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2014, Number 2

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Rev Latinoam Cir 2014; 4 (2)

Congenital cystic dilation of the biliary duct

Keuchkerian M, Varela D, Hernández N

Language: Spanish
References: 51
Page: 95-102
PDF size: 788.31 Kb.


ABSTRACT

Introduction: Congenital dilatation of the common bile duct (CDBD) is a rare embryological malformation and most cases are diagnosed in adults. They are usually symptomatic and at risk of developing associated cholangiocarcinoma.
Material and methods: Retrospective analysis of clinical presentation, laboratory results, surgical procedure, morbidity and mortality, extracted from medical records of patients with CDBD diagnosed between 2000 and 2014.
Results: Nine men and four women, aged between 18 and 84 years, with CDBD were identified; seven of these (54%) with exclusive extrahepatic disease, three with extra and intrahepatic unilobar involvement (23%), and the remaining three cases (23%) with diffuse Caroli’s disease. All cases were symptomatic and diagnosed by imaging. The biliopancreatic junction was possible to assess in nine cases (69%) and no cholangiocarcinoma was identified. The treatment of choice was resection for extrahepatic disease and sectoral Caroli’s disease. Biliary reconstruction was performed with hepatico/jejunostomy and Roux-in-Y intestinal loop. Diffuse Caroli’s disease was transplanted in one case. No mortality occurred. Morbidity was 23%, one patient requiring reoperation because choleperitoneum. Long term follow up was possible in nine (69%) patients with an average age of 8 years (0-14), all asymptomatic.
Conclusion: CDBD are infrequent, symptomatic, and may undergo malignant transformation. The treatment of extrahepatic forms is a total surgical resection; in the diffuse Caroli´s disease, the only solution is liver transplantation.


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