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. | ||
Latest revision as of 20:14, 17 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.
- Patients often present with recurrent episodes of fever/chills and abdominal pain due to cholangitis.
- Occurs in about a third.
- Portal HTN and Varices
- 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.
- Renal tubular ectasia or renal cystic disease
- More common in these patients.