Excellent long-term outcome of hepaticojejunostomy for biliary atresia with a hilar cyst

Yukiko Takahashi, Toshiharu Matsuura, Isamu Saeki, Yoshio Zaizen, Tomoaki Taguchi

研究成果: ジャーナルへの寄稿学術誌査読

15 被引用数 (Scopus)


Background: Biliary atresia (BA) with hilar cyst is an uncommon variant, which constitutes less than 10% of all types of BA, and the operative procedure for this type of BA remains controversial. Methods: We have had 200 cases with BA from 1963 to 2008 in our institute and our branch hospitals, in which 12 cases (6%) were BA with a hilar cyst. The clinical records of all cases were evaluated retrospectively. Results: Twelve BA patients with a hilar cyst included 2 boys and 10 girls. The diagnosis of BA was confirmed by intraoperative cholangiography (cloudy or treelike pattern). In all 12 cases, a hepaticojejunostomy was performed at a median age of 71.6 days (range, 24-136 days). The follow-up periods were 1.2 to 23.2 years. The current mean total bilirubin level was 0.8 mg/dL (range, 0.2-3.5 mg/dL), and the mean direct bilirubin level was 0.2 mg/dL (range, 0.0 to 0.8 mg/dL). Methylprednisolone or oral prednisolone was administered in 8 cases after operation, and 10 of 12 cases achieved a jaundice free state. The postoperative complications were cholangitis (n = 10), gastroesophageal varices (n = 7), splenomegaly (n = 3), ileus (n = 1), and pulmonary hypertension (n = 1). The overall survival rate with a native liver was 10 (83.3%) of 12 cases, of which 9 cases were jaundice-free and only 1 case showed recurrent jaundice. The other 2 cases underwent living-donor liver transplantation at age of 2 and 20 years, respectively, and they are currently doing well. Conclusions: Most cases of BA with a hilar cyst achieved excellent clinical outcome after a hepaticojejunostomy.

ジャーナルJournal of Pediatric Surgery
出版ステータス出版済み - 12月 2009

!!!All Science Journal Classification (ASJC) codes

  • 外科
  • 小児科学、周産期医学および子どもの健康


「Excellent long-term outcome of hepaticojejunostomy for biliary atresia with a hilar cyst」の研究トピックを掘り下げます。これらがまとまってユニークなフィンガープリントを構成します。