Late-presenting congenital diaphragmatic hernia in childhood

Aim: To characterize the clinical manifestations of late-presenting diaphragmatic hernia and its associated anomalies, diagnostic methods and outcomes. Methods: The records of patients aged 1 month-18 years old diagnosed with Bochdalek diaphragmatic hernia from February 1987-June 2008 were reviewed. Results: Fifteen children (nine boys, six girls) met inclusion criteria. Median age was 1.5 years (range, 38 days-9.9 years). Eleven (73%) had left-sided and four (27%) had right-sided diaphragmatic hernias. Six (40%) patients presented with respiratory symptoms, six (40%) with gastrointestinal symptoms and three (20%) with both. Five (33%) patients had failure to thrive. Six (40%) were diagnosed by chest radiography alone. The others required gastrointestinal contrast series, or chest computed tomography to confirm the diagnosis. One referred patient had been misdiagnosed as having left pneumothorax. Cases of bowel malrotation and gastric volvulus associated with the hernia were found in one patient each. One patient required mechanical ventilation because of respiratory failure before surgery. Primary repair without patch was performed in all patients. The overall survival in this series was 100%. Conclusion: Late-presenting diaphragmatic hernia should be suspected in cases of unexplained acute or chronic respiratory or gastrointestinal symptoms, and abnormal chest radiographic findings. The prognosis is favourable with correct diagnosis and prompt surgical repair.