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L. M. Amorese, M. Jancevski, M. Dacey, C. Foster; Atypical Presentation of Von Hippel-Lindau Disease Masquerading as Recalcitrant Posterior Scleritis. Invest. Ophthalmol. Vis. Sci. 2010;51(13):5855.
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To report and discuss the atypical presentation of retinal capillary hemangioma masquerading as posterior scleritis.
Review of medical record and ancillary tests: fundus photos, fluorescein angiogram and retinal OCT, findings demanded further assessment with B-scan, Orbit and brain MRI and CT scan with contrast. Routine blood work and serology review.
A 54 year old male with history of steroid-dependent, recurrent anterior scleritis for 10 years treated with oral Prednisone 10mg/day and Methotrexate 25mg/week complained of vision drop OD, daily right-sided headache resolving with ibuprofen, chronic floaters OU, and occasional arc of light in the periphery of his OD. Visual acuity 20/60 OD and 20/20 OS. Anterior segment exam disclosed tenderness OD, trace injection, chemosis, and multifocal scleral thinning OD more than OS. Red-free images obtained from fluorescein angiogram disclosed prominent choroidal striae with peripapillary, perifoveal and peripheral sites of leakage OD. Retina OCT revealed a macular thickness of 435 OD compared to 301 OS. B-scan disclosed a sub-scleral collection of fluid and foci of edema in surrounding tissue. The B-scan also showed a space-occupying lesion in the choroid OD. MRI and CT scan with contrast revealed a mass located on the posterolateral aspect of the orbit, as well as a cerebellar mass. Complete body-imaging to exclude additional lesions was negative. The ring-enhancing, multicystic, right-sided cerebellar mass, complicated with edema and compression of the fourth ventricle prompted emergency sub-occipital craniotomy and brain biopsy. Pathology of the lesion disclosed hemangioblastoma. This prompted the presumptive diagnosis of Von Hippel-Lindau Disease. A retinal-choroidal biopsy was deemed not an acceptable option due to the patient’s visual acuity.
The evidence of focal posterior inflammation with posterior fluid collection and the presence of prominent choroidal striae are typical features of posterior scleritis. However, the serendipitous discovery of a mass lesion warrants further investigation as to the possibility of an intraocular tumor.
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