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Congenital diaphragmatic hernia in 120 infants treated consecutively with permissive hypercapnea/spontaneous
Judd Boloker1, David A Bateman, Jen-Tien Wung
1Children's Hospital of New York, Divisions of Neonatology and Surgery, New York, USA.
Insights
A novel care strategy for congenital diaphragmatic hernia (CDH) involving permissive hypercapnia, spontaneous respiration, and elective repair significantly improves survival rates. This approach challenges traditional views, offering better outcomes for infants with this severe condition.
Area of Science:
- Pediatric Surgery
- Neonatal Critical Care
- Respiratory Physiology
Background:
- Congenital diaphragmatic hernia (CDH) is historically associated with poor prognosis, including a 50% survival rate and significant morbidity.
- Conventional management strategies for CDH often involve aggressive ventilation, which can lead to complications.
Purpose of the Study:
- To evaluate the efficacy of a care strategy combining permissive hypercapnia, spontaneous respiration, and elective surgical repair in infants with CDH.
- To challenge traditional management paradigms for CDH by assessing outcomes under this alternative approach.
Main Methods:
- A retrospective review of 120 infants with CDH treated between August 1992 and February 2000.
- All infants received a respiratory care strategy of permissive hypercapnia and spontaneous respiration, coupled with elective surgical repair.
- Outcome measures included need for extracorporeal membrane oxygenation (ECMO), discharge to home, supplemental oxygen requirements, and the impact of ancillary therapies.
Main Results:
- An overall survival rate of 75.8% was observed; excluding cases with lethal anomalies or severe pulmonary hypoplasia, survival to discharge was 84.4%.
- Non-ECMO ancillary therapies (surfactant, nitric oxide, high-frequency oscillatory ventilation) did not significantly impact survival rates.
- Extracorporeal membrane oxygenation (ECMO) was required in 13.3% of cases. Transabdominal surgery with prosthetic repair was used in 7%.
Conclusions:
- The implemented care strategy of permissive hypercapnia, spontaneous respiration, and elective surgery leads to high survival rates in infants with life-threatening CDH.
- This approach results in minimal pulmonary morbidity at discharge for the majority of treated infants.
Background/Purpose:
Poor prognosis (approximately 50% survival rate and significant morbidity) traditionally has been associated with congenital diaphragmatic hernia (CDH). The authors reviewed a single institution experience and challenged conventional wisdom in the context of a care strategy based on permissive hypercapnea/spontaneous respiration/elective repair.
Methods:
From August 1992 through February 2000, all infants with CDH and (1) respiratory distress requiring mechanical ventilation, (2) in-born or (3) transferred preoperatively within hours of birth are reported. All respiratory care strategy used permissive hypercapnea/spontaneous respiration and combined with elective repair. Arterial blood gas values and concomitant ventilator support were recorded. Outcome markers were (1) need for extracorporeal membrane oxygenation ECMO, (2) discharge to home, (3) supplemental oxygen need at discharge, and (4) influence of non-ECMO ancillary therapies (surfactant, nitric oxide, high-frequency oscillatory ventilation).
Results:
One hundred twenty consecutive infants were reviewed. Overall survival rate was 75.8%, but, excluding 18 of 120 not treated (6 lethal anomalies, 10 overwhelming pulmonary hypoplasia, 3 prerepair ECMO-related neurocomplications), 84.4% survived to discharge. A total of 67/120 were inborn. Non-ECMO ancillary treatments had no impact on survival rate. ECMO was used in 13.3%. Surgery was transabdominal; prosthetics were used in 7%. Tube thoracostomy was rare. Every inborn patient (n = 11) requiring a chest tube for pneumothorax died. Respiratory support before surgery was peak inspiratory pressure (PIP), 22, FIO(2),.43 with PaO(2), 66 torr; PaCO(2), 41 torr; and pH, 7.32. The survivors discharged on oxygen (n = 2) died at 4 and 7 months.
Conclusions:
The majority of infants with life-threatening CDH treated with permissive hypercapnea/spontaneous respiration/elective surgery survive to discharge with minimal pulmonary morbidity.
