Congenital portosystemic shunts: experience of a tertiary Tunisian referral center

Abstract

Congenital portosystemic shunt is a rare condition in which communications between the systemic venous circulation and the portal veins drain blood directly into the systemic circulation. Diagnosis may occur from the prenatal period to adulthood. Nevertheless, diagnosing and treating a congenital portosystemic shunt, particularly in the perinatal stage, remain challenging, as multiple complications can occur. This study aimed to describe the clinical characteristics of 3 cases of congenital portosystemic shunts diagnosed during pregnancy or the neonatal period, the diagnostic procedures, and their outcomes. This study reported 3 cases of full-term newborns with a congenital portosystemic shunt diagnosed at neonatal age. Case 1 was antenatally diagnosed with a congenital portosystemic shunt, which was confirmed postnatally via computed tomography and was associated with malformed ductus venosus and hypoplasia of the right portal vein. Cases 2 and 3 were siblings: a boy who had diffuse hemangiomatosis and a congenital portosystemic shunt complicated with severe persistent pulmonary hypertension and a girl who presented with a congenital portosystemic shunt and Kell alloimmunization. Congenital portosystemic shunts can be detected on prenatal ultrasounds during the etiologic workup of one of its complications or may be incidentally identified later in life. Children with congenital portosystemic shunts may develop various biological abnormalities, such as pulmonary hypertension, hypoxemia, encephalopathy, and liver tumors. A multidisciplinary approach and standardized protocols are required to optimize the management of congenital portosystemic shunts and minimize the short- and long-term consequences of congenital portosystemic shunts.