Bile duct cyst: Difference between revisions
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==Presentation== | ==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 | 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 [[abdominal pain|intermittent abdominal pain]], [[jaundice]], and a [[right upper quadrant]] [[abdominal mass]] is found only in minority of patients. | ||
==References== | ==References== | ||
Revision as of 01:06, 8 April 2009
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| 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.
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MRI - T2: Type 4 bile duct cyst
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MRI - T2: Type 4 bile duct cyst
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MRI - T2: Type 4 bile duct cyst
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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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