Neurofibromatosis type I with severe dystrophic kyphoscoliosis and its operative management via a simultaneous anterior-posterior approach: a case report and review of the literature.

BACKGROUND CONTEXT

Neurofibromatosis is an autosomal-dominant hereditary disorder with two subtypes: NF-1 (type I) and NF-2 (type II). NF-1 is a complex disorder with a constellation of manifestations that can also entail skeletal abnormalities, including spinal deformity of a noncongenital nature with early age onset. The short, sharp, angular curve usually present in the thoracic region, as exhibited in NF-1, presents a quandary in its surgical management. Various studies have reported on the efficacy of anterior correction as opposed to posterior alone, whereas others have advocated a sequential, combined approach to diminish the degree of deformity and achieve solid arthrodesis. However, despite solid arthrodesis, curve progression may still ensue. Nonetheless, a simultaneous anterior-posterior approach to treat such a condition of NF-1 with severe dystrophic kyphoscoliosis is a rare occurrence.

PURPOSE

To describe the presentation and operative management of a patient with NF-1 and severe dystrophic kyphoscoliosis.

STUDY DESIGN

A case report and review of the literature.

METHODS

A clinical and radiographic review of a 51-year-old male patient who presented with NF-1, a 165-degree thoracic kyphotic deformity, associated scoliosis, varied degree of vertebral destruction of T9-T11, and paraparesis below T10.

RESULTS

Operative intervention of the deformity consisted of a simultaneous anterior-posterior approach and entailed posterior cord exposure, anterior vertebrectomy of T9-T11, cord decompression, posterior osteotomy (posterior elements were auto-fused), anterior distraction and kyphosis correction, anterior strut grafting, anterior rod instrumentation, and posterior compression instrumentation and fusion from T6-L2. The deformity was reduced, sold fusion was noted, and the patient was asymptomatic.

CONCLUSIONS

A simultaneous anterior-posterior approach for the surgical treatment of severe dystrophic kyphoscoliosis in neurofibromatosis type I is an avenue to properly visualize the spinal cord, achieve solid arthrodesis, and to minimize as well as prevent the progression of deformity.