @ disc
Mannitol or Hypertonic Saline
 ↓ICP short-term, but may → rebound ↑ICP later
 Mannitol 1g/kg (20% @ 5ml/kg)

Herniation Syndromes

Tonsillar (Coning)
 ↑ pressure in posterior fossa → displacement of cerebellar tonsils through foramen magnum
 → compression of brainstem and cardioresp centres in medulla
 CN6 palsy, upgoing plantars → irregular breathing → apnoea

Transtentorial / Uncal
 Lateral supratentorial mass → compression of ipsilateral inferomedial temporal lobe (uncus) against free margin of
tentorium cerebelli.
 Ipsilateral CN3 palsy: mydriasis (dilation) then down-and-out
 Ipsilateral corticospinal tract: contralateral hemiparesis
 May → compression of contralateral corticospinal tracts → ipsilateral hemiparesis (Kernohan’s Notch: False Localising)

Subfalcine
 Frontal mass
 Displacement of cingulate gyrus (medial frontal lobe) under falx cerebri
 Compression of ACA → stroke
 Contralateral motor/sensory loss in legs>arms
 Abulia (pathological laziness)

© Alasdair Scott, 2012 182

 Parkinsonism
Causes

Degenerative
1. Parkinson’s disease

2. Parkinson’s-plus syndromes (basal ganglia degeneration + other system)

a. Multiple Systems Atrophy / Shy-Drager

 Autonomic dysfunction: post hypotension, bladder dysfunction
 Cerebellar + pyramidal signs
 Rigidity > Tremor

b. Progressive Supranuclear Palsy

 Postural instability → falls
 Speech disturbance (+ dementia)
 Palsy: vertical gaze

c. Corticobasilar Degeneration:
 Aphasia, dysarthria, apraxia
 Akinetic rigidity in one limb
 Astereognosis (cortical sensory loss)
 Alien limb phenomenon

d. Lewy Body Dementia:

 Fluctuating cognition
 Visual hallucinations

Infection
 Syphilis
 HIV
 CJD

Vascular: Multiple infarcts in SN

Drugs: Antipsychotics, metoclopramide

Trauma: dementia pugilistica

Genetic: Wilson’s disease

Symptoms

Tremor
 Worse at rest
 Exacerbated by distraction
 4-6hz, pill-rolling

Rigidity
 ↑ tone in all muscle groups: lead-pipe rigidity
 Rigidity + tremor → cog-wheel rigidity

Bradykinesia
 Slow initiation of movement c̄ reduction of amplitude on repetition
 Expressionless face
 Monotonous voice
 Micrografia

Gait
 ↓ arm swing
 Festinance
 Freezing (esp. in doorways)

© Alasdair Scott, 2012 183

 Parkinson’s Disease
Epidemiology Ix
 Mean onset 65yrs  DaTSCAN
 2% prevalence

Mx
Pathophysiology  MDT: neurologist, PD nurse, physio, OT, social worker,
 Destruction of dopaminergic neurones in pars compacta GP and carers
of substantia nigra.  Assess disability
 β-amyloid plaques  e.g. UPDRS: Unified Parkinson’s Disease Rating Scale
 Neurofibrillary tangles: hyperphosphorlated tau  Physiotherapy: postural exercises
 Depression screening

Features: TRAPPS PD Medical

 Asymmetric onset: side of onset remains worst  Young onset ± biologically fit
1. Da agonists: ropinirole, pramipexole
2. MOA-B inhibitors: rasagiline, selegiline
 Tremor: ↑ by stress, ↓ by sleep
3. L-DOPA: co-careldopa or co-beneldopa
 Rigidity: lead-pipe, cog-wheel
 Akinesia: slow initiation, difficulty c̄ repetitive movement,
 Biologically frail ± comorbidities
micrographia, monotonous voice, mask-like face 1. L-DOPA
 Postural instability: stooped gait c̄ festination 2. MOA-B inhibitors
 Postural hypotension: + other autonomic dysfunction
 Sleep disorders: insomnia, EDS, OSA, RBD  Other therapies
 Psychosis: esp. visual hallucinations  COMT inhibitor: tolcapone, entacapone
 Depression / Dementia / Drug SEs  Lessen end-of-dose effect
 Apomorphine: potent Da agonist
Sleep Disorder  SC rescue pen for sudden “off” freezing
 Affects ~90% of PD pts.  Amantidine: weak Da agonist
 Insomnia + frequent waking → EDS  Rx of drug-induced dyskinesias
 Inability to turn  Atypical antipsychotics: e.g. quetiapine
 Restless legs  Disease-induced psychosis
 Early morning dystonia (drugs wearing off)  SSRIs: citalopram, sertraline
 Nocturia  Depression
 OSA
 REM Behavioural sleep Disorder Surgical
 Loss of muscle atonia during REM sleep  Interrupt basal ganglia
 Violent enactment of dreams  Deep brain stimulation
 Da SEs: insomnia, drowsiness, EDS

Autonomic Dysfunction
 Combined effects of drugs and neurodegeneration Prognosis
 Postural hypotension  ↑ mortality
 Constipation  Loss of response to L-DOPA w/i 2-5yrs
 Hypersalivation → dribbling (↓ ability to swallow saliva)
 Urgency, frequency, Nocturia
 ED Differential
 Hyperhidrosis  Parkinson plus syndromes
 Multiple infarcts
L-DOPA SEs: DOPAMINE  Drugs: neuroleptics
 Dyskinesia  Inherited: Wilson’s
 On-Off phenomena = Motor fluctuations  Infection: HIV, syphilis, CJD
 Psychosis  Dementia pugilistica
 ABP↓
 Mouth dryness
 Insomnia
 N/V
 EDS (excessive daytime sleepiness)

Motor Fluctuations
 End-of-dose: deterioration as dose wears off c̄
progressively shorter benefit.
 On-Off effect: unpredictable fluctuations in motor
performance unrelated to timing of dose.

© Alasdair Scott, 2012 184

 Multiple Sclerosis
Definition
 A chronic inflammatory condition of the CNS characterised by multiple plaques of demyelination disseminated in time and
space.

Epidemiology Ix
 Lifetime risk: 1/1000  MRI: Gd-enhancing or T2 hyper-intense plaques
 Age: mean @ onset = 30yrs  Gd-enhancing = active inflammation
 Sex: F>M = 3:1  Typically located in periventricular white matter
 Race: rarer in blacks  LP: IgG oligoclonal bands (not present in serum)
 Abs
Aetiology  Anti-MBP
 Genetic (HLA-DRB1), environmental, viral  NMO-IgG: highly specific for Devic’s syn.
 Evoked potentials: delayed auditory, visual and sensory
Pathophysiology
 CD4 cell-mediated destruction of oligodendrocytes → Diagnosis: clinical
demyelination and eventual neuronal death.  Demonstration of lesions disseminated in time and space
 Initial viral inflam primes humoral Ab responses vs. MBP  May use McDonald Criteria
 Plaques of demyelination are hallmark
Differential
Classification: Inflammatory conditions may mimic MS plaques:
 Relapsing-remitting: 80%  CNS sarcoidosis
 Secondary progressive  SLE
 Primary progressive: 10%  Devic’s: Neuromyelitis optica (NMO)
 Progressive relapsing  MS variant c̄ transverse myelitis and optic atrophy
 Distinguished by presence of NMO-IgG Abs
Presentation: TEAM
 Tingling Mx
 Eye: optic neuritis (↓ central vision + eye move pain)  MDT: neurologist, radiologist, physio, OT, specialist
 Ataxia + other cerebellar signs nurses, GP, family 
 Motor: usually spastic paraparesis
Acute Attack
Clinical features  Methylpred 1g IV/PO /24h for 3d
Sensory: Motor  Doesn’t influence long-term outcome
 Dys/paraesthesia  Spastic weakness  ↓ duration and severity of attacks
 ↓ vibration sense  Transverse myelitis
Preventing Relapse: DMARDs
 Trigeminal neuralgia
 IFN-β: ↓ relapses by 30% in relapsing remitting MS
Eye: Cerebellum:  Glatiramer: similar efficacy to IFN-β
 Diplopia  Trunk and limb ataxia  
 Visual phenomena  Scanning dysarthria Preventing Relapse: Biologicals
 Bilateral INO  Falls  Natalizumab: anti-VLA-4 Ab
 ↓ Relapses by 2/3 in RRMS
 Optic neuritis → atrophy
 Alemtuzumab (Campath): anti-CD52
nd
GI: Sexual/GU:  2 line in RRMS
 Swallowing disorders  ED + anorgasmia
Symptomatic
 Constipation  Retention
 Fatigue: modafinil
 Incontinence
 Depression: SSRI (citalopram)
Lhermitte’s Sign  Pain: amitryptylline, gabapentin
 Neck flexion → electric shocks in trunk/limbs  Spasticity: physio, baclofen, dantrolene, botulinum
 Urgency / frequency: oxybutynin, tolterodine
Optic Neuritis  ED: sildenafil
 PC: pain on eye movement, rapid ↓ central vision  Tremor: clonazepam
 Uhthoff’s: vision ↓ c̄ heat: hot bath, hot meal, exercise
Prognosis  
 o/e: ↓ acuity, ↓ colour vision, white disc, central scotoma,
RAPD  
Poor Prognostic Signs Better Prognostic Signs
INO / ataxic nystagmus / conjugate gaze palsy  Older  Female 
 Disruption of MLF connecting CN6 to CN3  Male   <25 
 Weak adduction of ipsilateral eye  Motor signs @ onset  Sensory signs @ onset 
 Nystagmus of contralateral eye  Many relapses early on  Long interval between
 Convergence preserved  Many MRI lesions relapses 
 Axonal loss  Few MRI lesions 

© Alasdair Scott, 2012 185

 Cord Compression
Symptoms Cauda Equina and Conus Medullaris Lesions
 Deep, local spinal pain  Spinal cord tapers to its end at L1
 Stabbing, radicular pain in a dermatomal distribution  Compared c̄ lesions higher up the cord, these lesions
and LMN weakness @ lesion level are flaccid and areflexic (cf, spastic and hyperreflexic)
 Progressive UMN weakness and sensory loss below
lesion Conus Medullaris Lesions
 Bladder hesitancy, frequency → painless retention  Mixed UMN/LMN weakness
 Faecal incontinence or constipation  Early constipation and retention
 Back pain
Signs  Sacral sensory disturbance
 Look for motor, reflex and sensory level  ED
 Shooting, radicular pain @ level, anaesthesia below
 LMN signs @ level, UMN signs below level Cauda Equina Lesions
 Tone and reflexes are usually reduced in acute cord  Saddle anaesthesia
compression  Back pain
 Radicular pain down legs
Causes  Bilateral flaccid, areflexic lower limb weakness
 Trauma  Incontinence / retention of faeces / urine
 Infection: epidural abscess, TB  Poor anal tone
 2O to malignancy: breast, thyroid, bronchus, kidney,
prostate Mx
 Disc prolapse  As for cord compression
 Haematoma: warfarin  These are neurosurgical emergencies and require
 Intrinsic cord tumour urgent imaging and surgical decompression
 Myeloma

Ix
 MRI is definitive modality
 CXR for primaries

Rx
 This is a neurosurgical emergency
 Malignancy
 Dexamethasone IV
 Consider chemo, radio and decompressive
laminectomy
 Abscess: abx and surgical decompression

Differential
 Transverse myelitis
 MS
 Cord vasculitis
 Spinal artery thrombosis
 Aortic dissection

© Alasdair Scott, 2012 186

 Cervical and Lumbar Degeneration
Spondylosis Lumbosacral Spondylosis
 Degeneration due to trauma or ageing
 IV disc / vertebral collapse Presentation
 Osteophytes  L5 and S1 roots most commonly compressed by
 May → central (myelopathy) and/or lateral prolapse of L4/5, L5/S1 discs.
(radiculopathy) pathology  May present as severe pain on sneezing/coughing, a
few days after low back strain
 Lumbago – low back pain
Cervical Spondylosis  Sciatica – shooting radicular pain down buttock and
 90% of men > 60 and women > 50 thigh

Presentation Signs
 Usually asympto  Limited spinal flexion
 Neck stiffness ± crepitus  Pain on straight-leg raise
 Stabbing / dull arm pain (brachialgia)
 Upper limb motor and sensory disturbances according L4/5 → L5 Root Compression
to compression level (often C7)  Weak hallux extension ± foot drop
 Can → myelopathy c̄ quadraparesis and sphincter  In foot drop due to L5 radiculopathy, weak
dysfunction inversion (tib. post.) helps distinguish from
peroneal N. palsy.
Specific Signs  ↓ sensation on inner dorsum of foot
 Lhermitte’s sign: neck flexion → tingling down spine
 Hoffman reflex: flick to middle finger pulp → brief pincer L5/S1 → S1 Root Compression
flexion of thumb and index finger  Weak foot plantarflexion and eversion
 Loss of ankle-jerk
Typical Deficits  Calf pain
 ↓ sensation over sole of foot and back of calf
Root Disc Motor Weakness Sensory
C5 C4/5 Deltoid Numb elbow Central Compression
Supraspinatus  Cauda equina syndrome
↓ supinator jerk
C6 C5/6 Biceps Numb thumb and index Ix
Brachioradialis finger  MRI is definitive (emergency if cauda-equina syndrome)
↓ biceps jerk
C7 C6/7 Triceps Numb middle finger Rx
Finger extension  Conservative: rest, analgesia, mobilisation/physio
↓ triceps jerk  Medical: transforaminal steroid injection
C8 C7/T1 Finger flexors Number ring and little  Surgical: discectomy or laminectomy may be
Intrinsic hand fingers considered in cauda-equina syndrome, continuing pain
or muscle weakness.
Ix
 MRI
Spinal Stenosis
Rx  Developmental predisposition ± facet joint osteoarthritis
 Conservative: stiff collar, analgesia → generalized narrowing of lumbar spinal canal.
 Medical: transforaminal steroid injection
 Surgical: decompression: laminectomy or laminoplasty Presentation
 Spinal claudication
Differential  Aching or heavy buttock and lower limb pain on
 MS walking
 Nerve root neurofibroma  Rapid onset
 SACD  May c/o paraesthesiae/numbness
 Pain eased by leaning forward (e.g. on bike)
 Pain on spine extension
 Negative straight leg raise

Ix
 MRI

Rx
 Corsets
 NSAIDs
 Epidural steroid injection
 Canal decompression surgery

© Alasdair Scott, 2012 187

 Facial Nerve Palsy
Bell’s Palsy Other Causes of Facial Palsy
 Inflammatory oedema from entrapment of CNVII in
narrow facial canal May be suggested by
 Probably of viral origin (HSV1)  Bilat symptoms: Lyme, GBS, leukaemia, sarcoid, MG
 75% of facial palsy  UMN signs: sparing of frontalis and orbicularis oculi
 Due to bilateral cortical representation
Features  Other CN palsies (but seen in 8% of Bell’s)
 Sudden onset: e.g. overnight  Limb weakness
 Complete, unilateral facial weakness in 24-72h  Rashes
 Failure of eye closure → dryness
 Bell’s sign: eyeball rolls up on attempted closure Intracranial
 Drooling, speech difficulty  Vascular, MS, SOL
 Numbness or pain around ear  Motor cortex → UMN
 ↓ taste (ageusia)  Assoc. c̄ ipsilateral hemiplegia
 Hyperacusis: stapedius palsy  Brainstem nuclei → LMN
 Usually assoc. c̄ CN6 palsy + contralateral
Ix hemiplegia
 Serology: Borrelia or VZV Abs  Cerebello-pontine angle: acoustic neuroma, meningioma
 MRI: SOL, stroke, MS th th
 Both may be accompanied by 5 , 6 and 8 CN
th

 LP palsies and cerebellar signs

 Loss of corneal reflex
Mx  Sensorineural deafness, tinnitus, vertigo
 Give prednisolone w/i 72hrs  DANISH
 60mg/d PO for 5/7 followed by tapering
 Valaciclovir if zoster suspected Intra-temporal
 Otherwise antivirals don’t help  Cholesteatoma
 Protect eye  Ramsay Hunt
 Dark glasses  Otitis media
 Artificial tears  Trauma
 Tape closed @ night
 Plastic surgery may help if no recovery Infra-temporal
 Parotid tumours
Prognosis  Trauma
 Incomplete paralysis: recovers completely w/i wks
 Complete: 80% get full recovery Systemic
 Remainder have delayed recovery or permanent  Peripheral neuropathy
neurological / cosmetic abnormalities.  Demyelinating: GBS
 Axonal: DM, Lyme, HIV, Sarcoid
Complications: Aberrant Neural Connections  Pseudopalsy: MG, botulism
 Synkinesis: e.g. blinking causes up-turning of mouth
 Crocodile tears: eating stimulates unilateral
lacrimation, not salivation

Ramsay Hunt Syndrome

 American neurologist James Ramsay Hunt in 1907
 Reactivation of VZV in geniculate ganglion of CNVII

Features
 Preceding ear pain or stiff neck
 Vesicular rash in auditory canal ± TM, pinna, tongue,
hard palate (no rash = zoster sine herpete)
 Ipsilateral facial weakness, ageusia, hyperacusis,
 May affect CN8 → vertigo, tinnitus, deafness

Mx
 If Dx suspected give valaciclovir and prednisolone w/i
first 72h

Prognosis
 Rxed w/i 72h: 75% recovery
 Otherwise: 1/3 full recovery, 1/3 partial, 1/3 poor

© Alasdair Scott, 2012 188

 Mononeuropathies
 Lesions of individual peripheral or cranial nerves
 Usually local cause: trauma, entrapment
 Mononeuritis multiplex: 2 or more peripheral nerves affects
 Usually systemic cause: DM most commonly
 WAARDS PLC: Wegener’s, AIDS, Amyloid, RA, DM, Sarcoidosis, PAN, Leprosy, Carcinomatosis
 Electromyography (EMG) helps define site of lesion

Nerve Location / Cause Motor Features Sensory Loss/ Features

Median: C6-T1 Wrist: carpal tunnel, trauma LLOAF muscles Radial 3½ fingers and palm
Thenar wasting Aching pain in hand
↓ 2-point discrimination
Tinel’s and Phalen’s +ve
Ulnar: C7-T1 Elbow trauma Partial claw hand Ulnar 1½ fingers
- e.g. supracondylar # Hypothenar wasting
Can’t do good luck sign
Weakness and wasting of 1st dorsal
interosseous
Froment’s +ve

ii
Radial: C5-T1 Low: wrist Low: finger drop Dorsal thumb root (snuff box)
High: humerus High: wrist drop
V. high: Axilla V. high: triceps paralysis, wrist drop
Brachial plexus Trauma High (C5-6): Erb’s palsy → waiter’s tip High: C5-6 dermatome
Radiotherapy (e.g. breast) Low (C8-T1): Klumpke’s → claw hand Low: C8-T1 dermatome
Phrenic: C3-5 Neoplastic: Orthopnoea + raised hemidiaphragm
Lung Ca
Myeloma
Thymoma
Mechanical:
Cervical spondylosis
Big left atrium
Infective:
C3-5 zoster
HIV
Lyme
TB
Lat. cut. N. Entrapment under inguinal Meralgia paraesthetica –
thigh: L2-3 ligament anterolat. burning thigh pain
Sciatic: L4-S3 Pelvic tumours Hamstrings Below knee laterally and foot
Pelvic or femur #s All muscles below knee
Common Fibular head: trauma, sitting Foot drop: can’t walk on heals Below knee laterally
peroneal: L4-S1 cross legged Weak ankle dorsiflexion, eversion
NB. inversion intact!
Tibial: L4-S3 Can’t plantar flex → can’t stand on tiptoe Sole of foot
Foot inversion
Toe flexion

© Alasdair Scott, 2012 189

 Polyneuropathies
Features Sensory Neuropathy
 Generalised disorders of peripheral or cranial nerves
 Distribution is symmetrical and widespread Main Causes
 Distal weakness and sensory loss (glove + stocking)  Alcohol
 Classification  B12
 Time-course: acute, chronic  CRF and Ca (paraneoplastic)
 Function: motor, sensory, autonomic, mixed  DM
 Path: demyelination, axonal degeneration, both  Every vasculitis

General Features
Causes  Glove and stocking distribution: length dependent
 Deep tendon reflexes may be ↓ or absent
Metabolic (mostly axonal) Vasculitis  E.g. loss of ankle jerks in diabetic neuropathy
 DM  PAN  Signs of trauma or joint deformity (Charcot’s joints)
 Renal failure / uraemia  RA  Diabetic and alcoholic neuropathies are painful
 Hypothyroid  Wegener’s  Some aetiologies favour loss of particular fibres
 ↓B1 or ↓B12 (EtOH)
  Large Myelinated Fibres (Aα): e.g. B12
Inflammatory Infection  Loss of proprioception → ataxia
 GBS  HIV  Pins and needles
 Sarcoidosis  Syphilis
 Leprosy Small Unmyelinated Fibres (C): e.g. EtOH
 Lyme  Loss of pain and temperature sensation
   Painful dysesthesia: e.g. burning, hyperalgesia
Inherited Toxins
 CMT  Lead
 Refsum’s syndrome   Motor Neuropathy
Main Causes
Drugs Other  GBS (+ botulism)
 Isoniazid  Amyloid  HMSN / CMT
 EtOH  Paraproteinaemias  Paraneoplastic
 Phenytoin  Lead poisoning
 Vincristine
Features
 Weakness/clumsiness of hands, difficulty walking
History  LMN signs
 Time-course  CN: diplopia, dysarthria, dysphagia
 Precise symptoms  Involvement of respiratory muscles → ↓FVC
 Assoc. events
 D&V: GBS
 ↓wt: Ca
 Arthralgia: connective tissue
Autonomic Neuropathy
 Travel, EtOH, drugs
Causes
 DM
 HIV
Ix
 SLE
 LFTs, U+E, glucose, ESR, B12
 GBS, LEMS
 TFTs B1, ANA, ANCA
 Genetic tests: e.g. PMP22 in CMT
Features
 Nerve conduction studies  Postural hypotension
 EMG  ED, ejaculatory failure
 ↓ sweating
 Constipation / Nocturnal diarrhoea
 Urinary retention
 Horner’s

Autonomic Function Tests

 BP: Postural drop > 20/10mmHg
 ECG: variation >10bpm c̄ respiration

Primary Autonomic Failure

 Idiopathic or part of MSA or PD

© Alasdair Scott, 2012 190

Guillain-Barré Syndrome
Classification
 AIDP: Acute autoimmune demyelinating polyneuropathy
 AMAN: Acute motor (± sensory) axonal neuropathy
 Miller-Fisher: Ophthalmoplegia + ataxia + areflexia
Charcot-Marie-Tooth Syndrome
= Peroneal Muscular Atrophy
= Hereditary Motor and Sensory Neuropathy
Pathophysiology
 Group of inherited motor and sensory neuropathies

Causes HMSN1
 Abs cross-react to gangliosides  Commonest form
 No precipitant identified in 40%  Demyelinating
 Bacteria: C. jejuni, mycoplasma  AD mutation in the peripheral myelin protein 22 gene
 Viruses: CMV, EBV, HSV, HIV, flu
 Vaccines: esp. rabies HMSN2
 Second commonest form
 Axonal degeneration (near normal conduction velocity)
Features and Ix: GBS=AIDP
Growing Weakness Clinical Features
 Symmetrical, ascending flaccid weakness / paralysis  Onset at puberty
 LMN signs: areflexia, fasciculations may occur
 Proximal > distal (trunk, respiratory, CNs [esp. 7]) Nerves
 Progressive phase lasts ≤ 4wks  Thickened, enlarged nerves: esp. common peroneal

Breathing and Bulbar Problems Motor

 Foot drop → high stepping gait
Back Pain  Weak ankle dorsiflexion and toe extension
 Back / limb pain is common  Absent ankle jerks
 Symmetrical muscle atrophy: mainly distal
Sensory disturbance  Peroneal muscles → “Champagne Bottle”
 Paraesthesia in extremities  Hand and arm muscles → “Claw Hand”
 Sensory ataxia in Miller-Fisher  Pes cavus (high-arched feet)

