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Transient Vision Loss in a Healthy Older Woman

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

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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Article Information

Corresponding Author: Carol L. Shields, MD, Oncology Service, Wills Eye Hospital, Thomas Jefferson University, 840 Walnut St, Ste 1440, Philadelphia, PA 19107 (carolshields@gmail.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.

References
1.
Williams  BK  Jr , Di Nicola  M , Ferenczy  S , Shields  JA , Shields  CL .  Iris microhemangiomatosis: clinical, fluorescein angiography, and optical coherence tomography angiography features in 14 consecutive patients.   Am J Ophthalmol. 2018;196:18-25. doi:10.1016/j.ajo.2018.08.011PubMedGoogle ScholarCrossref
2.
Shields  JA , Bianciotto  C , Kligman  BE , Shields  CL .  Vascular tumors of the iris in 45 patients: the 2009 Helen Keller Lecture.   Arch Ophthalmol. 2010;128(9):1107-1113. doi:10.1001/archophthalmol.2010.188PubMedGoogle ScholarCrossref
3.
Shields  CL , Kancherla  S , Patel  J ,  et al.  Clinical survey of 3680 iris tumors based on patient age at presentation.   Ophthalmology. 2012;119(2):407-414. doi:10.1016/j.ophtha.2011.07.059PubMedGoogle ScholarCrossref
4.
Kang  AS , Welch  RJ , Sioufi  K , Say  EAT , Shields  JA , Shields  CL .  Optical coherence tomography angiography of iris microhemangiomatosis.   Am J Ophthalmol Case Rep. 2017;6:24-26. doi:10.1016/j.ajoc.2017.02.003PubMedGoogle ScholarCrossref
5.
Roberts  PK , Goldstein  DA , Fawzi  AA .  Anterior segment optical coherence tomography angiography for identification of iris vasculature and staging of iris neovascularization: a pilot study.   Curr Eye Res. 2017;42(8):1136-1142. doi:10.1080/02713683.2017.1293113PubMedGoogle ScholarCrossref
6.
Blanco  NA , Tello  A , Galvis  V , Acuña  MF , Pedraza-Concha  A .  Spontaneous microscopic hyphema secondary to iris vascular tufts: case report with video documentation.   Indian J Ophthalmol. 2019;67(12):2080-2082. doi:10.4103/ijo.IJO_1200_19PubMedGoogle ScholarCrossref
7.
Ni  N , Johnson  TV , Koval  MS , Shields  CL .  Iris microhemangiomatosis with videographically documented active bleeding and vision loss.   JAMA Ophthalmol. 2013;131(12):1649-1651. doi:10.1001/jamaophthalmol.2013.6211PubMedGoogle ScholarCrossref
8.
Sarmad  A , Alfaqawi  F , Chakrabarti  M , Mitra  A , Mushtaq  B .  Active iris vascular tufts bleeding successfully treated with argon laser photocoagulation.   Eur J Ophthalmol. 2018;28(2):241-242. doi:10.5301/ejo.5001021PubMedGoogle ScholarCrossref
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