Spinal deformity in Marfan syndrome.
Fourteen patients with classic Marfan syndrome and spinal deformity treated at Texas Scottish Rite Hospital for Crippled Children (Dallas, TX) after 1975 have been conservatively treated to maturity or are at least 2 years after spinal fusion. Brace treatment was successful in one of nine patients. Nine patients underwent fusion. Three had a solid fusion, two lost 20 degrees of correction, and four required revision for multiple pseudarthroses. These latter four all had or developed a kyphotic deformity. Although Marfan syndrome patients can achieve solid fusion, they require massive bone graft, secure segmental internal fixation, and careful observation for pseudarthrosis. Kyphotic deformities require anterior fusion.
D000293:Adolescent; D000328:Adult; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007738:Kyphosis / Q000175:diagnosis* / Q000601:surgery; D008297:Male; D008382:Marfan Syndrome / Q000175:diagnosis*; D012600:Scoliosis / Q000175:diagnosis* / Q000601:surgery; D013123:Spinal Fusion