A DUMBBELL (HOURGLASS) NEUROFIBROMA OF THE SPINE IN A PATIENT WITH VONRECKLINGHAUSENS DISEASE - A CASE-REPORT WITH 12-YEAR FOLLOW-UP

A five-and-one-half-year-old boy, referred for orthopaedic evaluation of spinal curvature, was diagnosed as having neurofibromatosis (von Recklinghausen's disease) with a 44-degree right thoracolumbar curve from T-10 to L-3. A soft-tissue mass was identified in the area of T-7 to T-12 and, when neurological signs developed, the mass was removed by extraperitoneal laparothoracotomy. Tumor root was noted to extend into the spinal canal, and subsequent surgery was undertaken to remove this tissue. There was no postoperative increase in the neurologic deficit, although the scoliosis progressed to the point where spinal fusion was required. This was followed by brace treatment to maintain correction during the growth years. Long-term follow-up (12 years) revealed that the patient's spine was balanced, the fusion mass appeared to be solid radiographically, and the neurological examination was normal. © 1984 Springer-Verlag.