April 2010
Volume 51, Issue 13
ARVO Annual Meeting Abstract  |   April 2010
Atypical Presentation of Von Hippel-Lindau Disease Masquerading as Recalcitrant Posterior Scleritis
Author Affiliations & Notes
  • L. M. Amorese
    Ophthalmology, MERSI, Cambridge, Massachusetts
  • M. Jancevski
    Ophthalmology, MERSI, Cambridge, Massachusetts
  • M. Dacey
    Ophthalmology, MERSI, Cambridge, Massachusetts
  • C. Foster
    Ophthalmology, MERSI, Cambridge, Massachusetts
  • Footnotes
    Commercial Relationships  L.M. Amorese, None; M. Jancevski, None; M. Dacey, None; C. Foster, None.
  • Footnotes
    Support  None.
Investigative Ophthalmology & Visual Science April 2010, Vol.51, 5855. doi:
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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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      © ARVO (1962-2015); The Authors (2016-present)

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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 

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