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膈疝:脐静脉异常引流的一种产后并发症。

Diaphragmatic hernia: a postnatal complication of anomalous drainage of the umbilical vein.

机构信息

Imperial College, Reproductive Biology at Queen Charlotte's and Chelsea Hospital, London, UK.

出版信息

Ultrasound Obstet Gynecol. 2013 May;41(5):589-91. doi: 10.1002/uog.12381.

DOI:10.1002/uog.12381
PMID:23288807
Abstract

The ductus venosus (DV) connects the portal venous system to the inferior vena cava. Rarely, the umbilical venous drainage is anomalous, either connecting to the portal sinus within the liver or having an extrahepatic connection, bypassing the liver and draining to one of a variety of sites, including to the heart directly. Prenatally, there is a recognized association of anomalous umbilical venous drainage with aneuploidy and other structural malformations. The fetus may also develop right heart failure because of unregulated volume loading. We report the postnatal development of diaphragmatic hernia in three fetuses with absent DV and umbilical venous drainage to the right atrium directly in two cases and to the coronary sinus in the third. In all fetuses, the abnormality was well-tolerated in pregnancy, with only a modest degree of right heart dilatation. All three neonates underwent repair of the diaphragmatic hernia and made a good recovery.

摘要

静脉导管(DV)将门静脉系统与下腔静脉连接。罕见情况下,脐静脉引流异常,要么连接到肝脏内的门静脉窦,要么存在肝外连接,绕过肝脏并引流到多种部位,包括直接引流到心脏。在胎儿期,异常的脐静脉引流与非整倍体和其他结构畸形有关。胎儿也可能因不受调节的容量负荷而出现右心衰竭。我们报告了三例 DV 缺失且脐静脉直接引流至右心房(两例引流至右心房,一例引流至冠状窦)胎儿的膈疝的产后发育情况。在所有胎儿中,该异常在妊娠期间耐受良好,仅右心轻度扩张。所有三名新生儿均接受了膈疝修补术,恢复良好。

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1
Diaphragmatic hernia: a postnatal complication of anomalous drainage of the umbilical vein.膈疝:脐静脉异常引流的一种产后并发症。
Ultrasound Obstet Gynecol. 2013 May;41(5):589-91. doi: 10.1002/uog.12381.
2
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
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Pitfall: anomalous umbilical vein and absent ductus venosus in association with right congenital diaphragmatic hernia.陷阱:异常脐静脉和静脉导管缺如合并右侧先天性膈疝。
Pediatr Radiol. 1997 Aug;27(8):651-3. doi: 10.1007/s002470050205.
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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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Aberrant drainage of the umbilical vein into the coronary sinus without ductus venosus agenesis.脐静脉异常引流至冠状窦,无静脉导管缺如。
J Ultrasound Med. 2014 Mar;33(3):535-42. doi: 10.7863/ultra.33.3.535.
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Diaphragmatic Hernia Associated With Absent Ductus Venosus and Anomalous Connection of an Obliterated Umbilical Vein to the Coronary Sinus.膈疝合并静脉导管缺如及闭锁脐静脉异常连接冠状窦。
World J Pediatr Congenit Heart Surg. 2020 Jul;11(4):NP80-NP82. doi: 10.1177/2150135117710927. Epub 2017 Sep 21.
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Functional morphology of ductus venosus in human fetus.人类胎儿静脉导管的功能形态学
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Prenatal diagnosis of abnormal course of umbilical vein and ductus venosus agenesis: report of three cases.脐静脉走行异常及静脉导管缺如的产前诊断:三例报告
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Diagnosis of absent ductus venosus in a population referred for fetal echocardiography: association with a persistent portosystemic shunt requiring postnatal device occlusion.胎儿超声心动图检查人群中静脉导管缺如的诊断:与出生后需要装置封堵的持续性门体分流的关联。
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Preferential streaming of the ductus venosus toward the right atrium is associated with a worse outcome despite a higher rate of invasive procedures in human fetuses with left diaphragmatic hernia.尽管患有左侧膈疝的胎儿进行有创操作的比例较高,但静脉导管偏向右侧心房与更差的结局相关。
Ultraschall Med. 2013 Dec;34(6):568-72. doi: 10.1055/s-0032-1330702. Epub 2013 May 21.

引用本文的文献

1
Prenatal sonographic characteristics and postnatal outcomes of congenital portosystemic shunt diagnosed during the fetal period: a systematic review.胎儿期诊断的先天性门体分流的产前超声特征及产后结局:一项系统评价
Orphanet J Rare Dis. 2025 May 27;20(1):257. doi: 10.1186/s13023-025-03811-3.
2
Prenatal diagnosis of an aberrant ductus venosus draining into the coronary sinus using two- and three-dimensional echocardiography: a case report.应用二维和三维超声心动图对异常静脉导管引流至冠状窦的产前诊断:病例报告。
BMC Pregnancy Childbirth. 2021 May 20;21(1):392. doi: 10.1186/s12884-021-03870-x.