Successful surgical intervention and management of biliary atresia in a 4-month-old infant: a case report.

INTRODUCTION AND IMPORTANCE: Biliary atresia (BA) is a progressive fibro-obliterative disorder of the biliary tract that causes cholestasis, cirrhosis, and liver failure if left untreated. Kasai portoenterostomy (KP) is the standard initial treatment, with better outcomes when performed early. We report a rare case of successful surgical management of late-presenting BA at 4 months of age in a resource-limited setting. CASE PRESENTATION: A 4-month-old girl presented with persistent jaundice, pale stools, dark urine, and hepatomegaly. She had previously been treated for neonatal hepatitis without improvement. Laboratory evaluation showed conjugated hyperbilirubinemia (total bilirubin 18.8 mg/dL, direct bilirubin 17.2 mg/dL). Ultrasonography demonstrated an atretic gallbladder and a triangular cord sign suggestive of BA. Due to limited access to advanced diagnostic modalities, the diagnosis was established based on clinical, laboratory, ultra-sonographic, and intraoperative findings. Extended hilar dissection and a wide Roux-en-Y hepatic portoenterostomy were performed. Postoperative management included corticosteroids and ursodeoxycholic acid. Progressive improvement in liver function tests and clinical status was observed, with complete resolution of jaundice during follow-up. CLINICAL DISCUSSION: Although KP performed within 60-90 days provides the best outcomes, selected late-presenting patients may still achieve successful bile drainage. This case emphasizes the importance of careful patient selection, meticulous surgical technique, and optimized postoperative management, even beyond the traditional therapeutic window. CONCLUSION: Delayed presentation should not automatically exclude infants with BA from surgical treatment. Early recognition, referral, and appropriate surgical management remain essential to improve outcomes in late-presenting cases.