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Iris cysts may be associated with dissecting aortic aneurysms

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PHILADELPHIA — A type of congenital iris cyst may be associated with life-threatening dissecting aortic aneurysms, a speaker said at the Wills Eye Alumni Conference here.

Bilateral multifocal iris pigment epithelial cysts, also known as iris flocculi, may result from a shared genetic mutation in smooth muscle that affects the iris and aorta, Jerry A. Shields, MD, said.

“The take-home points are that iris flocculi are usually benign and require no treatment,” Shields said. “Some of them are symptomatic, however, and in those cases the cysts have been reviewed. Good surgical planning is important to remove the cysts, and it can be combined with cataract surgery. [Sometimes] there is a genetic relationship between these iris cysts that are present since birth and aortic aneurysms that occur later in life and are life-threatening conditions. Patients with iris flocculi should have genetic studies and be monitored periodically for a life-threatening aneurysm in the aorta.”

For 30 years, Shields and colleagues followed one patient with iris lesions in both eyes. The cysts grew and receded but caused no visual impairment, Shields said.

Iris flocculi were characterized by a black area around the cysts and were anterior to the crystalline lens, he said.

“It’s relatively uncommon,” Shields said. “They’re usually isolated. However, in some cases there’s a very interesting familial association with thoracic aortic aneurysms, and this is what happened in our patient. There’s an interesting mutation in the smooth muscle gene.”

At age 55 years, the patient presented with 20/40 vision and underwent cataract surgery. A chest X-ray showed a dissecting aortic aneurysm, and the patient was treated for congestive heart failure stemming from the aneurysm.

The cysts were removed during cataract surgery, Shields said. – by Matt Hasson

Reference:

Shields JA. Relationship between congenital iris cysts (iris flocculi) and dissecting aortic aneurysms. Presented at: Wills Eye Alumni Conference; March 10-12, 2016; Philadelphia.

Disclosure: Shields reports no relevant financial disclosures.