右先天性膈疝：产前评估与结局

Right congenital diaphragmatic hernia: Prenatal assessment and outcome.

作者信息

Hedrick Holly L, Crombleholme Timothy M, Flake Alan W, Nance Michael L, von Allmen Daniel, Howell Lori J, Johnson Mark P, Wilson R Douglas, Adzick N Scott

机构信息

Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA 19104-4399, USA.

出版信息

J Pediatr Surg. 2004 Mar;39(3):319-23; discussion 319-23. doi: 10.1016/j.jpedsurg.2003.11.006.

DOI:10.1016/j.jpedsurg.2003.11.006

PMID:15017545

Abstract

PURPOSE

To understand the natural history of right congenital diaphragmatic hernia (CDH), the authors retrospectively reviewed 27 cases of right CDH that presented for prenatal evaluation or postnatal treatment.

METHODS

Between 1995 and September 2002, a total of 194 cases of fetal CDH were evaluated and included 22 right-sided defects. The authors reviewed prenatal diagnostic studies (ultrasound scan, magnetic resonance imaging [MRI] echocardiography) and pre- and postnatal outcomes in these 22 cases of right CDH. Five additional cases of right CDH without a prenatal diagnosis were reviewed.

RESULTS

The mean gestational age at evaluation was 26.1 weeks. The lung area to head circumference ratio (LHR) ranged from 0.32 to 2.5. In all cases, the fetal liver was herniated into the right chest. Associated anomalies were common. There were no karyotype abnormalities (17 of 22 tested). There were 4 terminations. Nine of the 18 (50%) continuing pregnancies had polyhydramnios, premature rupture of membranes, or preterm labor. The mean gestational age at birth was 36.8 weeks. One patient underwent tracheal occlusion at 27 weeks, and 2 patients died before postnatal repair. Overall survival rate (22 prenatal plus 5 postnatal diagnoses) was 19 of 27 (70%). Postnatal survival rate was 19 of 23 (83%). A Gore-tex (W. L. Gore and Associates, Flagstaff, AZ) patch was utilized in 14 of 21 neonates undergoing surgery. Twelve of 23 (52%) required extracorporeal membrane oxygenation (ECMO) with a 75% survival rate. Significant morbidity occurred in 10 of 19 survivors and included neurologic sequelae in 6 of 19 (32%).

CONCLUSIONS

MRI was helpful in the determination of liver position and confirmation of diagnosis. The high incidence of preterm complications, frequent need for ECMO, and high prevalence of comorbidities are indicative of the severity of this CDH population and warrant close prenatal surveillance and delivery at a tertiary care center with ECMO capability.

摘要

目的

为了解右侧先天性膈疝（CDH）的自然病史，作者回顾性分析了27例因产前评估或产后治疗而就诊的右侧CDH病例。

方法

1995年至2002年9月期间，共评估了194例胎儿CDH，其中包括22例右侧缺陷。作者回顾了这22例右侧CDH的产前诊断研究（超声扫描、磁共振成像[MRI]、超声心动图）以及产前和产后结局。另外回顾了5例未进行产前诊断的右侧CDH病例。

结果

评估时的平均孕周为26.1周。肺面积与头围比（LHR）范围为0.32至2.5。所有病例中，胎儿肝脏均疝入右胸腔。合并畸形很常见。未发现核型异常（22例检测中的17例）。有4例终止妊娠。18例继续妊娠的孕妇中有9例（50%）发生羊水过多、胎膜早破或早产。出生时的平均孕周为36.8周。1例患者在27周时接受了气管阻塞治疗，2例患者在产后修复前死亡。总体生存率（22例产前诊断加5例产后诊断）为27例中的19例（70%）。产后生存率为23例中的19例（83%）。21例接受手术的新生儿中有14例使用了戈尔特斯（W.L.戈尔公司，弗拉格斯塔夫，亚利桑那州）补片。23例中有12例（52%）需要体外膜肺氧合（ECMO），生存率为75%。19例幸存者中有10例发生了严重并发症，其中19例中有6例（32%）出现神经系统后遗症。