Type 1 Congenital Extrahepatic Portosystemic Shunt

Abstract

ongenital extrahepatic portosystemic shunts (CEPS) are rare. Only 60 to 70 cases are reported in the literature. 1 Afflicted patients have a wide range of ages, from 31 weeks in utero to 76 years. Morgan and Superina 2 proposed a classification system for these shunts in 1994 that placed these anomalies into 2 classes. A type 1 shunt is associated with aplasia of the intrahepatic portal system, whereas a type 2 shunt has an intact intrahepatic portal system. The type 1 CEPS has a female predominance, whereas the type 2 CEPS has no sex predilection.A CEPS is important to recognize for several reasons. First, there is a strong association with cardiac, urinary tract, and skeletal anomalies as well as biliary atresia and polysplenia. 1 Second, substantial mesenteric shunting results in elevated levels of toxins and ammonia, causing encephalopathy and central nervous system damage. Third, there is an increased incidence of hepatic tumors, including hepatocellular carcinoma, hepatoblastoma, and sarcoma.Most cases of CEPS are discovered in the postnatal period, when imaging is done while investigating a cardiac anomaly or abnormal liver function test results. The remainder are discovered during evaluation of patients with hepatic encephalopathy and hyperammonemia. Here we report a case of a type 1 CEPS detected by sonography associated with portal venous atresia. Abbreviations CEPS, congenital extrahepatic portosystemic shunt

Prenatal diagnosis of extrahepatic umbilicoportosystemic shunt: impact on postnatal management

“…Aft er birth, neonates with PSS can develop several complications, including worsening CHF, pulmonary hypertension, jaundice, elevated liver enzymes, progressive encephalopathy and liver tumours (Acherman et al 2007;Uchino et al 1999;Achiron et al 2009;Sista and Filly 2009). Th erefore, treatment should be attempted early to avoid long-term complications.…”