Pediatric presentation → abdominal mass + obstructive jaundice

Adult presentation→ biliary or pancreatic symptoms AND symptomatic cholelithiasis

Dx in adults: CT A/P

ASIANS common

Caused by reflux of pancreatic enzymes 2/2 to abnormal biliopancreatic junction, having a long common channel

• An anomalous pancreatobiliary junction (APBJ) is associated with majority of choledochal cysts
• APBJ - defined as a fused, long common pancreatic and biliary channel. This anomaly allows pancreatic enzymes to reflux into the biliary tree → inflammation → cystic degeneration

Dx: RUQ US 1st. 2nd MRCP next to define anatomy (diagnostic test of choice). Need to look at all bile ducts intra-extrahepatic

Tx:

Type I - MC 85% Saccular/fusiform dilation of CBD

Tx: CBD resection, Roux-en-y hepaticojejunostomy, and cholecystectomy

Type II - diverticulum off CBD

Tx: Resection off CBD, and close primarily. If communicating with biliary tree you will need Roux en y hepaticojejunostomy

Type III or choledochocele - Dilation of duodenal CBD or where pancreas joins CBD. Low risk of malignancy

Tx: < 3 cm - ERCP with sphincterotomy and marsupialization of the cyst

Special cases: > 3 cm → transduodenal ampullary resection + re-implantation of pancreatic duct

Type IVa - Fusiform both intra and extra hepatic

Tx: If only affecting one lobe → partial hepatectomy with hepaticoJ. Or liver transplant

Type IVb - multiple extra hepatic cysts

Tx: same as type I: CBD resection, Roux-en-y hepaticojejunostomy, and cholecystectomy

Type V (Caroli’s disease) - intrahepatic cyst only, get hepatic fibrosis, associated with congenital hepatic fibrosis and medullary sponge kidney

Tx: Partial liver resection or transplant