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Biliary atresia with extrahepatic biliary cysts--cholangiographic patterns influencing the prognosis
H Komuro1, S I Makino, T Momoya
1Departments of Surgery and Pediatrics, Jichi Medical School, Minamikawachi-machi, Tochigi, Japan.
Insights
Biliary atresia with extrahepatic biliary cysts can have varying outcomes. Intraoperative findings of intrahepatic biliary cysts and type III disease indicate a poor prognosis for biliary atresia patients.
Area of Science:
- Pediatric Surgery
- Hepatology
- Gastroenterology
Background:
- Biliary atresia (BA) with extrahepatic biliary cysts (EHBC) is often considered correctable with a good prognosis.
- Understanding variants of BA with EHBC is crucial for predicting outcomes.
Purpose of the Study:
- To review variants of biliary atresia with extrahepatic biliary cysts based on cholangiographic findings.
- To analyze the outcomes associated with these variants.
Main Methods:
- Retrospective review of 40 patients with BA who underwent surgery.
- Intraoperative cholangiography to classify BA with EHBC (Type I vs. Type III).
- Analysis of outcomes including bile drainage, complications, and need for liver transplantation.
Main Results:
- 6 of 8 patients with BA and EHBC had Type I BA with good bile drainage post-surgery.
- 2 patients with intrahepatic biliary cysts (IHBC) experienced recurrent cholangitis, one requiring liver transplantation.
- 2 patients with Type III BA with EHBC required revision surgery, with one achieving jaundice-free status and another succumbing to hepatic failure.
Conclusions:
- Intrahepatic biliary cysts identified during surgery are a poor prognostic factor in BA with EHBC.
- Type III biliary atresia in the presence of EHBC is associated with adverse outcomes.
- Cholangiographic findings are critical for predicting prognosis in BA with EHBC.
Purpose:
Biliary atresia (BA) with extrahepatic biliary cysts (EHBC) has been recognized generally as "correctable" BA, which indicates a good prognosis. The variants of BA with EHBC according to cholangiographic findings and their outcomes were reviewed.
Methods:
An EHBC was observed in 8 (20%) of 40 patients with BA who underwent operation at our institute. Intraoperative cholangiographic patterns included visualization of the intrahepatic bile ducts (type I BA with EHBC) in 6 patients and no visualization (type III BA with EHBC) in 2. Intrahepatic biliary cysts (IHBC) and EHBC were observed simultaneously in 2 patients diagnosed at older age. The follow-up periods ranged between 4 months and 20 years.
Results:
Good bile drainage after a hepaticoenterostomy or portoenterostomy was obtained in all 6 patients with type I BA with EHBC. Two who showed IHBC on intraoperative cholangiography had complications caused by postoperative recurrent cholangitis, which led to a liver transplantation in 1. Revision after the portoenterostomy was required in 2 patients with type III BA with EHBC. One became jaundice free after revision, whereas the other died of hepatic failure without bile drainage.
Conclusion:
Intraoperative cholangiographic findings showing IHBC and type III BA are poor prognostic factors in patients with BA with EHBC.