1994 Spinal deformity in Ehlers-Danlos syndrome

Description: J Bone Joint Surg Br. 1994 Sep;76(5):773-7. Spinal deformity in Ehlers-Danlos syndrome. Five patients treated by spinal fusion.McMaster MJ. Princess Margaret Rose Orthopaedic Hospital, Edinburgh, Scotland. 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.