Hepatic Lesions in Children Related to Congenital Intrahepatic Portal Venous Shunts

We present two patients with hepatic lesions associated with congenital portosystemic shunts (CPSS), a rare vascular malformation, in order to emphasize the variable clinical presentation of this condition and different management strategies in each case. CPSS can give rise to several complications such as hepatic encephalopathy, portopulmonary hypertension and hepatic tumors. These hepatic lesions though most commonly benign in nature have an increased risk of malignant transformation. Therefore, we underline the necessity of considering CPSS in the differential diagnoses for all cases of space-occupying hepatic lesions in pediatric patients.