Clinical Review & Education

JAMA Ophthalmology Clinical Challenge

Peculiar Protuberance Michael I. Seider, MD; David G. Hwang, MD; Bertil E. Damato, MD, PhD, FRCOphth

Figure 1. Ocular lesion that prompted patient to seek ophthalmic care.

A 47-year-old woman complained of a lesion under her left lower eyelid. The patient noticed the lesion when looking in a mirror 3 years prior to presentation. The patient has had no visual changes or other symptoms but thinks the lesion may have grown slightly since first detected. The patient wears spectacles for myopia but has no other ocular history and Quiz at jamaophthalmology.com denies a history of trauma. Her medical history is unremarkable, and her review of systems was negative, except for occasional back pain for which she takes tramadol hydrochloride orally. On examination, her corrected acuity was 20/20 OD and 20/ 25+ OS. The results of pupil, motility, and confrontational visual field examinations were normal. The intraocular pressure was 17 mm Hg OD and 18 mm Hg OS. The patient’s manifest refraction was −14.50 + 2.50 × 089 diopters OD and −16.00 + 4.00 × 077 diopters OS. The results of an anterior segment examination are that both the right eye and the left eye, except for the lesion identified (Figure 1), are normal. The results of a dilated fundoscopic examination are that the right eye is normal but that the left eye reveals a small area of shallow retinal detachment with associated retinal pigment epithelial pigment changes in the inferotemporal periphery.

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WHAT WOULD YOU DO NEXT?

A. Excisional biopsy of lesion B. Incisional biopsy of lesion C. Episcleral radioactive plaque treatment, with or without concomitant needle biopsy D. Observe

JAMA Ophthalmology December 2014 Volume 132, Number 12

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Clinical Review & Education JAMA Ophthalmology Clinical Challenge

A

B

Eyelid

Probe tip

Figure 2. A, The subconjunctival lesion transilluminates diffusely, suggesting a hollow architecture. B, Ultrasonographic biomicroscopy reveals a scleral defect, the hollow and compressible nature of the lesion, and an associated retinal detachment.

Sclera

Retina

Diagnosis Equatorial staphyloma

What To Do Next D. Observe

Discussion The patient presented with an idiopathic equatorial staphyloma. This type of staphyloma occurs in patients with high myopia, constitutes an outpouching of the uvea through a weakened sclera, and is usually asymptomatic, although cases of associated clinically significant retinal detachment have been reported.1 These staphylomas may occur at the equator because of a focal weakness caused by nearby perforating vortex veins. Most equatorial staphylomas are not large enough to be visualized with the slitlamp and are only noted during or after dissection of the overlying conjunctiva and the Tenon fascia during placement of a scleral buckle.1-5 Equatorial staphylomas that are not associated with clinically significant retinal detachment may be managed conservatively. We advised the patient to follow up at regular intervals, wear her spectacles at all times, avoid ARTICLE INFORMATION Author Affiliations: Department of Ophthalmology, University of California, San Francisco. Corresponding Author: Michael I. Seider, MD, Department of Ophthalmology, University of California, San Francisco, 10 Koret Way, K301, San Francisco, CA 94143 ([email protected]). Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest, and Dr Hwang reported that he was a consultant to Alcon Inc and Inspire Pharmaceuticals.

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rubbing her eyes or any trauma, and report to an emergency department if even moderate ocular trauma occurs. The diagnosis of equatorial staphyloma can be made from ophthalmoscopic examination of the external and internal portions of the lesion alone. An external examination reveals a relatively homogenous, pigmented subconjunctival lesion with overlying normal conjunctiva and Tenon fascia. If the lesion is protuberant, as in this case, the edges may be well circumscribed. An ophthalmoscopic examination of the internal aspects of the lesion reveals the absence of an internal mass and a focal retinal detachment with surrounding retinal pigment epithelial changes. A scleral depressed examination of these delicate tissues should be avoided. Transillumination is particularly illustrative and confirms the hollow nature of the lesion, thereby differentiating it from malignant lesions, including nodular conjunctival melanoma or an extrascleral extension of uveal melanoma (Figure 2A). Ultrasonographic biomicroscopy is also a useful imaging adjunct and reveals the lesion to be acoustically hollow, deformable with only slight pressure from the fluid-filled probe tip, and associated with a scleral defect and retinal detachment (Figure 2B).

Funding/Support: This work was supported by an unrestricted grant from Research to Prevent Blindness and by Core Grant for Vision Research (EY002162) from the National Institutes of Health/ National Eye Institute.

2. Watzke RC. Scleral staphylomas and retinal detachment. Arch Ophthalmol. 1963;70:796-804.

Role of the Funder/Sponsor: The sponsors had no role in the design and conduct of the study; collection, management, analysis, or interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.

4. Faris B, Freeman HM, Schepens CL. Scleral dehiscences, anterior staphyloma, and retinal detachment—Part 1: incidence and pathogenesis. Trans Sect Ophthalmol Am Acad Ophthalmol Otolaryngol. 1975;79(6):OP851-OP853.

REFERENCES 1. Vail D. Equatorial scleral staphyloma and retinal detachment cured by excision. Trans Am Ophthalmol Soc. 1940;38:53-58.

3. Young CA Jr. Equatorial scleral staphyloma; surgical treatment in a case with retinal detachment. Am J Ophthalmol. 1955;40(1):12-14.

5. Robertson DM. Retinal detachment with equatorial staphyloma. Arch Ophthalmol. 1996;114 (4):496-497.

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