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Current surgical management of congenital diaphragmatic hernia: a report from the Congenital Diaphragmatic Hernia
Insights
Congenital diaphragmatic hernia (CDH) management has shifted to delayed repair, with prosthetic patching common. However, a 63% survival rate indicates significant challenges remain for this condition.
Area of Science:
- Pediatric Surgery
- Neonatal Care
- Thoracic Surgery
Background:
- Congenital diaphragmatic hernia (CDH) management has evolved, moving from emergent to delayed repair strategies.
- Current understanding is limited by single-institution reports, hindering the establishment of standardized care.
- A need exists for large-scale data to guide optimal CDH treatment protocols.
Purpose of the Study:
- To establish a multicenter database for congenital diaphragmatic hernia (CDH) management.
- To analyze current surgical practices and outcomes in a large cohort of CDH patients.
- To identify trends and challenges in the contemporary treatment of CDH.
Main Methods:
- Formation of the CDH Study Group in 1995, collecting data from North America, Europe, and Australia.
- Registry of all live-born infants with CDH during 1995-1996, including demographics, surgical management, and outcomes.
- Data collection from 62 participating centers, encompassing 461 patients.
Main Results:
- Overall survival rate of 63% (280/442 patients).
- Prosthetic patching used in 51% of cases, predominantly with polytetrafluoroethylene.
- Delayed surgical repair is common, with most repairs occurring during standard working hours.
Conclusions:
- Multicenter data provides a snapshot of current CDH management, highlighting increased use of prosthetic patches and delayed repair.
- The 63% survival rate underscores the ongoing challenges in treating congenital diaphragmatic hernia.
- A call for centralized databases and cooperative studies to advance CDH care and improve outcomes.
Background:
Repair of congenital diaphragmatic hernia (CDH) has changed from an emergent procedure to a delayed procedure in the last decade. Many other aspects of management have also evolved since the first successful repair. However, most reports are from single institutions. The lack of a large multicenter database has hampered progress in the management of congenital diaphragmatic hernia (CDH) and makes determination of the current standard difficult.
Methods:
The CDH study group was formed in 1995 to collect data from multiple institutions in North America, Europe, and Australia. Participating centers completed a registry form on all live-born infants with CDH during 1995 and 1996. Demographic information, data about surgical management, and outcome were collected for all patients.
Results:
Sixty-two centers participated, with 461 patients entered. Overall survival was 280 of 442 patients (63%) where survival was recorded. The defect was left-sided in 78%, right-sided in 21%, and bilateral in 1%. A subcostal approach was used in 91% of patients, with pleural drainage used in 76%. A patch of some kind was used in just over half (51%) of the patients, with polytetrafluoroethylene being the most commonly used material (81%) in those patients with a patch. The mean surgical time was 102 minutes, with an average blood loss of 14 mL (range, 0 to 500 mL). The overwhelming majority of patients underwent repair between 6:00 AM and 6:00 PM (289 of 329, 88%). Nineteen percent of patients had surgical repair on extracorporeal membrane oxygenation (ECMO) at a mean time of 170 hours into the ECMO course (range, 10 to 593 hours). The mean age at surgery in patients not treated with ECMO was 73 hours (range, 1 to 445 hours).
Conclusions:
The multicenter nature of this report makes it a snapshot of current management. The data would indicate that prosthetic patching of the defect has become common, that after-hours repair is infrequent, and that delayed surgical repair has become the preferred approach in many centers. Furthermore, the mean survival rate of 63% indicates that despite decades of individual effort, the CDH problem is far from solved. This highlights the need for a centralized database and cooperative multicenter studies in the future.