Obstetrics and Prenatal Medicine, University Hospital Bonn, Germany.
Division of Prenatal Medicine and Gynecologic Sonography, Department of Obstetrics and Gynecology, University Hospital Cologne, Germany.
Ultraschall Med. 2019 Jun;40(3):333-339. doi: 10.1055/s-0043-115109. Epub 2017 Sep 21.
To assess the anatomic variants, associated anomalies and postnatal outcome of fetuses with a prenatally diagnosed agenesis of ductus venosus (ADV).
Retrospective study of 119 cases with agenesis of ductus venosus diagnosed by prenatal ultrasound in two tertiary referral centers from 2006 to 2014. The type and location of the umbilical venous drainage site was noted. Charts were reviewed for associated structural or chromosomal anomalies, pregnancy outcome and postnatal course.
In 24 cases (20.2 %) ADV was an isolated finding, while 95 cases (79.8 %) had associated anomalies. We identified 84 cases (70.6 %) with intrahepatic and 35 cases (29.4 %) with extrahepatic drainage of the umbilical vein. 58.8 % of neonates were alive at follow-up. There was no statistical association between drainage site and associated anomalies or outcome. Postnatal outcome was determined by the presence and severity of associated anomalies. There was no adverse outcome in the isolated group related to ADV. Overall, there were 6 persistent portosystemic shunts, 3 of them with a spontaneous closure, and one total agenesis of the portal venous system with lethal outcome.
Postnatal outcome in cases with ADV mainly depends on the presence of associated anomalies. In isolated cases the prognosis is generally good, but neonates with a prenatally diagnosed portosystemic shunt should be followed until its occlusion. Portal venous system agenesis is rare but should be ruled out on prenatal ultrasound.
评估产前诊断为静脉导管缺如（ADV）的胎儿的解剖变异、相关异常和产后结局。
对 2006 年至 2014 年在两个三级转诊中心通过产前超声诊断为静脉导管缺如的 119 例病例进行回顾性研究。记录脐静脉引流部位的类型和位置。查阅图表以了解相关的结构或染色体异常、妊娠结局和产后病程。
24 例（20.2%）ADV 为孤立性发现，95 例（79.8%）存在相关异常。我们发现 84 例（70.6%）为肝内引流，35 例（29.4%）为肝外引流。随访时 58.8%的新生儿存活。引流部位与相关异常或结局之间无统计学关联。产后结局取决于相关异常的存在和严重程度。孤立组 ADV 相关无不良结局。总体而言，有 6 例持续性门体分流，其中 3 例自发性闭合，1 例门静脉系统完全缺如导致致命结局。
ADV 病例的产后结局主要取决于相关异常的存在。孤立性病例的预后通常良好，但应在产前超声随访存在产前诊断的门体分流的新生儿，直至其闭塞。门静脉系统缺如是罕见的，但应在产前超声排除。
