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Abstract #78468 Published in IGR 20-1

Iris Microhemangiomatosis: Clinical, Fluorescein Angiography, and Optical Coherence Tomography Angiography Features in 14 Consecutive Patients

Williams BK; Di Nicola M; Ferenczy S; Shields JA; Shields CL
American Journal of Ophthalmology 2018; 196: 18-25


PURPOSE: To describe the clinical and imaging findings and treatment options in a series of patients with iris microhemangiomatosis. DESIGN: Retrospective observational case series. METHODS: Setting: Single institution. STUDY POPULATION: Twenty-two eyes of 14 consecutive patients with iris microhemangiomatosis were reviewed. OBSERVATION PROCEDURES: Clinical examination and slit-lamp photography were performed on every patient. Anterior segment optical coherence tomography (AS-OCT), anterior segment optical coherence tomography angiography (AS-OCTA), anterior segment intravenous fluorescein angiography (AS-IVFA), and ultrasound biomicroscopy were performed when possible. MAIN OUTCOME MEASURES: Clinical and imaging features and treatment strategies. RESULTS: Twenty-two eyes of 14 patients with iris microhemangiomatosis were included in this series. Mean patient age was 70 years (range, 58-82 years), and all patients were white. The iris hemangiomatosis appeared as subtle, multifocal, ectatic vascular channels arranged circumferentially at the pupillary margin. Iris color was blue (n = 19 eyes) or green (n = 3 eyes), and the features were unilateral (n = 6 patients) or bilateral (n = 8 patients). Additional features included reduced visual acuity (n = 13 eyes), hyphema (n = 12 eyes), and glaucoma (n = 7 eyes). There was no history of ocular trauma or systemic vasculopathic conditions. AS-OCT documented the fine tuft at the pupillary margin, AS-OCTA showed vascular flow, and AS-IVFA demonstrated pupillary margin vascular lesions with mild staining. Observation was advised in 20 cases (91%), and argon laser photocoagulation was required for repetitive hyphema in 2 cases (9%). CONCLUSION: Iris microhemangiomatosis is a rare ocular condition, often manifesting with spontaneous unilateral hyphema and/or secondary glaucoma. AS-IVFA confirms the diagnosis, and intervention with laser photocoagulation is necessary in only 9% of eyes.

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, USA.

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Classification:

9.4.3.5 Other (Part of: 9 Clinical forms of glaucomas > 9.4 Glaucomas associated with other ocular and systemic disorders > 9.4.3 Glaucomas associated with disorders of the iris and ciliary body)



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