Abstract
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.
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