Dr.

Sayyam Fatima
PGT SU-I
 CONTENTS

1 Case History

2 Differential Diagnosis

3 Investigations

4 The Discussion
Sumaira w/o Aqeel ,24 years, female, married, muslim,
resident of khushab, got admitted via OPD on
16/9/2019.

1- Pain in Right Hypochondrium --- 6 months

2- Vomiting --- few days

 HOPI

 Experienced RHC pain for last 6 months

• Sudden in onset

• Moderate in intensity

• Intermittent in nature

• Dull in character

• Radiating towards back

• Relieved temporarily by analgesics

• Associated with nausea and sometimes vomiting

 HOPI cont..

 Associated with nausea and vomiting for few days

• Always associated with pain

• Non projectile

• Non bilious

• Yellowish in color

• No specific odour

• Containing undigested food particles

 HOPI cont..

• No history of fever OR malaise

• No history of yellowish discoloration of skin and sclera

• No history of pruritus

• No history of dark colored urine or clay colored stool

• No history of altered bowel habits

• No history of anorexia and undocumented weight loss

 SYTEMIC ENQUIRY

• Systemic enquiry was insignificant

 PAST HISTORY

• No h/o D.M, T.B, I.H.D, H.T.N, Asthma

• History of 1 LSCS 2 years back

 PERSONAL HISTORY

• House wife , mother of 1 child , belongs to a

middle class family

• Non addict

• Normal sleep habits

 FAMILY HISTORY

• No h/o D.M., T.B., I.H.D., H.T.N., Asthma in family

 DRUG HISTORY

• No history of any prior drug intake

• No history of allergy to any medication

GENERAL PHYSICAL EXAMINATION

A young woman of average height and built, lying comfortably

in bed, well oriented in time and space…

VITALS

• Pulse 76 beats/min, regular, normal volume

• B.P. 110/80 mm Hg

• R.R. 16 breaths/min

• Temp 98 F
• Pallor

• Jaundice

• Cyanosis -ve

• Clubbing

• Lymph nodes

• JVP

• Thyroid not enlarged

SYSTEMIC EXAMINATION

• Examination was performed:

• Informed consent

• Adequate exposure and light

• Supine Position
Gastrointestinal system

Inspection:
• Protuberent abdomen, moving with respiration, umbilicus
central & inverted, normal skin with striae gravidarum

• No visible swelling, prominent veins, peristalsis

• 1 scar mark of previous LSCS

• Hernial orifices were intact

PALPATION

• Soft & mild tenderness in right hypochondrium

• No palpable mass

• Liver palpable about 2 cm below right costal margin, non

tender, smooth surface, well defined margins

• No lymph nodes palpable

PERCUSSION AND AUSCULTATION

• Shifting dullness -ve

• Fluid thrill -ve

• Bowel sounds audible

Digital rectal examination

• Sphincter tone normal
• Soft stool in rectum
• No PR bleed or discharge
RESPIRATORY SYSTEM
• Bilateral equal air entry

• No added sounds

• Normal vesicular breathing

CARDIOVASCULAR SYSTEM
• S1 + S2 + no added sounds

CENTRAL NERVOUS SYSTEM

• Intact
 CASE SUMMARY

“A 23 years old female , presented in Surgical OPD with

complaints of intermittent right hypochondrium pain, associated

with vomating. no fever and no s/s of obstructive jaundice.

On examination, mild tenderness in Right hypochondrium and no

palpable mass.
Differential
diagnosis:
Admitted in ward for further work up &
management
WORKUP
Hb 11.6
TLC 8,200
PLT 210,000

Sr. Bilirubin 0.5

ALT 24
ALP 107
AST 32

HBsAg - ive
Anti HCV - ive
 UREA 32

CREATININE 0.8

Na+ 138

K+ 4.0

Cl- 99

PT 13/13

aPTT 36/36
ULTRASOUND ABDOMEN
(19/8/2019)
• Multiple calculi are seen in lumen of GB
• Normal wall thickness
• No pericholecystic fluid seen

• CBD 31mm. dilated in fusiform shape with multiple calculi in it.

• No intra hepatic biliary dilatation seen

IMPRESSION:

 Cholelithisis
 Choledochal cyst type 1 with
 Choledocholithiasis
MRCP (21/09/2019)

• Marked fusiform dilatation of common bile duct.

