Congenital diaphragmatic hernia.

Department of Pediatric Surgery, Erasmusmc-Sophia, Rotterdam, The Netherlands.
Seminars in Pediatric Surgery (Impact Factor: 1.94). 08/2010; 19(3):180-5. DOI: 10.1053/j.sempedsurg.2010.03.001
Source: PubMed

ABSTRACT Congenital diaphragmatic hernia (CDH) is a congenital anomaly consisting of a posterolateral defect in the diaphragm also known as a Bochdalek hernia. It occurs in 1 in 2000 to 3000 newborns and is associated with a variable degree of pulmonary hypoplasia (PH) and persistent pulmonary hypertension (PPH). Despite remarkable advances in neonatal resuscitation and intensive care and the new postnatal treatment strategies, many newborns with CDH continue to have high rates of mortality and morbidity as the result of severe respiratory failure secondary to PH and PPH. The pathogenesis of CDH and associated PH and PPH is poorly understood. Herein, we aim to review diaphragm and pulmonary development and correlate this to the abnormalities found in CDH.

  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: PURPOSE: This study examined survival in newborn patients after congenital diaphragmatic hernia (CDH) repair. METHODS: We analyzed the Kids' Inpatient Database Years 2000, 2003, and 2006 for patients admitted at fewer than 8 d of age undergoing CDH repair. We analyzed patient demographics, clinical characteristics, socioeconomic measures, hospital type, operative case volume, and survival using Fisher's exact test and a multivariate binary logistic regression model. RESULTS: Of 847 patients identified, most were male (61%) and white (57%), were treated at urban (99.8%) and teaching (96%) hospitals, and had private insurance (57%). Survival to discharge was 95% in non-extracorporeal membrane oxygenation (ECMO) patients versus 51% for those requiring ECMO (P < 0.0001). Univariate analysis revealed significantly lower survival rates in blacks, Medicaid patients, and patients undergoing repair after 7 d of life. Among ECMO patients, we noted higher survival rates at hospitals conducting four or more ECMO cases per year (66% versus 47%; P = 0.03). Multivariate analysis identified ECMO (hazards ratio [HR] 16.23, P < 0.001), CDH repair at >7 d of age (HR 2.70, P = 0.004), and ECMO patients repaired at hospitals performing <4 CDH ECMO cases per year (HR 3.59, P = 0.03) as independent predictors of mortality. CONCLUSIONS: We conclude that ECMO hospital volume is associated with survival in patients requiring ECMO for CDH repair.
    Journal of Surgical Research 05/2012; 178(2). DOI:10.1016/j.jss.2012.05.046 · 2.12 Impact Factor
  • Source
  • [Show abstract] [Hide abstract]
    ABSTRACT: Right-sided congenital diaphragmatic hernia (R-CDH) occurs in 14 to 25 % of all CDH cases. The current literature comparing the outcome of R-CDH vs left CDH (L-CDH) is inconsistent, with some studies reporting higher and others lower mortality in R-CDH compared to L-CDH. The aim of our multicentre study was to analyse characteristics and outcome of R-CDH. We retrospectively reviewed the medical records of 178 consecutive infants with CDH who underwent surgical repair of CDH at three European tertiary pediatric surgical centres from three different countries between 2000 and 2009. The analysis focused on demographic data, morbidity and mortality in R-CDH compared with L-CDH. Out of a total of 178 children, 32 (18.0 %) right-sided and 146 (82.0 %) left-sided cases of CDH were identified. Prenatal diagnosis was made in 8 R-CDH vs 67 L-CDH (25.0 vs 45.9 %, p = 0.030). Median gestational age in R-CDH was 39 weeks (range 29-42 weeks) and 39 weeks in L-CDH (range 28-43 weeks, p = 0.943). Median birth weight in R-CDH was 3233 g (range 905-4480 g) and in L-CDH was 3060 g (range 1065-5240 g, p = 0.184). Major associated anomalies were present in 19 R-CDH vs 46 L-CDH (59.4 vs 31.5 %, p = 0.003). Extracorporeal membrane oxygenation (ECMO) was required in 3 R-CDH vs 19 L-CDH (9.4 vs 13.0 %, p = 0.571). A diaphragmatic patch was used in 13 R-CDH and 59 L-CDH (40.6 vs 40.4 %, p = 0.982). Fundoplication for GERD was required in 1 R-CDH and 19 L-CDH (3.1 vs 13.0 %, p = 0.109). No significant differences were observed in recurrence rate (9.4 vs 8.9 %, p = 0.933). Postoperative mortality rate was significantly higher in R-CDH compared to L-CDH (21.9 vs 8.2 %, p = 0.023). In R-CDH, prenatal diagnosis and patch repair correlated with mortality by univariate regression (p = 0.005 and p = 0.019). This multicentre study shows that prenatal diagnosis and patch repair were associated with an increased mortality rate in R-CDH. However, the morbidity following repair of R-CDH was not significantly different from that in L-CDH in survivors.
    Pediatric Surgery International 03/2015; 31(5). DOI:10.1007/s00383-015-3695-y · 1.06 Impact Factor