Bile duct cyst

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	Bile duct cyst;
	MRCP: Type 4 bile duct cyst. ; Image courtesy of RadsWiki

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Overview

Bile duct cysts are congenital conditions associated with benign cystic dilatation of bile ducts. They are uncommon in western countries^[1] ^[2] but not as rare in East Asian nations like Japan and China.

Classification

According to the Todani system, there are five types of bile duct cysts.^[3].

Type 1: Choledochal Cyst

Account for 80% to 90% of all bile duct cysts
Characterized by fusiform dilation of the extrahepatic bile duct
Theorized that choledochal cysts form as the result of reflux of pancreatic secretions into the bile duct via anomalous pancreaticobiliary junction.
Cyst should be resected completely to prevent associated complications (i.e. ascending cholangitis and malignant transformation).

Type 2: Diverticulum

Accounts for 3% of all bile duct cysts
Represents a true diverticulum.
Saccular outpouchings arising from the supraduodenal extrahepatic bile duct or the intrahepatic bile ducts.

Type 3: Choledochocele

Accounts for 5% of all bile duct cysts
Represents protrusion of a focally dilated, intramural segment of the distal common bile duct into the duodenum.
Choledochoceles may be successfully managed with endoscopic sphincterotomy, surgical excision, or both, in symptomatic patients.

Type 4: Multiple Communicating Intra and Extrahepatic Duct Cysts

Second most common type of bile duct cysts (10%)
Subdivided into subtypes A and B.
- Type 4A: Fusiform dilation of the entire extrahepatic bile duct with extension of dilation of the intrahepatic bile ducts
- Type 4B: Multiple cystic dilations involving only the extrahepatic bile duct.

Images courtesy of RadsWiki

MRI - T2: Type 4 bile duct cyst
MRI - T2: Type 4 bile duct cyst
MRI - T2: Type 4 bile duct cyst
MRCP: Type 4 bile duct cyst

Type 5: Caroli's Disease

Caroli's disease is a rare form of congenital biliary cystic disease manifested by cystic dilations of intrahepatic bile ducts
Association with benign renal tubular ectasia and other forms of renal cystic disease.

Presentation

Most of them present in 1st year of life; adult presentation is rare and usually at this stage is associated with complication . Classic triad of intermittent abdominal pain, jaundice, and a right upper quadrant abdominal mass is found only in minority of patients.

References

↑ Liu YB, Wang JW, Devkota KR; et al. (2007). "Congenital choledochal cysts in adults: twenty-five-year experience". Chin. Med. J. 120 (16): 1404–7. PMID 17825168.
↑ Yu, Jinxing, Turner, Mary Ann, Fulcher, Ann S., Halvorsen, Robert A. Congenital Anomalies and Normal Variants of the Pancreaticobiliary Tract and the Pancreas in Adults: Part 1, Biliary Tract. Am. J. Roentgenol. 2006 187: 1536-1543
↑ Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K (1977). "Congenital bile duct cysts: Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst". Am. J. Surg. 134 (2): 263–9. PMID 889044.

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[pmid17825168-1] Liu YB, Wang JW, Devkota KR; et al. (2007). "Congenital choledochal cysts in adults: twenty-five-year experience". Chin. Med. J. 120 (16): 1404–7. PMID 17825168.

[2] Yu, Jinxing, Turner, Mary Ann, Fulcher, Ann S., Halvorsen, Robert A. Congenital Anomalies and Normal Variants of the Pancreaticobiliary Tract and the Pancreas in Adults: Part 1, Biliary Tract. Am. J. Roentgenol. 2006 187: 1536-1543

[3] Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K (1977). "Congenital bile duct cysts: Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst". Am. J. Surg. 134 (2): 263–9. PMID 889044.

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