• Length of dilated segment is app. 7.6cm with max diameter of
3.9cm
• Multiple small filling defects suggesting calculi
• Few filling defects in gall bladder
• Common hepatic duct—9mm (normal upto 7mm)
• Extra pancreatic CBD—39 mm (normal upto 9mm)

IMPRESSION:
 Choledochal cyst type 1
 Cholelithiasis with choledocholithiasis
WHAT WE DECIDED…
 PRE-OPERATIVE PREPARATION

 Informed written consent obtained & patient counselled

 Anesthetist consultation sought

 Blood products arranged

 Pre-operative I.V. antibiotics

• Inj. Tienam 1 gm I.V 1 x stat

• Inj. Vancomycin 500 mg I.V 1 x stat

PER-OPERATIVE FINDINGS:
• Type1 choledochal cyst , extending upto ampulla of vater
and pancreas

• Densely adherent with deudenum

• Multiple small calculi in cyst along with greenish cystic fluid

• Friable wall of hepatic duct

Intra operatively

“Partial cyst excision+ cholecystectomy +

Roux-en-Y Hepaticojejunostomy”
• Shifted to PACU

Post operative medication…

• Nill per oral … till further orders

• Inf. R/L 1000 ml i.v. BD

• Inf. 5% D/W 1000 ml i.v OD

• Inj. Tienam 1 gm i.v. 1 x TDS

• Inf. Vancomycin 500 mg i.v. BD

• Inj. Omeprazole 40 mg i.v. 1 x OD (Inf. in Paeds chamber)

• Inj. Ketorolac 30 mg i.v. 1 x TDS

• Inj. Provas 1 g i.v TDS

• Inj. Sandostatin 50ug BD

• Cystic fluid sent for C/S

• Cyst and GB sent for histopathology

• Post ope Cbc, PT/Aptt, S.electrolytes and s.amylase levels

• 4 FFPs transfused
 POSTOPERATIVE COURSE

Patient re-explored on 6th post operative day

Per ope findings:

• 1.5 lit frank bile in abdomen

• Bile staining of abd viscera

• Site of leakage was hepaticojejunostomy with

dense adhesions around it

POSTOPERATIVE COURSE CONT…

• Post operatively patient developed sudden shortness

of breath

• Shifted to SICU

• Intubation under sedation done by anesthesia

department due to RESPIRATORY DISTRESS.

• Currently she is out of ventilatory support

• Under strict observation in SICU

Histopatology
specimen of Cyst is
“Consistent with choledochal cyst
And Negative for malignancy”
 Case

D/D

Investigations Discussion
Diagnosis
DISCUSSION
 OBJECTIVES

• Brief Overview of Choledochal cyst

• Diagnostic Workup

• Management Options

• Recent Advances
Anatomy of biliary tree
 BLOOD SUPPLY LYMPHATIC DRAINAGE
GB is supplied by cystic It drains into cystic lymph
artery –right hepatic artery nodes near neck of GB. From
here, lymph vessels pass to
Cystic vein drains directly into
hepatic nodes along the course
portal vein.
of hepatic artery and then to
NERVE SUPPLY celiac nodes.
Sympathetic and
parasympathetic vagal
fibers form celiac plexus.
GB contracts in response
to cholecystokinin produce
by mucous membrane of
deudenum on arrival of
fatty food from stomach
 INTRODUCTION

CHOLEDOCHAL CYSTS:

“Congenital dilations of intra and /or extra

hepatic biliary system are known as choledochal cysts.”

• An uncommon but correctable cause of biliary

obstruction.
 EPIDEMIOLOGY
• Can occur at any age but found more frequently in
pediatric population

INCIDENCE
• 1 in 100,000-150,000 live births in western population

• 1 in 13,500 live births in America

• Maximum incidence in Asian population 1 in 1000 live births

• Among all cases reported, 25% are detected in adults

• Unexplained female : male preponderence , reported as 4:1 to

3:1
 PATHOGENESIS

• No strong unifying etiologic theory exist for

choledochal cysts

• Most widely accepted pathogenic mechanism is

‘pancreaticobiliary malunion’

‘In pancreaticobiliary malunion, the pancreatic duct

joins the common bile duct more than 1cm proximal to ampulla
of vater ,which allows reflux of pancreatic enzymes into CBD’
PANCREATICOBILIAR MALUNION

NORMAL ANOMALOUS
 PATHOGENISIS

Babbitt’s theory of cysts:

When this malunion occus, bile duct can be
demaged in two ways:
(a) activated pancreatic enzymes cause epithelial
injury
(b) increased flow within the duct leads to an
increased in intraluminal pressure
as a result ,wall of the bile duct weakens and
dilates.

