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A case report: Obstructive jaundice associated with polycystic liver disease

2016; Elsevier BV; Volume: 18; Linguagem: Inglês

10.1016/j.hpb.2016.02.819

1477-2574

E. Anchante Castillo, Jorge Ferrándiz Quiroz, V. Torres Cueva, C. Yeren Paredes, Jian Ju, Verónica Paz Sihuas,

Pediatric Hepatobiliary Diseases and Treatments

Introduction: Polycystic liver disease (PCLD) is a rare autosomal dominant disorder characterized by multiple diffuse cystic lesions of the liver parenchyma. PCLD usually occurs in association with autosomal dominant polycystic kidney disease (ADPKD). Liver cysts usually remain asymptomatic. Jaundice due to biliary obstruction occurs in less than five percent of cases. We hereby report a case of ADPKD with an infrequent presentation. Methods: A female 61 y/o, history of ADPKD since 2005, was admitted with two months history of abdominal discomfort with rapidly progressing jaundice following nausea and weight loss. Liver function tests showed a picture of obstructive jaundice total bilirubin 15.38 mgr./dl, and direct bilirubin 14.4 mgr./dl. GGTP: 1136 IU/L, Renal function was normal.Ca 19–9 84 IU/l. Hepatitis virus test was negative. MR Cholangiopancreatography revealed a bilobar polycystic liver with cysts at the porta hepatis that caused a marked narrowing at bifurcation of the common hepatic duct. Mild dilation of intrahepatic right bile ducts was seen. Results: She underwent successful fenestration procedure of tense cyst al the hilius and caudate lobe. Serum bilirubin level decreased promptly to 7.57 mg/dL on a week and 1.5 mg/dL a month after. Conclusion: The commonest symptoms in patients with PLD are related to increase in liver size and include abdominal discomfort and respiratory compromise. The liver function is usually normal.Jaundice due to compression of biliary tree is an unusual feature of ADPKD. To date cyst fenestration remains the most favored option. [Figure 1]