Abstract
Purpose: :
To report and discuss the atypical presentation of retinal capillary hemangioma masquerading as posterior scleritis.
Methods: :
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.
Results: :
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.
Conclusions: :
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.
Keywords: sclera • retina • vascular endothelial growth factor