Autonomic Neuropathy Sensory

 Arrhythmias, ↑ HR  Variable loss of sensation in a stocking distribution
 Labile BP  Neuropathic pain in some
 Sweating
 Urinary retention Ix
 Genetic tests: PMP22 gene mutation
Immune  Nerve conduction studies: ↓ conduction speed in CMT1
 Serology for anti-ganglioside Abs
 Evidence of infection: e.g. stool sample Mx
 Supportive
Demyelinating Nerve Conduction Studies  Physio
 Slow conduction velocities  Podiatry
 Orthoses: e.g. ankle braces
Protein in CSF
 Protein often > 5.5g/L
 Normal white cell count

Mx
Supportive
 Airway / ventilation: ITU if FVC < 1.5L
 Analgesia: NSAIDs, gabapentin
 Autonomic: may need inotropes, catheter
 Antithrombotic: TEDS, LMWH

Immunosuppression
 IVIg
 Plasma exchange

Physiotherapy
 Prevent flexion contractures

Prognosis
 85% complete recovery
 10% unable to walk alone at 1yr
 5% mortality
© Alasdair Scott, 2012 191
 Motor Neurone Disease
Characteristics
 Cluster of degenerative disease characterised by
axonal degeneration of neurones in the motor cortex,
CN nuclei and anterior horn cells.
 UM and LM neurones affected (cf. polyneuropathy)
 No sensory loss or sphincter disturbance (cf. MS)
 Never affects eye movements (cf. MG)
Epidemiology
 Prevalence: 6/100,000
 Sex: M>F=3:2
 Median age @ onset = 60yrs
 Often fatal in 2-4yrs
Classification
Amyotrophic Lateral Sclerosis: 50%
 Loss of motor neurones in cortex and anterior horn →
UMN signs and LMN wasting + fasciculation
Progressive Bulbar Palsy: 10%
 Only affects CN 9-12 → bulbar palsy
Progressive Muscular Atrophy: 10%
 Anterior horn cell lesion → LMN signs only
 Distal to proximal
 Better prognosis cf. ALS

Primary Lateral Sclerosis

Causes  Loss of Betz cells in motor cortex → mainly UMN signs
 Unknown
 Marked spastic leg weakness and pseudobulbar palsy
 ~10% familial: SOD1 mutation in 20% of those
 No cognitive decline
Ix
 Brain/cord MRI: exclude structural cause
 Cervical cord compression → myelopathy Bulbar Palsy
 Brainstem lesions  Diseases of nuclei of CN 9-12 in the medulla
 LP: exclude inflammatory cause  LMN lesions of tongue, talking and swallowing
 EMG: shows denervation  Signs
 Flaccid, fasciculating tongue
 Speech: quiet or nasal (“Donald Duck” speech)
Features  Normal / absent jaw jerk
 UMN: spasticity, ↑ reflexes, ↑ plantars  Loss of gag reflex
 LMN: wasting, fasciculation of tongue / abdo / thigh  Causes
 Speech or swallowing impairment  MND
 Fronto-temporal dementia  GBS
 MG
Diagnosis  Central pontine myelinolysis (CPM)
 MRI to exclude structural lesions
 LP to exclude inflammation
 EMG shows acute denervation Pseudobulbar / Corticobulbar Palsy
 Use Revised El Escorial Diagnostic Criteria  Commoner than bulbar palsy
 Bilateral lesions above mid-pons (e.g. corticobulbar
Mx tracts) → UMN lesions of swallowing and talking
 MDT: neurologist, physio, OT, dietician, specialist  CN motor nuclei have bilateral cortical
nurse, GP, family representation except lower half of CN7
 Discussion of end-of-life decisions  Signs
 E.g. Advanced directive  Spastic tongue
 DNAR  Slow tongue movements c̄ slow deliberate
speech: “hot-potato” speech
Specific  Brisk jaw jerk
 Riluzole: antiglutamatergic that prolongs life by ~3mo  Emotional incontinence
 Causes
Supportive  MS
 Drooling: propantheline or amitriptyline  MND
 Dysphagia: NG or PEG feeding  Stroke
 Respiratory failure: NIV  CPM
 Pain: analgesic ladder
 Spasticity: baclofen, botulinum

Prognosis
 Most die w/i 3yrs
 Bronchopneumonia and respiratory failure
 Worse prog: elderly, female, bulbar involvement
Polio
 RNA virus
 Affects anterior horn cells
 Fever, sore throat, myalgia
 0.1% develop paralytic polio
 Asymmetric LMN paralysis
 No sensory involvement
 May be confined to upper or lower limbs or both
 Respiratory muscle paralysis can → death

© Alasdair Scott, 2012 192

 Myopathies
Myopathy vs. Neuropathy Myotonic Dystrophy
Myopathy  AD Cl- channelopathy
 Gradual onset  Onset in 20s
 Symmetrical, proximal weakness: difficulty combing  Tonic muscle spasm (myotonia)
hair, climbing stairs, getting up from chairs
 Dystrophies usually affect specific muscle groups Presentation
 Preserved tendon reflexes  Face
 Myopathic facies: long, thin, expressionless
Neuropathy  Wasting of facial muscles and SCM
 Paraesthesia, bladder problems  Bilateral ptosis
 Distal weakness  Dysarthria: myotonia of tongue and pharynx
 Hands
Other Pointers  Myotonia: slow relaxation
 Rapid onset: neuropathy, or drug, toxic or metabolic  E.g. inability to release hand after shake
myopathy  Wasting and weakness of distal muscles +
 Fatiguability: MG, LEMS areflexia → wrist drop
 Spontaneous pain and tenderness @ rest: inflam  Percussion myotonia: percuss thenar eminence
myopathy → involuntary thumb flexion
 Pain on exercise: ischaemia or metabolic myopathy  Other
 Oddly firm muscles: pseudohypertrophy in muscular  Frontal balding
dystrophies  Cataracts
 Fasciculation: anterior horn cell or root disease  DM
 Cardiomyopathy, tachy- / brad-arrhythmias
 Dysphagia
Ix
 Testicular atrophy
 ESR, CK, AST, LDH
 EMG Rx
 No Rx for weakness
 Phenytoin may improve myotonia
Muscular Dystrophies  Caution c̄ GA: high risk of anaesthetic complications
 Group of genetic disease c̄ progressive degeneration
and weakness of specific muscle groups. Prognosis
 Most die in middle-age of intercurrent illness
DMD
 Commonest: 3/1000 male births
 X-linked recessive, 30% spontaneous → non-functional Acquired myopathies of late onset
dystrophin
 Usually part of systemic disease
 Presentation
 Hyperthyroidism, Cushing’s, ↑/↓Ca2+
 ~4yrs old
 Difficulty standing  Drugs: steroids, statins, EtOH
 Calf pseudohypertrophy
 Respiratory failure
 Ix: ↑↑CK Inflammatory Myopathies
 Prog: some survive > 20yrs  Inclusion body myositis
 Polymyositis
BMD  Dermatomyositis
 0.3/1000 male births
 X-linked recessive Inclusion Body Myositis
 Partially functioning dystrophin  Asymmetric weakness affecting distal and prox muscles
 Presents later, is less severe and has better prognosis  Early involvement of quads, ankle dorsiflexors
and wrist/finger flexors
Facioscapulohumeral MD (Landouzy-Dejerine)  → loss of grip strength and ↓dexterity
 Almost as common as DMD  Dysphagia is very common
 AD inheritance  Myalgia is relatively uncommon
 Presentation
 Onset @ 12-14yrs
 Difficulty puffing cheeks and raising arms above
head
 Signs
 Weakness of face, shoulders and upper arms
(often asymmetric c̄ deltoids spared)
 Winging of scapula
 Foot drop.
 Prog: <20% need wheelchair by 20yrs

© Alasdair Scott, 2012 193

 Myasthenia Gravis
Pathophysiology Associations
 Autoimmune disease mediated by Abs vs. nicotinic Ach  <50yrs MG is commoner in women and assoc. c̄ other
receptors. AI disease (DM, RA, Graves) and thymic hyperplasia.
 Interferes c̄ NM transmission via depletion of working  >50yrs MG is commoner in men and assoc. c̄ thymic
post-synaptic receptor sites atrophy or thymic tumour.

Presentation Treatment
 ↑ing muscular fatigue
 Extra-ocular: bilateral ptosis, diplopia Symptom Control
 Bulbar: voice deteriorates on counting to 50  Anticholinesterase: e.g. pyridostigmine.
 Face: myasthenic snarl on attempting to smile  Cholinergic SEs = SLUDGEM
 Neck: head droop
 Limb: asymmetric, prox. weakness Immunosuppression
 Normal tendon reflexes  Rx relapses c̄ pred
 Weakness worsened by pregnancy, infection, emotion,  Steroids may be combined c̄ azathioprine or
drugs (β-B, gent, opiates, tetracyclines) methotrexate

Investigations Thymectomy
 Tensilon Test  Consider if young onset and disease not control by
 Give edrophonium IV anticholinesterases
 +ve if power improves w/i 1min  Remission in 25%, benefit in further 50%.
 Anti-AChR Abs: ↑ in 90%, MuSK Abs
 EMG: ↓ response to a train of impulses Complications
 Respiratory function: ↓FVC
 Thymus CT Myasthenic Crisis
 TFTs  Weakness of respiratory muscles during relapse may
be lethal.
Differential of Muscle Fatigability  Monitor FVC: vent support if <20ml/kg
 Polymyositis  Plasmapheresis or IVIg
 SLE  Rx trigger for relapse (drugs, infection…)
 Botulism
Prognosis
 Relapsing or slow progression

Lambert-Eaton Myasthenic Syndrome (LEMS)

Pathophysiology
 Abs to VGCC ↓ influx of Ca2+ during presynaptic excitation →
↓ presynaptic ACh-vesicle fusion.

Causes
 Paraneoplastic: e.g SCLC
 Autoimmune

Presentation
 As for MG except: LEMS
 Leg weakness early (before eyes)
 Extra: Autonomic and areflexia
 Movement improves symptoms
 Small response to edrophonium
 Anti-VGCC Abs

Mx
 3,4-diaminopyridine or IVIg
 Do regular CXRs / HRCTs as symptoms my precede Ca by 4yrs

Botulism
 Botulinum toxin prevents ACh vesicle release
 Descending flaccid paralysis c̄ no sensory signs
 Anti-cholinergic effects: mydriasis, cycloplegia, n/v, dry mouth, constipation
 Rx: benpen + antiserum
© Alasdair Scott, 2012 194
Neurofibromatosis 1 – von Neurofibromatosis 2
Recklinghausen’s
Epidemiology
Epidemiology  AD inheritance (Chr 22), but 50% are de novo
 Prev: 1/35,000
 AD Chr 17
 Variable expression
 Prev: 1/2500 Signs
Café-au-lait spots
Features: CAFÉ NOIR
 Rare
 Fewer cf. NF1
Café-au-lait spots
 1st yr of life
Bilateral Vestibular Schwannomas
 ↑ in size and no. c̄ age
 Characteristic
 Adults: >6, >15mm across  Symptomatic by 20yrs
 SNHL is first sign, then tinnitus, vertigo
Axillary Freckling
 in skin folds Juvenile Posterior Subcapsular Lenticular Opacity
 Form of cataract
Fibromas, neuro-:
 Bilateral
 Subcutaneous
 Occur before other manifestations and may be useful
 Small, gelatinous, violaceous nodules
for screening those @ risk
 Appear @ puberty
 May itch
 Nos. ↑ c̄ age Complications
 Plexiform  Tender schwannomas of cranial and peripheral nerves
 Overgrowth of nerve trunk and overlying tissue and spinal nerve roots.
 Large cutaneous mass  Meningiomas: often multiple
 Complications  Gliomas
 Sarcomatous change
 Compression: Mx
 Nerve roots: weakness, pain, paraesthesia  Hearing tests from puberty in affected families
 GI: bleeds and obstruction  MRI brain if abnormality detected

Eye Prognosis
 Lisch nodules  Mean survival from Dx is 15yrs
 Brown/translucent iris hamartomas
 Use a slit lamp
 Optic N. glioma

Neoplasia
 CNS: meningioma, ependyoma, astrocytoma
 Phaeochromocytoma
 Chronic or acute myeloid leukaemia

Orthopaedic
 Kyphoscoliosis
 Sphenoid dysplasia

IQ↓ and Epilepsy

Renal
 RAS → ↑ BP

Mx
 MDT orchestrated by GP
 Yearly BP and cutaneous review
 Excise some neurofibromas
 Genetic counselling

Café-au-Lait Spots Differential

 NF
 McCune-Albright
 Multiple Lentigenes
 Urticaria Pigmentosa

© Alasdair Scott, 2012 195

Intrinsic Cord Disease Syringomyelia
Presentation Characteristics
 Painless  Syrinx: tubular cavity in central canal of the cervical cord.
 Early sphincter / erectile dysfunction  Onset @ 30yrs
 Bilateral motor and sensory disturbance below  Symptoms may be static for yrs but then worsen fast
lesion  e.g. on coughing, sneezing as ↑ pressure → extension
 Commonly located in cervical cord
Causes: DIVINITY  Syrinx expands ventrally affecting:
 Decussating spinothalamic neurones
Degenerative  Anterior horn cells
 MND  Corticospinal tracts

Developmental Causes
 Friedrich’s Ataxia  Blocked CSF circulation c̄ ↓ flow from posterior fossa
 Hereditary spastic paraparesis  Arnold-Chiari malformation (cerebellum herniates
through foramen magnum)
Infection  Masses
 Viral: HIV, HTLV-1  Spina bifida
 Syphilis: Tabes Dorsalis  2O to cord trauma, myelitis, cord tumours and AVMs

Vascular Infarction Cardinal Signs

 Aortic dissection/aneurysm 1. Dissociated Sensory Loss
 Thromboembolism  Absent pain and temperature → scars from burns
 Atheroma  Preserved touch, proprioception and vibration.
 Vasculitis: esp. PAN  Root distribution reflects syrinx location
 Usually upper limbs and chest: “cape”
NB. ant. spinal A. infarction → spinothalamic and 2. Wasting/weakness of hands ± Claw hand
corticospinal tract loss → bilat loss of pain/temp and 3. Loss of reflexes in upper limb
spastic paresis 4. Charcot Joints: shoulder and elbow
 = Beck’s Syndrome
Other Signs
Inflammation  UMN weakness in lower limbs c̄ upgoing plantars
 Demyelination: MS
 Horner’s syndrome
 Transverse myelitis
 Syringobulbia: cerebellar and lower CN signs
 Kyphoscoliosis
Neoplasia
 Glioma
 Ependymoma
Ix
 MRI spine
Injury
Surgery
Toxin / Nutrition  Decompression at the foramen magnum for Chiari mal
 B12 deficiency

sYringomyelia

Brown-Sequard Syndrome
 Hemi-cord lesion
 Ipsilateral loss of proprioception and vibration
sense
 Ipsilateral UMN weakness
 Contralateral loss of pain sensation

© Alasdair Scott, 2012 196

 Miscellaneous Conditions
Friedrich’s Ataxia
 Auto recessive progressive degeneration of DRGs,
spinocerebellar and corticospinal tracts and
cerebellar cells
 Mitochondrial disorder
 Onset in teenage years
 Assoc. c̄ HOCM and mild dementia

Presentation
 Pes cavus and scoliosis
 Bilateral cerebellar signs
 Ataxia
 Dysarthria
 Nystagmus
 Leg wasting + areflexia but extensor plantars
 Loss of lower limb proprioception and vibration
sense
 Optic atrophy
 Cardiac: HOCM → ESM + 4 heart sound
th

 DM → hyperglycaemia

Hereditary Spastic Paraparesis

 Lower limb spasticity
 Ataxia
 Extrapyramidal signs

Human T-lymphotropic Virus-1

 Retrovirus
 ↑ prevalence in Japan and Caribbean

Features
 Adult T cell leukaemia / lymphoma
 Tropical spastic paraplegia / HTLV myelopathy
 Slowly progressing spastic paraplegia
 Sensory loss and paraesthesia
 Bladder dysfunction

© Alasdair Scott, 2012 197

Patterns of Presentation Rheumatological Investigations
Monoarthritis Joint Aspiration
 Septic arthritis  The key investigation in a monoarthritis
 Crystal arthritis: gout, CPPD  Appearance
 Osteoarthritis  Send for
 Trauma: haemarthrosis  WCC
 Gram stain and culture
Oligoarthritis (≤5 joints)  Polarized light microscopy
 Crystal arthritis
 Psoriatic arthritis
 Reactive arthritis Bloods
 Ankylosing spondylitis  Basic: FBC, U+E, ESR, CRP, urate
 Osteoarthritis  Culture: septic arthritis
 Abs: RF, ANA, others
Polyarthritis (>5 joints)  HLA-B27
 Viral serology, urine chlamydia PCR: reactive arthritis
Symmetrical
 RA
 Osteoarthritis Radiology
 Viruses: Hep A,B,C, mumps  CXR: RA, SLE, Vasculitis, TB, Sarcoid
 US/MRI: more sensitive for synovitis, enthesitis, infection
Asymmetrical
 Reactive arthritis Osteoarthritis
 Psoriatic arthritis  Loss of joint space
 Osteophytes
Either  Subchondral cysts
 Systemic disease: SLE, sarcoid, endocarditis  Subchondral sclerosis

Rheumatoid Arthritis
 Loss of joint space
 Soft tissue swelling
 Peri-articular osteopenia
 Deformity
 Subluxation

Gout
 Normal joint space
 Soft tissue swelling
 Periarticular erosions

© Alasdair Scott, 2012 199

 Back Pain
Red Flags Ix
 Age <20 or >55yrs  Usually only necessary if red flags present
 Neurological disturbance (inc. sciatica)  FBC, ESR, CRP, ALP, se electrophoresis, PSA
 Sphincter disturbance  MRI
 Bilateral or alternating leg pain
 Current or recent infection Mx
 Fever, wt. loss, night sweats  Neurosurgical referral if neurology
 History of malignancy  Conservative
 Thoracic back pain  Max 2d bed rest
 Morning stiffness  Education: keep active, how to lift / stoop
 Acute onset in elderly people  Physiotherapy
 Constant or progressive pain  Psychosocial issues re. chronic pain and disability
 Nocturnal pain  Warmth
 Medical
 Analgesia: paracetamol ± NSAIDs ± codeine
 Muscle relaxant: low-dose diazepam (short-term)
Causes  Facet joint injections
 Mechanical
 Surgical
 Strain/idiopathic
 Decompression
 Trauma
 Prolapse surgery: e.g. microdiscectomy
 Pregnancy
 Disc prolapse
 Spondylolisthesis (forward shift of one vertebra)
 Degenerative: spondylosis, vertebral collapse, stenosis Neurosurgical Emergencies
 Inflammatory: Ank spond, Paget’s
 Neoplasm: Mets, myeloma Acute Cord Compression
 Infection: TB, abscess  Bilateral pain: back and radicular
 LMN signs at compression level
 UMN signs and sensory level below compression
 Sphincter disturbance
Nerve Root Lesions
Acute Cauda Equina Compression
Root Weakness Reflex
 Alternating or bilateral radicular pain in the legs
L2 Hip flexion + adduction
 Saddle anaesthesia
L3 Knee extension Knee Jerk
 Loss of anal tone
Hip adduction
 Bladder ± bowel incontinence
L4 Foot inversion + dorsiflexion Knee Jerk
Knee extension
Rx
L5 Great toe dorsiflexion
 Large prolapse: laminectomy / discectomy
Foot inversion + dorsiflexion
Knee Flexion  Tumours: radiotherapy and steroids
Hip extension + abduction  Abscesses: decompression
S1 Foot eversion Ankle Jerk
Foot and toe plantarflexion
Knee flexion

© Alasdair Scott, 2012 200

Osteoarthritis Septic Arthritis
Definition Pathophysiology
 Degenerative joint disorder in which there is  Source: local or haematogenous.
progressive loss of hyaline cartilage.  Organisms
 Staph: commonest overall (60%)
Aetiology / Risk Factors  Gonococcus: commonest in young sexually active
 Age (80% > 75yrs)  Streps
 Obesity  Gm-ve bacilli
 Joint abnormality  RFs
 Joint disease (e.g. RA)
Classification  CRF
 Immunosuppression (e.g. DM)
 Primary: no underlying cause
 Prosthetic joints
 Secondary: obesity, joint abnormality
Symptoms
Symptoms
 Acutely inflamed tender, swollen joint
 Affects: knees, hips, DIPs, PIPs, thumb CMC
 ↓ROM
 Pain: worse c̄ movement, background rest/night pain,
 Systemically unwell
worse @ end of day.
 Stiffness: especially after rest, lasts ~30min (e.g. AM)
Investigations
 Deformity
 Joint aspiration for MCS
 ↓ ROM
 ↑↑ WCC (e.g. >50,000/mm3): mostly PMN
 ↑ESR/CRP, ↑WCC, Blood cultures
Signs  X-ray
 Bouchard’s (prox), Heberden’s (dist.) nodes
 Thumb CMC squaring
Management
 Fixed flexion deformity
 IV Abx: vanc + cefotaxime
 Consider joint washout under GA
Hx and Ix  Splint joint
 Focus on ADLs and social circumstances
 Physiotherapy after infection resolved
 X-ray
Complications
Differential  Osteomyelitis
 Septic
 Arthritis
 Crystal
 Ankylosis: fusion
 Trauma
Differential
Mx  Crystal arthropathy
 Reactive arthritis
Conservative
 ↓ wt.
 Alter activities: ↑ rest, ↓ sport
 Physio: muscle strengthening
 Walking aids, supportive footwear, home mods

Medical
 Analgesia
 Paracetamol
 NSAIDs: e.g. arthrotec (diclofenac + misoprostol)
 But misoprostol → diarrhoea
 Tramol
 Joint injection: local anaesthetic and steroids

Surgical
 Arthroscopic washout: esp. knee.
 Trim cartilage, remove foreign bodies.
 Arthroplasty: replacement (or excision)
 Osteotomy: small area of bone cut out.
 Arthrodesis: last resort for pain management
 Novel Techniques
 Microfracture: stem cell release → fibro-cartilage
formation
 Autologous chondrocyte implantation

© Alasdair Scott, 2012 201

 Rheumatoid Arthritis
Definition Ix
 Chronic systemic inflammatory disease characterised by  Bloods: FBC (anaemia, ↓PMN, ↑plat), ↑ESR, ↑CRP
a symmetrical, deforming, peripheral polyarthritis.  RF +ve in 70%
 High titre assoc. c̄ severe disease, erosions and
Epidemiology extra-articular disease
 Prev: 1% (↑ in smokers)  -ve = “seronegative rheumatoid”
 Sex: F>M=2:1  Anti-CCP: 98% specific (Ag derived from collagen)
 Age: 5th-6th decade  ANA: +ve in 30%
 Genetics: HLA-DR4/DR1 linked  Radiography, US, MRI

Features: ANTI CCP Or RF Mx

Conservative
Arthritis  Refer to rheumatologist
 Symmetrical, polyarthritis of MCPs, PIPs of hands and  Regular exercise
feet → pain, swelling, deformity  PT
1. Swan neck  OT: aids, splints
2. Boutonniere Medical
3. Z-thumb  DAS28: Monitor disease activity
4. Ulnar deviation of the fingers  DMARDs and biologicals: use early
5. Dorsal subluxation of ulnar styloid  Steroids: IM, PO or intra-articular for exacerbations
 Morning stiffness >1h  Avoid giving until seen by rheumatologist
 Improves c̄ exercise  NSAIDs: good for symptom relief
 Larger joints may become involved  Mx CV risk: RA accelerates atherosclerosis
 Prevent osteoporosis and gastric ulcers
Nodules Surgical
 Commonly elbows also fingers, feet, heal  Ulna stylectomy
 Firm, non-tender, mobile or fixed  Joint prosthesis
 Lungs
DMARDs
Tenosynovitis  1st line for treating RA
 De Quervain’s  Early DMARD use assoc. c̄ better long-term outcome
 Atlanto-axial subluxation
 All DMARDs can → myelosuppression → pancytopenia
Immune
Main agents
 AIHA
 Methorexate: hepatotoxic, pulmonary fibrosis
 Vasculitis
 Sulfasalazine: hepatotoxic, SJS, ↓ sperm count
 Amyloid
 Hydroxychloroquine: retinopathy, seizures
 Lymphadenopathy
Other Agents
 Leflunomide: ↑ risk of infection and malignancy
Cardiac: pericarditis + pericardial effusion
 Gold: nephrotic syndrome
Carpal Tunnel Syndrome  Penicillamine: drug-induced lupus, taste change

