Choledochal cysts: Difference between revisions
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'''Choledochal cysts''' are [[congenital]] conditions associated with [[benign]] [[cyst]]ic dilatation of [[bile ducts]]. They are uncommon in western countries<ref name="pmid17825168">{{cite journal |author=Liu YB, Wang JW, Devkota KR, ''et al'' |title=Congenital choledochal cysts in adults: twenty-five-year experience |journal=Chin. Med. J. |volume=120 |issue=16 |pages=1404–7 |year=2007 |pmid=17825168 |doi=}}</ref> but not as rare in East Asian nations like Japan and China. | '''Choledochal cysts''' are [[congenital]] conditions associated with [[benign]] [[cyst]]ic dilatation of [[bile ducts]]. They are uncommon in western countries<ref name="pmid17825168">{{cite journal |author=Liu YB, Wang JW, Devkota KR, ''et al'' |title=Congenital choledochal cysts in adults: twenty-five-year experience |journal=Chin. Med. J. |volume=120 |issue=16 |pages=1404–7 |year=2007 |pmid=17825168 |doi=}}</ref> but not as rare in East Asian nations like Japan and China. | ||
== | ==Genetics== | ||
Choledochal cysts may be associated with [[Caroli disease]]. | |||
== | ==Classification scheme== | ||
Choledochal cysts were classified into 5 types by Todani in 1977<ref>{{cite journal |author=Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K |title=Congenital bile duct cysts: Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst |journal=Am. J. Surg. |volume=134 |issue=2 |pages=263-9 |year=1977 |pmid=889044}}</ref>. | |||
[[Image:Choledochal_cysts.png|thumb|300px|center|Different types of choledochal cysts]] | [[Image:Choledochal_cysts.png|thumb|300px|center|Different types of choledochal cysts]] | ||
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*'''Type IV:''' Dilatation of both intrahepatic and extrahepatic biliary duct. | *'''Type IV:''' Dilatation of both intrahepatic and extrahepatic biliary duct. | ||
*'''Type V''' or '''Caroli's disease:''' Cystic dilatation of intra hepatic biliary ducts. | *'''Type V''' or '''Caroli's disease:''' Cystic dilatation of intra hepatic biliary ducts. | ||
==Signs and Symptoms== | |||
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. A hepatic cyst may be present, as may short stature. A fever of unknown origin may be observed. [[Pancreatitis]] may be present. | |||
==Treatments== | ==Treatments== | ||
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Overview
| Choledochal cysts | |
| ICD-10 | Q44.4 |
|---|---|
| ICD-9 | 751.69 |
| DiseasesDB | 2527 |
| eMedicine | med/349 |
| MeSH | D015529 |
Template:Search infobox Choledochal cysts are congenital conditions associated with benign cystic dilatation of bile ducts. They are uncommon in western countries[1] but not as rare in East Asian nations like Japan and China.
Genetics
Choledochal cysts may be associated with Caroli disease.
Classification scheme
Choledochal cysts were classified into 5 types by Todani in 1977[2].
Classification was based on site of the cyst or dilatation. Type I to IV has been subtyped.
- Type I: Most common variety involving saccular or fusiform dilatation of a portion or entire common bile duct (CBD) with normal intrahepatic duct.
- Type II: Isolated diverticulum protruding from the CBD.
- Type III or Choledochocele: Arise from dilatation of duodenal portion of CBD or where pancreatic duct meets.
- Type IV: Dilatation of both intrahepatic and extrahepatic biliary duct.
- Type V or Caroli's disease: Cystic dilatation of intra hepatic biliary ducts.
Signs and Symptoms
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. A hepatic cyst may be present, as may short stature. A fever of unknown origin may be observed. Pancreatitis may be present.
Treatments
Choledochal cysts are treated by surgical excision of the cyst with the formation of a roux-en-Y anastamosis to the biliary duct.
Future complications include cholangitis and a 2% risk of malignancy, which may develop in any part of the biliary tree.
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.
- ↑ 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.
Template:SIB Template:Cystic diseases Template:Congenital malformations and deformations of digestive system