This was a 55-year-old woman who has a peripapillary mass in her right eye causing decreased vision for approximately 5 months. The patient had no known systemic disease and was not on any systemic or ocular drugs. Family history of the patient was also unremarkable. The BCVAs were 20/70 in the right eye and 20/25 in the left eye. IOPs were within normal limtis and anterior segment examinations of both eyes were normal except for mild nuclear sclerosis.
Dilated fundus examinations of the right eye revealed a yellow–white raised peripapillary mass about three disk areas in size, located in the temporal region of the optic disk. A few retinal vessels could be observed on the lesion while the left eye had a normal appearance.
Late-phase image of fundus fluorescein angiography of the right eye demonstrated staining of the peripapillary lesion without significant leakage
whereas the left eye had a normal angiographic appearance.
Horizontal and vertical optical coherence tomography scans passing through the lesion showed a hyperreflective mass with underlying shadowing, that elevated the peripapillary retina and caused intraretinal cysts extending to the fovea. On the other hand, there were no pathologic findings on the optic coherence tomography of the left eye.
The peripapillary lesion appeared hypoautofluorescent on autofluorescence imaging.
In addition to these ocular imaging modalities, cranial and orbital MRI and chest computed tomography were performed for malignancy screening and no abnormality was detected.
In the light of imaging characteristics, the features of this richly vascularized, yellowish-pink tumor involving the optic disk and
juxtapapillary retina were highly characteristic of solitary juxtapapillary retinal capillary hemangioblastoma. In the case presented here, the lesion’s classic appearance on biomicroscopy and multimodal imaging was sufficient to make the diagnosis. However, smaller or less typical juxtapapillary retinal hemangioblastomas may some times be confused with juxtapapillary choroidal neovascular membranes or other retinal tumors such as retinoblastoma and astrocytic hamartoma.
Credit: Kemal Tekin, M.D., from Ulucanlar Eye Training and Research Hospital
Instagram accounts: @retina.academy and @dr.kemaltekin