Review of Lymphoedema Services (2005)

Lymphoedema: Diagnosis and Management in General Practice

Diagnostic delays
All types of lymphoedema= 3.3 years (average) Primary lymphoedema = 9.4 years (average)

By improving awareness in general practice

Improve access to timely and accurate diagnosis Facilitate referral for assessment and management

DHS, 2005

What is Lymphoedema?
Chronic swelling of a body part (usually limbs) caused by accumulation of fluid and protein in the tissue spaces due to a disruption in the lymphatic system 27,000 Australians are estimated to have lymphoedema

Types of Lymphoedema Primary

Caused by abnormal development of the lymphatic system May be present from:
birth (congenital), or develop in adolescence (lymphoedema praecox), or middle age (lymphoedema tarda)

International Society of Lymphology, 2003; DHS, 2005

Primary Lymphoedema

Types of Lymphoedema Secondary (most common)

Acquired following surgery, radiotherapy, trauma or other damage to the lymphatic system Can develop at any time after surgery or radiotherapy Early warning signs can be present for 3 years or more prior to the development of swelling

Provided by Dr Y. Zwar, Mercy Lymphoedema Clinic

Secondary Lymphoedema

Types of Lymphoedema Mixed

Mixed lymphoedema describes lymphatic decompensation or failure associated with: venous disease lipoedema obesity immobility chronic neurological disorders skin grafting vein stripping or harvesting arthroscopy

Provided by Dr Y. Zwar, Mercy Lymphoedema Clinic

Mixed Lymphoedema

Lipoedema
Lipoedema, which is caused by abnormal deposition of subcutaneous adipose tissue, can be misdiagnosed as lymphoedema With lipoedema:
swelling is bilateral and generally does not involve the feet which distinguishes it from lymphoedema there is pain on indent pressure and a tendency to bruise patients can have mixed lipooedema/ lymphoedema oedema develops due to overloading of the functional capacity of the normal lymphatic system
Keeley, 2006

Provided by Dr Y. Zwar, Mercy Lymphoedema Clinic

Risk factors for lymphoedema

Any surgery (not just cancer surgery) where there is damage to the lymphatic system Radiotherapy to the lymphatic system History of infection in the affected limb/body part Injury or trauma to the lymphatic system Immobility Obesity Filariasis Genetic predisposition
Lymphoedema Framework, 2006; MSAC, 2006; White et al, 2006

Cancer treatment-related risk

Reported incidence of post-treatment cancer-related lymphoedema
Breast 080% *SLNB 022%, ALND 1343.3% Prostate 2566% Melanoma 658% Uterine 18% Ovarian 7% Cervical 18% Vulval 747% Bladder 1320%

*SLNB reduces but does not eliminate risk

MSAC, 2006; White et al, 2006

Early warning signs

Transient swelling following exercise or physical activity Feelings of heaviness in the affected limb/body part Pain or tension in the affected limb/body part Tightness and fullness (a bursting feeling) in the limb/body part Clothing or jewellery becoming tighter

Case study 1 Mr FW
Presentation
49 year-old male truck driver, diabetic Presents with complaint of heaviness and persistent swelling in his legs Reports no pain in his legs Has an ulcer on right leg below his knee Overweight

Lymphoedema Framework, 2006; Piller, 2006

What would you do next?

Case study 1 Mr FW
Medical history
Swelling present for 3 months 12-year history of type 2 diabetes Patient poorly compliant with diet/exercise regimen Diabetes controlled on insulin Moderate hypertension, on combined diureticACE inhibitor therapy

Case study 1 Mr FW
Medical history (cont.)
No cancer treatment or surgery No prior injury or infection in legs (excl. ulcer) Not on any other medication

What differential diagnoses for the oedema would you consider at this stage?

What further information would you seek?

Differential diagnoses for oedema

Congestive heart failure Chronic venous insufficiency Renal dysfunction Hepatic dysfunction Hypoproteinaemia Thyroid disease Dependency or stasis oedema Drug induced (eg calcium channel blockers, steroids, NSAIDs) Lipoedema Lymphoedema
Lymphoedema Framework, 2006

Case study 1 Mr FW
Physical examination
BP 135/105 mmHg BMI 31.8 kg/m2 Legs and feet swollen Skin dry and cracked No tinea pedis No varicose veins
What else would you look for on physical examination?

Physical evaluation
Assess extent of swelling using measuring tape (www.lymphology.asn.au) Assess skin condition (dry, cracked, infection, bruising, etc) Assess subcutaneous tissue (pitting/non-pitting oedema, tissue tone, etc) Check for the presence of Stemmers sign Cardiac and respiratory parameters Examination for presence of masses

Positive Stemmers Sign

Provided by Dr Y. Zwar, Mercy Lymphoedema Clinic

Case study 1 Mr FW
Physical examination (cont.)
Cardiac and respiratory exam unremarkable No abdominal or pelvic masses palpable Lower legs and feet
marked pitting oedema/symmetrical bilaterally +ve Stemmers sign bilaterally pedal pulses palpable bilaterally

Initial investigations
Standard biochemical tests
To exclude other systemic causes of oedema

Chest X-ray
To exclude cardiac or respiratory causes of oedema

CT scan
To exclude masses/tumours

What initial diagnostic tests would you order?