Pulmonary Biologicals
 Fibrosing alveolitis (lower zones) Anti-TNF
 Pleural effusions (exudates)  Severe RA not responding to DMARDs
 Screen and Rx TB first
Ophthalmic  Infliximab: chimeric anti-TNF Ab
 Epi-/scleritis  Etanercept: TNF-receptor
 2 Sjogren’s Syndrome
O
 Adalimumab: human anti-TNF Ab
 SEs: ↑ infection (sepsis, TB), ↑ AI disease, ↑ Ca
Raynaud’s
Rituximab (anti-CD20 mAb)
Felty’s Syndrome  severe RA not responding to anti-TNF therapy
 RA + splenomegaly + neutropenia
 Splenomegaly alone in 5%, Felty’s in 1% Anatomy of Rheumatoid Hands
 Boutonierre’s: rupture of central slip of extensor
Dx – 4/7 of: expansion → PIPJ prolapse through “button-hole”
1. Morning stiffness >1h (lasting >6wks) created by the two lateral slips.
2. Arthritis ≥3 joints  Swan: rupture of lateral slips → PIPJ hyper-extension
3. Arthritis of hand joints
4. Symmetrical Differential of Rheumatoid Hands
5. Rheumatoid nodules
 Psoriatic arthritis: nail changes and plaques
6. +ve RF
 Jaccoud’s arthropathy: reducible in extension
7. Radiographic changes
 Chronic crystal arthritis
© Alasdair Scott, 2012 202
Gout Ca Pyrophosphate Dehydrate
Arthropathy
Pathophysiology  Pseudogout / Chondrocalcinosis
 Deposition of monosodium urate crystals in and around
joints → erosive arthritis
 May be ppted by surgery, infection, fasting or diuretics Acute CPPD
 Presents as acute monoarthropathy
Presentation  Usually knee, wrist or hip
 M>F=5:1  Usually spontaneous and self-limiting
 Acute monoarthritis c̄ severe joint inflammation
 ~60% occur @ great toe MTP = Podagra
 Also: ankle, foot, hand joints, wrist, elbow, knee Chronic CPPD
 Also: asymmetric oligoarthritis  Destructive changes like OA
 Urate deposits in pinna and tendons = Tophi  Can present as poly-arthritis (pseudo-rheumatoid)
 Renal disease: radiolucent stones and interstitial
nephritis
Risk Factors
Differential  ↑ age
 Septic arthritis  OA
 Pseudogout  DM
 Haemarthrosis  Hypothyroidism
 Hyperparathyroidism
Causes  Hereditary haemochromatosis
 Hereditary  Wilson’s disease
 Drugs: diuretics, NSAIDs, cytotoxics, pyrazinamide
 ↓ excretion: 1O gout, renal impairment
 ↑ cell turnover: lymphoma, leukaemia, psoriasis, Ix
haemolysis, tumour lysis syndrome  Polarized light microscopy
 EtOH excess  Positively birefringent rhomboid-shaped crystals
 Purine rich foods: beef, pork, lamb, seafood  X-ray may show chondrocalcinosis
 Soft-tissue Ca deposition (e.g. knee cartilage)
Associations
 Check for:
 HTN Rx
 IHD  Analgesia
 Metabolic syndrome  NSAIDs
 May try steroids: PO, IM or intra-articular
Ix
 Polarised light microscopy
 Negatively birefringent needle-shaped crystals
 ↑ serum urate (may be normal)
 X-ray changes occur late
 Punched-out erosions in juxta-articular bone
 ↓ joint space

Acute Rx
 NSAID: diclofenac or indomethacin
 Colchicine
 NSAIDs CI: warfarin, PUD, HF, CRF
 SE: diarrhoea
 In renal impairment: NSAIDs and colchicine are CI
 → Use steroids

Prevention
 Conservative
 Lose wt.
 Avoid prolonged fasts and EtOH excess
 Xanthine Oxidase Inhibitors: Allopurinol
 Use if recurrent attacks, tophi or renal stones
 Introduce c̄ NSAID or colchicine cover for 3/12
 SE: rash, fever, ↓WCC (c̄ azathioprine)
 Use febuxostat (XO inhibitor) if hypersensitivity
 Uricosuric drugs: e.g. probenecid, losartan
 Rarely used
 Recombinant urate oxidase: rasburicase
 May be used pre-cytotoxic therapy
© Alasdair Scott, 2012 203
 Seronegative Spondyloarthropathies
Definition Psoriatic Arthritis
 Group of inflammatory arthritidies affecting the spine and  Develops in 10-40% and may predate skin disease
peripheral joints w/o production of RFs and associated
c̄ HLA-B27 allele. Patterns of joint involvement
 Asymmetrical oligoarthritis: 60% (commonest)
 Distal arthritis of the DIP joints: 15% (classical)
Common Features  Symmetrical polyarthritis: 15% (like RA but c̄ DIPJs)
 Axial arthritis and sacroiliitis  Arthritis mutilans (rare, ~3%)
 Asymmetrical large-joint oligoarthritis or monoarthritis  Spinal (like AS)
 Enthesitis
 Dactylitis Other Features
 Extra-articular: iritis, psoriaform rashes, oral ulcers,  Psoriatic plaques
aortic regurg, IBD  Nail changes
 Pitting
 Subungual hyperkeratosis
Ankylosing Spondylitis  Onchyolysis
 Chronic disease of unknown aetiology characterised by  Enthesitis: Achilles tendonitis, plantar fasciitis
stiffening and inflammation of the spine and sacroiliac  Dactylitis
joints.
X-Ray
Epidemiology  Erosion → “pencil-in-cup” deformity
 Sex: M>F=6:1
 Age: men present earlier – late teens, early 20s Rx
 Genetics: 95% are HLA-B27+ve  NSAIDs
 Sulfasalazine, methotrexate, ciclosporin
Presentation  Anti-TNF
 Gradual onset back pain
 Radiates from SI joints to hips and buttocks
 Worse @ night c̄ morning stiffness Reactive Arthritis
 Relieved by exercise.  Sterile arthritis 1-4wks after urethritis or dysentery
 Progressive loss of all spinal movements  Urethritis: chlamydia, ureaplasma
 Schober’s test <5cm  Dysentery: campy, salmonella, shigella, yersinia
 Some develop thoracic kyphosis and neck
hyperextension = question mark posture Presentation
 Enthesitis: Achilles tendonitis, plantar fasciitis  Asymmetrical lower limb oligoarthritis: esp. knee
 Costochondritis  Iritis, conjunctivitis
 Keratoderma blenorrhagica: plaques on soles/palms
Extra-articular manifestations  Circinate balanitis: painless serpiginous penile ulceration
 Osteoporosis: 60%  Enthesitis
 Acute iritis / anterior uveitis: 30%  Mouth ulcers
 Aortic valve incompetence: <3%
 Apical pulmonary fibrosis Ix
 ↑ESR, ↑CRP
Ix  Stool culture if diarrhoea
 Clinical Dx as radiological changes appear late  Urine chlamydia PCR
 Sacroliliitis: irregularities, sclerosis, erosions
 Vertebra: corner erosions, squaring Mx
syndesmophytes (bony proliferations)  NSAIDs and local steroids
 Bamboo spine: calcification of ligaments,  Relapse may need sulfasalazine or methotrexate
periosteal bone formation
 FBC (anaemia), ↑ESR, ↑CRP, HLA-B27
 DEXA scan and CXR
Enteropathic Arthritis
Mx  Occurs in 15% of pts. c̄ UC or Crohn’s
 Conservative
 Exercise Presentation
 Physio  Asymmetrical large joint oligoarthritis mainly affecting
 Medical the lower limbs.
 NSAIDs: e.g. indomethacin  Sacroiliitis may occur
 Anti-TNF: if severe
 Local steroid injections Rx
 Bisphosphonates  Treat the IBD
 Surgical  NSAIDs or articular steroids for arthritis
 Hip replacement to ↓ pain and ↑ mobility  Colectomy → remission in UC

© Alasdair Scott, 2012 204

 Autoimmune Connective Tissue Disease
Types Sjogren’s Syndrome (= Keratoconjunctivitis Sicca)
 SLE
 Systemic Sclerosis Classification
 1O Sjogren’s  Primary
 Myositis  F>>M=9:1
 Mixed Connective Tissue Disease  Onset 4th-5th decade
 Relapsing Polychondritis  Secondary
 Behcet’s Disease  RA
 SLE
 Systemic Sclerosis
Autoantibodies
Features
RF
 ↓ tear production → dry eyes
 Sjogren’s 100%
 ↓ salivation → xerostomia
 Felty’s 100%
 Bilateral parotid swelling
 RA 70%
 Vaginal dryness → dyspareunia
ANA  Systemic
 SLE >95%  Polyarthritis
 Raynaud’s
 AIH 75%
 Bibasal pulmonary fibrosis
 Sjogren’s 70%
 Vasculitis
 Myositis
dsDNA SLE
Histone Drug-induced lupus
Associations
Centromere CREST Syndrome
 AI: thyroid disease, AIH, PBC
ENAs
SLE, Sjogren’s, heart block  Marginal zone MALT lymphoma
- Ro
- La SLE, Sjogren’s
- Sm SLE Ix
- RNP SLE, MCTD  Schirmer tear test
- Jo-1 Polymyositis, Dermatomyositis  Abs: ANA – Ro and La, RF
- Scl70 Diffuse Systemic Sclerosis  Hypergammaglobulinaemia
- RNA pol 1/2/3 Diffuse Systemic Sclerosis  Parotid biopsy

Relapsing Polychondritis Rx
 Rare inflammatory disease of cartilage  Artificial tears
 Saliva replacement solutions
Presentation  NSAIDs and hydroxychloroquine for arthralgia
 Tenderness, inflammation and destruction of cartilage  Immunosuppression for severe systemic disease
 Ear → floppy ears
 Nose → saddle-nose deformity
 Larynx → stridor Raynaud’s Phenomenon
 Peripheral digital ischaemia precipitated by cold or
Associations emotion.
 Aortic valve disease
 Polyarthritis Classification
 Vasculitis  Idiopathic / 1O: Raynaud’s Disease
 Secondary: Raynaud’s Syndrome
Rx  Systemic sclerosis, SLE, RA, Sjogren’s
 Immunosuppression  Thrombocytosis, PV
 β-blockers
Behcet’s Disease
Presentation
 Systemic vasculitis of unknown cause
 Digit pain + triphasic colour change: WBC
 White, Blue, Crimson
Presentation
 Digital ulceration and gangrene
 Turks, Mediterraneans and Japanese
 Recurrent oral and genital ulceration Rx
 Eyes: ant/post uveitis  Wear gloves
 Skin lesions: EN  CCBs: nifedipine
 Vasculitis  ACEi
 Joints: non-erosive large joint oligoarthropathy  IV prostacyclin
 Neuro: CN palsies
 GI: diarrhoea, colitis
MCTD / UCTD
Ix: skin pathergy test (needle prick → papule formation)  Combined features of SLE, PM, RA, SS
Rx: immunosuppression  Ab: RNP
© Alasdair Scott, 2012 205
Systemic Sclerosis Polymyositis and Dermatomyositis
 Striated muscle inflammation
Epidemiology
 F>M=3:1 Principal Features
 30-50yrs  Progressive symmetrical proximal muscle weakness.
 Wasting of shoulder and pelvic girdle
Limited Systemic Sclerosis: 70%  Dysphagia, dysphonia, respiratory weakness
(includes CREST syndrome)  Assoc. myalgia and arthralgia
 Calcinosis  Commoner in females
 Raynaud’s  Often a paraneoplastic phenomenon
 Esophageal and gut dysmotility → GOR  Lung, pancreas, ovarian, bowel
 Sclerodactyly
 Telangiectasia Dermatomyositis = myositis + skin signs
 Skin involvement limited to face, hands and feet  Heliotrope rash on eyelids ± oedema
 Beak nose  Macular rash (shawl sign +ve: over back and shoulders)
 Microstomia  Nailfold erythema
 Pulmonary HTN in 15%  Gottron’s papules: knuckles, elbows, knees
 Mechanics hands: painful, rough skin cracking of finger
Diffuse Systemic Sclerosis: 30% tips
 Diffuse skin involvement  Retinopathy: haemorrhages and cotton wool spots
 Organ fibrosis  Subcutaneous calcifications
 GI: GOR, aspiration, dysphagia, anal incontinence
 Lung: fibrosis and PHT Extra-Muscular Features
 Cardiac: arrhythmias and conduction defects  Fever
 Renal: acute hypertensive crisis (commonest  Arthritis
cause of death)  Bibasal pulmonary fibrosis
 Raynaud’s phenomenon
Ix  Myocardial involvement: myocarditis, arrhythmias
 Bloods: FBC (anaemia), U+E (renal impairment)
 Abs Ix
 Centromere: limited  Muscle enzymes: ↑CK, ↑AST, ↑ALT, ↑LDH
 Scl70 / topoisomerase: diffuse  Abs: Anti-Jo1 (assoc. c̄ extra-muscular features)
 RNA pol 1,2,3: diffuse
 EMG
 Urine: stix, PCR
 Muscle biopsy
 Imaging
 Screen for malignancy
 CXR: cardiomegaly, bibasal fibrosis
 Tumour markers
 Hands: calcinosis
 CXR
 Ba swallow: impaired oesophageal motility
 Mammogram
 HiRes CT
 Pelvic/abdo US
 Echo
 CT
 ECG + Echo: evidence of pulmonary HTN
Differential
Mx
 Inclusion body myositis
 Conservative
 Muscular dystrophy
 Exercise and skin lubricants: ↓ contractures
 Polymyalgia rheumatica
 Hand warmers: Raynaud’s
 Endocrine / metabolic myopathy
 Medical
 Immunosuppression  Drugs: steroids, statins, colchicine, fibrates
 Raynaud’s: CCBs, ACEi, IV prostacyclin
 Renal: intensive BP control – ACEi 1st line Mx
 Oesophageal: PPIs, prokinetics (metoclopramide)  Screen for malignancy
 PHT: sildenafil, bosentan  Immunosuppression
 Steroids
 Cytotoxics: azathioprine, methotrexate

© Alasdair Scott, 2012 206

 Systemic Lupus Erythematosus
Definition Drug-Induced Lupus
 Multisystemic autoimmune inflammatory disease in  Causes: procainamide, phenytoin, hydralazine, isoniazid
which autoAbs to a variety of autoantigens result in the  Anti-histone Abs in 100%
formation and deposition of immune complexes.  Mostly skin and lung signs
 Disease remits if drug stopped

Epidemiology
 Prev: 0.2% Anti-Phospholipid Syndrome
 Sex: F>>M=9:1
 Age: child-bearing age Classification
 Genetic: ↑ in Afro-Caribs and Asians  Primary: 70%
 Secondary to SLE: 30%

Features Pathology
 Relapsing, remitting history  Anti-phospholipid Abs: anti-cardiolipin and lupus
 Constitutional symptoms: fatigue, wt. loss, fever, myalgia anticoagulant

A RASH POINts an MD Features

 Arthritis  CLOTs: venous (e.g. DVT) and arterial (e.g. stroke)
 Non-erosive, involving peripheral joints  Coagulation defect: ↑APTT
 Jaccoud’s: reducible deforming arthropathy  Livido reticularis
 Obstetric complications: recurrent 1 trimester abortion
st
 Renal
 proteinuria and ↑BP  Thrombocyotpenia
 ANA (+ve in 95%)
 Serositis Rx
 Pleuritis: pleuritic pain, dyspnoea, effusion  Low-dose aspirin
 Pericarditis: chest pain relieved by sitting forwards  Warfarin if recurrent thromboses: INR 3.5
 Haematological
 AIHA
 ↓WCC Mx
 ↓Plats  In specialist SLE and lupus nephritis clinics
 Photosensitivity
 Oral ulcers Severe Flares: Acute SLE
 Immune phenomenon  AIHA, nephritis, pericarditis or CNS disease
 Anti-dsDNA  High-dose prednisolone + IV cyclophosphamide
 Anti-Sm
 Anti-phospholipid Cutaneous Symptoms
 Neurological  Treat c̄ topical steroids and prevent c̄ sun cream
 Seizures, psychosis
 Malar Rash Maintenance
 Facial erythema sparing the nasolabial folds  For joint and skin symptoms
 Discoid Rash  NSAIDs and hydroxychloroquine ± low-dose steroids
 Erythema → pigmented hyperkeratotic papules →
atrophic depressed lesions Lupus Nephritis
 Mainly affects face and chest  Proteinuria: ACEi
 Aggressive GN: immunosuppression

Immunology Rx Complications
 95% ANA+ve  ↑ risk of osteoporosis and CV disease
 dsDNA is very specific (sensitivity 60%)
 30% ENA+ve: Ro, La, Sm, RNP
 Anticardiolipin Abs → false +ve syphilis serology Prognosis
 80% survival @ 15yrs

Monitoring Disease Activity

 Anti-dsDNA titres
 Complement: ↓C3, ↓C4
 ↑ESR

Other Ix
 Bloods: FBC, U+E, CRP, clotting (usually normal)
 Urine: stix, PCR

© Alasdair Scott, 2012 207

 Vasculitis
Classification Takayasu’s Arteritis = Pulseless Disease
Large Vessel Epidemiology
 Giant Cell Arteritis  Geo: Rare outside Japan
 Takayasu’s Arteritis  Sex: F>M
 Age: 20-40yrs
Medium Vessel
 Polyarteritis Nodosa Features
 Kawasaki Disease  Constitutional symptoms: fever, fatigue, wt. loss
 Weak pulses in upper extremities
Small Vessel  Visual disturbance
 pANCA  HTN
 Churg-Straus
 Microscopic Polyangiitis
 cANCA: Wegener’s Granulomatosis Polyarteritis Nodosa
 ANCA –ve
 Henoch-Schonlein Purpura Epidemiology
 Goodpasture’s Disease
 Prev: rare in UK
 Cryoglobulinaemia
 Sex: M>F=2:1
 Cutaneous Leukocytoclastic Vasculitis
 Age: young adults
Giant Cell Arteritis / Temporal Arteritis Features
 Common in elderly (rare <55)  Assoc. c̄ Hep B
 Assoc. c̄ PMR in 50%
 Constitutional symptoms
 Rash
Features
 Renal → HTN
 Systemic signs: fever, malaise, fatigue
 GIT → melaena and abdo pain
 Headache
 Temporal artery and scalp tenderness Rx
 Jaw claudication  Pred + cyclophosphamide
 Amaurosis fugax
 Prominent temporal arteries ± pulsation

Mx and Ix Kawasaki’s Disease

 If suspect GCA: Do ESR and start pred 40-60mg/d PO  Childhood PAN variant
 ↑↑ESR and CRP
Features
 ↑ALP
 5-day fever
 ↓Hb (normo normo), ↑Plats
 Bilat non-purulent conjunctivitis
 Temporal artery biopsy w/i 3d: but skip lesion occur
 Oral mucositis
Continuing Rx  Cervical lymphadenopathy
 Taper steroids gradually, guided by symptoms and ESR  Polymorphic rash (esp. trunk)
 PPI and alendronate cover (~2yr course usually)  Extremity changes (erythema + desquamation)
 Coronary artery aneurysms
PMR
Rx
Presentation  IVIg + aspirin
 >50yrs old
 Severe pain and stiffness in shoulders, neck and hips
 Sudden / subacute onset
 Symmetrical
 Worse in the morning: stops pt. getting out of bed
 No weakness (cf. myopathy or myositis)
 ± mild polyarthritis, tenosynovitis and carpal tunnel syn.
 Systemic signs: fatigue, fever, wt. loss
 15% develop GCA

Ix
 ↑↑ESR and CRP (+ ↑plasma viscosity)
 ↑ALP
 Normal CK

Rx
 Pred 15mg/d PO: taper according to symptoms and ESR
 PPI and alendronate cover (~2yr course usually)
© Alasdair Scott, 2012 208
ANCA Positive Vasculitidies ANCA Negative Vasculitidies
Wegener’s Granulomatosis HSP (Childhood IgA nephropathy variant)
 Necrotizing granulomatous inflammation and small vessel  Children 3-8yrs
vasculitis c̄ a predilection for URT, LRT and Kidneys  Post-URTI
 Palpable purpura on buttocks
Features  Colicky abdo pain
URT  Arthralgia
 Chronic sinusitis  Haematuria
 Epistaxis
 Saddle-nose deformity
Goodpastures
LRT
 Anti-GBM Abs
 Cough
 RPGN
 Haemoptysis
 Haemoptysis
 Pleuritis
 CXR: Bilat lower zone infiltrates (haemorrhage)
Renal
Rx
 RPGN
 Immunosuppresion + plasmapheresis
 Haematuria and proteinuria

Other
 Palpable purpura Cryoglobulinaemia
 Ocular: conjunctivitis, keratitis, uveitis
Simple
Ix  Monoclonal IgM
 cANCA  2O to myeloma / CLL / Waldenstroms
 Dipstick: haematuria and proteinuria  → Hyperviscosity
 CXR: bilat nodular and cavity infiltrates  Visual disturbance
 Bleeding from mucus mems
 Thrombosis
 Headache, seizures
Churg-Strauss
 Late-onset asthma
Mixed (80%)
 Eosinophilia
 Polyclonal IgM
 Granulomatous small-vessel vasculitis
 2 to SLE, Sjog, HCV, Mycoplasma
O
 RPGN
 → Immune complex disease
 Palpable purpura
 GN
 GIT bleeding
 Palpable purpura
 pANCA
 Arthralgia
 May be ssoc. c̄ montelukast  Peripheral neuropathy

Microscopic Polyangiitis Cutaneous Leukocytoclastic Vasculitis

 RPGN  Palpable purpuric rash
 Haemoptysis  ± arthralgia ± GN
 Palpable purpura  Causes
 pANCA  HCV
 Drugs: sulphonamides, penicillin

© Alasdair Scott, 2012 209

Fibromyalgia
Epidemiology
 10% of new rheum referrals
 Prev: 0.5-4%
 Sex: F>>M=10:1

Risk Factors
 Neurosis: depression, anxiety, stress
 Dissatisfaction at work
 Overprotective family or lack of support
 Middle age
 Low income
 Divorced
 Low educational status

Associations
 Chronic fatigue syndrome
 Irritable bowel syndrome
 Chronic headache syndromes

Features
 Chronic, widespread musculoskeletal pain and
tenderness
 Morning stiffness
 Fatigue
 Poor concentration
 Sleep disturbance
 Low mood

Ix
 All normal
 Rule out organic cause: FBC, ESR, CRP, CK, TFTs, Ca

Mx
 Educate pt.
 CBT
 Graded exercise programs
 Amitriptyline or pregabalin
 Venlafaxine

