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

Congenital portosystemic shunts constitute a rare disorder in childhood with a wide spectrum of symptoms and signs, one of which may be cyanosis.
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.
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.