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

Pediatric Organization - Pedbase [at] Gmail.com