Congenital agenesis of the portal venous system (CAPVS) with ex-trahepatic umbilicoportosystemic (EHUPS) shunt is an extremely rare abnormality that permits the diversion of the portal flow to the systemic circulation. The absence of any flow regulation (absent ductus venosus) results in increased volume and pressure in the systemic system which may compromise the haemodynamic stability of the fetus and newborn (Gorincour et al. 2005). Correct prenatal diagnosis promotes early neonatal management and decreases morbidity. We present a case of prenatal diagnosis of EHUPS, which was confirmed and managed postnatally.
展开▼
