[Scoliosis and congenital diaphragmatic agenesis]. / Scolioses associées aux aplasies de coupole diaphragmatique.

PURPOSE OF THE STUDY: Diaphragmatic agenesis is the most extreme anatomic form of congenital diaphragmatic defects. Clinically this pathology is not different from posterolateral diaphragmatic hernia described by Bochdalek. We describe our observations of scoliosis in 16 neonates with diaphragmatic agenesis. MATERIAL AND METHODS: Between 1987 and 1996, 69 neonates underwent surgery for a congenital diaphragmatic hernia (CDH) at our institution. Sixteen of these patients had the most extreme form of CDH. Ten of these 16 patients survived. Spinal and/or chest wall deformities were observed in nine patients, including isolated scoliosis in two patients, scoliosis associated with a chest wall deformity in three, and isolated chest wall deformity (pectus excavatum or pectus carnium) in four. These deformities developed between 13 months and three and a half years of age (mean: two years). RESULTS: For two patients the deformity was mild (Cobb angle<15 degrees ) and required only observation. A Milwaukee brace was used in three patients that had severe kyphoscoliosis. Isolated chest wall deformities were treated with observation. No patient required operative treatment for these skeletal deformities. DISCUSSION: The preliminary results of this study indicate that chest wall deformities and scoliosis frequently occur during surgery for diaphragmatic agenesis. Nonoperative treatment is usually sufficient, however, follow-up of these patients until adulthood is recommended.