Non-Parasitic Hilar Peribiliary Cyst Causing Obstructive Juandice

Abstract

Purpose: Peribiliary cysts are a poorly recognized and under-reported pathologic entity. They are believed to represent cystic dilatations of obstructed intrahepatic peribiliary glands in the periductal connective tissue. They are more commonly associated with the cirrhotic liver and with polycystic kidney disease. Typically, they are discovered incidentally at autopsy and rarely cause symptoms in healthy individuals. We present a case of a 60 year old patient in general good health presenting with obstructive jaundice and dull right upper quadrant abdominal discomfort. Radiological imaging demonstrated a solitary (7 cm) well-defined, smooth, thin-walled cystic lesion at the porta hepatis. Endoscopic retrograde cholangiopancreatography revealed lack of communication with the biliary tree. Attempted catheter drainage of the cyst yielded incomplete resolution of thecyst. Cyst fluid was negative for any fungal, bacterial or parasitic elements. Cytology was also negative. A wide excision of the roof of the cyst was then performed and the patient's jaundice resolved dramatically. Histologic examination of the cyst wall confirmed the cyst to benign. This case demonstrates an unexpected cause of obstructive jaundice in a patient without underlying hepatobiliary disease.