AI Summary & Key Takeaways
AI InsightAssociated congenital anomalies were frequently observed, supporting consideration of multidisciplinary multisystem evaluation in children diagnosed with CPSS.
📌 3 Key Takeaways
Abstract
Aims: Congenital portosystemic shunts (CPSS) are rare vascular malformations diverting portal venous blood into the systemic circulation. Data describing their clinical spectrum, associated congenital anomalies, and natural history in children remain limited. Methods: We retrospectively evaluated nine pediatric patients diagnosed with CPSS at the Department of Pediatric Gastroenterology, Ankara Etlik City Hospital, between January 2023 and October 2025. Demographic, clinical, laboratory, radiological, and follow-up data were analyzed descriptively. Results: Seven patients (77.8%) had intrahepatic and two (22.2%) had extrahepatic (Abernethy type II) shunts. The most common presentations were neonatal hypoxemia/cyanosis (4/9), cholestasis (3/9), and hypoglycemia (2/9). Associated congenital anomalies, most frequently cardiovascular, hepatobiliary, and renal, were identified in eight of nine patients (88.9%). During follow-up (mean 21.6±14.1 months), complete spontaneous closure occurred in four of seven intrahepatic shunts (57.1%) and regression in two (28.6%), whereas both extrahepatic shunts remained patent. Conclusion: In this small series, intrahepatic CPSS diagnosed in infancy frequently demonstrated spontaneous closure or regression, whereas both extrahepatic shunts remained patent during follow-up, consistent with previous reports, although this finding should be interpreted cautiously given the small number of extrahepatic cases. Associated congenital anomalies were frequently observed, supporting consideration of multidisciplinary multisystem evaluation in children diagnosed with CPSS.