© Alasdair Scott, 2012 210

 Major Disease Patterns

?
Disease Biochemistry
Dehydration ↑↑ U, ↑Cr
↑albumin
↑HCT
Low GFR ↑U, ↑Cr, ↑H, ↑K, ↑urate
↑PO4, ↓Ca
Tubular Dysfunction Normal U and Cr
↓K, ↓urate, ↓PO4, ↓HCO3
Thiazide and Loop Diuretics ↓Na, ↓K, ↑HCO3, ↑U
Hepatocellular Disease EtOH: AST:ALT>2, ↑GGT
Viral: AST:ALT<2
↑bilirubin, ↑ALP, ↓albumin, ↑PT (↑APTT if end-stage)
Cholestasis ↑↑ALP, ↑↑GGT, ↑Bilirubin, ↑AST
Excess EtOH intake ↑GGT, ↑MCV, evidence of hepatocellular disease
Addison’s ↑K, ↓Na
Cushing’s May show: ↓K, ↑Na, ↑HCO3
Conn’s ↓K, ↑Na, ↑HCO3
DM ↑glucose
DI ↑Na, ↑ serum osmolality, ↓ urine osmolality
SIADH ↓Na, ↓ serum osmolality, ↑ urine osmolality (>500), ↑ urine Na

© Alasdair Scott, 2012 212

Hyponatraemia Hypernatraemia
Presentation Presentation
 <135: n/v, anorexia, malaise  Thirst
 <130: headache, confusion, irritability  Lethargy
 <125: seizures, non-cardiogenic pulmonary oedema  Weakness
 <115: coma and death  Irritability
 Confusion, fits, coma
Causes  Signs of dehydration

Hypovolaemic Causes
 U Na >20mM (= renal loss)  Usually caused by dehydration (↓ intake or ↑ loss)
 Diuretics
 Addison’s Hypovolaemic
 Osmolar diueresis (e.g. glucose)  GI loss: diarrhoea, vomiting
 Renal failure (diuretic phase)  Renal loss: diuretics, osmotic diuresis (e.g. DM)
 U Na >20mM (= extra-renal loss)  Skin: sweating, burns
 Diarrhoea
 Vomiting Euvolaemic
 Fistula  ↓ fluid intake
 SBO  DI
 Burns  Fever
Hypervolaemic Hypervolaemic
 Cadiac failure  Hyperaldosteronism (↑BP, ↓K, alkalosis)
 Nephrotic syndrome  Hypertonic saline
 Cirrhosis
 Renal failure
Mx
 Give water PO if possible
Euvolaemic
 Otherwise, 5% dextrose IV slowly
 U osmolality >500
 SIADH  Use 0.9% NS if hypovolaemic or Na >170mM
 Causes less marked fluid shifts
 U osmolality <500
 Water overload  Aim for Na ↓ ≤12mM/d
 Severe hypothyroidism  Too fast → cerebral oedema
 Glucocorticoid insufficiency
Free Water Deficit
 TBW (L) = coeff x wt.
Mx  Coeff = men:0.6, women:0.5
 Correct the underlying cause
 Def (L) = TBW x (1-[140/Na])
 Replace Na and water at the same rate they were lost
 Too fast → central pontine myelinolysis
 Chronic: 10mM/d Diabetes Insipidus
 Acute: 1mM/hr  Polyuria
 Asymptomatic chronic hyponatraemia  Polydipsia
 Fluid restrict  Dehydration
 Symptomatic / acute hyponatraemia / dehydrated
 Cautious rehydration with 0.9% NS Causes
 If hypervolaemic consider frusemide  Nephrogenic
 Emergency: seizures, coma  Inherited
 Consider hypertonic saline (e.g. 1.8%)  ↑Ca
 Drugs: Li, demeclocycline
 Post-obstructive uropathy
SIADH  Cranial
 Concentrated urine: Na >20mM, osmolality >500  Idiopathic
 Hyponatraemia or plasma osmolality <275  Congenital: DIDMOAD
 Absence of hypovolaemia, oedema or diuretics  Tumor
 Trauma
Causes  Vascular: haemorrhage
 Resp: SCLC, pneumonia, TB  Infection: meningoencephalitis
 CNS: meningoencephalitis, head injury, SAH
 Endo: hypothyroidism Ix
 Drugs: cyclophosphamide, SSRIs, CBZ  ↑Na
 Dilute urine
Rx  Dx: Water deprivation test
 Rx cause and fluid restrict
 Vasopressin receptor antagonists Rx
 Demeclocycline  Treat cause
 Vaptans  Desmopressin if cranial
© Alasdair Scott, 2012 213
Hypokalaemia Hyperkalaemia
Symptoms Symptoms
 Muscle weakness  Fast, irregular pulse
 Hypotonia  Palpitations
 Hyporeflexia  Chest pain
 Cramps  Weakness
 Tetany
 Palpitations
 Arrhythmias ECG
 Tall tented T waves
NB. ↓K exacerbates digoxin toxicity  Flattened P waves
 ↑ PR interval
 Widened QRS
ECG  Sine-wave pattern → VF
 Result from delayed ventricular repolarisation
 Flattened / inverted T waves
 Prominent U waves (after T waves) Causes
 ST depression
 Long PR interval Artefact
 Long QT interval  Haemolysis
 K2EDTA contamination from FBC bottle
 Leucocytosis, thrombocytosis
Causes  Drip arm

Internal Distribution Internal Distribution

 Alkalosis  Acidosis
 ↑ insulin  ↓ insulin
 β-agonists  Cell death / tissue trauma / burns
 Digoxin poisoning
↑ Excretion  Suxamethonium
 GI: vomiting, diarrhoea, rectal villous adenoma
 Renal: RTA (esp. type 2), Bartter syn. ↓ Excretion
 Drugs: diuretics, steroids  Oliguric renal failure
 Endo: Conn’s syn., Cushing’s syn.  Addison’s
 Drugs: ACEi, NSAIDs, K -sparing diuretics
+

↓ Input
 Inappropriate IV fluid management ↑ Input
 Excessive K therapy
 Massive transfusion
Mx
 1mM K ↓ = ~200-300mmol total deficit
 Don’t give K if oliguric Mx
 Never give STAT fast bolus
Non-urgent
Mild: K >2.5  Treat cause: review meds
 Oral K supplements  Polystyrene sulphonate resin (Calcium Resonium)
 ≥80mmol/d  Binds K in the GIT and ↓K over days

Severe: K <2.5 and/or dangerous symptoms Emergency: evidence of myocardial instability or K >6.5
 IV KCl cautiously  10ml 10% calcium gluconate
 10mmol/h (20mmol/h max)  100ml 20% glucose + 10u insulin (Actrapid)
 Best to give centrally (burning sensation peripherally)  Salbutamol 5mg nebulizer
 Max central conc: 60mM  Haemofiltration (usually needed if anuric)
 Max peripheral conc: 40mM  Calcium resonium 15g PO or 30g PR

Mg Replacement
 Pts. are often Mg deplete too
 Until Mg is replaced the K will not return to normal
levels despite K replacement
 Give empiric Mg replacement

© Alasdair Scott, 2012 214

Ca and PO4 Physiology Hypocalcaemia
PTH Presentation: SPASMODIC
 Overall: ↑Ca and ↓PO4  Spasms (carpopedal = Trousseau’s sign)
 ↓ ionised Ca → PTH release  Perioral paraesthesia
 Anxious, irritable
Renal  Seizures
 ↑ Ca reabsorption  Muscle tone ↑: colic, wheeze, dysphagia
 ↓ PO4 reabsorption  Orientation impaired (confusion)
 ↑ 1α-hydroxylation of 25-OH-Vit D3 in kidney  Dermatitis: atopic, exfoliative
 ↑ HCO3 excretion (may → mild met acidosis)  Impetigo herpetiformis (↓Ca + pustules in cyesis)
 Chovsteks, Cardiomyopathy (↑ QTc  TdP)
Bone  ↑ Ca → ↑threshold for action potential
 ↑ osteoclast activity → ↑Ca, ↑PO4
Causes
 Commonest cause is CRF
Vitamin D and Calcitriol
 Hepatic 25 hydroxylation → 25-OH-Vit D3 (Calcidiol) With ↑ PO4
 Renal 1α-hydroxylation in kidney → 1,25(OH)2 Vit D3  CKD
(Calcitriol)  Hypoparathyroidism / pseudoyhpoparathyroidism
 ↑ by ↑PTH, ↓Ca or ↓PO4  ↓ Mg
 Acute rhabdomyolysis (muscle Ca deposition)
GIT
 ↑Ca and ↑PO4 absorption With normal or ↓ PO4
 Inhibition of PTH release  Osteomalacia
 Active pancreatitis
Renal  Respiratory alkalosis
 ↑Ca and ↑PO4 reabsorption
Rx
Magnesium Mild
 ↓Mg prevents PTH release  Ca 5mmol QDS PO
 May → ↓Ca  Daily Ca levels

CKD
Plasma Binding  Alfacalcidol (1-OH-Vit D3)
 2.2-2.6mM
 50% albumin bound ( ↓Alb →↓Ca) Severe
 Labs measure total Ca  10ml 10% Ca gluconate IV (2.25mmol) over 30min
 cCa: ↑ Ca by 0.1mM for every 4g/L albumin↓  Repeat as necessary
below 40g/L
 Alkalosis → ↓albumin protonation → ↑Ca binding →
↓[Ca]

NB. Prolonged tourniquet application → ↑ albumin → ↑ Ca

 Take uncuffed specimen if concerned

© Alasdair Scott, 2012 215

Hypercalcaemia Osteoporosis
Presentation Definition
 Stones  ↓ bone mass
 Renal stones  1O: age-related
 Polyuria and polydipsia (nephrogenic DI)  2O: drugs or other condition
 Nephrocalcinosis
 Bones Risk Factors: SHATTERED
 Bone pain  Steroids
 Pathological #s  Hyperthyroidism, HPT, HIV
 Moans: depression, confusion  Alcohol and Cigarettes
 Groans  Thin (BMI <22)
 Abdo pain  Testosterone Low
 n/v and constipation
 Early Menopause
 Pancreatitis
 Renal / liver failure
 PUD (↑gastrin secretion)
 Erosive / inflam bone disease (e.g. RA, myeloma)
 Other:
2+
 ↑ BP (check Ca in all with HTN)  Dietary Ca low / malabsorption
 ↓ QT interval
Presentation
 Vertebral crush #s
 # NOF and other long bone #s
Causes
 Most commonly malignancy or 1O HPT
Ix: Bone profile, FBC, U+E
With ↑ PO4
 ↑ ALP (e.g. ↑ bone turnover) DEXA Scan
 Bone mets  Indications
 thyroid, breast, lung, kidney, prostate, colon  Low-trauma #
 Sarcoidosis  Women ≥65yrs c̄ one or more risk factors
 Thyrotoxicosis  Before giving long-term steroids (>3mo)
 Lithium  Parathyroid disorders, myeloma, HIV
 Normal ALP  Interpretation
 Myeloma  T: no. of SDs away from youthful average
 Hypervitaminosis D  Z: no. of SDs away from matched average
 Sarcoidosis  T > -1 = normal
 Milk alkali syn.  T -1 - -2.5 = osteopenia
 T <-2.5 = osteoporosis
With normal or ↓ PO4
 1O or 3O HPT Rx
 Familial benign hypercalciuria  Decision to instigate pharmacological Rx is based
 AD upon age, RFs, and BMD.
 ↑ Ca-sensing receptor set-point  FRAX can estimate 10yr # risk
 Paraneoplastic: PTHrP (but ↓PTH)
Conservative
 Stop smoking, ↓ EtOH
Ix  Wt. bearing or balancing exercise (e.g. Tai Chi)
 ↑PTH = 1O or 3O HPT  Ca and vit-D rich diet
 ↓PTH: most likely Ca  Home-based fall-prevention program c̄ visual
 FBC, protein electrophoresis, CXR, bone scan assessment.

Primary and secondary prevention of #s

 Bisphosphonates: alendronate is 1 line
st
Mx  Ca and Vit D supplement’s: e.g Calcium D3 Forte
 Dx and Rx underlying cause
 Strontium ranelate: bisphosphonate alternative
Rehydrate
Alternative for 2O prevention of osteoporotic #s
 1L 0.9% NS / 4h
 Raloxifene: SERM, ↓ breast Ca risk cf. HRT
 Monitor pts. hydration state
 Teriparetide: PTH analogue → new bone formation
 Denosumab: anti-RANKL → ↓ osteoclast activation
Frusemide
 Only start once pt. is volume replete
Bisphosphonate SEs
 Calciuric + makes room for more fluids
 GI upset
 Oesophageal ulceration / erosion
Bisphosphonates
 Take c̄ plenty of water on an empty stomach
 Ca bisphosphonate can’t be resorbed by osteoclasts
and refrain from lying and don’t eat for 30min.
 Only used in hypercalcaemia of malignancy
 Diffuse musculoskeletal pain
 Can obscure Dx as → ↓Ca, ↓PO4 and ↑PTH
 Osteonecrosis of the jaw
 E.g. Pamidronate, Zoledronate (IV)
© Alasdair Scott, 2012 216
Osteomalacia Paget’s Disease / Osteitis Deformans
Definition Pathophysiology
 ↓ bone mineral content  Affects 3% over 55yrs
 Excess uncalcified osteoid and cartilage  ↑ bone turnover → bone remodelling, enlargement,
weakness and deformity.
Presentation  ↑ bone mass but disordered and weak
 Commoner in temperate climes and Anglo-Saxons
Rickets: children
 Knock-kneed / bow-legged 3 Phases
 Bone pain  Osteolytic
 Craniotabes  Mixed Osteolytic-osteoblastic
 Osteochondral swelling: rachitic rosary  Quiescent osteosclerotic stage
 Harrison’s sulcus
Presentation
Osteomalacia: adults (after epiphyseal fusion)  Asymptomatic in 70%
 Bone pain and tenderness  Predominantly affects the axial skeleton
 #s: esp. NOF  Pelvis, lumbar spine, skull, femur and tibia
 Proximal myopathy due to ↓PO4  Polyostotic in 85%
 Bone pain
Causes  Pathological #
 Vitamin D deficiency: malabsorption, poor diet, ↓sunlight  Deformity
 Renal osteodystrophy: ↓ 1α hydroxylation  Sabre tibia
 Drugs: AEDs → ↑ hepatic vit D metabolism  Head enlargement
 Vit D resistance: inherited conditions
 Type 1: ↓ renal hydroxylation Complications
 Type 2: end-organ resistance  Nerve compression: deafness, radiculopathy
 Hepatic disease: malabsorption and ↓ 25 hydroxylation  High output CCF
 Malignancy: oncogenic hypophosphataemia  Osteosarcoma (<1% after 10yrs)
 ↑ fibroblast growth factor-23 → hyperphosphaturia  Sudden onset or worsening of bone pain

Ix Ix
 ↓Ca, ↓PO4, ↑ALP, ↑PTH, ↓25-OH Vit D3 (unless resistance)  ↑↑ALP, (Ca and PO4 normal)
 X-ray  Bone scan: hot spots
 Loss of cortical bone  X-ray
 Looser’s zones: pseudofractures  Bone enlargement
 Cupped metaphyses in Rickets  Sclerosis
 Patchy cortical thickening
Rx  Deformity
 Wedge-shaped lytic lesions
 Dietary: Calcium D3 Forte
 Osteoporosis circumscripta
 Malabsorption or hepatic disease
 Well-defined lytic skull lesions
 Vit D2 (ergocalciferol) PO
 Parenteral calcitriol
 Renal disease or vit D resistance Mx
 1α-OH-Vit D3 (alfacalcidol)  Analgesia
 ↓1,25-(OH)2 Vit D3 (calcitriol)  Alendronate: ↓ pain and/or deformity
 Monitor plasma Ca

© Alasdair Scott, 2012 217

 Hyperlipidaemia
Biology Presentation
 Chylomicrons  CVD
 Mainly TG  Xanthomata
 Carry dietary TG tissues  Eruptive: itchy nodules in crops (↑TG)
 VLDL  Tuberous: plaques on elbows, knees
 Mainly TG  Planar: ornage streaks in palmer creases
 Take endogenous TG from liver → tissue  Remnant hyperlipidaemia
 LDL  Xanthelasma: eyelids
 Mainly cholesterol  Arcus: cornea
 Carry cholesterol from liver to tissues  Pancreatitis (↑TG)
 HDL
 Mainly phospholipid
 Take cholesterol from tissues → liver for excretion
 Apolipoproteins
Ix
 Contained in lipoproteins and control transport and  Plasma cholesterol
uptake.  Plasma HDL
 ApoB contained in LDL and binds to cell receptors  Fasting TGs
→ uptake  TC:HDL ratio is best predictor of CV risk

Classification Mx
 Common 1 hyperlipidaemia
O

 70% Aims
 Dietary and genetic factors  TC <4
 ↑ LDL only  TC:HDL ratio <4.5
 Familial primary hyperlipidaemia
 Multiple phenotypes Lifestyle
 ↑↑ risk of CVD  Lose wt.
 2O hyperlipidaemia  ↓ saturated fat, ↑ fibre, ↑ fruit and veg
 ↑ LDL  ↑ exercise
 Nephrotic syn.
 Cholestasis: lipoprotein x Treatment Priorities
 Hypothyroidism  Known CVD
 Cushing’s  Those c̄ DM
 Drugs: thiazides, steroids  Those c̄ 10yr CVD risk >20%
 Mixed: ↑LDL and ↑TG  Irrespective of baseline lipids
 T2DM
st
 EtOH 1 -line: Statins
 CRF  E.g. simvastatin 40mg PO nocte
 HMG-CoA reductase inhibitors → ↓ cholesterol synth.

Familial Primary Hyperlipidaemias 2nd-line:

 Fibrates: PPARα antagonists, ↓ TGs
1O Hypercholesterolaemia  Ezetimibe: inhibits cholesterol absorption
 Commonest  Niacin / nicotinic acid: ↑HDL, ↓LDL
 ApoB (LDL receptor) defect → ↑↑ LDL-C

Combined Hyperlipidaemia
 2nd commonest
 ↑LDL-C + ↑TG

Lipoprotein Lipase Deficiency

 ↑↑ chylomicrons

Hypertriglyceridaemia
Remnant Particle Disease

© Alasdair Scott, 2012 219

 Porphyrias
Pathogenesis Porphyria Cutanea Tarda
 Deficiencies of enzymes in haem biosynthesis  Commonest porphyria
 → overproduction of toxic haem precursors  Cutaneous manifestations only
 Porphyrins: induce photosensitivity  Uropophyrinogen decarboxylase deficiency
 Porphyrin precursors: neurotoxic
 Genetic or acquired Presentation
 EtOH, lead and Fe → abnormal porphyrin  Photosensitivity: blistering skin lesions
metabolism  Facial hyperpigmentation and hypertrichosis

Precipitants
Acute Intermittent Porphyria  Sunlight
 The Madness of King George
 EtOH
 2nd commonest porphyria
Ix
Pathophysiology
 ↑ urine and se porphyrins
 AD c̄ partial penetrance
 ↑ se ferritin
 Porphyrobilinogen deaminase deficiency
 F>M = 2:1 Rx
 Presents @ 20-40yrs  Avoid sun
 Phlebotomy / iron chelators
Presentation  Chloroquine
 GI: Abdo pain, n/v, constipation
 CV: ↑HR, ↑BP (sympathetic overactivity)
 Neuropsych: peripheral motor neuropathy, seizures,
psychosis
 Other: Red urine, fever, ↑WCC

NB. Can mimic surgical abdomen (anaesthesia = disaster)

Precipitants
 P450 inducers: AEDs, EtOH, OCP/HRT
 Infection / stress
 Fasting
 Pre-menstrual

Ix
 ↑ urine PBG and ALA

Rx
 Supportive
 Analgesia: opiate (avoid oxycodone)
 IV fluids
 Carbohydrate
 Rx ↑HR and ↑BP c̄ β-B
 Specific
 IV haematin

© Alasdair Scott, 2012 220

Rare Inherited Metabolic Disorders
Homocystinuria
 Auto recessive
 Cystathione β-synthetase deficiency
 Accumulation of homocystine

Presentation
 Marfanoid habitus
 Downward lens dislocation
 Mental retardation
 Heart rarely affected
 Recurrent thrombosis

Rx
 Some response to high-dose pyridoxine

Gaucher’s Disease
 Commonest lysosomal storage disease
 Autosomal recessive glucocerebrosidase deficiency
 → accumulation of glucosylceramide in the
lysosomes of the reticuloendothelial system.
 Liver
 BM
 Spleen
 High incidence in Ashkenazi Jews (1/450)

Presentation
 3 clinical types: type 1 is commonest
 Presents in adulthood
 Gross HSM
 Brown skin pigmentation: forehead, hands
 Pancytopenia
 Pathological fractures

Mx
 Most have normal life expectancy in type 1
 Can give recombinant enzyme replacement

© Alasdair Scott, 2012 221

Genetic Oncology Epidemiology
 Cancer affects 30% of the population
Familial Breast and Ovarian Ca  20% of people die from cancer
 ~10% of breast Ca is familial
 ~5% is caused by BRCA1 or BRCA2 mutations
 Both TSGs Commonest Incidence
 BRCA1: Breast Ca – 80%, Ovarian Ca – 40% 1. Skin cancers
 BRCA2: Breast Ca – 80%, male breast Ca 2. Breast / Prostate
 May opt for prophylactic mastectomy and oophrectomy. 3. Lung
4. CRC

Familial Prostate Ca
 ~5% of those c̄ prostate Ca have +ve fam Hx Commonest Mortality
 Multifactorial inheritance  Lung
 BRCA1/2 → moderately ↑ risk  Breast / Prostate
 CRC

Familial CRC
 ~20% of those c̄ CRC have +ve fam Hx
 Relative risk of CRC for individual c̄ FH related to
 Closeness of relative
 Age of relative when Dx.

Familial Adenomatous Polyposis

 Mutation in APC gene on Chr 5
 TSG
 Promotes β-catenin degredation
 β-catenin is an oncogene which → cell
proliferation
 Cells then acquire another mutation to become Ca
 p53
 kRAS
 AD transmission
 ~100% risk of CRC by 50yrs

HNPCC
 Familial clustering of cancers
 Lynch 1: CRC
 Lynch 2: CRC + other Ca
 Ovarian
 Endometrial
 Pancreas
 Small Bowel
 Renal pelvis
 Mutations in DNA mismatch repair genes
 AD transmission (variable penetrance)
 Often Right-sided CRC
 Present @ young age: <50yrs

Peutz-Jehgers
 AD transmission
 Multiple GI hamartomatous polyps
 Mucocutaneous hyperpigmentation
 Lips
 Palms
 10-20% lifetime risk of CRC
 Also ↑ risk of other Ca
 Pancreas
 Lung
 Breast
 Ovaries and uterus
 Testes

© Alasdair Scott, 2012 223

 Oncological Emergencies
Febrile Neutropenia Hypercalcaemia
 PMN <1x109/L  Affects 10-20% of those c̄ Ca
 Isolation + barrier nursing  Affects 40% of those c̄ myeloma
 Meticulous antisepsis
 Broad-spectrum Abx, anti-fungals, anti-virals Causes
 Prophylaxis: co-trimoxazole  Lytic bone metastases
 Production of PTHrP

Spinal Cord Compression Symptoms

 Confusion
Presentation  Renal stones
 Back pain, radicular pain  Polyuria and polydipsia → dehydration
 Motor, reflex and sensory level  Abdominal pain, constipation
 Bladder and bowel dysfunction  Depression
 Lethargy
Causes  Anorexia
 Usually extradural metastasis
 Crush fracture Ix
 ↑ Ca (often >3mM)

O
Ix ↓ PTH (key to exclude 1 HPT)
 Urgent MRI spine  CXR
 Isotope bone scan
Mx
 Dexamethasone Rx
 Discuss c̄ neurosurgeon and oncologist  Aggressive hydration
 Consider radiotherapy or surgery  0.9% NS (e.g. 1L/4h)
 Monitor volume status
 Frusemide when full to make room for more fluid
 If 1 HPT excluded, give maintenance therapy c̄
O
SVCO c̄ Airway Compromise
bisphosphonate: zoledronate is good
 SVCO not an emergency unless there’s tracheal
compression c̄ airway compromise.

