[Spinal deformities in Marfan disease].
Spinal deformities are frequent in Marfan's disease. 37 patients were examined at the average age of 11 years. 32 showed a spinal deformity. The treatment of the spinal deformity was a brace in 15 cases, surgery in 16 cases (including 5 cases after brace-failure). 7 cases were just followed-up without treatment. Bracing was efficient only for mild curves, this treatment was satisfying 4 times out of 11 with adequate follow-up. The surgical treatment in 16 cases was a posterior fusion twice associated with anterior fusion. We used the same technique as for idiopathic scoliosis with Harrington instrumentation 5 times, Harrington with sublaminar wires 4 times and Cotrel-Dubousset instrumentation 7 times. The correction of scoliosis was achieved in 48.2 per cent. The use of segmental instrumentation compared with the use of the Harrington instrumentation can explain the small number of non unions and the improvement of the lateral spinal balance. We did not note any cardiovascular complication during or early after the operation. Aortic lesions were responsible of one death in the long term, three patients went through a surgical replacement of aortic valves or the aorta.
Robin
H
H
Service d'Orthopédie et de Chirurgie Réparatrice de l'Enfant, Hôpital Trousseau, Paris.
Damsin
J P
JP
Filipe
G
G
Lebard
J P
JP
Carlioz
H
H
D000293:Adolescent; D002318:Cardiovascular Diseases / Q000209:etiology; D002370:Casts, Surgical; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007738:Kyphosis; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000601:surgery; D012600:Scoliosis / Q000209:etiology / Q000601:surgery; D013122:Spinal Diseases / Q000209:etiology*; D013123:Spinal Fusion; D013168:Spondylolisthesis / Q000209:etiology / Q000601:surgery
Déformations rachidiennes de la maladie de Marfan.