静脉导管缺如：来自两个三级医疗中心的病例系列
Absent ductus venosus: case series from two tertiary centres.
作者信息
Maruotti Giuseppe Maria, Saccone Gabriele, Ciardulli Andrea, Mazzarelli Laura Letizia, Berghella Vincenzo, Martinelli Pasquale
机构信息
a Department of Neuroscience, Reproductive Sciences and Dentistry, School of Medicine , University of Naples "Federico II" , Naples , Italy.
b Department of Obstetrics and Gynecology, Division of Maternal Fetal Medicine , Sidney Kimmel Medical College of Thomas Jefferson University , Philadelphia , PA , USA.
出版信息
J Matern Fetal Neonatal Med. 2018 Sep;31(18):2478-2483. doi: 10.1080/14767058.2017.1344637. Epub 2017 Jul 12.
INTRODUCTION
Congenital absence of the ductus venosus (ADV) is a rare vascular anomaly often associated with fetal cardiac and extracardiac anomalies, aneuploidies, and hydrops. The prognosis depends on the patterns of abnormal venous circulation, on the associated malformations and on chromosomal aberrations.
METHODS
We performed a retrospective audit of all consecutive cases with ADV referred in our centres and analysed the outcomes.
RESULTS
A total of six cases with prenatally diagnosed ADV were identified. The gestational age at diagnosis ranged from 15 to 35 weeks. Karyotyping was performed in all cases. Normal karyotype was found in five out of the six cases. Overall, four neonates survived at 28 days follow-up. The other two died 48 h after delivery: both of them had extrahepatic ADV.
DISCUSSION
Absence of the ductus venosus may be compatible with normal fetal development without relevant disturbance of circulation and oxygenation independently from type of abnormal venous circulation.
引言
先天性静脉导管缺如（ADV）是一种罕见的血管异常，常与胎儿心脏和心脏外异常、非整倍体及水肿相关。预后取决于异常静脉循环模式、相关畸形及染色体畸变情况。
方法
我们对本中心所有连续转诊的ADV病例进行了回顾性审核并分析了结果。
结果
共确诊6例产前诊断为ADV的病例。诊断时的孕周为15至35周。所有病例均进行了核型分析。6例中有5例核型正常。总体而言，4例新生儿在28天随访时存活。另外2例在分娩后48小时死亡：二者均为肝外ADV。
讨论
静脉导管缺如可能与正常胎儿发育兼容，而不受异常静脉循环类型影响，不会对循环和氧合产生相关干扰。
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