Causes Raised ICP

 Usually lung Ca
 Thymus malignancy Presentation
 LNs  Headache: worse AM and bending over
 SVC thrombosis: central lines, nephrotic syndrome  n/v
 Fibrotic bands: lung fibrosis after chemo  Focal neuro and fits
 Papilloedema
Presentation
 Headache Ix
 Dyspnoea and orthopneoa  CT/MRI
 Plethora + thread veins in SVC distribution
Rx
 Swollen face and arms
 Dexamethasone
 Engorged neck veins
 Radio- / chemo-therapy
Pemberton’s Sign
 Lifting arms above head for >1min → facial plethora,
↑JVP and inspiratory stridor Tumour Lysis Syndrome
 Due to narrowing of the thoracic inlet  Massive cell destruction
 High count leukaemia or bulky lymphoma
Ix  ↑K, ↑urate, ↑PO4, ↓Ca
 Sputum cytology  → renal failure
 CXR  Prevention
 CT  ↑ fluid intake + allopurinol 24h before chemo
 Venography  Rasburicase is an option

Mx
 Dexamethasone
 Consider balloon venoplasty + SVC stenting
 Radical or palliative chemo / radio

© Alasdair Scott, 2012 224

 Cancer Therapy
Cancer must be managed in an MDT Radiotherapy
Chemotherapy Mechanism
 Neoadjuvant  Ionising radiation → free radicals which damage DNA
 Shrink tumour to ↓ need for major surgery  Normal cells better at repairing damage cf. Ca cells.
 Control early micrometastases  Radiation dose: gray (Gy)
 Primary Therapy  Given in daily fractions
 E.g. sole Rx in haematological Ca  India ink tattoo for reproducible targeting
 Adjuvant
 ↓ chance of relapse: e.g. breast and GI Ca Radical Rx
 Palliative  Curative intent
 Provide relief from symptoms  40-70Gy
 Prolong survival  15-30 daily fractions

Important Cytotoxic Classes Palliation

 Symptom relief
Alkylating agents  Bone pain, haemoptysis, cough, dyspnoea,
 Cyclophosphamide, chlorambucil, busulfan bleeding.
 8-30Gy
Antimetabolites: methotrexate, 5-FU  1-10 fractions

Vinca alkaloids: vincristine, vinblastine Early Reactions

 Tiredness
Cytotoxic Abx: doxorubicin, bleomycin, actinomycin D  Skin reactions: erythema → ulceration
 Mucositis
Taxanes: paclitaxel  n/v: occur c̄ stomach, liver or brain Rx
 Diarrhoea: pelvic or abdo Rx
Immune Modulators: thalidomide, lenalidomide
 Cystitis
MAbs  BM suppression
 Trastuzumab (anti-Her2): breast Ca
Late Reactions (months or yrs)
 Bevacizumab (anti-VEGF): RCC, CRC, lung
 Brachial plexopathy
 Cetuximab (anti-EGFR): CRC
 Follows axillary radiotherapy
 Rituximab (anti-CD20): NHL
 Numb, weak, painful arm
 Lymphoedema
Tyrosine Kinase Inhibitors
 Pneumonitis
 Erlotinib: lung Ca
 Dry cough ± dyspnoea
 Imatinib: CML
 Rx: prednisolone
 Xerostomia
Endocrine Modulators: tamoxifen, anastrazole
 Benign strictures
 Fistulae
Common Side Effects
 ↓ fertility
 n/v: prophylactic granisetron + dexamethasone
 Panhypopituitarism
 Alopecia
 Neutropenia: 10-14d after chemo
 Extravasation of chemo agent
 Pain, burning, bruising @ infusion site
Surgery
 Stop infusion, give steroids, apply cold pack  Diagnostics: tissue biopsy or complete removal
 Liaise early c̄ plastics  Excision: GI, soft-tissue sarcomas, gynae
 Often with neo-/adjunctive chemo or radiotherapy
 Palliation: e.g. bypass procedures, stenting
Specific Problems
Drug Problems
Cyclophosphamide Haemorrhagic cystitis: give mesna
Analgesia
Hair loss  Oral where possible
BM suppression  Fixed interval to give continuous relief
Doxorubicin Cardiomyopathy  Stepwise approach
Bleomycin Pulmonary fibrosis
Vincristine Peripheral neuropathy (don’t give IT)
Carboplatin Peripheral neuropathy
n/v
Nephrotoxic
Paclitaxel Hypersenstivity
5-FU Mucositis

© Alasdair Scott, 2012 225

Tumour Markers

7?
Marker Malignant Non-malignant
AFP HCC Hepatitis
Teratoma Cirrhosis
Pregnancy
CA 125 Ovary Cirrhosis
Uterus Pregnancy
Breast
CA 15-3 Breast Benign breast disease
CA 19-9 Pancreas Cholestasis
Cholangiocarcinoma Pancreatitis
CRC
CA 27-29 Breast
Neuron-specific SCLC
enolase
CEA CRC Pancreatitis
Cirrhosis
βHCG Germ cell tumour Pregnancy
PSA Prostate BPH
Mono Ig Multiple myeloma
S-100 Melanoma Sarcoma
PLAP Seminoma
Acid phosphatases Prostate Ca
Thyroglobulin Thyroid Ca

© Alasdair Scott, 2012 226

 Malignant and Pre-malignant Skin Lesions
Malignant Melanoma Squamous Cell Carcinoma
 Ulcerated lesion c̄ hard, raised everted edges
Epidemiology  Sun exposed areas
 F>M = 1.5:1
 UK incidence = 10 000/yr and 2000 deaths/yr Causes
 ↑80% in 20yrs  Sun exposure: scalp, face, ears, lower leg
 May arise in chronic ulcers: Marjolin’s Ulcer
Features  Xeroderma pigmentosa
 Asymmetry
 Boarder: irregular Evolution
 Colour: non-uniform  Solar/actinic keratosis → Bowen’s → SCC
 Diameter > 6mm  Lymph node spread is rare
 Evolving / Elevation
Rx
Risk Factors  Excision + radiotherapy to affected nodes
 Sunlight: esp. intense exposure in early years.
 Fair skinned (Low Fitzpatrick Skin Type)
 ↑ no. of common moles Actinic Keratoses
 +ve FH  Irregular, crusty warty lesions.
 ↑ age  Pre-malignant (~1%/yr)
 Immunosuppression
Rx
Classification  Cautery
 Superficial Spreading: 80%  Cryo
 Irregular boarders, colour variation  5-FU
 Commonest in Caucasians
 Imiquimod
 Grow slowly, metastasise late = better prognosis
 Photodynamic phototherapy
 Lentigo Maligna Melanoma
 Often elderly pts.
 Face or scalp
 Acral Lentiginous Bowen’s Disease
 Asians/blacks  Red/brown scaly plaques
 Palms, soles, subungual (c̄ Hutchinson’s sign)  SCC in situ
 Nodular Melanoma
 All sites Rx
 Younger age, new lesion  As for AKs
 Invade deeply and metastasis early = poor prog
 Amelanotic
 Atypical appearance → delayed Dx Keratoacanthoma
 A well differentiated SCC that arises in a hair follicle.
Staging and Prognosis  Fast-growing, dome-shaped c̄ a keratin plug.
 Breslow Depth  Usually regress but may be excised
 Thickness of tumour to deepest point of dermal
invasion
 <1mm = 95-100% 5ys Basal Cell Carcinoma
 >4mm = 50% 5ys  Commonest cancer
 Clark’s Staging  Pearly nodule c̄ rolled telangiectactic edge
 Stratifies depth by 5 anatomical levels  May ulcerate
 Stage 1: Epidermis
 Typically on face in sun-exposed area
 Stage 5: sc fat
 Above line from tragus → angle of mouth
Metastasis
Behaviour
 Liver
 Low-grade malignancy → very rarely metastasise
 Eye
 Locally invasive
Mx
 Excision + 2 margin excision depending on Bres depth
O Rx
 ± lymphadenectomy  Excision
 Mohs: complete circumferential margin
 ± adjuvant chemo (may use isolated limb perfusion)
assessment using frozen section histology
Poor prognostic indicators  Cryo/radio may be used.
 Male sex (more tumours on trunk cf females)
 ↑ mitoses
 Satellite lesions (lymphatic spread)
 Ulceration
© Alasdair Scott, 2012 233
 Psoriasis
Epidemiology Mx
 Prevalence: 2% of Caucasians
 Age: peaks in 20s and 50s Education
 Sex: F=M  Avoid triggers
 Genetics: 30% have FH
 Genetic predisposition Soap Substitute
 Aqueous cream
Pathology  Dermol cream
 TIV hypersensitivity reaction  Epaderm ointment
 Epidermal proliferation
 T-cell driven inflammatory infiltration Emollients
 Histo  Epaderm
 Acanthosis: thickening of the epidermis  Dermol
 Parakeratosis: nuclei in stratum corneum  Diprobase
 Munro’s microabscesses: neutrophils
Topical Therapy
Triggers  Vit D3 analogue: e.g. calcipotriol
 Stress  Steroids: e.g. betamethasone
 Dovobet = Calcipotriol + betamethasone
 Infections: esp. streps
 Tar: mainly reserved for in-patient use
 Skin trauma: Kobner phenomenon
 Dithranol
 Drugs: β-B, Li, anti-malarials, EtOH
 Retinoids: e.g. tazarotene
 Smoking
UV Phototherapy
Signs  Causes local immunosuppression
 Narrow band UVB
Plaques
 Psoralen + UVA: PUVA
 Symmetrical well-defined red plaques c̄ silvery scale
 Psoralen is a photsensitising agent and can be
 Extensors: elbows, knees topical or oral
 Flexures (no scales): axillae, groins, submammary  PUVA is more effective but ↑ skin Ca risk
 Scalp, behind ears, navel, sacrum
Non-Biologicals
Nail Changes (in 50%)  Methotrexate
 Pitting  Ciclosporin
 Onycholysis  Acetretin (oral retinoid / vit A analogue)
 Subungual hyperkeratosis  SE: ↑ lipids, ↑ glucose

10-40% Develop Seronegative Arthritis Biologicals

 Mono-/oligo-arthritis: DIPs commonly involved  Infliximab
 Rheumatoid-like  Etanercept
 Asymmetrical polyarthritis  Adalimumab
 Psoriatic spondylitis
 Arthritis mutilans
 May → dactylitis

Variants
Guttate
 Drop-like salmon-pink papules c̄ fine scale
 Mainly on trunk
 Occurs in children assoc. c̄ strep infection

Pustular
 Sterile pustules
 May be localised to palms and soles

Erythroderma and Generalised Pustular

 Generalised exfoliative dermatitis
 Severe systemic upset: fever, ↑WCC, dehydration
 May be triggered by rapid steroid withdrawal

Differential
 Eczema
 Tinea: asymmetrical
 Seborrhoeic dermatitis
© Alasdair Scott, 2012 234
 Eczema
Presentation Mx of Atopic Eczema
 Extremely itchy
 Poorly demarcated rash Education
 Acute: oozing papules and vesicles  Avoid triggers: e.g. soap
 Subacute: red and scaly
 Chronic eczema → lichenification Soap Substitute
 Skin thickening c̄ exaggeration of skin markings  Aqueous cream
 Dermol cream
 Epaderm ointment
Pathology
Emollients
 Epidermal spongiosus
 Epaderm
 Dermol
 Diprobase
Atopic Eczema  Oilatum (bath oil)
 TH2 driven inflammation c̄ ↑IgE production
 Affects 2% of infants Topical Therapy
 Most children grow-out of it by 13yrs  Steroids
 1% Hydrocortisone: face, groins
Cause  Eumovate: can use briefly (<1wk) on face
 FH of atopy common  Betnovate
 Specific allergens  Dermovate: very strong, brief use on thick skin
 House dust mite  Palms, soles
 Animal dander
 Diet: e.g. dairy products 2
nd
line Therapies
 Topical tacrolimus
Presentation  Phototherapy
 Face: esp. around eyes, cheeks  Ciclosporin or azathioprine
 Flexures: knees, elbows
 May become 2 infected
O

 Staph → fluclox
 HSV → aciclovir
Pruritus
Associations
Generalised
 Asthma
 CRF
 Hay fever
 Cholestasis
Ix  Haematological
 Polycythaemia
 ↑ IgE
 Hodgkin’s
 RAST testing: identify specific Ag
 Leukaemia
 Iron deficiency
 Endocrine
Irritant Contact Dermatitis  DM
 Everyone is susceptible to irritants  Hyper- / hypo-thyroidism
 Causes: detergents, soaps, oils, solvents, venous stasis  Pregnancy

Very Itchy Dermatological Diseases

Allergic Contact Dermatitis  Eczema
 Type IV hypersensitivity reaction  Urticaria
 Common allergens  Scabies
 Nickel: jewellery, watches, coins  Dermatitis herpetiformis
 Chromates: leather
 Lanolin: creams, cosmetics
 Location: correlates sharply c̄ allergen exposure
 Ix: Patch testing

Adult Seborrhoeic Dermatitis

 Red, scaly, rash
 Cause: overgrowth of skin yeasts (e.g. malassezia)
 Location: scalp (dandruff), eyebrows, cheeks,
nasolabial folds
 Rx: mild topical steroids / antifungal
 Daktacort: miconazole + hydrocortisone
© Alasdair Scott, 2012 235
 Skin Infections
Tinea Erysipelas
 A superficial mycosis caused by dermatophytes  Sharply defined superficial infection by S. pyogenes
 Microsporum  Often affects the face
 Epidermophyton  High fever + ↑ WCC
 Trichophyton  Rx
 Benpen IV
Presentation  Pen V and fluclox PO
 Round scaly lesion
 Itchy Cellulitis
 Central clearing  Acute infection of skin and soft tissues
 Scalp, body, foot, groin, nails
Cause: β-haemolytic Strep + staph. aureus
Rx
 Skin: terbinafine or topical ketoconazole / miconazole Presentation
 Scalp: griseofulvin or terbinafine  Deeper and less well defined than erysipelas
 Nails: terbinafine  Pain, swelling, erythema and warmth
 Systemic upset
 ± lymphadenopathy
Candida
 Common Rx
 RFs: immunosuppression, Abx, steroid inhalers  Empiric: fluclox IV
 Confirmed Strep: Benpen or Pen V
Presentation  Pen allergic: clindamycin
 Pink + white patches
 Moist Warts
 Satellite lesions  Caused by HPV infection
 Mouth, vagina, skin folds, toe web
Rx
Rx  Expectant
 Mouth: nystatin  Destructive
 Vagina: clotrimazole cream and pessary  Topical salicylic acid
 Cryotherapy
 Podophyllin
 Imiquimod
Pityriasis versicolor
 Caused by Malassezia furfur
Molluscum
 Pox virus
Presentation
 Pink papules c̄ umbilicated central punctum
 Common in hot and humid environments
 Circular hypo-/hyper-pigmented patches  Resolve spontaneaously
 Fine white scale
 Itchy Herpes Zoster / Shingles
 Back of neck and trunk  Recurrent VZV infection
 Dermatomal distribution of cropping vesicles and crust
Ix  Thoracic: 50%
 “Spaghetti and meatballs” appearance c̄ KOH stain  Ophthalmic: 20%
 Cornea affected in 50% → keratitis, iritis
Rx  May → post-herpetic neuralgia
 Selenium sulphate or ketoconazole shampoos  Rx: aciclovir or famciclovir PO if severe

Herpes Simplex
 Gingivostomatitis or recurrent genital or oral infections
Impetigo  Triggered by infection (e.g. CAP), sunlight and
 Contagious superficial skin rash caused by S. aureus immunosuppression
 May complicate eczema: eczema herpeticum
Presentation
 Grouped painful vesicles on an erythematous base
 Age: peak @ 2-5yrs
 Rx: acyclovir or famciclovir indicated if
 Honey-coloured crusts on erythematous base
immunosuppressed or recurrent genital herpes.
 Common on face

Rx Pityriasis Rosea
 Mild: topical Abx (fusidic acid, mupirocin)  HHV-6/-7
 More severe: fluclox PO  Herald patch precedes rash, mainly on the trunk

© Alasdair Scott, 2012 236

Miscellaneous Skin Disorders Acne Vulgaris
Lichen Planus Pathophysiology
 ↑ sebum production: androgens and CRH
Skin Presentation  P. acnes is a skin commensal that flourishes in the
 Flexors: wrists, forearms, ankles, legs anaerobic environment of the blocked follicle
 Display Kobner phenomenon  → inflammation
 Purple
 Pruritic Presentation
 Polygonal  Inflammation of pilosebaceus follicles
 Planar  Comedones (white- or black-heads), papules, pustules
 Papules nodules, cysts
 Lacy white marks: Wickham’s Striae  Face, neck, upper chest and back

Lesions Elsewhere Mx
 Scalp: scarring alopecia  Pt. education
 Nails: longitudinal ridges  Remember that topical therapy is difficult to apply to the
 Mouth: lacy white plaques on inner cheeks back.
 Genitals
Mild: topical therapy
Rx  Benzoyl peroxide
 Mild: topical steroids  Erythromycin, Clindamycin
 Severe: systemic steroids  Tretinoin / Isotretinoin

Moderate
Bullous Pemphigoid  Topical benzoyl peroxide + oral Abx (doxy or erythro)
 Autoimmune blistering disease due to auto-abs against
hemidesmosomes Severe
 Isotretinoin (vitamin A analogue)
Presentation  60-70% have no further recurrence
 Mainly affects the elderly  SE: teratogenic, hepatitis, ↑lipids, depression, dry
 Tense bullae on erythematous base skin, myalgia
 Can be itchy  Monitor: LFTs, lipids, FBC
 May try Dianette in women
Ix
 Biopsy shows linear IgG along the BM and subepidermal
bullae Acne Rosacea
 Chronic relapsing remitting disorder affecting the face
Rx
 Clobetasol (Dermavate) Presentation
 Chronic flushing ppted. by alcohol or spicy foods.
 Fixed erythema: chin, nose, cheeks, forehead
Pemphigus Vulgaris  Telangiectasia, papules, pustules (no comedones)
 Autoimmune blistering disease due to auto-abs against
desmosomes. Associations
 May be ppted by drugs  Rhinophyma: swelling and soft tissue overgrowth of the
 NSAIDs nose in males
 ACEi  Blepharitis: scaling and irritation at the eyelashes
 L-dopa
Rx
Presentation  Avoid sun exposure
 Younger pts.  Topical azelaic acid
 Large flaccid bullae which rupture easily  Oral doxycycline or azithromycin
 Nikolsky’s sign +ve
 Mucosa is often affected

Ix
 Intraepidermal bullae

Rx
 Prednisolone
 Rituximab
 IVIg

© Alasdair Scott, 2012 237

Drug Eruptions Infestations
Maculopapular Scabies
 Commonest type  Sarcoptes scabei
 Generalized erythematous macules and papules  Highly contagious: spread by direct contact
 ± fever and ↑ eosinophils  Female mite digs burrows and lays eggs
 Develops w/i two weeks of onset of drug
 Penicillins, cephalosporins, AEDs Presentation
 Burrows: short, serpiginous grey line, block dot
 Hypersensitivity rash: eczematous, vesicles
Urticaria  Extremely itchy → escoriation
 Particularly affects the finger web spaces (esp. 1 )
st
 Wheals ± angioedema and anaphylaxis
 Rapid onset after taking drug  Also: axillae, groin, umbilicus
 Immune (IgE)
 Penicillins Mx
 Cephalosporins  Permethrin cream: applied from neck down for
 Non-immune (direct mast cell degranulation) 24hrs
 Morphine  2nd line: Malathion
 Codeine  3rd line: oral ivermectin
 NSAIDs  Treat all members of the household
 Contrast

Headlice
Erythema Multiforme  Pediculus humanus capitis
 Symmetrical target lesions on palms, soles and limbs  Spread by head to head contact
 Occurs 1-2wks after insult  Nits = empty eggs
 Infections are commoner cause of EM
Presentation
Causes  Itch
 Idiopathic  Papular rash @ the nape of the neck
 Infections: HSV, mycoplasma
Rx
 Drugs
 Sulphonamides, NSAIDs, allopurinol, penicillin  Malathion
phenytoin  Combing

Stevens-Johnson Syndrome
 More severe variant of EM,
 Blistering mucosa: conjunctiva, oral, genital

Toxic Epidermal Necrolysis

 Extreme form of SJS
 Nearly always a drug reaction
 Features
 Severe mucosal ulceration
 Widespread erythema followed by epidermal necrosis
c̄ loss of large sheets of epidermis
 → dehydration
 ↑↑↑ risk in HIV+
 Rx: dexamethasone, IVIg
 Mortality: 30%

© Alasdair Scott, 2012 238

 Skin Manifestations of Systemic Disease
Skin Conditions Diseases
Erythema Nodosum Disease Skin Manifestation
 Painful blue-red lesions on anterior shins Rheumatic fever Erythema marginatum
 3 S’s: sarcoid, strep infections and sulfonamides Lyme Erythema chronicum migrans
 Also: OCP, IBD and TB, Behcet’s (Borrelia burgdorferi)
Crohn’s Perianal ulcers and fistulae
Erythema Multiforme EN
 Symmetrical target lesions on palms, soles and limbs Pyoderma gangrenosum
 Possibly mediated by IgM deposition Dermatomyositis Heliotrope rash on eyelids
 Usually 2 to infection
O
Shawl sign (macular rash)
 Infections: HSV (70%), mycoplasma Gottron’s Papules
 Drugs: SNAPP Mechanic’s hands
Nailfold erythema, telangiectasia
Stevens-Johnson Syndrome and TEN DM Ulcers
 Severe variants of EM Candida
 Nearly always drug-induced Kyrle disease
 Fever, mucosal and skin ulceration and necrosis Acanthosis nigricans
Necrobiosis lipoidica (shins)
Pyoderma Gangrenosum Granuloma annulare (hands, feet)
 Wide (10cm), deep ulceration c̄ violaceous border Sarcoidosis Erythema nodosum
Erythema multiforme
 Purulent surface
Lupus pernio
 Undermined edge Hypopigmented areas
 Commonly occurs on legs Red/violet plaques
 Heal c̄ cribriform (pitted) scars Coeliac Dermatitis herpetiformis (elbows)
 Assocs.: IBD, AIH, Wegener’s, RA, leukaemia Graves’ Pre-tibial myxoedema (lat mal)
 Rx: High-dose systemic steroids RA Rheumatoid nodules

Vitiligo
 Chalky white patches c̄ hyperpigmented borders
 Itch in sunlight
?i
SLE
Systemic sclerosis
Vasculitis (palpable purpura)
Facial butterfly rash
Calcinosis
Raynaud’s
 Assocs.: autoimmune disorders Sclerodactyly
Telangiectasia
Livedo Reticularis Generalised skin thickening
 Persistent mottled red/blue lesions that don’t blanch Liver disease Palmar erythema
 Commonly found on the legs Spider naevi
 Triggered by cold Gynaecomastia
 Causes ↓ 2O sexual hair
 Idiopathic Jaundice
 Vasculitis: RA, SLE, PAN Bruising
 Obstruction: anti-phos, cryoglobulinaemia Excoriations
 Sneddon’s syn.: LR + CVAs ESRD Assoc. c̄ Cause
- DM, vasculitis, sclero, RA, SLE
Assoc. c̄ ESRD
- Pruritus, xerosis, pigment change
Bullous disease
Assoc. c̄ Transplant
- Cushingoid, gingival hyperplasia,
Infections, BCC, SCC, melanoma
Kaposi’s