 Not all the patients with pancreaticobiliary malunion

develope CDC
 PATHOGENISIS

Congenital theory for CDC:

This theory states that embryologic over proliferation of epithelial
cells result in dilation during the cannulation period of development.

Davenport and Basu

Noted that all neonatal CCs they reviewed were cystic in nature,
and pathologically had fewer neurons and ganglions.
Their theory was that round cysts are congenital in nature, with
distal obstruction due to aganglionosis and proximal dilation (similar
to Hirschprung disease).
They believe that fusiform dilations are acquired lesions due to
APBDJ

Choledochal cyst in adults: aetiological considerations to

intrahepatic involvement.Cheng SP, Yang TL, Jeng KS, Liu
CL, Lee JJ, Liu TP
ANZ J Surg. 2004 Nov; 74(11):964-7.
[PubMed] [Ref list]
 Recent studies showed association of CDC with many
other congenital disorders. one of them is
‘Familial Adenomatous Polyposis’

Familial adenomatous polyposis is associated with mutations in

the adenomatosis polyposis coli tumour suppression gene,
which leads to interference with normal biliary cell–cell
adherence, and therefore may lead to cystic dilation.
• Reasons for the other associations remain unclear.

Review Type I choledochal cyst in association with familial adenomatous polyposis.Behrns KE, Shaheen NJ,
Grimm IS
• Am J Gastroenterol. 1998 Aug; 93(8):1377-9.
• [PubMed] [Ref list]
 CLASSIFICATION

Alonso-Lej and colleagues

proposed the first classification system for CCs
in 1959, describing 3 types of bile duct dilation

Todani and colleagues

expanded this system in 1977 to include the
occurrence of intrahepatic and multiple cysts
• most widely used classification
 TODANI CLASSIFICATION

Total 5 types:
Type I Cystic dilatation of common bile duct
(most common,50-80%)
IA: Cystic
IB: Saccular
IC: Fusiform
Type II Diverticulum of extrahepatic biliary tree
Type III Cystic dilatation of intraduodenal
portion of CBD (Chloledochocele)
Type IV Multiple cysts of intra and extra hepatic
biliary tree
IVA: Both intra and extra hepatic cysts (30-40%)
IVB: Multiple extra hepatic cysts
without intrahepatic (String of beads / bunch of
grapes appearance)
Type V Isolated intra hepatic biliary cystic disease
(Caroli’s disease / communicating cavernous
ectasia—20% )
Recent classification

• Type I- diffuse cystic dilatation

• Type II- localized dilatation of supra deudenal bile duct
• Type III- supra deudenal diverticulum
• Type IV- intra deudenal diverticulum or choledochocele
• Type V- solitary intrahepatic cyst
• Type VI- multiple intahepatic cysts(caroli disease)
• Type VII- multiple intra and extra hepatic cysts
CLINICAL PRESENTATION

Chief complaints

Infants:
- obstructive jaundice

- acholic stools

- palpable mass in RHC with hepatomegaly

Children:
- intermittent biliary obstruction with

- palpable mass in RHC and jaundice

- recurrent bouts of pancreatitis

Adults:
Classic triad—(10-20% )

- pain abdomen

- jaundice

- palpable mass in RHC

 There may be recurrent attacks of pancreatitis OR Cholangitis

Diagnosis
No laboratory studies are specific for diagnosis of choledochal cyst
but to rule out differentials
CBC:
Elevated TLC with increased and immature neutrophils –
cholangitis

Liver function studies:

Elevated hepatocellular enzymes and alkaline phosphatase
levels are non specific

Amylase and lipase levels:

Elevated in pancreatitis, biliary obstruction and cholangitis
IMAGING STUDIES:

