Diagnosis and Management of a Pediatric Portacaval Shunt: An Uncommon Cause of Cyanosis in Children

Introduction: Congenital portosystemic shunts constitute a rare disorder in childhood with a wide spectrum of symptoms and signs, one of which may be cyanosis. Case Presentation: We describe for the first time in Iran a 9-year-old boy with a portacaval shunt presenting with cyanosis. He was diagnosed, managed, and treated successfully. The postoperative period was complicated by portal vein thrombosis, which was gradually resolved with appropriate treatment. Conclusions: Cyanosis is one of the hallmarks of cardiopulmonary disease; however, as was the case in our patient, a secondary cause of pulmonary disease should be considered. The portacaval shunt is a rare but important cause of cyanosis and should be considered in all patients in whom cyanosis is unexplained.