American Academy of Optometry, 2014, Residents Day Submission

Title: Clinical Evaluation and Differential Diagnosis of Choroidal Lesions Author: Meaghan Horton, OD Kelly Thompson, OD, FAAO Cincinnati VA Medical Center The Ohio State University College of Optometry

Category: Ocular Disease

Abstract

An 81 year old Caucasian male presents with complaints of decreased acuity and a dark halo in vision, OD. Fundus examination reveals a 6DD lesion with associated edema, exudates and several intra-retinal hemorrhages.

I. Case History

 Demographics: 81 year old Caucasian male  Chief complaint: Dark halo around central vision of right eye that began 3 months ago  Ocular History: POAG, OS>OD; of Ota, OD; mild ERM, OU; moderate dry ARMD OD>OS; Pseudophakia, OU  Medical history: Hypertension, hyperlipidemia, OSH, BPA, Pulmonary hypertension, goiter  Medications: Cyclobenazaprine, fluticasone, furosemide, losartan, naproxen, potassium chloride, simvastatin, terazosin, travaprost

II. Pertinent findings

 Clinical o Visual Acuity: 20/400 OD (no improvement with pinhole); 20/25 OS o External: melanosis along lower right eyelid extending out to upper right cheek o Pupils: PERRL (-)APD, OD, OS o EOMs: FROM, OD, OS o CVF: FTFC, OD, OS o Slit Lamp Exam: diffuse conjunctival melanosis 360, OD; iris heterochromia, OD; arcus, OU; mild central SPK, OD o IOP: 14 mmHg OD, 15 mmHg OS o Fundus Exam: diffuse small/med soft drusen, mild RPE changes, OU; subretinal fluid and intraretinal fluid surrounding ONH around nasal aspect from 6 o/c to 11 o/c encompassing 6DD with subretinal elevation, mild inferior intraretinal hemes, bordering exudation and darkened pigmentation in central area of pigmentation, OD

 Others o OCT Macula . OD: diffuse RPE and PIL irregularities, most temporally, associated with outer retinal atrophic changes . OS: scattered RPE irregularities, mild PIL disruption o OCT 5 Line Raster . OD: intraretinal thickening; medium isoreflective lesion anterior to RPE and adjacent to optic disc margin o B-Scan . OD: posterior segment well visualized and within normal limits o Photos . OD: Correlates with DFE findings above

III. Differential diagnosis

 Initial/leading: Choroidal  Others: choroidal neovascular membrane, choroidal nevus, CHRPE, melanocytoma, choroidal , chorioretinal granumoma, choroidal metastatic lesions  (comparative table with photos will be used)

IV. Diagnosis and discussion

Differentials of a choroidal lesion can range from benign to life threatening conditions. Accurate and timely diagnosis can make a difference in maintaining sight, or possibly losing the eye. Important testing in the diagnosis of a choroidal lesion includes a dilated fundus exam, optical coherence tomography (OCT), B-Scan, A-Scan, ICG and fluorescein angiography.

One of the most ominous choroidal lesion diagnosis is . While uveal melanoma is a relatively rare finding, it is the most common malignant primary intraocular tumor in adults.1 90% of uveal are present in the choroid.2,3 The incidence has been found to be approximately 6 per 1 million individuals in the United States with the vast majority found in Caucasians with an average age of 60.1,2,4 Patients with uveal melanoma may be asymptomatic. As the lesion grows symptoms often increase. 5 The most common symptoms include blurry vision, photopsia, and visual field defects.4,5 There are no defined pathognomonic signs of choroidal melanomas so diagnosis can often be difficult.5 Choroidal melanomas generally appear as pigmented masses that are dome or collar-buttoned. This shape is created by the tumor breaking through Bruch’s membrane and creating an area of elevation.4 The color of the lesions can vary from darkly pigmented to amelanotic and can present in several different shapes.5 The most clinically useful diagnostic test when determining if the lesion is a true choroidal melanoma is a B-Scan.5

Choroidal neovascularization membrane (CNVM) is a condition that can initially look similar to a choroidal melanoma. In addition to performing an OCT, a B-Scan can be useful in distinguishing this finding from a choroidal melanoma. In this particular case, the initial leading diagnosis was a choroidal melanoma, but after further diagnostic testing, was determined to be a peripapillary CNV. CNV’s are often associated with wet age related macular degeneration, angiod streaks, inflammatory chorioretinal diseases, presumed ocular histoplasmosis, and degenerative myopia.6 In the patient presented above, the CNV was presumed to be due to ARMD, but the location of the lesion and the mild presentation of the ARMD detracted from an initial diagnosis of choroidal neovascularization.

Additional choroidal lesions and their associated findings that will be discussed include choroidal osteoma, choroidal metastatic lesions, choroidal nevus, and melanocytoma.

V. Treatment, management

The treatment of various choroidal lesions can depend on the final diagnosis. The leading diagnoses in this case were choroidal melanoma and CNVM, each having significantly different treatment and management.

The primary goal of treatment is to prevent metastasis of the melanoma. 50% of patients with uveal melanoma with develop metastatic disease.2,4 The most common site of metastasis is the liver, with approximately 90% of patients with metastasis having hepatic involvement.7 Patients presenting with a uveal melanoma should have a full systemic workup which includes a CT, chest x-ray, CBC, and liver enzyme analysis.6 Treatment options available include radiation therapy and enucleation. Treatment is often based on the size and location of the tumor, with radiation therapy most often used at the first-line option.

Treatment options of a CNV include anti-VEGF agents, PDT, and argon laser photocoagulation. The most common treatment selected in an intravitreal injection of an anti-VEGF agent (Lucentis, Avastin, Macugen, and Eylea). Based on the agent selected will determine how frequently the patient will be injected, which can vary from eight to twelve injections in the first year.

VI. Conclusion

Optometrists are the primary care providers of the eye and are often faced with the task of diagnosing various lesions that may be malignant or caused by underlying systemic conditions. It is the task of optometrists to be prepared to adequately diagnose these conditions, utilizing the aid of available diagnostic equipment to determine the appropriate treatment, management, and possible referrals necessary for these patients. The aim of this case is to highlight various choroidal lesions that may be difficult to initially diagnose based on fundus findings alone.

References

1. Egan, K. M., Seddon, J. M., Glynn, R. J., Gragoudas, E. S., & Albert, D. M. (1988). Epidemiologic aspects of uveal melanoma. Survey of ophthalmology, 32(4), 239-251. 2. Damato, B. (2010). Does ocular treatment of uveal melanoma influence survival? British journal of cancer, 103(3), 285-290. 3. Damato, B., Eleuteri, A., Taktak, A. F., & Coupland, S. E. (2011). Estimating prognosis for survival after treatment of choroidal melanoma. Progress in retinal and eye research, 30(5), 285-295. 4. Yonekawa, Y., & Kim, I. K. (2012). Epidemiology and management of uveal melanoma. Hematology/oncology clinics of North America, 26(6), 1169-1184. 5. Lederer, D. E., & Edelstein, C. (2004). Choroidal melanoma: clinical presentation and differential diagnosis. Canadian journal of ophthalmology. Journal canadien d'ophtalmologie, 39(4), 358-364. 6. Alexander, L. J. (2002). Primary care of the posterior segment. New York: McGraw-Hill. 7. Kanski, J. J., & Bowling, B. (2011). Clinical ophthalmology: A systematic approach. Edinburgh: Elsevier/Saunders.