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Published on: February 28, 2025
The Outcome of Patients With Cystic Biliary Atresia With Intact Proximal Hepatic Ducts Following
Akihiro Asai1,2, Jia-Feng Wu3, Kasper S Wang4,5
1From the Division of Gastroenterology, Hepatology, and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
Insights
For cystic biliary atresia, hepatic-cyst-jejunostomy offers favorable outcomes in patients with intact hepatic ducts. This surgical approach improves bile drainage and native liver survival in select pediatric cases.
Area of Science:
- Pediatric Surgery
- Hepatobiliary Surgery
- Congenital Anomalies
Background:
- Biliary atresia is a neonatal liver disease requiring surgical intervention.
- The Kasai hepatoportoenterostomy is a common initial procedure.
- Cystic biliary atresia presents unique anatomical considerations.
Purpose of the Study:
- To correlate hepatic duct anatomy with surgical outcomes in cystic biliary atresia.
- To compare the efficacy of Kasai hepatoportoenterostomy versus hepatic-cyst-jejunostomy.
- To identify optimal surgical strategies for improved patient outcomes.
Main Methods:
- International multicenter retrospective review of 287 patients.
- Identification of 33 cases with cystic biliary atresia.
- Categorization based on proximal hepatic duct anatomy: intact, hypoplastic, or obliterated.
Main Results:
- Patients with intact hepatic ducts undergoing hepatic-cyst-jejunostomy showed favorable bile drainage and native liver survival.
- Outcomes varied for hypoplastic hepatic ducts depending on the surgical procedure.
- Obliterated hepatic ducts primarily underwent Kasai hepatoportoenterostomy, with some requiring liver transplantation.
Conclusions:
- Hepatic-cyst-jejunostomy is associated with favorable outcomes in cystic biliary atresia with intact proximal hepatic ducts.
- Surgical strategy should be tailored to specific hepatic duct anatomy.
- Accurate anatomical assessment is crucial for optimizing surgical success.
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.
