Improved survival for congenital diaphragmatic hernia, based on prenatal ultrasound diagnosis and referral to a combined obstetric-pediatric surgical center

doi:10.1016/0022-3468(94)90821-4

Journal of Pediatric Surgery

Volume 29, Issue 9, September 1994, Pages 1268-1269

https://doi.org/10.1016/0022-3468(94)90821-4 Get rights and content

Abstract

Between January 1990 and January 1993, 36 patients with antenatal ultrasound (US) diagnosis and/or postnatal diagnosis of congenital diaphragmatic hernia (CDH) were referred to the authors' high-risk obstetric and pediatric hospital. Among the 36, there were four spontaneous abortions (11%), five deaths after live births (14%), one false-positive US examination, and 26 patients who underwent surgery, 23 of whom survived (66% overall, 74% of live births, and 89% postoperatively). Only one survivor had extracorporeal membrane oxygenation (ECMO). Thirty-five ultrasound examinations were performed in 24 patients; there were 18 true-positives results (51%), one false-positive (3%), and 16 false-negatives (46%). In this series, there were 25 left-sided CDHs, eight right-sided, one bilateral, and one central. Of the four right-sided hernias having antenatal US, only one was diagnosed prenatally; 15 of the 17 left-sided CDHs were diagnosed correctly (88%). All 19 babies with the prenatal diagnosis were born at the authors' institution. US diagnosis before 25 weeks' gestation and polyhydramnios separately resulted in a mortality rate of only 50%. Patients born at this institution tend to be sicker than those transferred from elsewhere, as reflected by the lower 1- and 5-minute Apgar scores (3.7 v 6.9, P < .001, and 5.4 v 6.9, P < .16, respectively), lower gestational ages (37.0 v 39.2 weeks, P < .007), and lower birth weights (2,525 v 3,049 g, P < .02). Nevertheless, transferred patients had a mortality rate (3 of 15 patients, 20%) similar to that of nontransferred patients (5 of 20 patients, 25%). The authors conclude that (1) systematic prenatal US can diagnose CDH and enable referral to a center that provides high-risk obstetric and pediatric surgical care, thus providing optimal postnatal treatment, and (2) contrary to other reports, antenatal diagnosis of CDH did not presage a high mortality rate.

References (4)

MS Caplan et al.
Congenital diaphragmatic hernia and abdominal wall defects
Clin Perinatol
(1989)
NS Adzick et al.
Congenital diaphragmatic hernia in the fetus: Outcome in 94 cases
J Pediatr Surg
(1985)

There are more references available in the full text version of this article.

Cited by (30)

