Title: Iris microhemangiomatosis
Authors: Abigail Jebaraj, MSIV; Bradley Katz, MD; Roger Harrie, MD; Theresa Long, MD.
Photographer: Glen Jenkins
Keywords/Main Subjects: iris, arteriovenous malformation, microhemangiomatosis, fluorescein angiography, hyphema
Diagnosis: Iris microhemangiomatosis of the right eye
Description of Image: A 56-year-old female presented with a history of two episodes of hyphema of the right eye causing blurry vision with no history of trauma. Her medical history was noncontributory with no other unusual bleeds, no blood thinner use, and no bleeding disorders. Her family history was significant for a brother with childhood glaucoma and fatal brainstem arteriovenous malformation. Visual acuity was 20/15 with normal intraocular pressures bilaterally. Gonioscopy showed a normal angle with no neovascularization bilaterally. The remainder of the exam was normal.
Ultrasound of the anterior segment of the right eye performed with high-frequency 40 MHz probe with an immersion technique revealed no ciliary body abnormalities, posterior iris abnormalities, masses, cysts, or other lesions. Fluorescein angiography of the iris revealed vessel leakage of the pupillary margin consistent with microhemangiomatosis of the iris. She was recommended to avoid strenuous activity, avoid aspirin and ibuprofen, use a drop of prednisolone forte 1% four times daily in the right eye, and use a drop of cyclopentolate 1% twice daily in the right eye.
Iris microhemangiomatosis is a benign vascular lesion of the iris that can cause spontaneous hyphema, blurry vision, and increased intraocular pressure. It is a type of vascular iris lesion, which is only 2% of all iris tumors. The majority of described cases are bilateral, but it can occur unilaterally. It can be associated with myotonic dystrophy and idiopathic juxtafoveal retinal telangiectasia. Diagnosis consists of a slit lamp exam, fluorescein angiography, and more recently optical coherence tomography angiography (OCTA) has been described as a diagnostic option. It is important to view under gonioscopy to differentiate iris microhemangiomatosis from iris neovascularization. Fluorescein angiography of the iris can demonstrate ectatic vasculature, particularly along the pupillary margin (pictured). Management includes observation, medication, or argon laser photocoagulation if the hyphemas are recurring. Medical treatment, as used in this case, can consist of topical corticosteroids and mydriatics for hemostasis. As elevated intraocular pressures can also be associated with iris microhemangiomatosis, appropriate intraocular pressure monitoring and control is an important component of management.
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Faculty Approval by: Griffin Jardine, MD