Congenital portosystemic shunt in children: Case reports

Abstract

Congenital portosystemic shunt is a rare vascular malformation characterized by an abnormal connection between the portal venous system and the systemic venous system. This results in partial or complete diversion of blood flow from the intestines and spleen into systemic circulation, bypassing the liver. The condition can be classified into four types according to the Bicêtre classification system, which aids in diagnosis and treatment planning. Major complications include liver disease, hepatic tumors, pulmonary arterial hypertension, and pulmonary arteriovenous shunting. Imaging plays a crucial role in diagnosing congenital portosystemic shunts and associated anomalies. Computed tomography is considered the first-line modality for identifying the shunt and its type, allowing for detailed visualization of blood vessels and small hepatic lesions. Digital subtraction angiography is essential in evaluating the feasibility of shunt closure. Treatment decisions depend on the shunt type and clinical presentation, and may involve surgical or endovascular shunt occlusion. In this report, we present three confirmed cases of congenital portosystemic shunt diagnosed via CT imaging. One case involved a type 2 side-to-side shunt (according to the Bicêtre classification) successfully treated with endovascular intervention; another was a type 4 shunt associated with multiple hepatic adenomas; and the third was a type 2 end-to-side shunt with coexisting congenital heart defects, which was successfully managed by open surgical shunt banding.