Biliary Atresia - Clinical Series

Bárbara Neto1, Mariana Borges-Dias1, Eunice Trindade2

  • 1Department of Pediatric Surgery, Centro Hospitalar São João, Faculdade de Medicina da Universidade do Porto, Porto, Portugal.

Insights

Biliary atresia treatment in children shows improved surgical success and survival rates. Early Kasai portoenterostomy before 72 days and avoiding recurrent cholangitis are key for better prognosis in pediatric liver disease.

Area of Science:

  • Pediatric Hepatology
  • Surgical Gastroenterology
  • Pediatric Liver Transplantation

Background:

  • Biliary atresia is a leading cause of pediatric liver failure and the primary indication for liver transplantation.
  • This study focuses on patients diagnosed and treated for biliary atresia between 2000 and 2015.

Purpose of the Study:

  • To analyze the epidemiological, clinical, biochemical, and imaging data of pediatric patients with biliary atresia.
  • To evaluate the outcomes, complications, and survival rates in this patient cohort.
  • To identify prognostic factors influencing the disease's progression and treatment success.

Main Methods:

  • A descriptive, observational, and retrospective study design was employed.
  • Data collected included patient demographics, clinical presentation, biochemical markers, imaging results, and treatment outcomes.
  • Analysis focused on patients diagnosed and treated between January 1, 2000, and December 31, 2015.

Main Results:

  • Eighteen patients were evaluated, with a median age of 63 days at Kasai portoenterostomy.
  • Surgical success was achieved in two-thirds of cases, with better prognosis for those operated on before 72 days.
  • Survival rates with native liver were 77.8% at 1 year, 72.2% at 5 years, and 64.2% at 10 years; recurrent cholangitis was associated with survival.

Conclusions:

  • The study observed higher surgical success and long-term survival rates compared to many published series.
  • Age at surgery and recurrent cholangitis were identified as significant prognostic factors.
  • Despite a small patient cohort, outcomes align with those reported by major reference centers.
Abstract

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