Journal article
The Outcome of Patients with Cystic Biliary Atresia with Intact Proximal Hepatic Ducts Following Hepatic-Cyst-Jejunostomy
Journal of pediatric gastroenterology and nutrition, Vol.75(2), pp.131-137
08/01/2022
DOI: 10.1097/MPG.0000000000003485
PMID: 35653389
Abstract
Objectives:
To determine the outcomes of patients with cystic biliary atresia by correlating the anatomy of the hepatic ducts with the choice of biliary reconstruction surgery.
Background:
The Kasai hepatoportoenterostomy (Kasai) is the initial surgical procedure offered to most patients with biliary atresia. In contrast, a hepatic-cyst-jejunostomy has been reported to be effective in patients with the cystic form of biliary atresia.
Methods and Results:
We performed an international multicenter retrospective review. Two hundred eighty-seven patients were included, and 33 cases of cystic biliary atresia were identified. Outcomes were the serum total bilirubin level 3 months post-surgery and native liver survival at 2 years of age and were compared between cases who received the Kasai versus hepatic-cyst-jejunostomy in correlation to the anatomy of proximal hepatic ducts. The patients were categorized into 3 anatomical groups: patent intact hepatic ducts (n = 10), patent hypoplastic hepatic ducts (n = 13), and obliterated hepatic ducts (n = 10). All 10 patients with patent intact hepatic duct group underwent hepatic-cyst-jejunostomy, and 9 experienced bile drainage and native liver survival. Among the 13 patients with hypoplastic hepatic ducts, 11 underwent the Kasai procedure, and 9 had bile drainage, whereas 2 underwent hepatic-cyst-jejunostomy, and one survived with the native liver. All of the patients with obliterated hepatic ducts underwent the Kasai procedure; 5 established biliary drainage and survived with the native liver. Of 5 who did not drain, 3 underwent liver transplantation.
Conclusions:
In patients with cystic biliary atresia, the subset with a connection between cyst and intrahepatic bile ducts via intact proximal hepatic ducts had favorable clinical outcomes following hepatic-cyst-jejunostomy.
Details
- Title: Subtitle
- The Outcome of Patients with Cystic Biliary Atresia with Intact Proximal Hepatic Ducts Following Hepatic-Cyst-Jejunostomy
- Creators
- Akihiro Asai - Cincinnati Children's Hospital Medical CenterJia Feng Wu - National Taiwan University HospitalKasper S. Wang - Children's Hospital of Los AngelesAtsuyuki Yamataka - Juntendo UniversityMasaki Nio - Tohoku UniversityDa Jyun Su - National Taiwan University HospitalCelia Short - Children's Hospital of Los AngelesKoichi Tsuboi - Juntendo UniversityTakanori Ochi - Juntendo UniversityHideyuki Sasaki - Tohoku UniversityRyuji Okubo - Tohoku UniversityToshifumi Yodoshi - Cincinnati Children's Hospital Medical CenterKenichiro Konishi - Cincinnati Children's Hospital Medical CenterMichael E. Rogers - Cincinnati Children's Hospital Medical CenterGregory M. Tiao - Cincinnati Children's Hospital Medical CenterPacific Biliary Atresia Study Group (PaBAS)
- Resource Type
- Journal article
- Publication Details
- Journal of pediatric gastroenterology and nutrition, Vol.75(2), pp.131-137
- DOI
- 10.1097/MPG.0000000000003485
- PMID
- 35653389
- NLM abbreviation
- J Pediatr Gastroenterol Nutr
- ISSN
- 0277-2116
- eISSN
- 1536-4801
- Number of pages
- 7
- Language
- English
- Date published
- 08/01/2022
- Academic Unit
- Stead Family Department of Pediatrics; Gastroenterology, Hepatology, Pancreatology, and Nutrition
- Record Identifier
- 9985214103802771
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