Management of Proximal Thoracic Kyphoscoliosis with Early Myelopathy in a Young Adult with Neurofibromatosis Type 1: A Case Report and Review of Literature.

INTRODUCTION

Neurofibromatosis (NF) 1 is associated with skeletal deformities. Scoliosis is seen in about 10-64% of NF-1 patients. NF-1 may be associated with dystrophic or non-dystrophic type of curve. There are technical difficulties in treating large dystrophic curve with proximal thoracic kyphoscoliosis in NF-1. This case report and subsequent review of literature attempts to provide a line of management for such difficult lesions.

MATERIALS AND METHODS

We present a case of 22-year-old male with NF-1 who came to us with gradually progressive upper back deformity and signs of early myelopathy for 2-3 months. The patient had a 100° proximal thoracic kyphosis and 100° scoliosis with no neurological deficit and brisk reflexes. A single stage posterior fixation with three column osteotomy at the apex of the curve was performed.

RESULTS

The kyphosis was corrected to 65° from 100, the scoliosis was corrected to 60° from 100. Clinically, the hump in the upper back was visibly reduced. At 1-year follow-up, the patient remained asymptomatic with no loss of correction and implant breakage on X-ray.

CONCLUSION

Large dystrophic proximal thoracic kyphoscoliosis is a difficult lesion to treat and can be managed with an all posterior approach, achieving good cosmetic and neurological outcomes, as demonstrated by this case.