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An Amelanotic Choroidal Mass in a Middle-aged Woman

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 57-year-old woman with a history of hypertension presented to the emergency department with blurry vision in her right eye for a duration of 2 weeks. Her visual acuity with correction was 20/40 OD and 20/25 OS. Her intraocular pressures were 14 mm Hg OD and 15 mm Hg OS. A dilated fundus examination of the right eye was notable for an amelanotic choroidal mass with overlying subretinal fluid (Figure 1A). Results of a dilated fundus examination of the left eye were unremarkable. Results of fluorescein angiographic imaging of the right eye showed late leakage corresponding to the choroidal mass; the left eye was unremarkable. Optical coherence tomography demonstrated a choroidal mass with an irregular surface contour and a mild overlying vitreous cell. On B-scan ultrasonography of the right eye, a superotemporal choroidal mass with an irregular surface contour and medium internal reflectivity was observed (Figure 1B). On further questioning, the patient reported a 9 pack-year smoking history. She had no known history of malignant conditions. She denied recent fevers, chills, weight loss, night sweats, and travel to foreign countries, and she was otherwise feeling well.

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Giant nodular posterior scleritis

A. Investigate for sarcoidosis and tuberculosis; if negative, start treatment with steroids

The patient was diagnosed with giant nodular posterior scleritis. Test results for tuberculosis, sarcoidosis, and syphilis were negative. She started receiving oral prednisone at a dosage of 60 mg daily. Giant nodular posterior scleritis is uncommon, and diagnosis can be challenging, because there is a broad differential diagnosis for an amelanotic choroidal mass, including choroidal metastasis, amelanotic choroidal melanoma, choroidal lymphoma, circumscribed choroidal hemangioma, and choroidal granuloma (most commonly seen in association with sarcoidosis and tuberculosis). When present, typical features, such as eye pain, headache, and loss of visual acuity, are helpful; however, up to 25% of cases of posterior scleritis are painless.1,2 Most studies show a female predominance. Systemic associations are found in 29% of cases; most commonly, these are rheumatoid arthritis, systemic vasculitis, and granulomatosis with polyangiitis.1,2 Coexisting anterior scleritis is seen in 36% of cases.2 Posterior manifestations include serous retinal detachment (21% of cases), optic disc edema (18%), and (rarely) an amelanotic choroidal mass (13%). Fluid in the Tenon space (the classic T sign) can be observed on ultrasonography (Figure 1B) and is supportive of the diagnosis; however, in a review of 11 patients with nodular posterior scleritis, only 4 (36%) had this feature.2 Most patients respond well to treatment with nonsteroidal anti-inflammatory drugs or oral steroids. Patients with recurrent disease may require steroid-sparing immunosuppressive therapy.

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Article Information

Corresponding Author: Mary E. Aronow, MD, Retina Service, Massachusetts Eye and Ear, Department of Ophthalmology, Harvard Medical School, 243 Charles St, Ste 850A, Boston, MA 02114 (mary_aronow@meei.harvard.edu).

Published Online: October 31, 2019. doi:10.1001/jamaophthalmol.2019.4134

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank John Hensel, BS, Massachusetts Eye and Ear, for providing the optimized color fundus photographs. He was compensated for his contribution. We also thank the patient for granting permission to publish this information.

References
1.
McCluskey  PJ, Watson  PG, Lightman  S, Haybittle  J, Restori  M, Branley  M.  Posterior scleritis: clinical features, systemic associations, and outcome in a large series of patients.  Ophthalmology. 1999;106(12):2380-2386. doi:10.1016/S0161-6420(99)90543-2PubMedGoogle ScholarCrossref
2.
Agrawal  R, Lavric  A, Restori  M, Pavesio  C, Sagoo  MS.  Nodular posterior scleritis: clinico-sonographic characteristics and proposed diagnostic criteria.  Retina. 2016;36(2):392-401. doi:10.1097/IAE.0000000000000699PubMedGoogle ScholarCrossref
3.
Shields  CL, Shields  JA, Gross  NE, Schwartz  GP, Lally  SE.  Survey of 520 eyes with uveal metastases.  Ophthalmology. 1997;104(8):1265-1276. doi:10.1016/S0161-6420(97)30148-1PubMedGoogle ScholarCrossref
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