Bilateral congenital diaphragmatic hernia

Holly Neville, Tom Jaksic, Jay M. Wilson, Pamela A. Lally, William D. Hardin, Ronald B. Hirschl, Kevin P. Lally

Research output: Contribution to journalArticle

44 Citations (Scopus)

Abstract

Background/Purpose: CDH occurs in approximately 1 in 2,450 live births. Bilateral CDH, previously identified through a limited number of case reports, is extremely rare. The care of CDH patients is a challenge for neonatologists and surgeons. This report details the management and outcome of patients with bilateral CDH. Methods: Records of all liveborn patients with CDH between 1995 and 2001 in 83 hospitals were entered into the CDH database. Those with bilateral CDH were reviewed retrospectively. Data were analyzed using the X2 test. Results: A total of 1833 patients were entered in the database, 17 of these had bilateral CDH (0.9%). Eleven were boys. The average birth weight was 2.6 kg. The average gestational age was 36.8 weeks. Sixteen patients experienced early distress requiring intubation (12 immediately), and 4 were placed on extracorporeal membrane oxygenation (ECMO). Seven patients were diagnosed prenatally. Twelve patients (70%) were found to have other anomalies, 3 had chromosomal abnormalities and 7 had cardiac anomalies. These included tetralogy of Fallot (TOF), VSD, absence of the pericardium, coarctation of the aorta (2), accessory SVC with aortic coarctation, and ASD with TOF. Only 9 of 17 (53%) patients underwent surgical repair (6 primary, 3 patch). Mortality rate was 65% compared with 33% of patients with unilateral CDH (P = 0.01). Seven patients died within 48 hours of birth. There was no significant difference in survival based on gender, weight, gestational age, presence of anomalies, or prenatal diagnosis. Conclusions: The management of infants with bilateral congenital diaphragmatic hernia remains a difficult problem with a significant mortality. Bilateral congenital diaphragmatic hernia is associated more frequently with other major anomalies than unilateral congenital diaphragmatic hernia and should prompt an evaluation for further anomalies.

Original languageEnglish
Pages (from-to)522-524
Number of pages3
JournalJournal of Pediatric Surgery
Volume38
Issue number3
DOIs
StatePublished - Mar 1 2003
Externally publishedYes

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Aortic Coarctation
Tetralogy of Fallot
Gestational Age
Databases
Congenital Diaphragmatic Hernias
Extracorporeal Membrane Oxygenation
Mortality
Pericardium
Live Birth
Prenatal Diagnosis
Intubation
Birth Weight
Chromosome Aberrations
Patient Care
Parturition
Weights and Measures
Survival
Neonatologists
Surgeons

Keywords

  • Bilateral congenital diaphragmatic hernia
  • Outcome

ASJC Scopus subject areas

  • Surgery

Cite this

Neville, H., Jaksic, T., Wilson, J. M., Lally, P. A., Hardin, W. D., Hirschl, R. B., & Lally, K. P. (2003). Bilateral congenital diaphragmatic hernia. Journal of Pediatric Surgery, 38(3), 522-524. https://doi.org/10.1053/jpsu.2003.50092

Bilateral congenital diaphragmatic hernia. / Neville, Holly; Jaksic, Tom; Wilson, Jay M.; Lally, Pamela A.; Hardin, William D.; Hirschl, Ronald B.; Lally, Kevin P.

In: Journal of Pediatric Surgery, Vol. 38, No. 3, 01.03.2003, p. 522-524.

Research output: Contribution to journalArticle

Neville, H, Jaksic, T, Wilson, JM, Lally, PA, Hardin, WD, Hirschl, RB & Lally, KP 2003, 'Bilateral congenital diaphragmatic hernia', Journal of Pediatric Surgery, vol. 38, no. 3, pp. 522-524. https://doi.org/10.1053/jpsu.2003.50092
Neville H, Jaksic T, Wilson JM, Lally PA, Hardin WD, Hirschl RB et al. Bilateral congenital diaphragmatic hernia. Journal of Pediatric Surgery. 2003 Mar 1;38(3):522-524. https://doi.org/10.1053/jpsu.2003.50092
Neville, Holly ; Jaksic, Tom ; Wilson, Jay M. ; Lally, Pamela A. ; Hardin, William D. ; Hirschl, Ronald B. ; Lally, Kevin P. / Bilateral congenital diaphragmatic hernia. In: Journal of Pediatric Surgery. 2003 ; Vol. 38, No. 3. pp. 522-524.
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abstract = "Background/Purpose: CDH occurs in approximately 1 in 2,450 live births. Bilateral CDH, previously identified through a limited number of case reports, is extremely rare. The care of CDH patients is a challenge for neonatologists and surgeons. This report details the management and outcome of patients with bilateral CDH. Methods: Records of all liveborn patients with CDH between 1995 and 2001 in 83 hospitals were entered into the CDH database. Those with bilateral CDH were reviewed retrospectively. Data were analyzed using the X2 test. Results: A total of 1833 patients were entered in the database, 17 of these had bilateral CDH (0.9{\%}). Eleven were boys. The average birth weight was 2.6 kg. The average gestational age was 36.8 weeks. Sixteen patients experienced early distress requiring intubation (12 immediately), and 4 were placed on extracorporeal membrane oxygenation (ECMO). Seven patients were diagnosed prenatally. Twelve patients (70{\%}) were found to have other anomalies, 3 had chromosomal abnormalities and 7 had cardiac anomalies. These included tetralogy of Fallot (TOF), VSD, absence of the pericardium, coarctation of the aorta (2), accessory SVC with aortic coarctation, and ASD with TOF. Only 9 of 17 (53{\%}) patients underwent surgical repair (6 primary, 3 patch). Mortality rate was 65{\%} compared with 33{\%} of patients with unilateral CDH (P = 0.01). Seven patients died within 48 hours of birth. There was no significant difference in survival based on gender, weight, gestational age, presence of anomalies, or prenatal diagnosis. Conclusions: The management of infants with bilateral congenital diaphragmatic hernia remains a difficult problem with a significant mortality. Bilateral congenital diaphragmatic hernia is associated more frequently with other major anomalies than unilateral congenital diaphragmatic hernia and should prompt an evaluation for further anomalies.",
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