Long-term outcomes beyond childhood in patients treated for congenital diaphragmatic hernia- a systematic review

Area of Science:

• Medical Sciences
• Pediatric Surgery
• Adult Medicine

Background:

• Improved survival rates for congenital diaphragmatic hernia (CDH) necessitate focus on long-term outcomes in adult survivors.
• Limited physician awareness of CDH sequelae hinders effective transition from pediatric to adult care.
• Growing population of adult CDH survivors requires comprehensive understanding of their ongoing health challenges.

Purpose of the Study:

• To systematically review and evaluate common long-term complications in adult survivors of congenital diaphragmatic hernia (CDH) aged 16 years and older.
• To synthesize evidence on pulmonary, gastrointestinal, musculoskeletal, and psychological sequelae following neonatal surgical repair of CDH.
• To inform the development of structured transition programs and multidisciplinary follow-up for adult CDH patients.

Main Methods:

• Systematic literature search of major databases (PubMed, MEDLINE, EMBASE, Web of Science, Cochrane Library) following PRISMA guidelines.
• Inclusion of 19 studies encompassing 250 adult CDH survivors (mean age 22.1 years).
• Analysis of reported long-term complications including chronic lung disease, dyspnea, GERD, scoliosis, and quality of life indicators.

Main Results:

• Chronic lung disease affected 33% of survivors; 28% reported dyspnea.
• Gastroesophageal reflux disease (GERD) was prevalent in 45% of patients.
• Severe scoliosis occurred in 2.4%, and 30% experienced depression, with lower scores in vitality and mental health domains.

Conclusions:

• Adult congenital diaphragmatic hernia (CDH) survivors frequently experience multisystem morbidities requiring specialized adult care.
• Structured transition programs and multidisciplinary follow-up are crucial for managing long-term CDH sequelae.
• Further research is needed to optimize outcomes and establish standardized care protocols for this population.