CC BY-NC-ND 4.0 · J Neurol Surg B Skull Base 2019; 80(S 04): S348
DOI: 10.1055/s-0039-1700511
Skull Base: Operative Videos
Georg Thieme Verlag KG Stuttgart · New York

Resection of von Hippel Lindau Related Brainstem Hemangioblastoma

Dominic Maggio
1  Surgical Neurology Branch, National Institute for Neurological Diseases and Stroke, Bethesda, Maryland, United States
,
1  Surgical Neurology Branch, National Institute for Neurological Diseases and Stroke, Bethesda, Maryland, United States
2  Neuro-Oncology Branch, National Cancer Institute, Bethesda, Maryland, United States
,
1  Surgical Neurology Branch, National Institute for Neurological Diseases and Stroke, Bethesda, Maryland, United States
3  Neurosurgery Unit for Pituitary and Inheritable Diseases, National Institute for Neurological Diseases and Stroke, Bethesda, Maryland, United States
› Author Affiliations
Further Information

Address for correspondence

Prashant Chittiboina, MD, MPH
Neurosurgery Unit for Pituitary and Inheritable Disorders, NINDS
Bethesda, MD 20892
United States   

Publication History

01 April 2019

25 August 2019

Publication Date:
22 October 2019 (online)

 

    Abstract

    Patients with von Hippel Lindau (VHL) disease develop multiple central nervous system hemangioblastomas (HB). The surgical resection of VHL-HBs is the standard of clinical care when these HBs become symptomatic due to tumor growth, edema, or cyst formation. VHL-HBs frequently present at the obex of the brainstem, making this a challenging surgical problem. Here, we present a case of a large symptomatic brainstem VHL-HB that was resected using a dorsal midline approach and midline myelotomy. This 35-year-old man with VHL disease and multiple prior resections of cerebellar hemangioblastomas presented with progressive bilateral upper extremity weakness, ataxia, and dysphagia. The accompanying two-dimensional (2D) video demonstrates the key techniques for resection of this brainstem hemangioblastoma, including recognizing the pial presentation, sharp pial/subpial dissection, and en bloc removal. Subsequently, we discuss circumferential dissection, identification, and division of feeding vessels. We also demonstrate key maneuvers to recognize and divide the main arterial pedicle while preserving the anterior spinal artery. The patient tolerated the resection without complication and the patient was discharged home after 6 days at his baseline neurologic function. This work was supported by the Intramural Research Program of the National Institutes of Health.

    The link to the video can be found at: https://youtu.be/mb2VqcLpxDQ.


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    Conflict of Interest

    None declared.

    Address for correspondence

    Prashant Chittiboina, MD, MPH
    Neurosurgery Unit for Pituitary and Inheritable Disorders, NINDS
    Bethesda, MD 20892
    United States