Long-term stability after multilevel cervical laminectomy for spinal cord tumor resection in von Hippel-Lindau disease

Abstract

Despite the frequent multiplicity and development of new spinal cord hemangioblastomas that require multiple resections in patients with von Hippel-Lindau (VHL) disease, the long-term effects of spinal surgery on spinal column stability in this neoplasia disorder are not known. To determine the effect of multilevel cervical laminectomy for spinal cord tumor resection in VHL, the authors analyzed long-term clinical and radiographic outcomes. The authors included consecutive patients enrolled in a prospective VHL disease natural history study who underwent cervical laminectomy(s) for spinal cord hemangioblastoma resection. Serial clinical examinations, neck disability indices, and radiographs (static and dynamic), as well as operative records, were analyzed. Twenty-five adult patients (16 female, 9 male) with VHL disease underwent 34 operations (mean 1.4 ± 0.7 [± SD]/patient) for the resection of cervical spinal cord hemangioblastomas (mean number of lamina removed/surgery 3.0 ± 1.3). The mean age at surgery was 33.9 ± 11.9 years (range 18-61 years), and the mean follow-up duration was 9.1 ± 5.6 years. At last follow-up, radiographic criteria indicated that 9 patients (36%) had spinal column instability, 13 patients (52%) developed a cervical spinal deformity, 4 patients (16%) developed moderate to severe neck disability, and 3 patients (12%) met the criteria for clinical instability. Removal of the C-2 lamina was associated with the development of clinical instability (p = 0.02, Fisher exact test); older age at surgery was associated with the development of cervical deformity (p = 0.05, logistic regression); and a greater number of operations (suboccipital-T4) were associated with increased neck disability indices (p = 0.01, linear regression). Whereas patients with VHL disease will often require multiple laminectomies for cervical spinal cord hemangioblastoma resection, a limited number of patients (12%) will develop clinical instability. Because prophylactic cervical instrumentation confers limited benefit at the time of spinal cord tumor resection for most patients, and because these patients need life-long MR imaging of the spinal cord, the quality of which may be affected by instrumentation, longitudinal clinical and radiological evaluation may be used to determine which patients will require stabilization.