The surgery of "correctable" biliary atresia

Area of Science:

• Pediatric Surgery
• Hepatobiliary Diseases
• Gastroenterology

Background:

• Biliary atresia is a rare neonatal liver disease.
• A specific variant presents with microscopic bile ducts and intrahepatic biliary hypoplasia.

Purpose of the Study:

• To characterize a variant of biliary atresia.
• To evaluate surgical outcomes for this biliary atresia variant.

Main Methods:

• Retrospective review of 114 biliary atresia patients.
• Analysis of surgical interventions including choledochoenterostomy and Roux-en-Y portoenterostomy.
• Histopathological examination of excised bile duct cysts.

Main Results:

• Six percent of patients had biliary atresia with microscopic bile ducts and intrahepatic biliary hypoplasia.
• Complete surgical excision of extrahepatic ducts and Roux-en-Y portoenterostomy was performed on six patients.
• Two patients died; five showed improvement but had residual liver damage.

Conclusions:

• So-called correctable biliary atresia occurs in less than 10% of cases.
• Intrahepatic biliary hypoplasia prevents a complete "cure".
• Complete surgical excision of extrahepatic bile ducts and Roux-en-Y portoenterostomy is the treatment of choice.