Hemangioblastoma of the optic nerve producing bilateral optic tract edema in a patient with von Hippel-Lindau disease.
BACKGROUND
The authors present a novel case of a hemangioblastoma of the optic nerve producing bilateral optic tract edema in a patient with von Hippel-Lindau disease (VHL). This is the only case in the literature documenting optic tract edema secondary to a hemangioblastoma of the optic nerve.


METHODS
The patient was a 34-year-old female in whom this lesion was causing retro-orbital pain and proptosis. She had previously lost vision in the symptomatic eye secondary to a retinal hemangioblastoma. The optic nerve lesion was excised by sectioning the optic nerve both proximally and distally to the lesion. There were no complications and patient's symptoms resolved postoperatively. A follow-up magnetic resonance imaging (MRI) scan revealed complete excision of the mass and resolution of the optic tract edema.


CONCLUSIONS
Optic nerve hemangioblastomas in patients with VHL are rare, but are manageable with meticulous microneurosurgery and with appropriate patient expectations. This is the first known case of an optic nerve hemangioblastoma producing bilateral optic tract edema, which resolved after resection of the prechiasmal tumor. Hemangioblastoma should remain in the differential diagnosis of optic nerve tumors, especially in the setting of VHL.
Department of Neurosurgery, Houston Methodist Neurological Institute, Methodist Hospital, Houston, TX, USA.
Blake N
BN
Staub
Department of Neurosurgery, Houston Methodist Neurological Institute, Methodist Hospital, Houston, TX, USA.
Andrew D
AD
Livingston
Department of Pathology and Laboratory Medicine and Ophthalmology, Weill Medical College of Cornell University, New York ; Department of Pathology and Genomic Medicine, Houston Methodist Hospital, Houston, TX, USA.
Patricia
P
Chévez-Barrios
Department of Neurosurgery, Houston Methodist Neurological Institute, Methodist Hospital, Houston, TX, USA.
David S
DS
Baskin
Hemangioblastoma; optic nerve mass; optic-tract edema; von Hippel–Lindau