title: A case of multiple hepatic peribiliary cysts which contributed to the obstructive jaundice and led to liver failure at the young man with von Recklinghausen's disease.
abstract: We treated a young man with obstructive jaundice, in whom multiple hepatic peribiliary cysts were detected at autopsy. He had been diagnosed with von Recklinghausen's disease in early childhood and had undergone surgery for a ventricular septal defect with massive blood transfusion at the age of 4 years. Examination at the age of 21 revealed prominent splenomegaly and a low platelet count. He underwent splenectomy and liver cirrhosis was confirmed by open biopsy of the liver. He was followed up at a local hospital and was admitted several times. When he was 41 years old, he was transferred to our hospital with severe jaundice and hepatic encephalopathy. Computed tomography showed marked dilation of the intrahepatic bile ducts and liver function tests showed that jaundice was mainly due to an increase of direct bilirubin. Despite endoscopic nasobiliary drainage, plasma exchange, and continuous hemodiafiltration, he died of hepatic failure after 5 days. At autopsy, multiple hepatic peribiliary cysts were found. Although peribiliary cysts have generally been considered to cause no symptoms, this report describes a patient with multiple hepatic peribiliary cysts which appear to have been responsible for the progression of obstructive jaundice.
authors.affiliation: Department of Internal Medicine, Hiroshima City Asa Hospital, 2-1-1, Kabeminami Asakita-ku, Hiroshima 731-0293, Japan.
authors.forename: Haruna
authors.initials: H
authors.lastname: Yokomichi
authors.forename: Keiji
authors.initials: K
authors.lastname: Tsuji
authors.forename: Yuzo
authors.initials: Y
authors.lastname: Hayashi
authors.forename: Mayumi
authors.initials: M
authors.lastname: Kaneko
authors.forename: Koichi
authors.initials: K
authors.lastname: Nakadoi
authors.forename: Yuki
authors.initials: Y
authors.lastname: Ishida
authors.forename: Takayasu
authors.initials: T
authors.lastname: Kuwabara
authors.forename: Takako
authors.initials: T
authors.lastname: Tsumuna
authors.forename: Tomonori
authors.initials: T
authors.lastname: Sumida
authors.forename: Shinji
authors.initials: S
authors.lastname: Nagata
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Ohgoshi
authors.forename: Sanshiro
authors.initials: S
authors.lastname: Uraki
authors.forename: Tohru
authors.initials: T
authors.lastname: Hidaka
authors.forename: Kazuaki
authors.initials: K
authors.lastname: Chayama