{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,17]],"date-time":"2026-06-17T16:17:01Z","timestamp":1781713021829,"version":"3.54.5"},"reference-count":27,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2020,11,5]],"date-time":"2020-11-05T00:00:00Z","timestamp":1604534400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Congenital Anomalies"],"published-print":{"date-parts":[[2021,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Umbilical vein varix is associated with a high incidence of fetal anomalies and perinatal complications. There are two types of umbilical vein varix: fetal intra\u2010abdominal and extra\u2010abdominal. Herein, a case is reported of severe fetal anemia with extra\u2010abdominal umbilical vein varix. A 33\u2010year\u2010old primigravida was referred to our hospital for fetal growth restriction, fetal cardiomegaly, and decreased fetal movements at 26\u2009weeks' gestation. A Doppler assessment showed an elevated middle cerebral artery peak systolic velocity at 2.2\u2009MoM, suggesting fetal anemia. Umbilical vein varix had caused intermittent turbulent flow, provoking hemolytic anemia. Intrauterine transfusion improved fetal circulatory failure and anemia and prolonged gestational period. At 33\u2009weeks' gestation, the patient underwent cesarean delivery due to nonreassuring fetal status. Pathological analysis revealed focal loss of vascular smooth muscle of the umbilical vein. Extra\u2010abdominal umbilical vein varix has been reported in 14 cases including this case. The antenatal diagnosis rate is reported to be 79%; fetal heartbeat abnormalities and fetal deaths were reported as 50% and 14%, respectively. Eighty\u2010six percent of patients had intra\u2010umbilical cord thrombosis, but currently this is the only case of hemolytic anemia. Furthermore, extra\u2010abdominal umbilical vein varix may present as fetal hydrops with anemia. During ultrasound examination of fetal anemia, umbilical cord screening should be performed with caution.<\/jats:p>","DOI":"10.1111\/cga.12397","type":"journal-article","created":{"date-parts":[[2020,10,25]],"date-time":"2020-10-25T06:30:48Z","timestamp":1603607448000},"page":"4-8","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Severe fetal anemia as a consequence of extra\u2010abdominal umbilical vein varix: A case report and review of the literature"],"prefix":"10.1111","volume":"61","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8769-134X","authenticated-orcid":false,"given":"Shingo","family":"Io","sequence":"first","affiliation":[{"name":"Department of Gynecology and Obstetrics Kyoto University Graduate School of Medicine  Kyoto Japan"},{"name":"Japan Society for the Promotion of Science  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eiji","family":"Kondoh","sequence":"additional","affiliation":[{"name":"Department of Gynecology and Obstetrics Kyoto University Graduate School of Medicine  Kyoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshiki","family":"Iemura","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Pathology Kyoto University Graduate School of Medicine  Kyoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sachiko","family":"Minamiguchi","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Pathology Kyoto University Graduate School of Medicine  Kyoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshitsugu","family":"Chigusa","sequence":"additional","affiliation":[{"name":"Department of Gynecology and Obstetrics Kyoto University Graduate School of Medicine  Kyoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haruta","family":"Mogami","sequence":"additional","affiliation":[{"name":"Department of Gynecology and Obstetrics Kyoto University Graduate School of Medicine  Kyoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masaki","family":"Mandai","sequence":"additional","affiliation":[{"name":"Department of Gynecology and Obstetrics Kyoto University Graduate School of Medicine  Kyoto 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enlargement of the umbilical cord owing to an intra\u2010amniotic varicosity associated with thrombocytopenia","volume":"47","author":"Trobs RB","year":"2012","journal-title":"J Pediatr Surg"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.placenta.2013.08.008"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1136\/bcr-2018-227424"},{"key":"e_1_2_6_26_1","first-page":"7154560","article-title":"A case report of umbilical vein Varix with thrombosis: prenatal Ultrasonographic diagnosis and management","volume":"2019","author":"Matsumoto Y","year":"2019","journal-title":"Case Rep Obstet Gynecol"},{"issue":"2","key":"e_1_2_6_27_1","first-page":"190","article-title":"Images in paediatrics: intra\u2010amniotic umbilical vein varix","volume":"105","author":"Hayden J","year":"2020","journal-title":"Arch Dis Child"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.1097\/00043426-200005000-00013"}],"container-title":["Congenital 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