Article
Spinal cord hemangioblastomas – Significance of intraoperative neurophysiological monitoring for surgical resection and long-term outcome
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Published: | June 9, 2017 |
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Objective: Spinal cord hemangioblastomas are rare tumors, developing sporadically or as part of von Hippel-Lindau disease (VHL). Although surgical resection is generally referred to be the treatment of choice, significance of intraoperative neurophysiological monitoring (IONM) for resection and postoperative outcome has hardly been evaluated before.
Methods: We retrospectively analyzed clinical, imaging and operative findings of 24 patients (m/f=1:1, sporadic/VHL=2.4:1) with 26 operations for resection of 27 spinal cord hemangioblastomas. Resection was performed under continuous IONM of somatosensory and transcranial motor evoked potentials (SSEPs and TcMEPs) and electromyography. Outcome parameters included short- and long-term neurological/functional status and a questionnaire evaluation of general performance and Oswestry Disability Index (ODI) at the end of follow-up. The correlation between IONM findings and postoperative deficits resp. outcome parameters was statistically assessed. Patients’ informed consent and approval by the local ethical review board was obtained.
Results: Preoperative symptoms included sensory changes (100.0%), pain (66.7%), and motor deficits (41.7%) with a median McCormick grade of II. Most tumors were located dorsally (92.6%), cervically (77.8%), and completely intramedullary (77.8%). Gross-total resection was achieved for 26 tumors (96.3%). IONM was non-pathological in 11 and pathological in 15 operations. Patients with non-pathological IONM had significantly less new or worsened postoperative sensorimotor deficits (p=0.005). Long-term follow-up (mean 7.9yrs, 7 patients lost) revealed a satisfying outcome with 88.2% of the patients having a stable/improved McCormick grade and 70.6% a Barthel Index (BI) of 100%, while mean ODI (11.4±12.5%) indicated only minimal disability. There was a significant correlation of pathological IONM findings and worse long-term BI resp. ODI (p=0.011 resp. p=0.024). VHL was identified as a risk factor for worse functional long-term prognosis (p=0.044).
Conclusion: Resection of spinal cord hemangioblastomas with IONM facilitates a satisfying long-term outcome. Non-pathological IONM is associated with a lower risk of new deficits and correlates with a better long-term BI and ODI. VHL is a risk factor for worse prognosis.