Outcome at 2 years of infants with congenital diaphragmatic hernia: a population-based study

Management of neonates with congenital diaphragmatic hernia (CDH) has undergone many changes associated with increased survival of high-risk CDH. However, little is known about the long-term outcome of CDH infants.

Follow-up was performed in 85 newborn infants with CDH admitted in our neonatal intensive care unit between January 1991 and December 1998. Early (< 2 months) and late mortality (> or = 2 months), and respiratory, nutritional, musculoskeletal, and neurosensory outcome at 2 years were recorded.

Surgical repair was performed in 59 infants (69%) at a median postnatal age of 124 (range, 38 to 246) hours. Extracorporeal membrane oxygenation was used in 26 (30%) newborn infants. Survival at 2 years was 51 of 85 (60%) (early death, 28/85 [33%]; late death, 6/85 [7%]). Late deaths occurred because of persistent pulmonary hypertension or iatrogenic complications. Twelve of 51 (24%) newborn infants were oxygen dependant at the postnatal age of 28 days, and 1 of 51 (1.9%) was still oxygen dependant at 2 years. Growth failure was noted in 9 of 51 (18%), mainly related to severe gastroesophageal reflux and oral aversion. Scoliosis was diagnosed in 2 infants. Neurologic examination at 2 years was normal in 45 of 51 (88%). Cerebral palsy and developmental delay were observed in 2 and 4 infants, respectively. Four infants (8%) experienced associated problems. Respiratory, nutritional, and musculoskeletal morbidity was higher in infants treated by extracorporeal membrane oxygenation (p < 0.05).

CDH infants are at risk for adverse nutritional and respiratory outcome. Despite severe respiratory failure at birth, prolonged oxygen therapy above 2 years of age is uncommon. Conversely, failure to thrive related at least in part to gastroesophageal reflux and oral dysfunction remains the major problem at 2 years of age. However, both nutritional and respiratory problems tend to improve with age.