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KMID : 0361720130240040300
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Korean Journal of perinatology
2013 Volume.24 No. 4 p.300 ~ p.305
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A Case of Non-Immune Hydrops Fetalis due to Umbilical Venous Malformation and Noonan Syndrome
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Yun Na-Ra
Kim Ji-soo
Lee Ju-Young
Shin Seung-Han
Ko Jung-Min
Kim Ee-Kyung
Kim Han-Suk
Choi Jung-Hwan
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Abstract
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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.
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KEYWORD
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Vascular malformations, Umbilical veins, Fetus, Non immune hydrops fetalis, Noonan syndrome
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