Congenital portosystemic shunts: experience of a tertiary Tunisian referral center

Abstract

BackgroundCongenital porto-systemic shunts (CPSS) are 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. Diagnosing and treating CPSS, particularly in the perinatal stage, remains nevertheless challenging, since multiple complications are possible. AimTo describe clinical characteristics of three cases of CPSS diagnosed either during pregnancy or the neonatal period, the diagnosis procedures, and their outcomes. ResultsWe reported three cases of full-term newborns with CPSS diagnosed at neonatal age. Case 1 was antenatally diagnosed with CPSS, confirmed postnatally via computed tomography, associated with malformed ductus venosus and hypoplasia of the right portal vein. Case 2 and 3 were siblings: a boy who had diffuse hemangiomatosis and CPSS complicated with severe persistent pulmonary hypertension, and a girl who presented with CPSS and Kell alloimmunization. ConclusionCPSS can be detected on prenatal ultrasounds, during the etiological workup of one of its complications, or may be incidentally identified later in life. Children with CPSS may develop various biological abnormalities, pulmonary hypertension, hypoxemia, encephalopathy, or even liver tumors. A multidisciplinary approach and standardized protocols are required to optimize the management of CPSS and minimize the short- and long-term consequences of CPSS.