Neoplasia Acanthosis nigricans

Dermatomyositis
Thrombophlebitis migrans
Acquired ichthyosis

© Alasdair Scott, 2012 239

 Health Information
Routine Data Clinical Governance
 Data that are routinely collected and recorded in an on-
going systematic way, often for administrative purposes. Definition
 A transparent system for the continual maintenance and
Advantages improvement of healthcare standards both on an
 Relatively cheap individual and organisational level within the NHS.
 Already collected and available
 Standardised collection procedures Elements
 Education and Training
Disadvantages  Clinical audit
 May not answer the question  Clinical effectiveness
 Variable quality  Research and Development
 Not every case captured  Openness
 Risk Management
Sources
 Demographics: census Agencies
 Health: births, deaths, admissions, prescriptions, hospital  The Care Quality Commission
episode statistics, cancer  Independently inspect healthcare services
 Exposures: smoking, air pollution, crime against standards set by the Department of
 Disease prevention: screening, immunisation uptake Health.
 Investigate serious failures in healthcare
services.
 Publish regular ratings of NHS trusts.
Hospital Episode Statistics
 Electronic record of every inpatient or day-case episode
of patient care in every NHS hospital. Clinical Audit
 300 fields of information, including:  Are pts. given the best care?
 Pt demographics
 Diagnosis using ICD-10 Definition
 Procedures using OPCS4  A quality improvement process that seeks to improve
pt. care and outcomes through the systematic review of
Advantages care against explicit criteria and the implementation of
 Comprehensive change.
 Unbiased
 Based on case notes

Disadvantages Outcome Measures

 Data may not be complete  Are pts. better and do they feel better?
 In-hospital death only
Patient Reported Outcomes (PROMs)
 Calculate the health gain from the pts. perspective after
surgical Rx using pre- and post-operative surveys.
Mortality  Arthroplasty, hernia repairs, varicose veins
 Hip replacement gives largest improvement in
UK specific condition and general health.
1. Heart and Circulatatory disorders: 33%
a. IHD (23%) Quality and Outcomes Framework (QOF)
b. Stroke (10%)  Voluntary annual reward and incentive programme for
2. Cancer: 30% all GP surgeries in England, detailing practice
a. Trachea, bronchus, lung achievement results.
b. Breast, prostate
c. Colorectum
3. Respiratory disorders: 13%
a. Pneumonia
b. COPD

Worldwide
1. IHD
2. Stroke and other cerebrovascular disease
3. LRTI
4. COPD
5. Diarrhoeal diseases

© Alasdair Scott, 2012 241

 Public Health Issues
Major Contributions to Health Smoking
 Drugs and medical intervention  Commonest cause of preventable death in the UK
 Obstetric and perinatal care  2nd hand smoking
 Immunisation  Respiratory infections
 Screening  Asthma
 Chronic disease Mx  SIDS
 ↑ lung Ca and CHD risk
 Social gradient in mortality is partially explained by
social gradient in smoking.
Major Mortality Risk Factors
 Heart and Circulatory Disorders Some Strategies
 Smoking
 Health education
 ↓ exercise
 Restrict advertising
 Diet: ↑fat, ↑calorie, ↑salt, EtOH
 ?Stress  Remove from display in shops
 Neoplasms  ↑ price
 Smoking  Smoking ban in public places
 ↓ exercise  Very brief advice
 Diet: ↓fruit and veg, ↓fibre, ↑fat
 Pulmonary Disease
 Smoking Alcohol
 ↓ exercise  5% of men and 2% of women report problems
 Occupation: asbestos, dust exposure  Recommended
 Men: 21u/wk or 3-4u/d
 Women: 14u/wk or 2-3u/d
Major Public Health Issues  NB. 1u = 10ml EtOH
 Smoking
Some Strategies
 Obesity / unhealthy diet
 National Alcohol Harm Reduction Strategy
 ↓ Exercise
 ↑ minimum price / unit
 EtOH abuse
 Sexual health
 Mental health
Mental Health
 Common: ~15% lifetime risk
 Depression and anxiety disorders most commonly
Obesity
 20% obese Some Strategies
 50% of females overweight or obese  GPs get rewarded for depression screening
 Expansion of psychological therapies (e.g. CBT)
Nurses’ Health Study
 Overwt. → ↓3yrs of life
 Obese → ↓7yrs of life
 Active lifestyle (30min walk/d) prevents 30% of obesity
and 45% of new DM.

Evidence for Changing Nutrition

 Mediterranean diet can ↓ cardiac death post-MI
 Low Na diet → ↓ BP

Sexual Health
 10% of 16-24yr olds have ≥1 STI
 ↑ in urban areas and amongst Blacks and Minorities
 → infertility, ectopics, Cervical Ca, HIV

Some Strategies
 Improving access to sexual health services
 Chlamydia screening

© Alasdair Scott, 2012 242

 Clinical Evidence
Hierarchy of Evidence Cohort Study
1. Systematic reviews and meta-analyses  Prospective study comparing development of disease in
2. RCTs exposed and non-exposed grps.
3. Cohort or case-control studies  Incidence of disease in each grp → relative risk
4. Descriptive studies
5. Case reports Examples
 Million Women study
Study Types  Framingham Heart Study
 Nurses’ Health Study
Observational
 Analytical / Aetiological Advantages
 Studies determinants of disease by judging  Good for evaluation of rare exposures
whether exposure causes or prevents disease  Can examine multiple effects of a single exposure
 Descriptive  Can elucidate temporal relationship
 Studies distribution of disease  Direct incidence calculation
 Person, place and time
Disadvantages
Experimental  Inefficient for evaluation of rare diseases
 RCTs  Expensive and time-consuming
 Loss to f/up affects results

Descriptive Studies
 Can be used to generate hypotheses RCTs
 Provide frequency data  Planned experiment designed to assess the efficacy of
 Incidence an intervention.
 Point prevalence  Randomisation: ↓ selection bias.
 Can’t determine causation  Blinding: ↓ measurement bias

Examples Advantages
 Cross-sectional Surveys  Most reliable demonstration of causality
 Household interview surveys asking questions
regarding illness, social circumstances and Disadvantages
demographics.  Non-compliance
 Cross-sectional Census  Loss to f/up
 Ecological studies  Validity depends on quality of study
 Use populations rather than individual as unit of  Ethical issues
observation.  Selection criteria may limit generalisability
 Subject to significant confounding: age, sex, SES
 Other routine data
Meta-Analyses
 Observational study of evidence
Case-Control Studies  Systematic identification of relevant trials and
 Retrospective study of exposure in a case grp c̄ the assessment of their quality
disease and a control grp w/o the disease.  Allow accurate interpretation of multiple RCTs
 Proportion of exposed in each group → odds ratio
Forest plots
Advantages  Square = OR
 Quick and cheep  Size = size of study
 Well-suited for diseases c̄ long latent periods  Line = 95% CI of OR
 Good for evaluation of rare diseases  Diamond = combined odds ratio
 Can examine multiple aetiological factors for a single  Width = 95% CI
disease.

Disadvantages
 Inefficient for evaluating rare exposures
 Cannot calculate incidence rates
 Temporal relationship between exposure and disease
can be difficult to establish
 Recall bias
 E.g. testicular cancer sufferers more likely to
report preceding trauma
 Selection bias
 E.g. non-random selection of study grps.

© Alasdair Scott, 2012 243

 Epidemiological Concepts
5 Steps of EBM Odds Ratio
1. Question
2. Search OR = odds of exposure in cases / odds of exposure in controls
3. Appraise = (A/C) / (B/D) = AD/BC
4. Apply
5. Evaluate Case Control
Exposed A B
Association and Causation Not Exposed C D
 Association: a relationship between two factors
 Causation: X → Y OR = ~RR if disease is rare (<10%)

Bradford Hill Causation Criteria Relative Risk

 Strong association: i.e ↑↑ RR or OR  ↑/↓ in probability of disease given a particular RF
 Consistency c̄ other investigations
 Specificity RR = incidence in exposed / incidence in unexposed
 Temporal relationship = (A/A+B) / (C/C+D)
 Dose response relationship
 Plausibility Risk Estimates
 Coherence  Absolute risk: probability of disease occurrence
 Experimental evidence  Relative risk: ↑/↓ in probability of disease given a RF
 Analogy  Attributable risk: measure of excess risk due a factor

Chance Number Needed To Treat

 Studies are based on estimates from samples and are  NNT = (1 / Absolute risk reduction) x 100
therefore subject to random variation.  Absolute risk reduction = 1- hazard ratio
 Random variation can be minimised by ↑ sample size.
 The role of chance is assessed by statistical significance Screening
tests and by calculating confidence intervals.
Modified Wilson Criteria
Bias  Disease
 Inaccurate data due to systematic error in selection,  Important health problem
measurement or analysis.  Well recognised pre-clinical stage
 Natural Hx well understood
Selection Bias  Test
 Systematic difference between the characteristics of  Valid and reliable
those selected for a study and those who were not or  Simple and cheap
differences between study grps.  Safe and acceptable
 E.g. self-selection, healthy worker effect  Dx and Rx
 Adequate facilities
Measurement Bias  Acceptable Rx
 Early intervention is of more benefit than later Rx
 Occurs when measurements or classifications of
disease or exposure are inaccurate.  Cost effective
 E.g. inaccuracies of the instruments, expectations of
observers or participants. Validity
 Sensitivity: detect true positives
Analysis Bias  Specificity: exclude true negatives
 Error caused by participants being lost to f/up or  PPV: how likely to have disease if positive
switching treatment groups.  NPV: how likely not to have disease if negative
 Take into account using an intention to treat analysis.  PPV and NPV depend on disease frequency

Confounding Disease No Disease

Positive A B PPV
 Error in the interpretation of an accurate measurement.
A/(A+B)
 Any factor which is prognostically linked to the outcome
Negative C D NPV
and is unevenly distributed between study groups.
C/(C+D)
 Not a confounder if it lies on the causal pathway
Sensitivity Specificity
between the variables of interest.
A/(A+C) B/(B+D)
 Common confounders: age, sex, SES
Effectiveness
Reducing Confounding
 Selection bias: healthiest come for screening
 Randomisation
 Lead-time bias: disease Dx earlier → ↑ survival time
 Stratification: e.g. stratify by age
 Length-time bias: outcome appears better because
 Standardisation: e.g. SMR
more disease c̄ good outcome detected.
 Ratio of observed:expected deaths
 Expected deaths derived from larger population
 © Regression
Alasdair Scott, 2012 244
Disease Prevention
Primordial Prevention
 Prevention of factors promoting the emergence of risk
factors: lifestyle, behaviours, exposure patterns
Examples
 Healthy eating programmes in schools
 Social policies to ↓ poverty and inequality
 Programmes that promote walking, cycling and public
transport.
 Encouragement of positive health behaviour.
Likelihood Ratios
Pre- and Post-test Probability
 Pre-: estimate of probability that pt. has a certain
disease based on presentation or epidemiology.
 Test: diagnostic test or sign or symptom that you elicit
 Post-: new probability taking into account the “test”
result
 Likelihood ratio: measure of how much the test alters
your probability of the disease.
Calculation

Odds = probability / (1- probability)

Primary Prevention  i.e. the ratio of the probability something will happen to
the probability that it won’t.
 Prevention of disease onset
Probability = odds / (odds +1)
Examples
 Vaccination
LR = p [test result if disease present] / p [test result if disease
 Smoking cessation absent]
 Healthy diet
 Condom use LR for positive test result = sensitivity / (1- specificity)
 Detection and Rx of hyperlipidaemia
LR for negative test result = (1- sensitivity) / specificity

Secondary Prevention Post-test odds = pre-test odds x likelihood ratio

 Halting progression of established disease
 Early detection followed by prompt, effective Rx
Examples
 Screening for Ca
 Smoking cessation after MI
Tertiary Prevention
 Rehabilitation of people c̄ established disease to
minimise residual disability and complications.
Example
 Pre-test probability could be disease incidence in that
population.
 Convert pre-test probability to odds.
 Calculate likelihood ratio based on the result of your test.
 Apply likelihood ratio to get post-test odds.
 Convert post-test odds to post-test probability

Examples
 Rapid Rx of MI or stroke to ↓ disability
 HAART

Disease Prevention in General Practice

 General advice for a healthy lifestyle
 Identifying and facilitating reduction of risk factors
 Ensuring availability of preventative interventions
 Identifying early disease and offering treatment

Prevention Paradox
 Many people exposed to a small risk may generate more
disease than a few exposed to a large risk.
  when many people receive a small benefit the total
benefit may be large.
 However, individual inconvenience may be high to the
many while benefit may only be to a few.

Health Promotion
 Process of enabling people to increase control over, and
to improve, their health.
 Public policy, supportive environments…
 Health promotion is specific requirement of the GMC
Good Medical Practice guidelines.

© Alasdair Scott, 2012 245

 Shock
If ECG unrecordable, Rx as cardiac arrest Causes : CHOD

Cardiogenic
ABCDE  MI
 Arrhythmia

Hypovolaemic
Raise foot of bed  Haemorrhage: internal and external
(unless cardiogenic)  Endocrine: Addisonion crisis, DKA
 Excess loss: burns, diarrhoea
 Third-spacing: pancreatitis
IV Access:
 2 wide bore (14g) cannula in each ACF Obstructive
 PE
 Tension pneumothorax
Fast infusion of crystalloid to raise BP Distributive
(unless cardiogenic)  Sepsis
 Anaphylaxis
 Neurogenic

Assessment
Appearance
History  Cold, clammy: cardiogenic or hypovolaemic
 Chest pain: MI, PE, dissection, anaphylaxis
 Warm, well perfused: septic
 Abdo pain: AAA, DKA, peritonitis, ruptured ectopic
 Urticaria, angioedema, wheeze: anaphylaxis
 Back pain: AAA

Hands JVP
 ↑CRT: hypovolaemia, cardiogenic  ↑: cardiogenic
 ↓: hypovolaemic, distributive
Pulse
 Tachy (unless β-B or bradyarrhythmia)
Abdomen
 Small, thread: hypovolaemia
 Tender, guarding: trauma, aneurysm, peritonitis.
 Bounding: sepsis
 Melaena: GI bleed
ABP
 ↓PP: hypovolaemia
 R-L differential > 20mmHg: dissection

Ix Specific Measures
 FBC, U+E, glucose, ABG, CRP, trop Anaphylaxis:
 X-match, clotting  Adrenaline (0.5mg), hydrocortisone (200mg), chlorphenamine
 Blood cultures, urine MCS (10mg), salbutamol
 ECG, CXR, USS, Echo, CT
Cardiogenic:
 Rx arrhythmias/MI. Consider dobutamine.

Septic shock:
Monitoring  IV Abx (e.g. meropenem 1g/8h IV + tazocin 4.5g/8h IV)
 Catheter (>30ml/hr)  Fluids, vasopressors (e.g. norad)
 Art line
 CVP line Hypovolaemic
 Fluid replacement: crystalloid, colloid, blood (grp specific/O neg)
 Titrate to: urine output, CVP, BP
 Haemodialysis if ATN

© Alasdair Scott, 2012 247

 Anaphylactic Shock
Pathogenesis Mx
 Type 1 IgE mediated hypersensitivity reaction
 TH2 driven IgE production following 1O allergen exposure Secure Airway and give 100% O2
 Re-exposure → biphasic inflammatory response  Intubate if respiratory obstruction
 Elevate the feet
Early phase: w/i minutes
 IgE cross-linking →mast cell degranulation
 Vasoactive mediator release
 Histamine Adrenaline 0.5mg IM
 Tryptase  0.5ml of adrenaline 1:1000
 Lipid mediator synthesis  Repeat every 5min if needed
 Leukotrienes: ↑ vasc perm + → bronchial
 Guided by cardiorespiratory function
constriction
 Prostacyclin
 Chemokines: e.g. eotaxin
 Promote leukocyte recruitment (esp. eosinophils)
Secure IV Access
Late Phase: 2-24hrs  Blood for mast cell tryptase
 Eosinophils recruited in early phase.  IVI 0.9% NS may be needed
 Release enzymes and stimulate mast cells.  E.g. 500ml over 15-30min
 Amplify and sustain the initial response  Titrate against BP

Common Precipitants Chlorphenamine 10mg IV

 Drugs: penicillin, contrast media +
 Food: peanuts, eggs, fish, semen Hydrocortisone 200mg IV
 Latex
 Stings

Salbutamol Nebs if wheeze

Presentation  5mg salbutamol + 0.5mg ipratropium
 Sweating, d/v
 Urticaria, itching, oedema
 Wheeze, laryngeal obstruction, cyanosis
Further Mx
 Tachycardia, hypotension
 Monitor closely for second deterioration.
 Possible improvement and then deterioration as late
phase initiates.
Discharge
 Teach adrenaline self-injection and ensure pt. has
≥2 0.3mg Epipens.
 Advise wearing of medic alert bracelet
 Advice re recognition and avoidance
 Arrange OPD f/up

OPD f/up
 Skin prick tests may help ID antigens
 RAST may be preferable

© Alasdair Scott, 2012 248

 Narrow Complex Tachycardias = SVT
Definition: Rate >100bmp, QRS width < 120ms Principles of Mx
 If pt. compromised → sedate + DC cardioversion
Differential  Otherwise ID rhythm and Rx accordingly
1. Sinus tachycardia  Key to ID irregular rhythm = AF = different Rx
 Vagal manoeuvres (carotid sinus massage, valsalva) transiently ↑
2. Atrial AV block and may unmask underlying atrial rhythm.
 AF  If manoeuvres unsuccessful, give adenosine while recording
 Atrial flutter rhythm strip
 Atrial tachycardia  → transient AV block, unmasking atrial rhythm
 → cardioverts AVNRT/AVRT to sinus rhythm
3. AV nodal re-entry tachycardia  If adenosine fails, choose from:
4. AV re-entry tachycardia  Digoxin
 Atenolol
 Verapamil (not if on β-blocker)
 Amiodarone
 If unsuccessful → DC cardioversion

Mx Flowchart

O2 + IV access

Regular rhythm? NO
i> Treat as AF
 Control rate c̄ β-B (e.g. metoprolol) or digoxin
YES  If onset <48h consider cardioversion c̄ amiodarone (as
below) or DC shock
 Continuous ECG trace  Consider anticoagulation c̄ heparin and/or warfarin
 Vagal manoeuvres
 Adenosine 6mg IV bolus NB. Avoid adenosine, CCB, β-B in AF/Flutter with WPW or Hx of
 Then 12mg, then 12mg WPW as may → VF. Use Amiodarone or flecainide.

YES
Adverse Signs? i> Sedation
 BP <90
 HF
 ↓ consciousness
 HR >200
¢ NO
I Synchronised cardioversion:
100→200→360J
I
Choose from:
Amiodarone:
 Digoxin (500ug over 30min)
 Amiodarone  300mg over 20-60min
 Verapamil  Then 900mg over next 23h
 Atenolol

Adenosine Prophylaxis
 MOA: temporary AVN block  β-B
 SEs: Transient chest tightness, dyspnoea, flushing, headache  AVRT: flecainide
 Relative CIs: asthma, 2nd/3rd degree block  AVNRT: verapamil
 Interactions
 fx ↑d by dipyridimole
 fx ↓d by theophylline

© Alasdair Scott, 2012 249

 Broad Complex Tachycardias
Definition: Rate >100bmp, QRS width > 120ms VT Causes: I’M QVICK
 Infarction (esp. c̄ ventricular aneurysm)
Differential  Myocarditis
1. VT  QT interval↑
2. Torsades de points  Valve abnormality: mitral prolapse, AS
3. SVT c̄ BBB  Iatrogenic: digoxin, antiarrhythmics, catheter
 Cardiomyopathy (esp. dilated)
 K↓, Mg↓, O2↓, acidosis

Mx Flowchart

Pulse? i> CPR

NO
YES

O2 + IV access

YES
 BP<90
Adverse Signs? i> Sedation

 HF
 Chest pain
 ↓ consciousness
Synchronised cardioversion:
 HR > 150 200→300→360

NO

Correct Electrolyte Problems Amiodarone:

 ↓K+: max 60mM KCL @ 20mmol/h  300mg over 20-60min
 ↓Mg2+: 4ml 50% MgSO4 in 30min  Then 900mg over next 23h

Assess Rhythm
Regular (i.e. VT):
 Amiodarone (see opposite)
 Or lignocaine 50mg over 2min

If irregular, Dx is usually:
 AF c̄ BBB
 Pre-excited AF: flec / amio Recurrent / Paroxysmal VT
 TDP: MgSO4 2g IV over 10 min
Medical:
 Amiodarone
Failure
 β-B
Synchronised Cardioversion
ICD

© Alasdair Scott, 2012 250


12 lead ECG
O2 2-4L aim for SpO2 94-98%
IV access
Bloods for FBC, U+E, glucose, lipids
Brief assessment
 Hx of CVD and risk factors
 Thrombolysis CIs
 CV exam
Antiplatelet
 Aspirin 300mg PO (then 75mg/d)
 Clopidogrel 300mg PO (then 75mg/d)
Analgesia
 Morphine 5-10mg IV
 Metoclopramide 10mg IV
Anti-ischaemia
 GTN 2 puffs or 1 tablet SL
 β-B atenolol 5mg IV (CI: asthma, LVF)
DVT Prophylaxis
Enoxaparin 40mg SC OD
Admit to CCU for monitoring
 Arrhythmias
 Continue meds except CCBs
Primary PCI or Thrombolysis
© Alasdair Scott, 2012
STEMI
Primary Percutaneous Coronary Intervention
 Rx of choice if <12h
 Angioplasty and stenting
 + GP IIb/IIIa antagonist (tirofiban) if high risk
 Delayed PCI, DM, complex procedure
Complications:
 Bleeding
 Emboli
 Arrhythmia
Thrombolysis
 CI beyond 24hrs from pain onset
ECG Criteria:
 ST elevation > 1mm in 2+ limbs or > 2mm in 2+ chest leads.
 New LBBB
 Posterior: Deep ST “depression” and tall “R” waves in V1-V3
Contraindications: AGAINST
 Aortic dissection
 GI bleeding
 Allergic reaction previously
 Iatrogenic: major surgery <14d
 Neuro: cerebral neoplasm or CVA Hx
 Severe HTN (200/120)
 Trauma, inc. CPR
st
Agents: 1 : streptokinase, alteplase (rt-PA), tenecteplase
Complications:
 Bleeding
 Stroke
 Arrhythmia
 Allergic reaction
Pts. not receiving any form of reperfusion therapy should be given
fondaparinux.
Continuing Therapy: address risk factors
 ACEi: start w/i 24hrs of MI (e.g. lisinopril 2.5mg)
 β-blocker: e.g. bisoprolol 1.25mg OD (or, CCB)
 Cardiac rehabilitation (group exercise and info) / Heart Manual
 DVT prophylaxis until fully mobile
 Continue for 3mo if large anterior MI
 Statin: regardless of basal lipids (e.g. atorvastatin 80mg)
Advice
 Stop smoking
 Diet: oily fish, fruit, veg, ↓ sat fats
 Exercise: 30min OD
 Work: return in 2mo
 Sex: avoid for 1mo
 Driving :avoid for 1mo
NB. Continue clopidogrel for 1mo following STEMI
Continue aspirin indefinitely.
251
 NSTEMI + UA

12 lead ECG + Admit to CCU

IVIV

O2 2-4L aim for SpO2 94-98%

IV access
Bloods for FBC, U+E, glucose, lipids,
Troponin

Brief Assessment
 Hx of CVD and risk factors
 CV exam

Antiplatelet
 Aspirin 300mg PO (then 75mg/d)
 Clopidogrel 300mg PO

Anti-coagulate
 Fondaparinux 2.5mg SC

Analgesia
 Morphine 5-10mg IV
 Metoclopramide 10mg IV

Anti-ischaemia
 GTN: 2 puffs or 1 tablet SL
 β-B: atenolol 50mg/24h PO (CI: asthma, LVF)
 IV GTN if pain continues

Assess Cardiovascular Risk: GRACE/TIMI

Intermediate- to High-Risk Low-Risk

 Persistent/recurrent ischaemia, ST depression, DM,
positive trop.
 GPIIb/IIIa antagonist (tirofiban)
 Angiography (±PCI) w/i 96hrs
 Clopidogrel 75mg/d for one year
 No further pain, flat or inverted T waves or normal
ECG, negative trop.
 May discharge if 12h trop is negative.
 Outpatient tests: angio, perfusion scan, stress echo

Continuing Therapy: address risk factors

 ACEi (e.g. lisinopril 2.5mg)
 β-blocker (e.g. bisoprolol 1.25mg OD) or,
 CCB (diltiazem or verapamil)
 Stop antithrombotic therapy when pain free (but give 3-5d)
 Statin (e.g. atorvastatin 80mg)

Advice as above
NB. Continue clopidogrel for 1yr following NSTEMI
Continue aspirin indefinitely.
© Alasdair Scott, 2012 252
 Severe Pulmonary Oedema

Sit pt. up Causes

¢
¢Iv¢
Cardiogenic
 MI
O2
 Arrhythmia
 15L/min via reservoir mask
 Fluid overload: renal, iatrogenic
 Target SpO2: 94-98%
Non-cardiogenic
 ARDS: sepsis, post-op, trauma
IV access + monitor ECG  Upper airway obstruction
 Bloods for FBC, U+E, troponin, BNP, ABG  Neurogenic: head injury
 Rx any arrhythmias (e.g. AF)

Symptoms
 Dyspnoea
Diamorphine 2.5-5mg IV  Orthopnea
+ Metoclopramide 10mg IV  Pink frothy sputum

Frusemide 40-80mg IV Signs

 Distressed, pale, sweaty, cyanosed
 ↑HR, ↑RR
 ↑JVP
GTN 2 puffs or 2 x 300ug tabs SL
 S3 / gallop rhythm
Unless SBP <90mmHg
IvIvIv
Iv  Bibasal creps
 Pleural effusions
 Wheeze (cardiac asthma)
Hx, Ex, Ix
 CXR: ABCDE
 ECG: MI, arrhythmias, pulsus alternans Differential
 Consider echo  Asthma/COPD
 Pneumonia
 PE
If SBP >100mmHg, start nitrate IVI
 ISMN 2-10mg/h IVI
 Keep SBP >90 Monitoring Progress
 BP
 HR and RR
If worsening, consider:  JVP
 CPAP  Urine Output
 More frusemide or ↑ nitrate infusion  ABG
 Haemofiltration / dialysis

If SBP <100mmHg: Rx as cardiogenic shock

 i.e. consider inotropes

Continuing Therapy
 Daily weights
 DVT prophylaxis
 Repeat CXR
 Change to oral frusemide or bumetanide
 ACEi + β-B if heart failure
 Consider spironolactone
 Consider digoxin ± warfarin (esp. if in AF)

Morphine in Pulmonary Oedema

 Make pt. more comfortable
 Pulm venodilators → ↓ pre-load → optimise position on Starling Curve
© Alasdair Scott, 2012 253
 Cardiogenic Shock

O2 Definition
 15L/min via reservoir mask  Inadequate tissue perfusion primarily due to
 Target SpO2: 94-98% cardiac dysfunction.
++
+++
++

Causes: MI HEART:
IV access + monitor ECG  MI
 Bloods for FBC, U+E, troponin, ABG  Hyperkalaemia (inc. electrolytes)
 Endocarditis (valve destruction)
 Aortic Dissection
Diamorphine 2.5-5mg IV (pain/anxiety)  Rhythm disturbance
+ metoclopramide 10mg IV  Tamponade

Obstructive
 Tension pneumo
Correct any:
 Massive PE
 Arrhythmias
 Electrolyte disturbance
 Acid-base abnormalities Presentation
 Unwell: pale, sweaty, cyanosed, distressed
 Cold clammy peripheries
Hx, Ex, Ix  ↑RR ± ↑HR
 CXR  Pulmonary oedema
 Echo
 Consider CT thorax (dissection/PE)

Monitoring
 CVP, BP, ABG, ECG, urine output.

