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Treatment and outcome of congenital diaphragmatic hernia
Insights
This study on congenital diaphragmatic hernia (CDH) found that noninvasive respiratory care and delayed surgery led to a survival rate of nearly 75%. This approach is viable for most hospitals without advanced ECMO technology.
Area of Science:
- Pediatric Surgery
- Neonatal Intensive Care
- Respiratory Medicine
Background:
- Congenital diaphragmatic hernia (CDH) presents a significant challenge with high mortality.
- Optimal therapeutic strategies for CDH remain an area of active investigation.
Purpose of the Study:
- To describe the clinical characteristics, treatment, and outcomes of infants with CDH.
- To evaluate the efficacy of a noninvasive respiratory care strategy combined with delayed surgical repair.
Main Methods:
- Retrospective analysis of 48 infants with CDH treated between 1987 and 1998.
- Ventilator strategy focused on permissive hypercapnia and avoiding hyperventilation.
- Delayed surgical repair (after 3 days of age) was employed in most cases.
Main Results:
- Bochdalek CDH was most common (46/48 cases); antenatal diagnosis in 10 cases.
- Respiratory distress was the primary manifestation, typically presenting at birth.
- Overall mortality was 27%, with major causes including respiratory failure and pulmonary hypertension.
Conclusions:
- A survival rate of nearly 75% was achieved in this cohort.
- Noninvasive respiratory care and delayed surgery represent an acceptable treatment strategy for CDH.
- This approach is feasible in institutions lacking extracorporeal membrane oxygenation (ECMO) support.
Background And Purpose:
Congenital diaphragmatic hernia (CDH) is a challenging condition and is associated with a high mortality rate; optimal therapy remains unclear. This retrospective study describes the clinical characteristics of treatment and outcome in 48 infants with CDH.
Methods:
Twenty-eight male (58%) and 20 female (42%) infants with CDH were treated from 1987 through 1998. The goals of the ventilator strategy were permissive hypercapnea (PaCO2 < or = 55 mm Hg) and avoidance of hyperventilation. Infants were initially ventilated with an intermittent mandatory rate of 40 to 60 per minute, peak inspiratory pressure of 20 to 25 cm H2O, and positive end-expiratory pressure of 5 cm H2O. High-frequency positive pressure ventilation was used if hypoxemia or severe hypercapnea (PaCO2 > 60 mm Hg) occurred. Most infants underwent repair after 3 days of age and only four infants underwent early repair within 24 hours of birth. A prophylactic chest tube was placed in the ipsilateral hemithorax postoperatively in all patients treated before 1996. The severity of respiratory distress was estimated by alveolar-arterial oxygen difference, oxygenation index, and alveolar-arterial ratio.
Results:
Forty-six patients presented with Bochdalek CDH, and two with Morgangni CDH. Antenatal diagnosis was made in 10 cases. Respiratory distress was the major manifestation and usually occurred immediately after birth. Six cases were diagnosed several months after birth and presented mainly with gastrointestinal symptoms. Eleven patients died before surgery and 37 patients underwent surgical repair. Two infants died postoperatively because of congestive heart failure and tension pneumothorax, respectively. The overall mortality rate was 27%. The major causes of mortality were severe respiratory failure, persistent pulmonary hypertension, pneumothorax, and associated anomalies.
Conclusion:
Nearly 75% of patients in this series survived. This suggests that noninvasive respiratory care combined with delayed surgery may be an acceptable strategy for the treatment of CDH, and can be used in most medical institutions without equipment for extracorporeal membrane oxygenation therapy.