Basil K Williams1, Maura Di Nicola1, Sandor Ferenczy1, Jerry A Shields1, Carol L Shields2. 1. Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, USA. 2. Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, USA. Electronic address: carolshields@gmail.com.
Abstract
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.
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.