Concurrent biliary atresia and intestinal atresia: a systematic review of clinical characteristics, pathogenesis, management, and outcomes.
Zaheer Hasan, Ramjee Prasad, Om Prakash Purbey et al.
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In 30 seconds
This systematic review examined clinical features, management, and outcomes in patients with concurrent biliary atresia (BA) and intestinal atresia (IA), drawing on case reports, case series, and retrospective reviews. Fifteen studies included 98 patients. Duodenal atresia was the most common intestinal association, and mortality in combined presentations exceeded 50%. The review reports better outcomes when Kasai portoenterostomy was performed before 60 days, while concomitant short bowel could constrain Roux-en-Y limb length and increase cholangitis risk.
Key findings
- Fifteen studies comprising 98 patients were included; mortality in combined BA and IA presentations exceeded 50%.
- Duodenal atresia was the most common intestinal association; the review reports it in 0–1.5% of BA patients.
- Kasai portoenterostomy before 60 days was associated with improved outcomes.
- Concomitant short bowel may limit Roux-en-Y limb length, increasing cholangitis risk.
Why it matters
The review highlights a serious diagnostic and management challenge: BA may be overlooked when neonatal care is focused on IA, despite the high mortality reported for combined presentations. Awareness of the association and attention to biliary findings and persistent cholestasis may support earlier recognition and timely assessment for biliary intervention.
What to keep in mind
The evidence came from case reports, case series, and retrospective reviews, so the findings are based on non-randomised, potentially incomplete reports.