Concurrent biliary atresia and intestinal atresia: a systematic review of clinical characteristics, pathogenesis, management, and outcomes.

Abstract

Purpose: The coexistence of biliary atresia (BA) and intestinal atresia (IA) is exceptionally rare. Delayed recognition of BA in neonates undergoing surgery for IA may adversely affect outcomes. This systematic review aimed to evaluate the available evidence regarding the clinical presentation, pathogenesis, management strategies, and outcomes of patients with concurrent BA and IA.

Methods: A systematic literature search of PubMed, MEDLINE, and Google Scholar was conducted for studies published up to December 2025. Relevant studies encompassing case reports, case series, and retrospective reviews were identified. Data regarding demographics, type of intestinal atresia, associated anomalies, diagnostic methods, surgical management, and outcomes were extracted.

Results: Fifteen studies comprising 98 patients met inclusion criteria. The association of BA with IA is rare, affecting 0.5-5% of BA patients. Duodenal atresia (DA) represents the most common intestinal association (0-1.5% of BA), followed by jejunoileal atresia (JIA) (0.5-5%). Among 98 identified cases, mortality exceeds 50% in combined presentations. Early Kasai portoenterostomy (KPE) before 60 days improves outcomes, but concomitant short bowel limits Roux-en-Y limb length, increasing cholangitis risk.

Conclusions: Concurrent BA and IA represent a rare but severe neonatal condition associated with substantial morbidity and mortality. Careful biliary assessment during IA surgery, prompt investigation of persistent cholestasis, and timely Kasai portoenterostomy are essential for improving outcomes.