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四例静脉导管缺如:三例合并严重胎儿水肿。

Four cases of absent ductus venosus: three in combination with severe hydrops fetalis.

作者信息

Jörgensen C, Andolf E

机构信息

Department of Obstetrics and Gynecology, University Hospital Lund, Sweden.

出版信息

Fetal Diagn Ther. 1994 Nov-Dec;9(6):395-7. doi: 10.1159/000264072.

DOI:10.1159/000264072
PMID:7880437
Abstract

Ductus venosus links the umbilical vein and the portal vein to the inferior vena cava. It is of great significance for the developing fetus but of minor importance to the growing infant where it normally obliterates and is transformed to the ligamentum venosum. An absent ductus venosus implies that the oxygenated blood from the umbilical vein has to circulate through the liver. We report 4 cases with this malformation. Prenatally, three fetuses had an extreme hydrops, especially hydrothorax. In two fetuses hydrops was severe already in the second trimester of pregnancy. Termination was performed. In the third case, hydrops was first diagnosed in the 36th week of gestation. The fourth case had an ultrasound examination in the 34th week of gestation. There was no hydrops but a disproportion with a very small head and abdominal diameter compared to femur length. Postpartum both these infants died. At autopsy, the only structural malformation in the three hydrops cases was an absent ductus venosus. In the fourth case there were other anomalies.

摘要

静脉导管将脐静脉与门静脉连接至下腔静脉。它对发育中的胎儿具有重要意义,但对成长中的婴儿来说重要性较小,在婴儿期它通常会闭塞并转化为静脉韧带。静脉导管缺如意味着来自脐静脉的氧合血必须通过肝脏循环。我们报告了4例这种畸形病例。产前,3例胎儿出现严重水肿,尤其是胸腔积液。2例胎儿在妊娠中期就已出现严重水肿,于是终止了妊娠。第3例在妊娠36周时首次诊断出水肿。第4例在妊娠34周时进行了超声检查,未发现水肿,但存在头围和腹径与股骨长度相比非常小的比例失调情况。产后这2例婴儿均死亡。尸检发现,3例水肿病例中唯一的结构畸形是静脉导管缺如。第4例还存在其他异常情况。

相似文献

1
Four cases of absent ductus venosus: three in combination with severe hydrops fetalis.四例静脉导管缺如:三例合并严重胎儿水肿。
Fetal Diagn Ther. 1994 Nov-Dec;9(6):395-7. doi: 10.1159/000264072.
2
Agenesis of the ductus venosus and its correlation to hydrops fetalis.静脉导管缺如及其与胎儿水肿的相关性。
J Perinat Med. 2000;28(1):69-73. doi: 10.1515/JPM.2000.010.
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Hydrops fetalis due to agenesis of the ductus venosus: new hepatic histological features.静脉导管缺如所致胎儿水肿:新的肝脏组织学特征
Pediatr Dev Pathol. 2009 May-Jun;12(3):239-43. doi: 10.2350/07-10-0363.1.
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Absence of the ductus venosus: report of 10 new cases and review of the literature.静脉导管缺如:10例新病例报告及文献复习
Ultrasound Obstet Gynecol. 2001 Dec;18(6):605-9. doi: 10.1046/j.0960-7692.2001.00599.x.
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Agenesis of the ductus venosus and its correlation to hydrops fetalis and the fetal hepatic circulation: case reports and review of the literature.
Pediatr Pathol Lab Med. 1995 Jan-Feb;15(1):39-50. doi: 10.3109/15513819509026938.
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Agenesis of the ductus venosus that is associated with extrahepatic umbilical vein drainage: prenatal features and clinical outcome.与肝外脐静脉引流相关的静脉导管缺如:产前特征及临床结局
Am J Obstet Gynecol. 2002 Oct;187(4):1031-7. doi: 10.1067/mob.2002.126292.
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Absent ductus venosus in the fetus: review of the literature and first report of direct umbilical venous drainage to the coronary sinus.胎儿静脉导管缺如:文献综述及直接经脐静脉引流至冠状窦的首例报道
Fetal Diagn Ther. 2003 Jul-Aug;18(4):247-51. doi: 10.1159/000070804.
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In utero diagnosis of congenital absence of the ductus venosus.胎儿期诊断先天性静脉导管缺如。
J Clin Ultrasound. 1997 Oct;25(8):456-8. doi: 10.1002/(sici)1097-0096(199710)25:8<456::aid-jcu9>3.0.co;2-i.
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[Agenesia of the canal of Arantius. A case report].[阿朗蒂乌斯管发育不全。病例报告]
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Missing ductus venosus: a case report.静脉导管缺如:一例报告
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Absence of Ductus Venosus: A Comparison of 2 Distinctive Fetal Autopsy Cases and Embryologic Perspectives.静脉导管缺如:2 例独特胎儿尸检病例的比较和胚胎学观点。
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