Are Two Surgeries Necessary for Correction of 1 Deformity- Cervical Kyphosis in Neurofibromatosis?- A Rare Case...

Dystrophic neurofibromatosis type I, involving upper cervical spines, is rare, which can cause serious complications. Myelopathy develops due to compression of the cord posteriorly. Surgical correction has its inherent risks and difficulties because of poor bone quality, difficult anterior approach because of bizarre deformities, and the necessary manipulation, which might cause more cord damage and ischemia. Anterior decompression with alignment correction was an early popular choice. But without posterior shortening, the technique proved unsatisfactory in restoring normal alignment. It tended to expose the graft bone at increased risk of insufficient union or extruding. So a combination of anterior decompression and posterior correction was generally recommended. This report describes surgical technique applicable to cases of severe cervical kyphosis using only anterior approach.

A 13-year-old boy, a case of neurofibromatosis type 1 presented with neck pain, and bilateral upper limb radiculopathy for 2 months with affected daily living, with intact neurology. Imaging demonstrated 46° kyphosis (C3-C5), marked dystrophic changes of the C4 and C5, extreme dorsal angulation indenting the cord. Anterior surgery was planned for the patient with neuromonitoring. Through left anterior approach, total C3, C4 and C5 corpectomy was accomplished, keeping the PLL intact. The defect was filled with a fibular cortical graft which was stabilized with 48 mm cervical screw plate, showing full correction of kyphosis postoperatively. At the follow-up 6 months postoperatively, lateral plain radiograph showed stability of the cervical spine fusion without correction loss. The patient had full relief from tingling and numbness with no neurological deficit and could resume his daily duties. Patient is asymptomatic at 2 years follow up.

Only anterior decompression and fusion are effective in satisfactorily correcting cervical kyphosis in cases of neurofibromatosis without neurologic compromise and avoids the risk of damage to vital neurovascular structures because of pedicle or lateral mass screw fixation.