Comparison of anterior and posterior surgical approaches in the treatment of ventral spinal hemangioblastomas in patients with von Hippel—Lindau disease

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Object. Von Hippel—Lindau (VHL) disease is an autosomal-dominant neoplastic syndrome with manifestations in multiple organs, which is evoked by the deletion or mutation of a tumor suppressor gene on chromosome 3p25. Spinal hemangioblastomas (40% of VHL disease—associated lesions of the central nervous system) arise predominantly in the posterior aspect of the spinal cord and are often associated with an intraspinal cyst. Rarely, the tumor develops in the anterior aspect of the spinal cord. Ventral spinal hemangioblastomas are a surgical challenge because of difficult access and because vessels feeding the tumor originate from the anterior spinal artery.

The goal of this study was to clarify whether an anterior or posterior surgical approach is better for management of hemangioblastomas of the ventral spinal cord.

Methods. The authors performed a retrospective analysis of clinical outcomes and findings on magnetic resonance (MR) imaging studies in eight patients (two women and six men with a mean age of 34 ± 15 years) who underwent resection of ventral spinal hemangioblastomas (nine tumors: five cervical and four thoracic). Two surgical approaches were used to resect these tumors. A posterior approach was selected to treat five patients (laminectomy and posterior myelotomy in four patients and the posterolateral approach in one patient); an anterior approach (corpectomy and arthrodesis) was selected to treat the remaining three patients.

Immediately after surgery, the ability to ambulate remained unchanged in patients in whom an anterior approach had been performed, but deteriorated significantly in patients in whom a posterior approach had been used, because of motor weakness (four of five patients) and/or proprioceptive sensory loss (three of five patients). This difference in ambulation, despite significant improvements over time among patients in the posterior access group, remained significant 6 months after surgery. In all cases, MR images revealed complete resection of the tumor and in five patients significant or complete resolution of the intramedullary cyst was demonstrated (present in six of eight patients).

Conclusions. The outcomes of these eight patients with hemangioblastomas of the ventral spinal cord indicate that both immediate and long-term results are better when an anterior approach is selected for resection.

Article Information

Address reprint requests to: Edward H. Oldfield, M.D., Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, 10 Center Drive, Building 10, Room 5D37, Bethesda, Maryland 20892. email: OldfielE@ninds.nih.gov.

© AANS, except where prohibited by US copyright law.

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Figures

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    Case 5. Magnetic resonance images demonstrating a T-2 hemangioblastoma in a 31-year-old man with VHL disease. The black arrows indicate the tumor and the white arrows indicate the associated cyst. A: Sagittal view. B: Axial T1-weighted image with contrast enhancement.

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    Case 7. Sagittal contrast-enhanced T1-weighted MR image obtained in a 43-year-old man with VHL disease, demonstrating a large thoracic cyst (white arrows) with two hemangioblastomas (black arrows).

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    Case 1. A: Sagittal contrast-enhanced T1-weighted MR image obtained in a 50-year-old man with VHL disease, demonstrating a ventral C-6 hemangioblastoma (arrow). B: Intraoperative photograph (rostral to the right) obtained during the first operation, which revealed no surface presentation of the hemangioblastoma. C: Photograph obtained during the second operation, which was performed using the anterior approach, showing the surface presentation of a 1-cm-diameter hemangioblastoma (black arrow) to the left of the midline and to the left of the anterior spinal artery (white arrow). D: Intraoperative photograph demonstrating the tumor bed after resection. White arrow indicates the anterior spinal artery.

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    A and B: Sagittal contrast-enhanced T1-weighted MR images obtained in a 21-year-old woman with VHL disease and a Chiari I malformation with syringomyelia, demonstrating a ventral C-7 hemangioblastoma (black arrows) and the syrinx (white arrows). A C-7 corpectomy was performed. C–F: Intraoperative photographs. After removal of the posterior longitudinal ligament, the hemangioblastoma is easily visualized through the intact dura (C: rostral to the right). The surface presentation of a predominantly exophytic (85% extramedullary) hemangioblastoma displacing the anterior spinal artery to the left side is revealed (D). The hemangioblastoma is clearly seen during resection. The inferior pole has been displaced rostrally, exposing the tumor bed in the spinal cord (E). The bed is viewed after tumor resection (F).

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