Multi-institutional follow-up of patients with congenital diaphragmatic hernia reveals severe disability and variations in practice.

BACKGROUND/PURPOSE: Survivors of congenital diaphragmatic hernia (CDH) have a high incidence of morbidity. Variability in follow-up practices between institutions may affect perception of disability and prevent population-based outcome analysis.

METHODS

A survey of follow-up practices at 16 centers within a population-based CDH network was performed. A descriptive analysis of outcomes (minimum 24 months postdischarge) of CDH survivors from the 2 largest centers was performed.

RESULTS

The nature of follow-up of CDH survivors was highly variable in 12 of 16 responding centers, ranging from ad hoc, community-based, and pediatrician-sponsored follow-up to a single perinatal center-based multispecialty CDH clinic. Outcomes at 24 to 36 months were reported from the 2 largest centers (n = 44). Among survivors, neurodevelopmental disability was most common (12/44; 27%) followed by gastrointestinal (9; 20.5%), pulmonary (5; 11.4%), musculoskeletal (5; 11.4%), and cardiac (2; 4.5%). Additional surgery was required in 17 patients (38.6%), including recurrent CDH repair in 7 (15.9%). Five patients (11.4%) had hearing loss. Among 41 children with available 24-month data, 32 (78%), 17 (41.5%), and 14 (34.1%) patients had weights below the 50th, 25th, and 3rd percentiles, respectively.

CONCLUSION

Congenital diaphragmatic hernia survivorship is associated with significant disability. Standardization of follow-up practices is essential to enable population-based outcomes analysis.