Hemangioblastoma of the optic nerve producing bilateral optic tract edema in a patient with von Hippel–Lindau disease
This patient is a 34-year-old right-handed female with a known history of VHL disease. She presented to the neurosurgical service at Houston Methodist Hospital with a chief complaint of right retro-orbital pain and proptosis. She had previously undergone removal of a right retinal hemangioblastoma and was blind in the right eye, but the progressive pain and proptosis led to the discovery of a progressively enlarging lesion of the right optic nerve. Past history was notable for VHL and gastroesophageal reflux disease. In addition to the retinal lesion, she has previously undergone suboccipital craniectomy for excision of a cerebellar hemangioblastoma as well as a left sided acoustic neuroma. She harbors intramedullary tumors at the craniocervical junction as well as in the cauda equina. Her family history is significant for a son who has VHL. She has no knowledge of other family members with VHL as she does not know her biological father. Neurological examination is significant for blindness of the right eye and numbness in the perineum.\nMagnetic resonance imaging (MRI) scans of the brain with and without gadolinium revealed an avidly enhancing mass of the right optic nerve distal to the optic chiasm involving the cisternal, canalicular, and orbital components of the nerve measuring 1.2 cm in maximal diameter []. The mass was noted to have doubled in size in comparison to imaging performed 5 years prior. In addition, increased FLAIR signal was noted in the optic tracts bilaterally, which was not observed in the prior study [], suggesting the diagnosis of an infiltrating optic nerve glioma.\nShe underwent elective craniotomy and excision of the mass, which was performed through a standard fronto-temporal craniotomy. The sphenoid wing was drilled flush with the orbital roof and the dura was opened in a curvilinear fashion based on the sphenoid wing. The proximal Sylvian fissure was dissected sharply and gentle frontal lobe retraction was applied. The tumor was noted to have a red-tan appearance and was noted to tent the dura propria of the optic canal upward. The dura propria was incised sharply, and the orbital roof was opened utilizing a high speed diamond burr and 1 and 2 mm Cloward punches. The distal optic nerve was identified and divided followed by section of the nerve 1-2 mm distal to the chiasm. The ophthalmic artery was then identified, coagulated and cut [].\nNeuropathological diagnosis confirmed hemangioblastoma with the tumor having marked vascularity with a vacuolated stroma [].\nThe patient had an initial uncomplicated postoperative course and was discharged to home. She returned to the hospital 3 days after discharge with headaches. Repeat MRI of the brain revealed gross total excision of the mass as well as resolution of the FLAIR signal in the bilateral optic tracts. She is presently doing well, with resolution of her retro-orbital pain and no visual defect in the left eye.