Type IVA choledochal cyst: a rare cause of cirrhosis in adults

Chantelli Iamblaudiot Razafindrazoto,Lova Hasina Ny Ony Narindra Rajaonarison,Andry Lalaina Rinà Rakotozafindrabe,Soloniaina Hélio Razafimahefa,Sonny Maherison,Domoina Harivonjy Hasina Laingonirina,Sakaiza Malala Randrianambininjanahary,Rado Manitrala Ramanampamonjy,Nitah Harivony Randriamifidy,Tovo Harimanana Rabenjanahary

doi:10.1186/s43162-022-00102-2

Chantelli Iamblaudiot Razafindrazoto, Lova Hasina Ny Ony Narindra Rajaonarison + Show 8 more

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https://doi.org/10.1186/s43162-022-00102-2

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Abstract

BackgroundCholedochal cysts (CDCs) are characterized by congenital cystic dilations of the intrahepatic or extrahepatic bile ducts or both. CDCs are very rarely reported in sub-Saharan Africa. This congenital anomaly of the bile ducts is rarely discovered at the stage of advanced chronic liver disease with portal hypertension. We report the first Malagasy case of a type IVA choledochal cyst discovered during an etiological work-up of cirrhosis.Case presentationA 23-year-old woman was hospitalized for jaundice and ascites. Biological and radiological investigations have led to the diagnosis of secondary biliary cirrhosis due to type IVA choledochal cyst. In view of the severity of the liver damage (Child-Pugh C score), a collegial decision (hepato-gastroenterologists and visceral surgeons) had rejected surgical intervention and opted only for the management of cirrhotic decompensation.ConclusionA type IVA choledochal cyst is a possible etiology of advanced chronic liver disease.

Highlights

Choledochal cysts (CDCs) are characterized by congenital cystic dilations of the intrahepatic or extrahepatic bile ducts or both
A type IVA choledochal cyst is a possible etiology of advanced chronic liver disease
We report the first Malagasy case of choledochal cyst (CDC) at the stage of secondary biliary cirrhosis (SBC) with portal hypertension

Summary

Introduction

Choledochal cysts (CDCs) are characterized by congenital cystic dilations of the intrahepatic or extrahepatic bile ducts or both. Conclusion: A type IVA choledochal cyst is a possible etiology of advanced chronic liver disease. Background Choledochal cysts (CDCs) are rare diseases of the bile ducts, characterized by congenital cystic dilation of the intrahepatic or extrahepatic bile ducts or both [1]. The etiology of these cysts remains unknown, but they are considered congenital as approximately 75% of cases are observed in children under 10 years of age.

Results

Conclusion