Consider need for dobutamine

Rx underlying cause

Tamponade

Causes: Signs: Ix:

 Trauma  Beck’s triad: ↓BP, ↑JVP, muffled heart sounds  Echo: diagnostic
 Lung/breast Ca  Kussmaul’s sign: ↑JVP on inspiration  CXR: globular heart
 Pericarditis  Pulsus paradoxus (pulse fades on inspiration)
 MI
 Bacteria (e.g. TB)

Mx:
 ABCs
 Pericardiocentesis (preferably under echo guidance)

© Alasdair Scott, 2012 254

 Meningitis
Features
Abx Therapy
Meningitic  Community: benpen 1.2g IV/IM
 Headache  <50: ceftriaxone 2g IVI/IM BD
 Neck stiffness  >50: ceftriaxone + ampicillin 2g IVI /4h
 Kernig’s: Straightening leg c̄ hip @ 90O  If viral suspected: aciclovir
 Brudzinski’s: lifting head → lifting of legs
 Photophobia Organisms
 n/v  Viruses: enteroviruses (Coxsackie, echovirus), HSV2
 Meningococcus
Neurological
 Pneumococcus
 ↓ GCS → coma
 Listeria
 Seizures (20%)
 Haemophilus
 Focal neuro (20%): e.g. CN palsies
 TB
 Cryptococcus
Septic
 Fever
 ↓BP, ↑HR Ix
 ↑CRT  Bloods: FBC, U+Es, clotting, glucose, ABG
 Purpuric rash  Blood cultures
 DIC  LP: MCS, glucose, virology/PCR, lactate

Acute Management

ABC
 O2 15L – SpO2 94-98%
 IVI fluid resus c̄ crystalloid

Mainly Septicaemic Mainly Meningitic

 Don’t attempt LP  If no shock or CIs do LP
 Ceftriaxone 2g IVI  Dexamethasone 0.15mg/kg IV QDS
 Consider ITU if shocked  Ceftriaxone 2g IVI post-LP

Continuing Management
 Ceftriaxone 2g BD IVI
 Meningococcus: 7d IV then review
 Pneumococcus: 14d IV then review
 Maintenance fluids
 UO 30ml/h
 SBP >80mmHg
 If response is poor, consider intubation ± inotropic support
 Rifampicin prophylaxis for household contacts.

CIs to LP: Try LP Unless ContraINdicated CSF Findings

 Thrombocytopenia
Findings Bacterial TB Viral
 Lateness (delay in antibiotic admin)
Appearance Turbid Fibrin web Clear
 Pressure (signs of raised ICP)
Cells PMN Lympho / Lympho /
 Unstable (Cardio + resp systems) mononuc mononuc
 Coagulation disorder Count 100-1000 10-1000 50-1000
 Infection at LP site Glucose ↓ (< ½ ↓ (< ½ > ½ plasma
 Neurology (focal neurological signs) plasma) plasma)
Protein (g/L) ↑↑ (>1.5) ↑↑↑ (1-5) mild ↑ (<1)

© Alasdair Scott, 2012 255

Encephalitis Cerebral Abscess
Presentation Pre-disposing Factors
 Infectious prodrome: fever, rash, LNs, cold sores,  Infection: ear, sinus, dental or periodontal
conjunctivitis, meningeal signs.  Skull #
 Bizarre behaviour or personality change  Congenital heart disease
 Confusion  Endocarditis
 ↓ GCS → coma  Bronchiectasis
 Fever  Immunosuppression
 Headache
 Focal neuro Organisms
 Seizures  Frontal sinus/teeth – strep. Milleri, oropharyngeal
 Hx of travel or animal bite anaerobes
 Ear – Bacteroides, other anaerobes
Causes
Signs
Usually viral  Seizures
 HSV1/2  Fever
 CMV, EBV, VZV  Localizing signs
 Arboviruses  Signs of ↑ ICP
 HIV  Signs of infection elsewhere

Non-viral Ix
 Any bacterial meningitis  CT/MRI – ring-enhancing lesion
 TB  ↑WCC, ↑ESR
 Malaria
 Lyme disease
Rx
 Neurosurgical referral
Ix  Abx – e.g. ceftriaxone
 Bloods: cultures, viral PCR, malaria film  Treat ↑ ICP
 Contrast CT: focal bilat temporal involvement suggests
HSV
 LP: ↑ CSF protein, lymphocytes, PCR
 EEG: shows diffuse abnormalities, may confirm Dx

Mx
 Aciclovir STAT: 10mg/kg/8h IVI over 1h for 14/7
 Supportive measures in HDU/ITU
 Phenytoin for seizures

Prognosis
 70% mortality if untreated

w/o fever, consider encephalopathy

 ↓ glucose
 Hepatic
 DKA
 Drugs
 SLE
 Uraemia
 Hypoxic brain injury
 Beri-Beri

© Alasdair Scott, 2012 256

 Status Epilepticus
Definition Drugs
 Seizure lasting >30min, or
 Repeated seizures w/o intervening consciousness Lorazepam
 2-4mg IV bolus over 30s
Ix  2nd dose if no response w/i 2min
 BM  Alternatives:
 Diazepam 10mg IV/PR (20mg max)
 Bloods: glucose, ABG, U+E, FBC, Ca2+
 Midazolam 10mg buccal
 ECG, EEG
 Consider: AED levels, tox screen, LP, βhCG, CT Phenytoin
 18mg/kg IVI @ 50mg/min
 100mg/6-8h maintenance
 Monitor ECG and BP
 CI: bradycardia or heart block

Diazepam Infusion
 100mg in 500ml 5% dex @ 40ml/hr (3mg/kg/24h)

Dexamethasone
 10mg IV if vasculitis / cerebral oedema (tumour)
possible

Acute Management

ABC
 Oral / nasal airway, intubate
 Suction
 100% O2
 Capillary blood glucose

IV Access + Bloods
 U+E, LFT, FBC, Glucose, Ca2+
 AED levels
 Tox screen

Reverse Potential Causes

 Thiamine 250mg IV if EtOH
 100ml 20% glucose unless glucose
known to be normal

Slow IV Bolus Phase

 Lorazepam 2-4mg IV
 2nd dose if no response w/i 2min

IV Infusion Phase
 Phenytoin 18mg/kg IVI (then 100mg/6-8h)
 Or, diazepam 100mg in 500ml 5% dex IVI

RSI Phase
Never spend >20min c̄ someone in status w/o
getting an anaesthetist

© Alasdair Scott, 2012 257

 Head Injury
Initial Mx Intubate
 GCS ≤ 8
1O Survey  PaO2 <9KPa on air / <13KPa on O2 or PCO2 >6KPa
 A: ? intubation, immobilise C-spine  Spontaneous hyperventilation: PCO2 <4KPa
 B: 100% O2, RR  Respiratory irregularity
 C: IV access, BP, HR
 D: GCS, pupils CT head guidelines: BANGS LOC
 Treat seizures  Break: open, depressed or base of skull
 Lorazepam 2-4mg IV  Amnesia > 30min retrograde
 Phenytoin18mg/kg IVI then 100mg/6-8h  Neuro deficit or seizure
 E: expose pt. and look for other obvious injuries  GCS: <13 @ any time or <15 2h after injury
 Sickness: vomited > once
2O Survey  LOC or any amnesia and any of:
 Look for:  Dangerous mechanism: RTA, great height
 Lacerations  Age ≥ 65
 Obvious facial/skull deformity  Coagulopathy (inc. warfarin)
 CSF leak from nose or ears
 Battle’s sign, Racoon eyes
 Blood behind TM
Risk of Intracranial Haematoma in Adults:
 C-spine tenderness ± deformity  Fully conscious, no skull # = <1:1000
 Head-to-toe examination for other injuries  Confused, no skull # = 1:100
 Log role  Fully conscious, skull # = 1:30
 Confused, skull # = 1:4
Hx if possible
 How and when? GCS
 GCS and other vitals immediately after injury
 Headache, fits, vomiting, amnesia, EtOH Eyes: 4
4 – Spontaneous eye opening
Ix 3 – Open to voice
 Bloods: FBC, U+E, glucose, clotting, EtOH level, ABG 2 – Open to pain
 ? CT head + c-spine 1 – No opening

Verbal: 5
Rx 5 – Orientated conversation
 Neurosurgical opinion if signs of ↑ICP, CT evidence of 4 – Confused conversation
intracranial bleed significant skull # 3 – Inappropriate speech
 Admit if: 2 – Incomprehensible sounds
 Abnormalities on imaging 1 – No speech
 Difficult to assess: EtOH, post-ictal
 Not returned to GCS 15 after imaging Motor: 6
 CNS signs: vomiting, severe headache 6 – Obeys commands
 Neuro-obs half-hrly until GCS 15 5 – Localises pain
 GCS 4 – Withdraws to pain
 Pupils 3 – Decorticate posturing to pain (flexor)
 HR, BP 2 – Decerebrate posturing to pain (extensor)
 RR, SpO2 1 – No movement
 Temperature

Discharge Advice
 Stay with someone for first 48hrs
 Give advice card advising return on:
 Confusion, drowsiness, unconsciousness
 Visual problems
 Weakness
 Deafness
 V. painful headache that won’t go away
 Vomiting
 Fits

© Alasdair Scott, 2012 258

 Raised ICP
Types of Cerebral Oedema
1. Vasogenic (↑ cap permeability): trauma, tumour, ischaemia, infection
2. Cytotoxic: e.g. from hypoxia
3. Interstitial: e.g. obstructive hydrocephalus, ↓Na+

Causes Acute Management

 Haemorrhage
ABC
 Tumours
 Infection: meningitis, encephalitis, abscess
 Hydrocephalus
 Status Treat seizures and correct hypotension
 Cerebral oedema

Signs and Symptoms Elevate bed to 40O

 Headache
 n/v
 Seizures Neuroprotective Ventilation
 Drowsiness → coma  PaO2: >13kPa (100mmHg)
 Cushing’s reflex: ↑BP, ↓HR, irregular breathing  PCO2: 4.5kPa
 6th CN palsy (may be false localising)  Good sedation ± NM blockade
 Cheyne-Stokes respiration
 Pupils – constriction → dilatation
 Papilloedema, loss of venous pulsation @ disc
Mannitol or Hypertonic Saline
 ↓ICP short-term, but may → rebound ↑ICP later
 Mannitol 1g/kg (20% @ 5ml/kg)

Herniation Syndromes

Tonsillar (Coning)
 ↑ pressure in posterior fossa → displacement of cerebellar tonsils through foramen magnum
 → compression of brainstem and cardioresp centres in medulla
 CN6 palsy, upgoing plantars → irregular breathing → apnoea

Transtentorial / uncal
 Lateral supratentorial mass → compression of ipsilateral inferomedial temporal lobe (uncus) against free margin of
tentorium cerebelli.
 Ipsilateral CN3 palsy: mydriasis (dilation) then down-and-out
 Contralateral hemiparesis
 Compression of contralateral corticospinal tracts → ipsilateral hemiparesis (Kernohan’s Notch)

Subfalcine
 Frontal mass
 Displacement of cingulate gyrus (medial frontal lobe) under falx cerebri
 Compression of ACA → stroke
 Contralateral motor/sensory loss in legs>arms
 Abulia (pathological laziness)

© Alasdair Scott, 2012 259

 Acute Severe Asthma
Presentation Mx
 Acute breathlessness and wheeze
O2, Nebs and Steroids
Hx 1. Sit-up
 Precipitant: infection, travel, exercise? 2. 100% O2 via non-rebreathe mask (aim for 94-98%)
 Usual and recent Rx? 3. Nebulised salbutamol (5mg) and ipratropium (0.5mg)
 Previous attacks and severity: ICU? 4. Hydrocortisone 100mg IV or pred 50mg PO (or both)
 Best PEFR? 5. Write “no sedation” on drug chart

Ix
 PEFR If Life Threatening
 ABG  Inform ITU
 PaO2 usually normal or slightly ↓  MgSO4 2g IVI over 20min
 PaCO2 ↓  Nebulised salbutamol every 15min (monitor ECG)
 If PaCO2 ↑: send to ITU for ventilation
 FBC, U+E, CRP, blood cultures
If Improving
Assessment  Monitor: SpO2 @ 92-94%, PEFR
 Continue pred 50mg OD for 5 days
Severe
 Nebulised salbutamol every 4hrs
 PEFR <50%
 RR >25
 HR >110
 Can’t complete sentence in one breath IV Rx if No Improvement in 15-30min:
 Nebulised salbutamol every 15min (monitor ECG)
Life Threatening  Continue ipratropium 0.5mg 4-6hrly
 PEFR <33%  MgSO4 2g IVI over 20min
 SpO2 <92%, PCO2 >4.6kPa, PaO2 <8kPa  Salbutamol IVI 3-20ug/min
 Cyanosis  Consider Aminophylline
 Hypotension  Load: 5mg/kg IVI over 20min
 Exhaustion, confusion  Unless already on theophylline
 Silent chest, poor respiratory effort  Continue: 0.5mg/kg/hr
 Tachy-/brady-/arrhythmias  Monitor levels
 ITU transfer for invasive ventilation
Differential
 Acute exacerbation of COPD
 Pneumothorax
 Pulmonary oedema Monitoring
 PEFR every 15-30min
 Pre- and post-β agonist
Admission Criteria
 SpO2: keep >92%
 Life-threatening attack
 ABG if initial PaCO2 normal or ↑
 Feature of severe attack persisting despite initial Rx
 May discharge if PEFR > 75% 1h after initial Rx

Discharge When
 Been stable on discharge meds for 24h
 PEFR > 75% c̄ diurnal variability < 25%

Discharge Plan
 TAME pt.
 PO steroids for 5d
 GP appointment w/i 1 wk.
 Resp clinic appointment w/i 1mo

© Alasdair Scott, 2012 260

 Acute Exacerbation of COPD
Causes
 Viral URTI (30%) Management
 Also bacterial infections
Controlled O2 Therapy
Presentation  Sit-up
 Cough + sputum  24% O2 via Venturi mask: Target SpO2 88-92%,
 Breathlessness  Vary FiO2 and SpO2 target according to ABG
 Wheeze  Aim for PaO2 >8 and ↑ in PCO2 of <1.5kPa

Hx
 Smoking status
 Exercise capacity Nebulised Bronchodilators
 Current treatment  Air driven c̄ nasal specs
 Previous exacerbations  Salbutamol 5mg/4h
 Ipratropium 0.5mg/6h
Ix
 PEFR
 Bloods: FBC, U+E, ABG, CRP, cultures
 Sputum culture Steroids (IV and PO)
 CXR: infection, pneumothorax  Hydrocortisone 200mg IV
 ECG  Prednisolone 40mg PO for 7-14d

Differential
 Pneumothorax Abx
 Pulmonary oedema  If evidence of infection
 PE  Doxy 200mg PO STAT then 100mg OD PO for 5d
 Asthma

Discharge NIV if no response:

 Spirometry
 Establish optimal maintenance therapy
 GP and specialist f/up
 Prevention using home oral steroids and Abx
 Pneumococcal and Flu vaccine
 Home assessment
 Repeat nebs and consider aminophylline IV
 Consider NIV (BiPAP) if pH<7.35 and/or RR >30
 Consider invasive ventilation if pH<7.26
 Depends on pre-morbid state: exercise capacity,
home O2, comorbidity

© Alasdair Scott, 2012 261

 Pulmonary Embolism
Causes Mx
 Usually arise from DVTs in proximal leg or iliac veins
 Rarely:
O2
 Right ventricle post MI
 Sit-up
 Septic emboli in right sided endocarditis
 100% O2 via non-rebreather mask
Risk Factors: SPASMODICAL
 Sex: F
 Pregnancy Analgesia
 Age: ↑  Morphine ± metoclopramide if distressed
 Surgery (classically 10d post-op straining at stool)
 Malignancy
 Oestrogen: OCP/HRT If critically ill c̄ massive PE consider thrombolysis
 DVT/PE previous Hx
 Immobility
 Alteplase 50mg bolus stat
 Colossal size  (Surgical or interventional embolectomy)
 Antiphospholipid Abs
 Lupus Anti-coagulant
LMWH Heparin
Presentation e.g. enoxaparin 1.5mg/kg/24h SC
 Symptoms and signs depend on size, number and
distribution of emboli

Symptoms SBP?
 Dyspnoea
 Pleuritic pain
 Haemoptysis
<90 >90
 Syncope
 500ml colloid  Start Warfarin
 Confirm Dx
Signs
 Fever
 Cyanosis
 Tachycardia, tachypnoea Inotropes if BP still ↓
 RHF: hypotension, ↑JVP, loud P2  Dobutamine: aim for SBP >90
 Evidence of cause: DVT  Consider addition of NORAD
 Consider thrombolysis (medical or surgical)
Ix
 Bloods: FBC, U+E, clotting, D-dimers
 ABG: normal or ↓PaO2 and ↓PaCO2, ↑pH On-going Mx
 CXR: normal or oligaemia, linear atelectasis
 ECG: sinus tachycardia, RBBB, right ventricular strain  TEDS stockings in hospital
(inverted T in V1-V4)  Graduated compression stockings for 2yrs if DVT:
 S1, Q3, T3 is rare prevent post-phlebitic syndrome (10-30%)
 Doppler US: thigh and pelvis (+ve in 60%)  Continue LMWH until INR >2 (at least 5d)
 CTPA + venous phase of legs and pelvis  Target INR = 2-3
 85-95% sensitivity  Duration
 V/Q scan no longer used  Remedial cause: 3mo
 No identifiable cause: 6mo
Dx  On-going cause: indefinite
1. Assess probability using Wells’ Score  VC filter if repeat DVT/PE despite anticoagulation
2. Low-probability → perform D-dimers
 Negative → excludes PE
 Positive → CTPA
3. High probability → CTPA

NB. –ve D-dimer has 95% NPV for PE

Prevention
 Risk assessment for all pts
 TEDS
 Prophylactic LMWH
 Avoid OCP/HRT if @ risk

© Alasdair Scott, 2012 262

 Pneumothorax
Definition Mx
 Accumulation of air in the pleural space c̄ 2O lung
collapse.
Tension PTX
 Resuscitate pt.
Classification  No CXR
 Closed: intact chest wall and air leaks from lung into  Large bore Venflon into 2nd ICS, mid-clavicular line
pleural cavity.
 Insert ICD
 Open: defect in the chest wall allows communication
between PTX and exterior: may be sucking.
 Tension: air enters pleural cavity through one-way Traumatic
valve and cannot escape → mediastinal compression.  Resuscitate pt.
 Analgesia: e.g. morphine
Causes  3-sided wet dressing if sucking

I7
Spontaneous  Insert ICD
 1O: no underlying lung disease
 Young, thin men (ruptured subpleural bulla)
 Smokers
1O PTX
 2O: underlying lung disease
 COPD SOB and/or rim ≥2cm?
No
Consider d/c
 Marfan’s, Ehler’s Danlos ++
 Pulmonary fibrosis, sarcoidosis Yes
Yes

Trauma Aspiration successful?

