Delayed surgical repair and ECMO improves survival in congenital diaphragmatic hernia

Karen W. West, Kristi Bengston, Frederick Rescorla, William A. Engle, Jay L. Grosfeld

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101 Citations (Scopus)

Abstract

One hundred ten infants with congenital diaphragmatic hernia (CDH) developed life-threatening respiratory distress in the first 6 hours of life. Associated anomalies were present in 33%. Twenty-eight of 65 infants (43%) treated before 1987 (pre-extracorporeal membrane oxygenation [ECMO] era) survived after immediate CDH repair, and mechanical ventilation with or without pharmacologic support. Only two of 16 (12.5%) infants requiring a prosthetic diaphragmatic patch survived. Since 1987, 31 of 46 (67.4%) infants with birth weight, gestational age, and severity of illness similar to the pre-1987 group survived. All patients were immediately intubated and ventilated. Seven (four with lethal chromosomal anomalies) infants died before treatment, and 30 stabilized (partial pressure of carbon dioxide [Pco2] < 50; partial pressure of oxygen [Po2] > 100; pH > 7.3) and underwent delayed CDH repair at 5 to 72 hours. Fifteen did well on conventional support and survived. Fifteen infants deteriorated after operation: 11 were placed on ECMO with eight survivors, and four infants were not considered ECMO candidates. Nine babies failed to stabilize initially and were placed on ECMO before CDH repair (alveolar-arterial gradient > 600 and oxygenation index > 40), and seven survived. The overall survival rate was 80% at 3 months in this ECMO-treated group. Early mortality was due to inability to wean from ECMO (one), intracranial hemorrhage (one), liver injury (one), and pulmonary hypoplasia (one). Nine of 11 babies requiring a prosthetic patch in the post-1987 ECMO group survived (81.8%). There were three late post-ECMO deaths (3 to 18 months) of right heart failure (two) and sepsis (one). Symptomatic gastroesophageal reflux occurred in nine cases, six requiring a fundoplication in the bypass babies. Recurrent diaphragmatic hernia occurred in nine cases (five ECMO). The overall survival rate was significantly improved in the delayed repair/ECMO group (67% versus 43%; p < 0.05) and was most noticeable in infants requiring a prosthetic diaphragm (81.2% versus 12.5%; p < 0.005). These data indicate that early stabilization, delayed repair, and ECMO improve survival in high-risk CDH. Early deaths are related to pulmonary hypertension and can be reversed by ECMO.

Original languageEnglish
Pages (from-to)454-460
Number of pages7
JournalAnnals of Surgery
Volume216
Issue number4
StatePublished - 1992

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Extracorporeal Membrane Oxygenation
Survival
Herniorrhaphy
Congenital Diaphragmatic Hernias
Survival Rate
Fundoplication
Diaphragmatic Hernia
Intracranial Hemorrhages
Partial Pressure
Lung Injury
Gastroesophageal Reflux
Diaphragm
Artificial Respiration
Pulmonary Hypertension
Birth Weight
Carbon Dioxide
Gestational Age
Survivors
Sepsis
Heart Failure

ASJC Scopus subject areas

  • Surgery

Cite this

West, K. W., Bengston, K., Rescorla, F., Engle, W. A., & Grosfeld, J. L. (1992). Delayed surgical repair and ECMO improves survival in congenital diaphragmatic hernia. Annals of Surgery, 216(4), 454-460.

Delayed surgical repair and ECMO improves survival in congenital diaphragmatic hernia. / West, Karen W.; Bengston, Kristi; Rescorla, Frederick; Engle, William A.; Grosfeld, Jay L.

In: Annals of Surgery, Vol. 216, No. 4, 1992, p. 454-460.

Research output: Contribution to journalArticle

West, KW, Bengston, K, Rescorla, F, Engle, WA & Grosfeld, JL 1992, 'Delayed surgical repair and ECMO improves survival in congenital diaphragmatic hernia', Annals of Surgery, vol. 216, no. 4, pp. 454-460.
West, Karen W. ; Bengston, Kristi ; Rescorla, Frederick ; Engle, William A. ; Grosfeld, Jay L. / Delayed surgical repair and ECMO improves survival in congenital diaphragmatic hernia. In: Annals of Surgery. 1992 ; Vol. 216, No. 4. pp. 454-460.
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abstract = "One hundred ten infants with congenital diaphragmatic hernia (CDH) developed life-threatening respiratory distress in the first 6 hours of life. Associated anomalies were present in 33{\%}. Twenty-eight of 65 infants (43{\%}) treated before 1987 (pre-extracorporeal membrane oxygenation [ECMO] era) survived after immediate CDH repair, and mechanical ventilation with or without pharmacologic support. Only two of 16 (12.5{\%}) infants requiring a prosthetic diaphragmatic patch survived. Since 1987, 31 of 46 (67.4{\%}) infants with birth weight, gestational age, and severity of illness similar to the pre-1987 group survived. All patients were immediately intubated and ventilated. Seven (four with lethal chromosomal anomalies) infants died before treatment, and 30 stabilized (partial pressure of carbon dioxide [Pco2] < 50; partial pressure of oxygen [Po2] > 100; pH > 7.3) and underwent delayed CDH repair at 5 to 72 hours. Fifteen did well on conventional support and survived. Fifteen infants deteriorated after operation: 11 were placed on ECMO with eight survivors, and four infants were not considered ECMO candidates. Nine babies failed to stabilize initially and were placed on ECMO before CDH repair (alveolar-arterial gradient > 600 and oxygenation index > 40), and seven survived. The overall survival rate was 80{\%} at 3 months in this ECMO-treated group. Early mortality was due to inability to wean from ECMO (one), intracranial hemorrhage (one), liver injury (one), and pulmonary hypoplasia (one). Nine of 11 babies requiring a prosthetic patch in the post-1987 ECMO group survived (81.8{\%}). There were three late post-ECMO deaths (3 to 18 months) of right heart failure (two) and sepsis (one). Symptomatic gastroesophageal reflux occurred in nine cases, six requiring a fundoplication in the bypass babies. Recurrent diaphragmatic hernia occurred in nine cases (five ECMO). The overall survival rate was significantly improved in the delayed repair/ECMO group (67{\%} versus 43{\%}; p < 0.05) and was most noticeable in infants requiring a prosthetic diaphragm (81.2{\%} versus 12.5{\%}; p < 0.005). These data indicate that early stabilization, delayed repair, and ECMO improve survival in high-risk CDH. Early deaths are related to pulmonary hypertension and can be reversed by ECMO.",
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