Dystrophic spinal deformities in neurofibromatosis. Treatment by anterior and posterior fusion

Abstract

Thirteen patients with dystrophic spinal deformities from neurofibromatosis treated by anterior and posterior fusion have been reviewed. The shortest follow-up was five years, the average seven years. Combined fusion produced satisfactory results in patients with a smooth kyphoscoliosis or with scoliosis without kyphosis, but it was unsatisfactory in patients with an angular kyphoscoliosis. Of the five patients with angular kyphoscoliosis, one had a persistent pseudarthrosis after operation and all had progression of the kyphosis despite the treatment. The morbidity rate also was high in this group of patients. Many of the complications were related to soft-tissue manifestations of the disease. It is recommended that very special attention be paid to the dystrophic angular deformity in neurofibromatosis; even anterior and posterior spinal fusion may fail to control its progression.

Corpectomy and circumferential spinal fusion in dystrophic neurofibromatous curves

“…Malaligned apical vertebrae with severe rotation would make effective anterior release and strut grafting difficult, and probably technically inadequate [5,8]. Corpectomy of the thoracic apical vertebrae, which are usually out of the support axis and are squeezed against each other in a complex three-dimensional pattern, provides better cord decompression, removes a major part of deformity, and produces some mobility for the correction of kyphosis with the strut bone graft [8,10,16].…”

“…It is grouped into dystrophic and non-dystrophic curves [2,5,6]. Non-dystrophic curves, which may modulate to dystrophic ones, have a more benign course and behave like idiopathic scoliosis [4,7].…”

Characteristics of soft tissues at the apex of the deformity in patients with kyphoscoliosis with underlying type 1 neurofibromatosis

“…Оценке состояния струк-тур и тканей в области предполагаемого оперативного лечения кифосколиоза уделяется недостаточное вни-мание, что приводит к нежелательным осложнениям [2,7,12,19].…”

Spinal Reconstruction with Pedicle Screw-Based Instrumentation and rhBMP-2 in Patients with Neurofibromatosis and Severe Dural Ectasia and Spinal Deformity

“…Intrinsic pathologic conditions, such as osteomalacia and general mesodermal dysplasia, can also contribute to spinal instability in a more occult fashion 13 . Evidence of these processes on imaging studies includes rib penciling, meningoceles, expanded neural foramina, vertebral scalloping and wedging, and soft-tissue masses [15][16][17] . Clinical sequelae, ranging from simple back pain to decreased pulmonary function and quadriparesis, have been associated with the spinal manifestations of type-1 neurofibromatosis 18,19 .…”

“…aspect of the spine 17 . Thus, Stone et al 10 described the use of posterior-only instrumentation and fusion to correct upper thoracic spontaneous vertebral dislocation associated with dural ectasia in a patient with type-1 neurofibromatosis.…”