Caroli's disease history and symptoms: Difference between revisions

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:* 80% present before 30 years of age.
:* 80% present before 30 years of age.
* [[Cholangitis]]  
* [[Cholangitis]]  
:* Patients often present with recurrent episodes of fever/chills and abdominal pain due to cholangitis.
:* Patients often present with recurrent episodes of fever/chills and [[abdominal pain]] due to [[cholangitis]].
* Biliary lithiasis
* [[Biliary]] lithiasis
:* Occurs in about a third.
:* Occurs in about a third.
* Portal HTN and [[Varices]]
* Portal HTN and [[Varices]]
:* Occasionally patients will present later in life with sequelae of liver disease and portal HTN.
:* Occasionally patients will present later in life with sequelae of [[liver disease]] and portal HTN.
* Ductal rupture  
* Ductal rupture  
:* Has been described with intrahepatic as well as extrahepatic bile duct cysts.  This can result in peritonitis.
:* Has been described with intrahepatic as well as extrahepatic bile duct cysts.  This can result in peritonitis.
* Liver abscess  
* Liver abscess  
:* Can occur in association with the intrahepatic cysts, abnormal biliary flow, cholangitis, and/or stone disease.
:* Can occur in association with the intrahepatic [[cysts]], abnormal biliary flow, [[cholangitis]], and/or stone disease.
* Malignancy
* [[Malignancy]]
:* Is more common in the affected tissues, including cancer of the gall bladder, biliary tree, and liver.   
:* Is more common in the affected tissues, including cancer of the [[gall bladder]], [[biliary tree]], and [[liver]].   
:* This often occurs in patients ~50 years of age, about 2 decades before the mean age of carcinoma in the general population, and at a much higher rate (~7%) than the general population.   
:* This often occurs in patients ~50 years of age, about 2 decades before the mean age of [[carcinoma]] in the general population, and at a much higher rate (~7%) than the general population.   
:* The reason for this increase in carcinoma is not clear, but may occur in association with pancreatic/biliary regurgitation.   
:* The reason for this increase in [[carcinoma]] is not clear, but may occur in association with [[pancreatic]]/[[biliary]] regurgitation.   
:* Removal of cysts does not necessarily reduce the risk.
:* Removal of [[cysts]] does not necessarily reduce the risk.
:* Once malignancy develops, prognosis is dismal.
:* Once [[malignancy]] develops, prognosis is dismal.
* Renal tubular ectasia or [[renal cystic disease]]
* Renal tubular [[ectasia]] or [[renal cystic disease]]
:* More common in these patients.
:* More common in these patients.



Revision as of 20:07, 11 March 2022

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

History and Symptoms

  • Patients often present in young adulthood
  • 80% present before 30 years of age.
  • Occurs in about a third.
  • Occasionally patients will present later in life with sequelae of liver disease and portal HTN.
  • Ductal rupture
  • Has been described with intrahepatic as well as extrahepatic bile duct cysts. This can result in peritonitis.
  • Liver abscess
  • Can occur in association with the intrahepatic cysts, abnormal biliary flow, cholangitis, and/or stone disease.
  • Is more common in the affected tissues, including cancer of the gall bladder, biliary tree, and liver.
  • This often occurs in patients ~50 years of age, about 2 decades before the mean age of carcinoma in the general population, and at a much higher rate (~7%) than the general population.
  • The reason for this increase in carcinoma is not clear, but may occur in association with pancreatic/biliary regurgitation.
  • Removal of cysts does not necessarily reduce the risk.
  • Once malignancy develops, prognosis is dismal.
  • More common in these patients.

References