Título

RETINAL CAPPILARY HEMANGIOMA IN VON HIPPLE-LINDAU DISEASE: CASE REPORT

Introdução / Purpose

A 31-years-old man has been referred for evaluation of the floaters in th left eye, intermittent, not affecting the visual axis for 7 years. The negative increase of the same, low visual acuity, trauma or eye surgeries. He reports as a personal history cervical spine surgery 9 years ago for removal of a benign tumor- which was descovered when he had progressive paralysis of the IV parts, whose biopsy report came as intramedullary hemangioblastoma grade I.
He also reports that 5 years ago, after acute holocranial headche without improvement with medication, he underwent emergency surgery to remove anothes benign tumor in the cerebellum region- which causing intracarnial hypertension.
A solid-cystic expansive formation in the posterior fossa, obliterating the left Luschka and Magendie foramens. Still, it presents anterior compression of the bulb and dilation of ventricular system. After urgency surgery, the biopsy result came is as cerebellar hemangioblastoma.
Upon examination, this patient best-corrected visual acuity (BCVA) was 20/20 and J1in both eyes. At fundoscopy, an important increase in caliber and tortuosity of vessels was observed, especially those nourishing the lesions in the middle periphery and
perilesional, with nutrient artery and dilated drainage veins in both eyes. In addition to the presence of “twin “vessels.
Angiofluoreceinography, shows na early hyperfluorescence of dilated vessels and nodular lesions, as well as na early and well-delimited hyperfluorescence of hemangioblastomas, with littel contrast extravasation throughout the examination. There was no evidence of hyper or hypofluorescence early or late in the macular region, resulting in the absence of edema.
The patient was referred for a multidiscplinary follow-up to be done genetic evaluation and screening of others systemic tumors. It was also advised when the course of na ocular disease, routine follow-up at the time.

Material e Método / Methods

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Resultados / Results

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Discussão e Conclusões / Discussion

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Palavras Chave

RETINAL CAPPILARY HEMANGIOMA; VON HIPPLE-LINDAU DISEASE