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A 72-year-old White woman, who was experiencing episodes of nontraumatic transient vision loss for 2 weeks, was evaluated by an ophthalmologist. On examination, visual acuity was 20/30 OU. Intraocular pressure (IOP) was 16 mm Hg OD and 41 mm Hg OS. The anterior segment was normal in the right eye. Anterior examination of the left eye revealed 10% hyphema. The patient was prescribed cyclopentolate, 1%, and prednisolone acetate, 1%, to reduce inflammation and lower the IOP in the left eye. Two days later, IOP was 16 mm Hg OD and 10 mm Hg OS. Slitlamp biomicroscopy revealed resolved hyphema and abnormal blood vessels at the superotemporal pupillary margin of the iris, prompting referral for further evaluation.
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Iris microhemangiomatosis causing spontaneous hyphema
A. Observe until repeated incident
Iris microhemangiomatosis is a rare condition, most often found in middle-aged or older (mean age, 70 years) White (100%) female individuals (73%).1 This ectatic vascular anomaly manifests at the pupillary margin (100%) and generally involves both eyes (57%), presenting with symptoms of blurred vision (59%) and findings of hyphema (55%) or glaucoma (32%). In no case has there been history or evidence of trauma, to our knowledge.1 The differential diagnosis includes entities such as diabetic retinopathy, central retinal vein occlusion, ocular ischemic syndrome, trauma, or tumor, especially juvenile xanthogranuloma. The findings on slitlamp evaluation and iris FA confirmed the suspected diagnosis. This condition has a low rate (2% per year) of repeated hyphema, so the best next step in the management would be to observe (choice A).2 Argon laser photocoagulation (choice B) is only recommended if a single ectatic lesion shows repetitive active bleeding. Administration of intravitreal bevacizumab injection (choice C) would be correct if this were iris neovascularization due to diabetic retinopathy, central retinal vein occlusion, or ocular ischemic syndrome. Surgical management with iridocyclectomy (choice D) would be inappropriate because iris microhemangioma is a benign condition without malignant transformation.
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Corresponding Author: Carol L. Shields, MD, Oncology Service, Wills Eye Hospital, Thomas Jefferson University, 840 Walnut St, Ste 1440, Philadelphia, PA 19107 (email@example.com).
Published Online: August 13, 2020. doi:10.1001/jamaophthalmol.2020.1421
Conflict of Interest Disclosures: Dr Shields reports having support provided by the Eye Tumor Research Foundation. No other disclosures were reported.
Additional Contributions: We thank the patient for granting permission to publish this information.
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