Congenital diaphragmatic hernia: the hidden morbidity

Area of Science:

• Pediatric Surgery
• Neonatology
• Developmental Pediatrics

Background:

• Congenital diaphragmatic hernia (CDH) survivors are often presumed to have isolated lung hypoplasia.
• Limited data exist on extrapulmonary complications in high-risk CDH survivors.

Purpose of the Study:

• To investigate the spectrum and prevalence of extrapulmonary problems in infants surviving high-risk congenital diaphragmatic hernia (CDH).

Main Methods:

• A multidisciplinary follow-up clinic was established in 1990.
• 33 infants with high-risk CDH (symptomatic within 6 hours of birth) were followed.
• 20 patients received extracorporeal membrane oxygenation (ECMO) treatment.

Main Results:

• Neurological issues were common: 21% needed hearing aids, 7 had abnormal brain-stem auditory evoked response (BAER) tests, 10 had head CT abnormalities, and 4 experienced seizures.
• Developmental delays affected 15 patients, growth and nutrition problems were noted in 13 (below 5th percentile for weight), and 6 required fundoplications.
• Other complications included recurrent hernias (2/10 patch repairs), bowel obstruction (6), pectus excavatum (11), scoliosis (4), undescended testicles (5), vesicoureteral reflux (2), and kidney stones (2).

Conclusions:

• High-risk congenital diaphragmatic hernia (CDH) survivors experience a high burden of extrapulmonary morbidities.
• These complications span neurological, developmental, gastrointestinal, orthopedic, and genitourinary systems.
• Comprehensive, multidisciplinary long-term follow-up is crucial for managing these complex patients.