Abdominal Ultrasonography:
• Test of choice for diagnosis of choledochal cyst
• Can be useful for antenatal disgnosis in 2nd trimester
• Sagro and colleagues detected caroli disease antenatally

ENDOSCOPIC ULTRASONOGRAPHY (EUS):

• Differentiate between choledochal and pancreatic cyst
• EUS guided fluid aspiration—differentiate between biliary cyst
and other cyctic structures

CT scanning and MRI:

• Help to delineate anatomyof leision and surrounding structures
• Detect the presence and extent of intrahepatic ductal
involvement
Magnetic resonance cholangiopancreatography:
• Gold standard
• Fitoz and colleagues reported dignostic accuracy of MRCP in
choledochal cyst –100 %
• Valuable in defining anomalous pancreaticobiliary junctions
INVASIVE DIAGNOSTIC STUDIES
PERCUTANEOUS TRANSHEPATIC CHOLANGIOGRAPHY
OR
ENDOSCOPIC RETROGRADE
CHOLANGIOPANCREATOGRAPHY
• Sensitivity –100 %
• Delineating associated extrahepatic or intrahepatic strictures
and stones
• Demonstrating presence of anamolous pancreaticobiliary
junction
MANAGEMENT

Treatment of choice for choledochal cyst is

“complete surgical excision with construction of

a biliary enteric anastomosis to restore the
continuity with gasterointestinal tract”

• Can be open or laproscopically

• No medical therapy specifically targets the etiology of
choledochal cysts

• Patients presenting at late stage( with liver cirrhosis and portal

hypertension) are not good candidates for surgery because of
prohibitive mortality and morbidity associated with these
comorbids

• Patients with cholangitis should receive broad spectrum

antibiotic therapy directed against common biliary pathogens
such as, Escherichia coli and Klebsiella
Type I + Type II
Complete excision of the involved portion of extrahepatic bile
duct
A Roux-en-Y hepaticojejunostomy is performed to restore
biliary enteric continuity

Type III
The dilated diverticulum comprising type III is excised in its
entirety
the resultant defect in CBD is closed over a T-Tube
Type IV (choledochocele)
If <3cm—endoscopic sphincterotomy
If >3cm—leisions are surgically excised with transdeudenal
approach
If pancreatic duct enters into choledochocele, it may be
reimplanted into the deudenum after cyst excision

Type V
Intrahepatic ductal disease does not require dedicated therapy
unless hepatolithiasis, intrahepatic ductal strictures and hepatic
abscess are present
In such instances ,affected segment or lobe of liver is resected
Type VI (caroli disease)
Disease limited to one hepatic lobe is amenable to treatment by
lobectomy
Bilobar disease with manifestation of liver faliure, biliary cirrhosis
or portal hypertension are candidates of liver transplantation

Type VII
Dilated extra hepatic duct is completely excised with Roun-en-Y
hepaticojejunostomy
For dilated intra hepatic portion, lobectomy or segmentectomy is
performed
LILLY Technique:
When cyst adheres densely to portal vein secondary to long lasting
inflammation.
Full thickness excision may not be possible

in this technique,
“Serosal surface of the cyst is left adherent to the portal
vein,while the mucosa of the cyst wall is obliterated by
currettage or cautery”
• Patients with choledochal cysts require life long follow up
because of increased risk of cholangiocarcinoma and
gallbladder carcinoma

Visser and colleagues

• reported a series of 39 adult patients with choledochal cysts.
Cholagiocarcinoma or gallbladder cancers were noted in 8 patients
after surgery.
• 7 of these patients had undergone partial cyst excision and one
undergone hepaticodeudenostomy
• No cancer was noted during the follow up care of patients who
underwent complete cyst excision
Shimotakhara and cowerkers
Compared Roun-en-Y hepaticojenunostomy to
hepaticodeudenostomy for biliary reconstruction following cyst
excision and concluded that

‘hepaticojejunostomy was a better choice

because of an unacceptably high rate of
deudenogastric bile reflux(33%) in
hepaticodeudenostomy group’
Recent advancement

Robotically assisted laproscopic resection of

choledochal cyst

• Relatively recent technique

• Was used in management of a 5 years old child with type I cyst
• Total robotic operative time was 390 minutes
• No complication occurred
• Patient was reported well after 6 months follow up