Hilar cysts in jaundiced neonates: Diagnostic challenges and surgical outcomes at a National Pediatric Referral Center in Colombia.
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OBJECTIVES: The cystic variant of biliary atresia (CBA) accounts for 5% to 10% of all cases. Although traditionally considered a condition with a more favorable prognosis, outcomes depend on the patient's age at the time of surgical diversion. This study aims to characterize outcomes of CBA patients at a high-volume national referral center. MATERIAL AND METHODS: We conducted a case series of patients diagnosed with cystic biliary atresia between 2020 and 2024 at Fundación Cardioinfantil (Bogotá, Colombia), a national pediatric referral center receiving patients from all regions of the country. Demographic, clinical, and therapeutic variables were described, along with patient outcomes, and disease progression was analyzed on a case-by-case basis. RESULTS: During the study period, our institution treated 46 patients with biliary atresia, of whom five (10.9%) presented the cystic variant and were included in this series. In addition to jaundice and acholic stools, initial presentations involved biliary ascites and a presumed retroperitoneal neoplasm with abdominal compartment syndrome. The mean TB at admission was 6.03 mg/dL (SD 2.06 mg/dL), while the mean DB was 3.33 mg/dL (SD 1.68 mg/dL). The average cyst diameter was 28 mm (SD 11.1). The median age at KPE was 29 days (14-67 days). Jaundice clearance was achieved within six months. No patients required liver transplantation or died during follow-up. CONCLUSIONS: This report is the first case series describing the management of hilar cysts in jaundiced neonates in Colombia, underscoring the diagnostic overlap between cystic biliary atresia and neonatal choledochal malformation. Our findings support the value of centralized, high-volume referral centers in achieving early diagnosis and favorable outcomes for this rare condition. We conclude that the presence of a hepatic hilar cyst in a jaundiced newborn should be presumed to be CBA until proven otherwise.