Surgical management of the spinal deformity in Ehlers-Danlos syndrome type VI.

Abstract

Five patients with Ehlers-Danlos syndrome type VI were surgically treated for their spinal deformities. All of them were female. All but one had a double-major thoracic and lumbar curve. One had a mild scoliotic curve but severe thoracic lordosis. Anterior discectomy and fusion and posterior instrumentation was performed in a single stage in two patients, while two had anterior and posterior surgery performed in two stages. The remaining patient underwent posterior surgery only, because of the relative mildness of the deformity and adequate flexibility. Two serious vascular complications were confronted. One patient experienced avulsion of segmental arteries and rupture of iliac artery and vein during anterior surgery. Another patient had avulsion of superior gluteal artery, which happened during subperiosteal dissection to harvest iliac bone graft. Fortunately, we did not see any temporary or permanent neurological complications. The spinal fusions appeared solid radiographically, with no implant failure and loss of correction after an average follow-up of 4 years.