[Spine deformity in Marfan's syndrome in childhood (author's transl)].
The authors have studied twenty cases of spine deformity in children suffering from Marfan's syndrome. They describe the main features of the disease and point out the difficulty of accurate diagnosis in children with minor involvement. The deformities are those of a dysplastic scoliosis as shown by the frequency of vertebral inversion on the lateral view: dorso-lumbar kyphosis, decreased lumbar lordosis. The treatment should be surgical because of the danger of a fatal outcome without treatment. The incidence of non-union is high and the authors recommend systematic revision of the fusion double curve scolioses with a moderate kyphosis. In cases of severe kyphosis, the addition of anterior grafting is recommended.
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007738:Kyphosis / Q000175:diagnosis / Q000209:etiology; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000175:diagnosis; D011183:Postoperative Complications; D011542:Pseudarthrosis / Q000517:prevention & control; D012600:Scoliosis / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D013131:Spine / Q000002:abnormalities
Les déviations rachidiennes de la maladie de Marfan chez l'enfant. Etude de 20 cas.