Congenital Diaphragmatic Hernia and Eventration
2012, Pediatric Surgery, 2-Volume Set: Expert Consult - Online and Print
Congenital Diaphragmatic Hernia and Eventration
2012, Pediatric Surgery
Congenital Diaphragmatic Hernia and Eventration
2006, Pediatric Surgery: Sixth Edition
Antenatal detection and impact on outcome of congenital diaphragmatic hernia: A 12-year experience in Auvergne (France)
2006, European Journal of Obstetrics and Gynecology and Reproductive Biology
To evaluate the detection rate of prenatal diagnosis and its impact on outcome in congenital diaphragmatic hernia (CDH).
We retrospectively studied 51 cases of CDH registered in the Auvergne area from January 1992 to December 2003 (Birth Defect Registry of Auvergne, Institut Européen des Génomutations). Our main outcome measurements were the detection rate of prenatal diagnosis, the incidence and types of associated anomalies and outcome (termination of pregnancy, in utero fetal demise, neonatal death, survival at the time of registration).
Twenty-nine cases of isolated CDH were identified of which 13 were detected prenatally (45%) at a mean gestational age of 26.1 weeks and 22 cases of CDH with associated anomalies with prenatal diagnosis of CDH or any associated anomaly in 16 (73%; p = 0.03) at a mean gestational age of 23.9 weeks. In the prenatally detected group (29 cases), there was 1 (3%) in utero fetal death (IUFD), 17 (59%) terminations of pregnancy (TOP) and 11 (38%) live births with early neonatal death in 7 (24%) cases despite delivery in a tertiary care centre in 10/11 cases (four survivors = 14%). Most of the undetected cases were isolated CDH (16/22 = 73%) of which 1 (5%) was a stillborn and 21 (95%) live births with 17 survivors (77%) although 15/21 (71%) were not born at the tertiary care centre (p = 0.001). The overall survival rate was 41% with a large variability depending on associated anomalies and prenatal diagnosis (p < 0.0001) (prenatally detected cases: 3/13 (23%) isolated CDH and 1/16 (6%) CDH with associated anomalies; undetected cases: 13/16 (81%) isolated CDH and 4/6 (67%) CDH with associated anomalies).
Prenatal diagnosis of CDH leads to the delivery of affected babies in tertiary care centres but it remains a challenge in particular for isolated CDH cases and it is associated with a lower survival rate. Associated anomalies contribute to prenatal detection, are related to a higher TOP rate but do not facilitate the detection of diaphragmatic defect per se.
Congenital diaphragmatic hernia in Scandinavia from 1995 to 1998: Predictors of mortality
2002, Journal of Pediatric Surgery
Citation Excerpt :
This observation is in accordance with those in recent large studies showing significantly increased mortality rate in CDH patients with a prenatal diagnosis compared with those without.4,8,13,31 However, some studies did not find any effect on mortality rate.1-2,32-33 There is reason to believe that increased statistical power based on a large sample was a prerequisite for this finding.
Background/Purpose: There is a lack of large contemporary studies on the management of congenital diaphragmatic hernia (CDH), and the prediction of mortality remains difficult. The aim of this study was to investigate the influence of perinatal factors on mortality rate in a contemporary multicenter study. Methods: The authors conducted a retrospective multicenter cohort study. Twelve of 13 Scandinavian pediatric surgical centers participated in the study. During a 4-year period (1995 through 1998) 195 children with CDH were included. The main endpoints were hospital mortality rate and total mortality rate (before 2001). Bivariate and multivariate survival analyses were performed using Kaplan-Meier plots, Log-rank test, and Cox regression. Results: Overall hospital mortality rate was 30%. Among 168 neonates with symptoms within 24 hours (early presenters) 35% died before discharge. All 61 deaths occurred in 157 neonates with symptoms within the first 2 hours of life. Among early presenters, 27% had prenatal ultrasound diagnosis, 26% were delivered by cesarean section, and 21% had associated major malformations. Bivariate analysis of early presenters showed increased risk of death in neonates with prenatal diagnosis, associated anomalies, right-sided diaphragmatic hernia (RCDH), low 1-minute and 5-minute Apgar scores, low birth weight, short gestational age, and cesarean delivery. Neonates with prenatal diagnosis were characterized by significantly lower Apgar scores, lower birth weight, and increased frequency of associated anomalies than those diagnosed after birth. Multivariate analysis found that prenatal diagnosis (P =.004), 1-minute Apgar (P =.001), and RCDH (P =.042) were independent predictors of total mortality rate. Conclusions: In a series of 195 CDH patients, all 61 deaths occurred in the 157 neonates presenting with symptoms within the first 2 hours of life. Prenatal diagnosis, 1-minute Apgar score, and RCDH were significant independent predictors of total mortality. J Pediatr Surg 37:1269-1275. Copyright 2002, Elsevier Science (USA). All rights reserved.
Congenital diaphragmatic hernia: A meta-analysis of mortality factors
2000, Journal of Pediatric Surgery
Citation Excerpt :
The current study shows that mortality rate is increased among prenatally diagnosed CDH patients compared with postnatally diagnosed patients. The highest pooled mortality rates were found in the group with fetuses diagnosed prenatally, and these findings support the conclusions from other reports,2,12,76 but this is in contrast to other reports.6,7,13,14,68 The magnitude of the effect of prenatal diagnosis on pooled mortality rate is relatively strong with an odds ratio between 2.0 and 4.0 depending on the study population.
Purpose: The aim of this study was to review all available studies reported in the English-language literature from 1975 through 1998, and by meta-analysis assess the importance of prenatal diagnosis, associated malformations, side of hernia, timing of surgery, and study population on mortality rates in patients with congenital diaphragmatic hernia (CDH). Methods: One-hundred-two studies were identified, and 51 studies (2,980 patients) fulfilled the prespecified inclusion criteria. Studies were grouped according to study population into: (I) fetuses diagnosed prenatally; (II) neonates admitted to a treatment center; and (III) population-based studies. Results: Pooled total mortality rate was significantly higher in category I than in category III (75.6% v 58.2%, P <.001). Pooled hidden postnatal mortality rate (deaths before admittance to a treatment center) in population-based studies was 34.9%. Prenatally diagnosed patients in both category II and III had significantly higher mortality rates than those diagnosed postnatally. Mortality rates were significantly higher among CDH infants with associated major malformations compared with isolated CDH in all 3 categories. An increased mortality rate in right-sided CDH was found in category II and III. Conclusions: Prenatal diagnosis of CDH, presence of associated major malformations, and the study population have a major influence on mortality rate. The very high mortality rate in studies of fetuses with a prenatal diagnosis of CDH should be taken into account in prenatal counselling. J Pediatr Surg 35:1187-1197. Copyright © 2000 by W.B. Saunders Company.

View all citing articles on Scopus

^☆: Presented at the 25th Annual Meeting of the Canadian Association of Paediatric Surgeons, Victoria, British Columbia, September 13–15, 1993.

View full text

Improved survival for congenital diaphragmatic hernia, based on prenatal ultrasound diagnosis and referral to a combined obstetric-pediatric surgical center☆

Abstract

Clin Perinatol

J Pediatr Surg