Duplex scan
To exclude venous insufficiency/DVT

Investigations for lymphoedema

Lymphoscintigraphy
Detects presence and degree of lymph flow impairment Performed by a nuclear medicine specialist Radioisotope is injected into the first web space of each hand or foot Radioisotope is tracked as it moves along the lymphatic system by a gamma camera Can involve a dynamic (exercise) component in addition to a static (resting) phase Refer to centre with experience in technique
Lymphoedema Framework, 2006

Investigations for lymphoedema

Bioimpedence analysis (BIA)
Bioimpedance is a measure of the opposition of the body to an applied current Using ECG-type electrodes, a low-strength alternating current is passed through the body and the impedance to the flow of this current is measured When lymphoedema is present there is an overall increase in the total extracellular fluid content of the limb, causing swelling As fluid builds in the limb, the impedance to the current decreases and this allows the presence or progression of lymphoedema to be measured BIA can also assess whole body composition, e.g. fat-free mass and fat mass in children, adults and obese patients

Case study 1 Mr FW
Test results
FBC/U&E/LFTs/TFTs/ESR normal Glucose control OK (HbA1c = 7.4) Creatinine slightly elevated (moderate renal impairment)

Case study 1 Mr FW
Diagnosis
Referred for assessment to lymphoedema clinic Mixed lymphoedema diagnosed

What are the immediate management issues for this patient?

Management essentials
Effective management can reduce symptom severity and improve quality of life Infection control is essential to reduce the risk of developing or exacerbating lymphoedema Acknowledging patient concerns and challenges of living with lymphoedema is important and should include practical and emotional aspects

Complex Physical Therapy (CPT)

CPT is the most effective management option, and may include one or more of the following: education on care of the limb/body part including skin care physical exercises designed to improve lymphatic flow lymphatic drainage massage compression bandaging/garment individually fitted by a lymphoedema practitioner N.B. not every patient will require compression bandaging/garment

Cheville et al, 2003; Erickson et al, 2001; Fldi, 1998

Case study 1 Mr FW
Lymphoedema management plan
Patient educated on care of affected limbs, including:
Skin care to reduce risk of infection Avoiding insulin injections and other clinical procedures to affected limbs Foot care Weight control

Case study 1 Mr FW
Management plan in GP
GPMP developed with patient TCA developed with local physiotherapist and endocrinologist or diabetes educator 6-monthly review

Discuss the team-care management plan

What are the long-term management issues for this patient?

Case study 2 - Ms DN
Presentation
45 year-old female accountant Developed oedema in left arm following a bus tour of Outback Australia two months ago Left arm swelling extending from her hand to upper arm Feeling of tightness and heaviness in the arm Swelling reduces somewhat at night

Case study 2 Ms DN
Medical history
Diagnosed with left breast cancer in 2002 Lumpectomy and ALND Later received chemotherapy followed by radiotherapy to the breast Walks 5 km daily, gym work twice a week

What would you look for on physical examination?

What information will you seek from the patient?

Case study 2 - Ms DN
Physical examination
Moderate swelling of left hand and forearm 3cm difference in circumference between left and right arms Soft, pitting oedema No skin infection Skin moist and supple Breast and axillary examination - no lumps palpable BMI 23 kg/m2

Case study 2 - Ms DN
Test results
Mammogram normal CT scans of chest and abdomen clear No DVT on Duplex scan

How would you manage this patient?

What differential diagnoses for the oedema would you consider at this stage? How would you investigate the oedema?

Case study 2 - Ms DN
Referral to lymphoedema practitioner Education on care of her arm, including skin care, and exercise program
Left arm circumference reduced by 1.5 cm

Case study 2 - Ms DN
Follow-up opportunity
Patient comes into surgery 5 months later asking for antibiotics for a throat infection On questioning about her lymphoedema
Says she is sick and tired of people asking her when her arm is going to get better Tells you she has stopped going to the gym and is upset that shes putting on weight She is concerned that returning to gym work will make the swelling worse

Daily exercises and occasional lymphatic massage for maintenance

What long-term management issues may arise for this patient?

How would you respond to her concerns?

Case study 2 - Ms DN
Management intervention in GP
Liaison with lymphoedema practitioner re: guidance on returning to gym work Referral for psychosocial support (e.g. social worker at lymphoedema clinic or local psychologist) Patient put in contact with peer support group through Lymphoedema Association of Victoria

Specific management principles

Cellulitis
Be aware of increased risk of cellulitis, prevention strategies and recommended treatment regimens To treat infection with either streptococci or staphylococci:
di/flucoxacillin 500 mg orally, 6qh for 7-10 days OR clindamycin 450 mg orally, q8h for 7-10 days if allergic to penicillin
Therapeutic Guidelines: Antibiotic, 2006

Specific management principles

Specific management principles

Skincare
Good skin care to ensure healthy skin acts as a barrier to infection Patient should avoid constrictions (e.g. jewellery, tight clothes) to the affected limb(s)

Clinical procedures
Use non-affected arm/area of the body for injections, IV drips, BP readings and other clinical procedures Take care when excising skin lesions and using liquid nitrogen

Footcare
Feet should be cleaned and dried daily Treat any infection/injury promptly

Weight control
Weight management is essential as excess body weight may slow lymphatic flow

Specific management principles

Compression Garment Program

Partial financial assistance is available to pension holders and low income earners to purchase compression garments Funded by DHS and administered by the Mercy Lymphoedema Clinic (www.mercy.com.au) Victorian residents with a diagnosis of lymphoedema can access the program through a lymphoedema practitioner

Overheating
Advise patients that hot baths, spas and saunas may exacerbate any swelling Patient should avoid strenuous activities (e.g. sport, gardening) in hot weather

Travel
Patient should seek specialist advice if air travel or long-haul land trip (> 4 hrs) is planned

Lymphoedema Association of Vic

Acknowledgements
Developed with the assistance of representatives from the:
Lymphoedema Association of Victoria General Practice Divisions Victoria National Breast Cancer Centre Lymphoedema Practitioners Education Group Royal Australasian College of Surgeons

Information, education and support Referral list for local practitioners and clinics
Ph: 1300 852 850 Website: www.lav.org.au

Project funded by the Department of Human Services With thanks to all our industry sponsors for event support