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

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

Research output: Contribution to journalArticle

8 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)2312-2315
Number of pages4
JournalJournal of Pediatric Surgery
Volume44
Issue number12
DOIs
Publication statusPublished - Dec 1 2009

Fingerprint

Biliary Atresia
Cysts
Jaundice
Bilirubin
Satellite Hospitals
Cholangitis
Cholangiography
Ileus
Living Donors
Splenomegaly
Operative Surgical Procedures
Methylprednisolone
Varicose Veins
Prednisolone
Pulmonary Hypertension
Liver Transplantation
Survival Rate
Liver

All Science Journal Classification (ASJC) codes

  • Surgery
  • Pediatrics, Perinatology, and Child Health

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Excellent long-term outcome of hepaticojejunostomy for biliary atresia with a hilar cyst. / Takahashi, Yukiko; Matsuura, Toshiharu; Saeki, Isamu; Zaizen, Yoshio; Taguchi, Tomoaki.

In: Journal of Pediatric Surgery, Vol. 44, No. 12, 01.12.2009, p. 2312-2315.

Research output: Contribution to journalArticle

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abstract = "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.",
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