 Penetrating
 Blunt ± rib #s No

Iatrogenic Insert ICD

 Subclavian CVP line insertion
 Positive pressure ventilation
 Transbronchial biospy 2O PTX
 Liver biopsy Yes
SOB and > 50yrs and Insert ICD
rim ≥2cm?
Presentation ++
No No
Symptoms
 Sudden onset Aspiration successful?
 Dyspnoea
 Pleuritic chest pain Yes
 Tension: respiratory distress, cardiac arrest
Admit for 24h
Signs
 Chest
 ↓ expansion
 Resonant percussion
 ↓ breath sounds
 ↓VR
 Tension: ↑JVP, mediastinal shift, ↑HR, ↓BP
 Crepitus: surgical emphysema

Ix
 ABG
 US
 CXR (expiratory film may be helpful)
 Translucency + collapse (2cm rim = 50% vol
loss)
 Mediastinal shift (away from PTX)
 Surgical emphysema
 Cause: rib #s, pulmonary disease (e.g. bullae)

© Alasdair Scott, 2012 263

 Upper GI Bleeding
Hx Management
 Previous bleeds
 Dyspepsia, known ulcers Resuscitate
 Liver disease or oesophageal varices  Head-down.
 Dysphagia, wt. loss  100% O2, protect airway
 Drugs and EtOH  2 x 14G cannulae + IV crystalloid infusion up to 1L.
 Co-morbidities  Bloods: FBC, U+E (↑ urea), LFTs, clotting, x-match
6u, ABG, glucose
o/e
 Signs of CLD
 PR:melaena Blood if remains shocked
 Shock?  Group specific or O- until X-matched
 Cool, clammy, CRT>2s
 ↓BP (<100) or postural hypotension (>20 drop)
 ↓ urine output (<30ml/h)
 Tachycardia Variceal Bleed
 ↓GCS  Terlipressin IV (splanchnic vasopressor)
 Prophylactic Abx: e.g. ciprofloxacin 1g/24h
Common Causes
 PUD: 40% (DU commonly)
 Acute erosions / gastritis:20% Maintenance
 Mallory-Weiss tear: 10%  Crystalloid IVI, transfuse if necessary (keep Hb≥10)
 Varices: 5%  Catheter + consider CVP (aim for >5cm H2O)
 Oesophagitis: 5%  Correct coagulopathy: vit K, FFP, platelets
 Ca Stomach / oesophagus:<3%  Thiamine if EtOH
 Notify surgeons of severe bleeds
Rockall Score: (Prof T Rockall, St. Mary’s)
 Prediction of re-bleeding and mortality
 40% of re-bleeders die
Urgent Endoscopy
 Initial score pre-endoscopy
Haemostasis of vessel or ulcer:
 Age
 Adrenaline injection
 Shock: BP, pulse
 Comorbidities  Thermal / laser coagulation
 Final score post-endoscopy  Fibrin glue
 Final Dx + evidence of recent haemorrhage  Endoclips
 Active bleeding
Variceal bleeding:
 Visible vessel
 2 of: banding, sclerotherapy, adrenaline,
 Adherent clot
coagulation
 Initial score ≥3 or final >6 are indications for surgery
 Balloon tamponade c̄ Sengstaken-Blakemore tube
 Only used if exsanguinating haemorrhage or
Oesophageal Varices failure of endoscopic therapy
 Portal HTN → dilated veins @ sites of porto-systemic
 TIPSS if bleeding can’t be stopped endoscopically
anastomosis: L. gastric and inferior oesophageal veins
 30-50% c̄ portal HTN will bleed from varices
 Overall mortality 25%: ↑ c̄ severity of liver disease.
After endoscopy
Causes of portal HTN  Omeprazole IV + continuation PO (↓s re-bleeding)
 Pre-hepatic: portal vein thrombosis  Keep NBM for 24h → clear fluids → light diet @ 48h
 Hepatic: cirrhosis (80% in UK), schisto (commonest  Daily bloods: FBC, U+E, LFT, clotting
worldwide), sarcoidosis.  H. pylori testing and eradication
 Post-hepatic: Budd-Chiari, RHF, constrict pericarditis  Stop NSAIDs, steroids et.c.

Bleed Prevention
 1 : β-B, repeat endoscopic banding
O
Indications for Surgery
 2O: β-B, repeat banding, TIPSS  Re-bleeding
 Bleeding despite transfusing 6u
Transjuglar Intrahepatic Porto-Systemic Shunt (TIPSS)
 Uncontrollable bleeding at endoscopy
 IR creates artificial channel between hepatic vein and
 Initial Rockall score ≥3, or final >6.
portal vein → ↓ portal pressure.
Open stomach, find bleeder and underrun vessel.
 Colapinto needle creates tract through liver
parenchyma which is expand using a balloon and
maintained by placement of a stent. NB. Avoid 0.9% NS in uncompensated liver disease (worsens
 Used prophylactically or acutely if endoscopic therapy ascites). Use blood or albumin for resus and 5% dex for
fails to control variceal bleeding. maintenance.
© Alasdair Scott, 2012 264
 Acute Renal Failure
Common Causes
 Pre-renal: shock (e.g. sepsis, hypovolaemia), HRS Resuscitate and Assess Fluid Status
 Renal: ATN, TIN, GN  A: ↓GCS may need airway Mx
 Post-renal: Stone, neoplasm, catheter  B: pulmonary oedema – sit up, high flow O2
 C: Assess fluid status:
CV Tissues End-organ
Presentation Postural BP CRT Mental state
 Usually presents in the context of critical illness JVP Cold / warm hands Urine output
 Uraemia HR Skin turgor
 Hyperkalaemia Mucus membranes
 Acidosis
 Oedema and ↑BP

Rx Life-Threatening Complications
Ix  Hyperkalaemia
 Bloods: FBC, U+E, LFT, glucose, clotting, Ca, ESR  Pulmonary oedema
 ABG: hypoxia (oedema), acidosis, ↑K+  Consider need for rapid dialysis
 GN screen: if cause unclear
 Urine: dip, MCS, chemistry (U+E, CRP, osmolality,
BJP) Rx Shock or Dehydration
 ECG: hyperkalaemia  Fluid challenge 250-500ml over 30min
 CXR: pulmonary oedema  Repeat as necessary: aim for CVP of 5-10cm
 Renal US: Renal size, hydronephrosis  Once replete, continue @ 20ml+UO/h

Hyperkalaemia
 ECG Features (in order) Monitor
 Peaked T waves  Cardiac monitor
 Flattened P waves  Urinary catheter
 ↑ PR interval  Consider CVP
 Widened QRS  Start fluid balance chart
 Sine-wave pattern → VF
 Mx
 10ml 10% calcium gluconate
 50ml 50% glucose + 10u insulin (Actrapid) Look for Evidence of Post-Renal Causes
 Salbutamol 5mg nebulizer  Palpable ± tender bladder
 Calcium resonium 15g PO or 30g PR  Enlarged prostate
 Haemofiltration (usually needed if anuric)  Catheter in situ
 Complete anuria

Pulmonary Oedema
 Sit up and give high-flow O2
 Morphine 2.5mg IV (± metoclopramide 10mg IV) Hx and Ix
Hx: Evidence of Acute vs. Chronic RF
 Frusemide 120-250mg IV over 1h
 Duration of symptoms
 GTN spray ± ISMN IVI (unless SBP <90)
 Co-morbidities
 If no response consider:
 CPAP  Previous blood results
 Haemofiltration / haemodialysis ± venesection
Ix
 Bloods, ABG
 Urine dip + MCS + chem
Indications for Acute Dialysis (AEIOU)
1. Persistent hyperkalaemia (>7mM)  ECG
2. Refractory pulmonary oedema  CXR and Renal US
3. Symptomatic uraemia: encephalopathy, pericarditis
4. Severe metabolic acidosis (pH <7.2)
5. Poisoning (e.g. aspirin)
Rx Sepsis
 Blood cultures and empirical Abx

Further Mx
 Call urologists if obstructed despite catheter
 Care with nephrotoxic drugs: e.g. gentamicin

© Alasdair Scott, 2012 265

 Poisoning: Recognition and Management
Drug Features Mx
Benzodiazepines ↓ GCS Flumazenil
Respiratory depression
β-Blockers Severe bradycardia or hypotension Atropine
Cyanide Inhibits the cytochrome system Dicobalt edentate
Almond smell
Phase 1: Anxiety ± confusion
Phase 2: ↓/↑ pulse
Phase 3: Fits, coma
Carbon monoxide Headache, dizziness, nausea Hyperbaric O2
Hypoxaemia (SpO2 may be normal)
Metabolic acidosis
Digoxin ↓ GCS Anti-digoxin antibodies
Yellow-green visual halos
Arrhythmias
Ethanol ↓ GCS
Respiratory depression
Ethylene glycol ↑ AG metabolic acidosis with ↑ OG Ethanol
Intoxication with no visual disturbance Haemodialysis
Found in antifreeze
Heparin Bleeding Protamine
Iron Nausea, vomiting, abdo pain Desferrioxamine
Lithium n/v Saline
Ataxia, coarse tremor
Confusion
Polyuria and renal failure
Methanol ↑ AG metabolic acidosis with ↑ OG Ethanol
Intoxication with visual disturbance Haemodialysis
Opiates Respiratory depression Naloxone
↓GCS
Pin-point pupils
Organophosphates SLUDGEM Atropine + Pralidoxime
e.g. malathion
Tricyclics ↑ QTc → Torsade de pointes Activated charcoal
Metabolic acidosis NaHCO3 IV
Anticholinergic effects
Warfarin Major bleed Vit K IV
Prothrombin complex or (FFP)

Aspirin Paracetamol
Effects Effects
 Respiratory stimulant → respiratory alkalosis  Normal metabolism overloaded and paracetamol
 Uncouples oxidative phosphorylation → met acidosis converted to highly toxic NAPQI by CyP450.
 NAPQI can be detoxified by glutathione conjugation
Presentation  Overwhelmed in OD
 Vomiting and dehydration
 Hyperventilation Presentation
 Tinnitus, vertigo  Vomiting, RUQ pain
 Hyper- or hypo-glycaemia  Jaundice and encephalopathy ± liver failure
 Respiratory alkalosis initially then lactic acidosis  Cerebral oedema → ↑ ICP
 Mixed picture usually  ↓HR, decerebrate posture, poor pupil responses

Mx Mx
 Activated charcoal if <1h since ingestion  Activated charcoal if <1h since ingestion
 Bloods  Bloods
 Paracetamol and salicylate levels  Paracetamol level 4h post ingestion
 Glucose, U+E, LFTs, INR, ABG  Glucose, U+E, LFTs, INR, ABG
 Alkalinise urine: NaHCO3 ± KCl  NAC: if levels above treatment line on graph
 Haemodialysis may be needed

© Alasdair Scott, 2012 266

 Diabetic Ketoacidosis
Pathogenesis Management
Ketogenesis
 ↓ insulin → ↑ stress hormones and ↑ glucagon Fluids
 → ↓ glucose utilisation + ↑ fat β-oxidation  0.9% NS infusion via large bore cannula
 ↑ fatty acids → ↑ ATP + generation of ketone bodies.  SBP<90 → 1L stat + more until SBP >90
 SBP>90 → 1L over 1h
Dehydration  Then: 1L over next 2h, 1L/2h, 1L/4h, 1L/4h, 1L/6h
 ↓ insulin → ↓ glucose utilisation + ↑ gluconeogenesis →  Add 10% dextrose 1L/8h when glucose <14mM
severe hyperglycaemia
 → osmotic diuresis → dehydration Start Potassium Replacement in 2nd Bag of Fluids
 Also, ↑ ketones → vomiting  >5.5mM → Nil
 3.5-5.5mM → 40mM/L
Acidosis  <3.5mM → consult senior for review
 Dehydration → renal perfusion
 Hyperkalaemia
Insulin Infusion
Precipitants  0.1u/kg/h Actrapid (6u if no wt., max 15u)
 Infection / stress ± stopping insulin
 New T1DM

Presentation Assessment
 Abdo pain + vomiting  Hx + full examination
 Gradual drowsiness  Investigations: capillary, urine, blood, imaging
 Sighing “Kussmaul” hyperventilation
 Dehydration
 Ketotic breath Additional Measures
 Urinary catheter (aim: 0.5ml/kg/hr)
Dx  NGT if vomiting or ↓GCS
 Acidosis (↑AG): pH <7.3 (± HCO3 <15mM)  Thromboprophylaxis c̄ LMWH
 Hyperglycaemia: ≥11.1mM (or known DM)  Refer to Specialist Diabetes Team
 Ketonaemia: ≥3mM (≥2+ on dipstix)  Find and treat precipitating factors

Ix
 Urine: ketones and glucose, MCS
 Cap glucose and ketones
 VBG: acidosis + ↑K
 Bloods: U+E, FBC, glucose, cultures
 CXR: evidence of infection
Subtleties
 Hyponatraemia is the norm
 Osmolar compensation for hyperglycaemia
 ↑/↔ Na indicates severe dehydration
 Avoid rapid ↓ in insulin once glucose normalised
 Glucose decreases faster than ketones and
insulin is necessary to get rid of them.
 Amylase is often ↑ (up to 10x)
 Excretion of ketones → loss of potential bicarbonate →
hyperchloraemic metabolic acidosis after Rx
Complications
 Cerebral oedema: excess fluid administration
 Commonest cause of mortality
 Aspiration pneumonia
 Hypokalaemia
 Hypophosphataemia → resp and skeletal muscle
weakness
 Thromboembolism
Monitoring
 Hrly capillary glucose and ketones
 VBG @ 60min, 2h and then 2hrly
 Plasma electrolytes 4hrly
Aims
 ↓ ketones by ≥0.5mM/h or ↑HCO3 by ≥3mM/h
 ↓ plasma glucose by ≥3mM/h
 Maintain K in normal range
 Avoid hypoglycaemia
Resolution
 Ketones <0.3mM + venous pH>7.3 (HCO3 >18mM)
 Transfer to sliding scale if not eating
 Transfer to SC insulin when eating and drinking
Transfer to SC Insulin
 When biochemically resolved and eating
 Start long-acting insulin the night before
 Give short-acting insulin before breakfast
 Stop IVI 30min after short acting

Mx: in HDU
 Gastric aspiration Pt. Education
 Rehydrate  ID precipitating factors and provide action plan
 Insulin infusion  Provision of ketone meter c̄ education on use.
 Potassium replacement
© Alasdair Scott, 2012 267
Hyperosmolar Non-Ketotic Coma Hypoglycaemia
The Patient Symptoms
 Usually T2DM, often new presentation
 Usually older Autonomic Neuroglycopenic
 Long hx (e.g. 1wk)  Sweating  Confusion
 Anxiety  Drowsiness
Metabolic Derangement  Hunger  Seizures
 Marked dehydration and glucose >35mM  Tremor  Coma
 No acidosis (no ketogenesis)  Palpitations  Personality change
 Osmolality >340mosmol/kg

Complications Cause
 Occlusive events are common: DVT, stroke  Usually Exogenous: insulin, gliclazide
 Give LMWH  Pituitary insufficiency
 Liver failure
Mx  Addison’s
 Rehydrate c̄ 0.9% NS over 48h  Insulinomas
 May need ~9L
 Wait 1h before starting insulin
 It may not be needed Mx
 Start low to avoid rapid changes in osmolality
 E.g. 1-3u/hr Alert and Orientated: Oral Carb
 Look for precipitant  Rapid acting: lucozade
 MI  Long acting: toast, sandwich
 Infection
 Bowel infarct Drowsy / confused but swallow intact: Buccal Carb
 LMWH  Hypostop / Glucogel
 Consider IV access

Unconscious or Concerned re Swallow: IV dextrose

 50ml 50% or 100ml 20% glucose

Deteriorating / refractory / insulin-induced / no access

 1mg glucagon IM/SC
 Won’t work in drunks + short duration of effect (20min)
 Insulin release may → rebound hypoglycaemia

© Alasdair Scott, 2012 268

Thyroid Storm Myxoedema Coma
Presentation Presentation
 ↑ temp  Looks hypothyroid
 Agitation, confusion, coma  Hypothermia
 Tachycardia, AF  Hypoglycaemia
 Acute abdomen  Heart failure: bradycardia and ↓BP
 Heart failure  Coma and seizures

Precipitants Precipitants
 Recent thyroid surgery or radio-iodine  Radioiodine
 Infection  Thyroidectomy
 MI  Pituitary surgery
 Trauma  Infection, trauma, MI, stroke

Mx Mx
1. Fluid resuscitation + NGT  Bloods: TFTs, FBC, U+E, glucose, cortisol
2. Bloods: TFTs + cultures if infection suspected  Correct any hypoglycaemia
3. Propranolol PO/IV  T3/T4 IV slowly (may ppt. myocardial ischaemia)
4. Digoxin may be needed  Hydrocortisone 100mg IV
5. Carbimazole then Lugol’s Iodine 4h later to inhibit  Rx hypothermia and heart failure
thyroid
6. Hydrocortisone
7. Rx cause

© Alasdair Scott, 2012 269

Addisonian Crisis Hypertensive Crisis
Presentation Cause
 Shocked: ↑HR, postural drop, oliguria, confused  Phaeochromocytoma
 Hypoglycaemia
 Usually known Addisonian or chronic steroid user Presentation
 Pallor
Precipitants  Pulsating headache
 Infection  Feeling of impending doom
 Trauma  ↑↑BP
 Surgery  ↑ ST and cardiogenic shock
 Stopping long-term steroids
Precipitants
Mx  Stress
 Bloods: cortisol, ACTH, U+E  Abdominal palpation
 Check CBG: glucose may be needed  General anaesthetic
 Hydrocortisone 100mg IV 6hrly  Parturition
 IV crystalloid  Contrast media
 Septic screen
 Treat underlying cause Rx
 Phentolamine 2-5mg IV (α-blocker) or labetalol 50mg IV
 Repeat to safe BP (e.g. 110 diastolic)
 Phenoxybenzamine 10mg/d PO when BP controlled
 May now give β-B to control tachycardia / arrhythmias
 Elective surgery after 4-6wks to allow full α-blockade
and volume expansion

NB. Never give β-B alone first

© Alasdair Scott, 2012 270

 Burns
Risk Factors Mx
 Age: children and elderly  Based on ATLS principals
 Co-morbidities: epilepsy, CVA, dementia, mental illness  Specific concerns c̄ burns
 Occupation  Secure airway
 Manage fluid loss
Classification  Prevent infection

Superficial Airway
 Erythema  Examine for respiratory burns
 Painful  Soot in oral or nasal cavity
 E.g. sunburn  Burnt nasal hairs
 Hoarse voice, stridor
Partial Thickness  Flexible laryngoscopy can be helpful
 Heal w/i 2-3wks if not complicated  Consider early intubation + dexamethasone (↓ inflam)
 Superficial
 No loss of dermis Breathing
 Painful  100% O2
 Blisters  Exclude constricting burns
 Deep  Signs of CO poisoning
 Loss of dermis but adnexae remain  Headache
 Healing from adnexae: e.g. follicles  n/v
 V. painful  Confusion
 Cherry red appearance
Full Thickness  ABG
 Complete loss of dermis  COHb level
 Charred, waxy, white, skin  SpO2 unreliable if CO poisoning
 Anaesthetic
 Heal from the edges → scar Circulation
 Fluid losses may be huge
 2x large-bore cannulae in each ACF
 Bloods: FBC, U+E, G+S/XM
Complications
 Start 2L warmed Hartmann’s immediately
Early
Parkland Formula to guide replacement in 1st 24hrs
 Infection: loss of barrier function, necrotic tissue, SIRS,
 4 x wt. (kg) x % burn = mL of Hartmann’s in 24h
hospital
 Replace fluid from time of burn
 Hypovolaemia: loss of fluid in skin + ↑ cap permeability
 Give half in 1st 8h
 Metabolic disturbance: ↑↑K, ↑↑myoglobin, ↑Hb → AKI
 Best guide is UO: 30-50mL/h
 Compartment syndrome: circumferential burns
 Peptic ulcers: Curling’s ulcers Muir and Barclay Formula to guide fluid replacement
 Pulmonary: CO poisoning, ARDS  (wt. x % burn)/2 = mL of Colloid per unit time
 Time units: 4, 4, 4, 6, 6, 12 = 36hrs total
Intermediate
 May need to use blood
 VTE
 Pressure sores Burn Treatments
 Analgesia: morphine
Late
 Dress partial thickness burns
 Scarring
 Biological: e.g. cadaveric skin
 Contractures  Synthetic
 Psychological problems  Cream: e.g. Flamazine (silver sulfadiazine) +
sterile film.
 Full thickness burns
Assessment  Tangential excision debridement
 Split-thickness skin grafts
Wallace rule of 9s: % BSA burnt  Circumferential burns may require escharotomy to
 Head and neck: 9% prevent compartment syndrome.
 Arms: 9% each  Anti-tetanus toxoid (o.5ml ATT)
 Torso: 18% front and back  Consider prophylactic Abx: esp. anti-pseudommonal
 Legs: 18% each
 Perineum: 1%
 (Palm: 1%)

NB. may also use Lund and Browder charts

© Alasdair Scott, 2012 271

 Hypothermia
Definition Ix
 Core (rectal) temperature <35 CO  Rectal / ear temperature
 FBC, U+E, glucose
 TFTs, blood gas
Pathophysiology  ECG
 Body heat is lost via 4 mechanisms  J waves: between QRS and T wave
 Arrhythmias
1. Radiation: 60%
 Infra-red emissions Mx
 Cardiac monitor
2. Conduction: 15%  Warm IVI 0.9% NS
 Direct contact  Urinary catheter
 1O means in cold water immersion  Consider Abx for prevention of pneumonia
 Routine if temp <32 and >65yrs
3. Convection: 15%
 Removes warmed air from around the body Slowly Rewarm
 ↑d in windy environments  Reheating too quickly → peripheral vasodilatation and
shock.
 Aim for 0.5 C /hr
O
4. Evaporation: 10%
 Removal of warmed water  Passive external: blankets, warm drinks
 ↑ in dry, windy environments  Active external: warm water or warmed air
 Active internal: mediastinal lavage and CPB
 Severe hypothermia only
Aetiological Classification
 Primary: environmental exposure
 Secondary: change in temperature set-point Complications
 E.g.: age-related, hypothyroidism, autonomic  Arrhythmias
neuropathy  Pneumonia
 Coagulopathy
Presentation  Acute renal failure

Mild: 32.2 – 35OC

 Shivering
 Tachycardia
 Vasoconstriction
 Apathy

Moderate: 28 – 32.2OC
 Dysrhythmia, bradycardia, hypotension
 J waves
 ↓ reflexes, dilated pupils, ↓ GCS

Severe: <28OC
 VT → VF → Cardiogenic shock
 Apnoea
 Non-reactive pupils
 Coagulopathy
 Oliguria
 Pulmonary oedema

© Alasdair Scott, 2012 272

 2010 Resuscitation
Guidelines
Resuscitation Council (UK)

Adult Advanced Life Support

Unresponsive?
Not breathing or
only occasional gasps

Call
resuscitation team

CPR 30:2
Attach defibrillator / monitor
Minimise interruptions

Assess
rhythm

Shockable Non-Shockable
(VF / Pulseless VT) (PEA / Asystole)

1 Shock Return of
150J Biphasic
spontaneous
360J Monophasic circulation

Immediately resume Immediate post cardiac Immediately resume

CPR for 2 min arrest treatment CPR for 2 min
Use ABCDE approach
Adrenaline 1mg Controlled oxygenation and Adrenaline 1mg as soon
+ amiodarone 300mg ventilation as IV access obtained
after 3rd shock 12-lead ECG
Treat precipitating cause
Repeat adrenaline every Repeat adrenaline every
Temperature control /
other cycle therapeutic hypothermia other cycle

/ During CPR \/ Reversible Causes \

Ensure high-quality CPR: 100/min, 4cm deep Hypoxia
Plan actions before interrupting CPR Hypovolaemia
Give oxygen Hypo- / hyperkalaemia / metabolic
Consider advanced airway and capnography Hypothermia
Continuous chest compressions when advanced
airway in place Thrombosis - coronary or pulmonary
Vascular access (intravenous, intraosseous) Tamponade - cardiac
Give adrenaline every 3-5 min Toxins
Correct reversible causes Tension pneumothorax
\
© Alasdair Scott, 2012
/\ / 273
 2010 Resuscitation
Guidelines
Resuscitation Council (UK)

Adult bradycardia algorithm

 Assess using the ABCDE approach

 Give oxygen if appropriate and obtain IV access
 Monitor ECG, BP, SpO2, record 12-lead ECG
 Identify and treat reversible causes
(e.g. electrolyte abnormalities)

[— i
Adverse features?
YES  Shock NO
 Syncope
 Myocardial ischaemia
 Heart failure

Atropine
500 mcg IV

Satisfactory YES
response?

l ]
NO

Interim measures: Risk of asystole?

 Atropine 500 mcg IV  Recent asystole
repeat to maximum of 3 mg  Mobitz II AV block
YES
 Isoprenaline 5 mcg min-1 IV  Complete heart block
 Adrenaline 2-10 mcg min-1 IV with broad QRS

l
 Alternative drugs *  Ventricular pause > 3 s
OR
 Transcutaneous pacing NO

Seek expert help ! Observe

Arrange transvenous pacing

{ }
* Alternatives include:
 Aminophylline
 Dopamine
 Glucagon (if beta-blocker or calcium channel blocker overdose)
 Glycopyrrolate can be used instead of atropine

© Alasdair Scott, 2012 275