A 35-year-old male with a father and brother affected by VHL presented for a background review of the eye.
She complained of mild blurred vision in her right eye, with no other associated systemic symptoms at that time.
Initial evaluation: Visual acuity (VA) of 1 in both eyes; with presence of two hemangiomas in right eye (OD) with mild exudate.
The rest of the exploration was normal.
A renal cyst was found in the systemic study without other findings.
Cranial CT scan was normal.
Six months later the patient presented with decreased VA in the right ear (AV).
Fluorescein angiography (FFA) showed a macular epiretinal membrane with little exudation associated with congenital hemangiomas.
Brain MRI was normal.
The patient was treated with transconjunctival cryotherapy of lesions.
After one year, VA in RE was counted toes to half meters, despite an apparent good response to treatment.
Two years later, the patient complained of vision loss in the left eye, which showed a peripheral angioma without exudation that was treated with photocoagulation.
Brain MRI showed lesions in both optic nerves with intense contrast uptake, greater in the right optic nerve, where it reached the chiasmatic zone.
These lesions were radiologically compatible with meningiomas, gliomas or even hemangioblastomas.
Four years later, the VA in the RE was hand movements and in the LE of the unit.
At that time, the patient was diagnosed with pheochromocytoma and periodic controls of eye fundus and orbital and cerebral MRI continued.
The neurosurgery department ruled out a possible intervention to preserve visual remnant, so there is no histopathological confirmation of the lesions.
Currently, seven years after the beginning of the process, right eye is auricle, and at the bottom of the eye, hemangiomas are seen as healed, folds, and papillary stenosis.
In the left eye the VA is 0.6 and the hemangioma treated in the temporal zone and papillary stenosis is observed.
The visual field of the left eye showed a central defect extending to the nasal hemifield.
