제대 정맥 기형에 의한 비면역성 태아수종 1례 = A Case of Non-Immune Hydrops Fetalis due to Umbilical Venous Malformation and Noonan Syndrome

다국어 초록 (Multilingual Abstract)

Anomalies of the fetal venous system are rare. Major portion of fetal venous anomalies are malformation of umbilical vein and ductus venosus. Abnormal umbilico-systemic shunt, bypassing the ductus venosus makes direct connection between the high-pressure umbilical system and the low-pressure systemic system. And it makes adverse to the fetal hemodynamics. Fetal hemodynamic distress may induce fetal growth retardation, hepatomegaly, cardiomegaly, hydrops fetalis and fetal death. We report a case of non-immune hydrops fetalis which was associated with abnormal umbilical vein pathway. Our patient had bifurcated umbilical veins. Main branch of umbilical vein was drained directly to the left internal iliac vein and another branch was drained to the portal vein. After birth, extra hepatic shunt through main branch of umbilical vein that bypassed the portal system was persisted and thrombocytopenia was combined due to consumption in thrombus of a dilated anomalous umbilical vein. Later this case was diagnosed as Noonan syndrome with a genetic testing.