Hemangioblastoma of the optic nerve producing bilateral optic tract edema in a patient with von Hippel-Lindau disease

Blaken Staub,Andrewd Livingston,Davids Baskin,Patricia Chévez-Barrios

doi:10.4103/2152-7806.128430

Abstract

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.Case Description: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.Conclusion: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.

Highlights

ConclusionOptic nerve hemangioblastomas in patients with von Hippel–Lindau disease (VHL) are rare, but are manageable with meticulous microneurosurgery and with appropriate patient expectations
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)
We report a case of an optic nerve hemangioblastoma in a patient with known VHL, which produced bilateral optic tract edema suggesting the diagnosis of an optic nerve glioma, and review the current literature

Summary

Conclusion

Optic nerve hemangioblastomas in patients with VHL are rare, but are manageable with meticulous microneurosurgery and with appropriate patient expectations. We report a case of an optic nerve hemangioblastoma in a patient with known VHL, which produced bilateral optic tract edema suggesting the diagnosis of an optic nerve glioma, and review the current literature. This patient is a 34‐year‐old right‐handed female with a known history of VHL disease. Increased FLAIR signal was noted in the optic tracts bilaterally, which was not observed in the prior study [Figure 1c], suggesting the diagnosis of an infiltrating optic nerve glioma She underwent elective craniotomy and excision of the mass, which was performed through a standard fronto‐temporal craniotomy. She is presently doing well, with resolution of her retro‐orbital pain and no visual defect in the left eye

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