Persistent right umbilical vein: an ominous prenatal finding?

Abstract

The persistence of a right umbilical vein is an uncommon finding, with only a dozen cases reported since 1826. The persistent right umbilical vein may replace the normal left umbilical vein or be supernumerary. The anomaly is associated with numerous and occasionally lethal malformations. In this series, only three of six fetuses (and another two in the literature) had no associated anomalies. All the others had a variety of associated lesions ranging from minor to lethal. The appearance at ultrasound is easy to recognize: The intrahepatic portion of the umbilical vein is lateral to the gallbladder, and the portal vein curves toward the stomach, instead of parallel to it. Since the recognition of the persistent right umbilical vein is simple and does not require additional scanning (it is visible in the section used to measure the abdominal perimeter), the author suggests using it as an indicator for more in-depth scanning.

Umbilical vein entering the right atrium: significance of in utero diagnosis

“…In none of the six cases reported by Jeanty was there direct extension of the right umbilical vein into the right atrium. 3 In fact, to our knowledge, only four reports of this anomaly, none of which were diagnosed prenatally, 4 -1 have been published. The importance of identifying this uncommon anomaly on prenatal sonograms is because of its asso· ciation with other structural anomalies.…”

“…3). 3 Other aberrant connections include umbilical vein drainage into the suprahepatic inf erlor vena cava (IVC), infrahepatic IVC, iliac veins, or subcutaneous collateral vessels into the superior vena cava. Prior sonographic experiences with aberrant connections between the umbilical vein are few.…”

“…To our knowledge, only one such report exists in the literature. 3 This includes six cases of persistent right umbilical vein diagnosed prenatally. In all six cases, the right umbilical vein connected directly to the portal venous system.…”

“…Anomalies that commonly occur with persistent umbilical vein involve the gastrointestinal, cardiovascular, genitourinary, and musculoskeleta) systems. 3 Gastrointestinal anomalies include, among others, duodenal atresia, imperforate anus, annular pancreas, and tracheoesophageal fistula . Cardiovascular anomalies may include simple ASD or atrial septa!…”

Four‐vessel umbilical cord

“…Persistence of the right umbilical vein can be intracorporeal or extracorporeal (within the umbilical cord). In the latter instance it will result in a four-vessel umbilical cord 10 . Supernumerary vessels of the umbilical cord are rare and may be isolated or associated with congenital anomalies: ectopia cordis, pulmonary stenosis, cleft lip and palate, bifid liver, situs inversus, tetralogy of Fallot and gastroschisis have been reported 11 -17 .…”