Spinal deformity in Ehlers-Danlos syndrome. Five patients treated by spinal fusion.
Five patients with classical Ehlers-Danlos syndrome developed severe spinal deformities. Two were shown to have type-VI collagen abnormalities. Three had a double structural scoliosis of the thoracic and lumbar regions, one had a single thoracic scoliosis and one had a thoracic kyphosis. The curves first developed before the age of four years, and were not controlled by bracing. Major corrective surgery with posterior fusion was performed at a mean age of 11 years 8 months. Excessive blood loss could be controlled and although wound haematoma and dehiscence were common, they did not provide major problems. The spinal fusions healed satisfactorily.
McMaster
M J
MJ
Princess Margaret Rose Orthopaedic Hospital, Edinburgh, Scotland.
D016063:Blood Loss, Surgical; D002675:Child, Preschool; D004535:Ehlers-Danlos Syndrome / Q000150:complications*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007738:Kyphosis / Q000209:etiology / Q000503:physiopathology / Q000601:surgery*; D012600:Scoliosis / Q000209:etiology / Q000503:physiopathology / Q000601:surgery*; D013123:Spinal Fusion / Q000379:methods*; D014945:Wound Healing