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Vascularized growth causes glare in elderly woman
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This image was taken at the initial visit.
This image was taken 5 days after the initial visit.
A less magnified version of the image taken on day 5.
Images: Hubbard K
An 85-year-old white female presented to the eye clinic complaining of light sensitivity and glare in her left eye. She stated that it had been bothering her for about 1 month and appeared to be changing. She was not experiencing any pain but mentioned that her vision was getting worse in her left eye.
Her ocular history listed dry moderate age-related macular degeneration in each eye and vitreal floaters. She had bilateral cataract surgery many years earlier without complications. At her last eye exam 1 year ago she was treated for blepharitis/meibomianitis with erythromycin ointment and lid scrubs. Her medical history revealed chronic obstructive pulmonary disease and hypertension that were controlled. The patient was taking potassium 4,700 mg/d, Norvasc (amlodipine besilate, Pfizer) 5 mg/d, captopril 150 mg twice daily, Advair (fluticasone propionate 45 µg and salmeterol 21 µg, GlaxoSmithKline) one puff twice daily and erythromycin 0.5% ointment in both eyes at bedtime. Blood pressure was 109/76 mm Hg.
Visual acuity was reduced to 20/60 in both eyes, but was noted to be similar to last year’s visit. Visual field screening showed central scotoma with scattered visual field defects and poor reliability. Intraocular pressure was 13 mm Hg OD and 17 mm Hg OS.
Pupillary findings were normal in the right eye but irregular in the left eye due to a mass that was causing a notch at 3 o’clock on the iris. A more detailed slit lamp exam revealed a vascularized growth at 3 o’clock on her left iris. The growth had damaged the iris to the point that the pupil was irregularly shaped and there were iris transillumination. The spot was well vascularized on the edges, elevated and had a “fluffy whitish” appearance. There were some trace cells in the anterior chamber and deposits on the back of the IOL implant.
The cornea was within normal limits and there was minor debris in the tear film secondary to blepharitis. Gonioscopy revealed elevated iris tissue temporally with no anterior synechiae and mild to moderate deposits in the trabecular meshwork of the left eye only.
Examination of the retina revealed soft and hard drusen in the macula of both eyes with mild retinal pigment epithelial changes. Retinal vasculature showed mild artery attenuation. The optic nerve heads had some pallor, without significant cupping, and the cup-to-disc ratio was 0.30 h/0.35 v. Dilation was not attempted in the left eye due to the concern that the mechanical stretching of the iris would further damage/tear the iris and cause more problems. Also, it was a concern that the lesion would touch the corneal endothelium or structures of the angle of the eye and cause anterior synechiae. Peripheral retinal views, although limited, showed no areas of concern.
Differential diagnosis, however limited in this case presentation, could include iris atrophy caused by normal aging, which can result in high IOP due to chronic inflammation. Essential iris atrophy is one of three iridocorneal endothelial syndromes (ICEs) and is a slowly progressive, rare disorder of the eye. It is commonly unilateral in presentation with a pupil distorted or out of place and holes in the iris. These abnormalities can lead to peripheral anterior synechiae and glaucoma.
Iris cysts can also be considered due their variability in presentation and location. Most epithelial (pigmented) iris cysts go undetected on the back of the iris and cause no symptoms. Neuro-epithelial iris cysts are the most common and are usually located at the root of the iris. Some iris cysts can cause schisis or splitting of the iris pigment epithelium, resulting in holes or elevation. However, iris cysts rarely progress. They may come to attention during evaluation of the iris, as they present as elevations. Ciliary body cysts may do the same and are clear. Structural changes to the iris can be caused by surgical trauma during cataract surgery as well as eye injuries.
Malignant melanomas on the iris are rare and have a tendency to present inferior temporal, below the horizontal midline. They are primarily unilateral, with a high amount of vascularization supplying the tumor. They present most commonly in patients with light-colored eyes in the fifth decade of life. Most of these patients have had at least one iris nevus since birth.
Iris melanomas grow rapidly and can cause a sectorial cataract near the site of the tumor. Also, small pieces of iris and cells can break off and float in the anterior chamber and cause a pseudohypopyon. They can also deposit on the anterior surface of the lens, on the posterior cornea and in the anterior chamber angle of the eye resulting in blurry vision, secondary iritis or secondary glaucoma. Due to the vascularized and fragile nature of iris melanomas, blood vessels can break and leak, causing a hyphema in the anterior chamber. Primary site iris malignant lesions can appear in many different shades and colors, but secondary site iris malignancies appear as a gray-white tapioca color and are round or oval in shape.
Diagnosis, prognosis, treatment
After examining the clinical findings, the patient was diagnosed with a metastatic iris neoplasm and was educated and referred for cancer screening to locate the site of the primary tumor. Metastatic iris neoplasms are rare and almost always unilateral. The primary site of the cancer is usually the breast or lung, but it can occur in other organs such as the kidneys, gastrointestinal tract and thyroid.
Because of the location of iris neoplasms near the angle of the eye, more than one-third of patients develop a secondary glaucoma. Once diagnosed, systemic prognosis is poor, and most of these patients do not live longer than 13 months.
Treatment options for primary site malignant lesions in the iris are determined by the size, severity and location of the lesion. Radiation and surgical removal can be successful but can cause other side effects and harm to vision and ocular structures. Larger primary site tumors in the eye require enucleation. When the lesion in the iris is the result of a metastasis from another organ in the body, chemotherapy and systemic treatments are given.
This patient’s management
MRI and systemic testing revealed that the primary site of the carcinoma was the breast tissue of this patient, with metastasis throughout the body. Treatment options for this type of metastasis usually include chemotherapy, radiation, hormone therapy and surgery.
Due to the frail nature of the patient and the extensive spread of the carcinoma, only estrogen blockers were given. Many treatments can improve the quality of life and suppress side effects even if the cancer continues to progress. The patient was also followed for secondary glaucoma to manage the increased eye pressure with drops.
Reports from the patient’s other specialist show that within 6 weeks the tumor had spread rapidly throughout parts of the anterior chamber and affected the corneal endothelium, causing corneal haze. Also, the angle of the eye became blocked, and IOP was elevated. Due to systemic progression the patient was heavily medicated to control pain, and treatment was focused on comfort and spending time with her family.