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Ann Thorac Surg 2003;75:250-256
© 2003 The Society of Thoracic Surgeons


Original article: general thoracic

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

Sophie M. Jaillard, MDa*, Véronique Pierrat, MDb, Angélina Dubois, MDb, Patrick Truffert, MDb, Pierre Lequien, MDb, Alain J. Wurtz, MDa, Laurent Storme, MDb

a Department of Thoracic Surgery, University Hospital of Lille, Lille, France
b Department of Neonatology, University Hospital of Lille, Lille, France

Accepted for publication August 8, 2002.

* Address reprint requests to Dr Jaillard, Clinique Chirurgicale, Hôpital Calmette, Centre Hôpitalier Régional et Universitaire de Lille, 59037 Lille, France.
e-mail: sjaillard{at}chru-lille.fr

BACKGROUND: 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.

METHODS: 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 (>= 2 months), and respiratory, nutritional, musculoskeletal, and neurosensory outcome at 2 years were recorded.

RESULTS: 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 gastro-esophageal 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).

CONCLUSIONS: 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.




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