PEDBASE.org - The Pediatric Database - Detailed information of CHOLEDOCHAL CYST

CHOLEDOCHAL CYST

DEFINITION:

A congenital weakness in the bile duct wall resulting in an obstructive cholangiopathy and conjugated hyperbilirubinemia.

EPIDEMIOLOGY:

incidence: over 500 cases reported
age of onset:
- 1st year (22%); 1-10 years (33%); by 30 years (80%)
risk factors:
- sporadic
- F > M (4:1)
- Japanese

PATHOGENESIS:

1. Etiology

unknown; may represent a congenital weakness in the bile duct wall -> obstructive cholangiopathy -> cholestasis - a disorder causing a direct hyperbilirubinemia and jaundice due to a persistent extrahepatic cholestasis

2. Types

Type(%) - Feature

I (77%) - dilation of the common bile duct (CBD)
II (2%) - CBD or gallbladder diverticulum
III (1%) - choledochocele
IV (18%) - intrahepatic & extrahepatic bile duct cysts
V (2%) - diffuse dilatation of intrahepatic and extra hepatic bile ducts

CLINICAL FEATURES:

1. Gastrointestinal Manifestations

1. Classic Triad

Right Upper Quadrant Mass (80%)
Jaundice (75%)
Pain (60%)

characterized by intermittent episodes of pain and jaundice associated with dark urine (50%), chills (23%), acholic stools (15%), nausea/vomiting (17%)
may weigh up to 10 litres in adults

2. Complications

cholangitis
obstruction of adjacent structures (duodenum)
pancreatitis
biliary cirrhosis
portal hypertension and varices
carcinoma

INVESTIGATIONS:

1. Imaging Studies

1. Intravenous Cholangiogram

may be diagnostic

2. Others

CT, MRI, ultrasound, HIDA scan

2. Serum

conjugated hyperbilirubinemia

MANAGEMENT:

1. Surgical

1. Biliary

Intestinal Drainage Procedure
Cyst Excision + Hepatoenterostomy

2. Prognosis

death from cholangitis and biliary cirrhosis by the end of the 3rd decade unless surgically corrected

Pediatric Database - CHOLEDOCHAL CYST

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