Portohepatic fusion mimics biliary aplasia

Area of Science:

• Pediatric Surgery
• Hepatobiliary Medicine
• Medical Imaging

Background:

• Biliary atresia is a rare neonatal condition causing jaundice.
• Persistent jaundice and elevated direct bilirubin in infants necessitate investigation.
• Atypical biliary duct anatomy presents diagnostic and surgical challenges.

Purpose of the Study:

• To report a rare case of biliary aplasia with hepatic plate-portal vein fusion.
• To highlight the successful surgical management of this rare anomaly.
• To emphasize the importance of timely intervention in biliary atresia.

Main Methods:

• Clinical presentation of persistent jaundice and elevated direct bilirubin.
• Diagnostic imaging to assess extrahepatic bile ducts.
• Intraoperative findings of absent extrahepatic biliary tree and hepatic plate-portal vein fusion.
• Modified Kasai portoenterostomy.

Main Results:

• Imaging confirmed absence of extrahepatic bile ducts, suggestive of biliary atresia.
• Intraoperative dissection revealed biliary hypoplasia and a unique hepatic plate-portal vein fusion.
• A modified Kasai portoenterostomy was successfully performed.
• The infant showed complete jaundice resolution and normalized liver function tests post-surgery.

Conclusions:

• This case presents a rare anatomical variant (hepatic plate-portal vein fusion) in biliary atresia.
• A timely modified Kasai portoenterostomy can lead to favorable outcomes even with atypical anatomy.
• This underscores the importance of thorough intraoperative assessment and tailored surgical approaches.