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先天性膈疝囊:产前影像学及相关产后结局

Congenital diaphragmatic hernia sacs: prenatal imaging and associated postnatal outcomes.

作者信息

Oliver Edward R, DeBari Suzanne E, Adams Samantha E, Didier Ryne A, Horii Steven C, Victoria Teresa, Hedrick Holly L, Adzick N Scott, Howell Lori J, Moldenhauer Julie S, Coleman Beverly G

机构信息

Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.

Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, PA, USA.

出版信息

Pediatr Radiol. 2019 May;49(5):593-599. doi: 10.1007/s00247-018-04334-9. Epub 2019 Jan 11.

Abstract

BACKGROUND

The presence of a hernia sac in congenital diaphragmatic hernia (CDH) has been reported to be associated with higher lung volumes and better postnatal outcomes.

OBJECTIVE

To compare prenatal imaging (ultrasound and MRI) prognostic measurements and postnatal outcomes of CDH with and without hernia sac.

MATERIALS AND METHODS

We performed database searches from January 2008 to March 2017 for surgically proven cases of CDH with and without hernia sac. All children had a detailed ultrasound (US) examination and most had an MRI examination. We reviewed the medical records of children enrolled in our Pulmonary Hypoplasia Program.

RESULTS

Of 200 cases of unilateral CDH, 46 (23%) had hernia sacs. Cases of CDH with hernia sac had a higher mean lung-to-head ratio (LHR; 1.61 vs. 1.17; P<0.01), a higher mean observed/expected LHR (0.49 vs. 0.37; P<0.01), and on MRI a higher mean observed/expected total lung volume (0.53 vs. 0.41; P<0.01). Based on a smooth interface between lung and herniated contents, hernia sac or eventration was prospectively questioned by US and MRI in 45.7% and 38.6% of cases, respectively. Postnatally, hernia sac is associated with shorter median periods of admission to the neonatal intensive care unit (45.0 days vs. 61.5 days, P=0.03); mechanical ventilation (15.5 days vs. 23.5 days, P=0.04); extracorporeal membrane oxygenation (251 h vs. 434 h, P=0.04); decreased rates of patch repair (39.0% vs. 69.2%, P<0.01); and pulmonary hypertension (56.1% vs. 75.4%, P=0.03).

CONCLUSION

Hernia sac is associated with statistically higher prenatal prognostic measurements and improved postnatal outcomes. Recognition of a sharp interface between lung and herniated contents may allow for improved prenatal diagnosis; however, delivery and management should still occur at experienced quaternary neonatal centers.

摘要

背景

据报道,先天性膈疝(CDH)中疝囊的存在与更高的肺容积和更好的产后结局相关。

目的

比较有无疝囊的CDH的产前影像学(超声和MRI)预后测量指标及产后结局。

材料与方法

我们对2008年1月至2017年3月手术确诊的有无疝囊的CDH病例进行了数据库检索。所有儿童均接受了详细的超声(US)检查,大多数还接受了MRI检查。我们回顾了纳入我们肺发育不全项目的儿童的病历。

结果

在200例单侧CDH病例中,46例(23%)有疝囊。有疝囊的CDH病例平均肺头比(LHR)更高(1.61对1.17;P<0.01),平均观察/预期LHR更高(0.49对0.37;P<0.01),MRI显示平均观察/预期总肺容积更高(0.53对0.41;P<0.01)。基于肺与疝入内容物之间的光滑界面,超声和MRI分别在45.7%和38.6%的病例中前瞻性地怀疑有疝囊或膈膨出。产后,疝囊与新生儿重症监护病房住院中位时间较短相关(45.0天对61.5天,P=0.03);机械通气时间较短(15.5天对23.5天,P=0.04);体外膜肺氧合时间较短(251小时对434小时,P=0.04);补片修补率降低(39.0%对69.2%,P<0.01);以及肺动脉高压发生率较低(56.1%对75.4%,P=0.03)。

结论

疝囊与统计学上更高的产前预后测量指标及改善的产后结局相关。认识到肺与疝入内容物之间的清晰界面可能有助于改善产前诊断;然而,分娩和管理仍应在经验丰富的四级新生儿中心进行。

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