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Pediatric Iris Cyst

Home / Pediatric Ophthalmology and Strabismus / Diseases of the Cornea, Anterior Segment and Iris

Title: Pediatric Iris Cyst
Author: Karl Andersen, MSIV — Geisinger Commonwealth School of Medicine
Photographer: Danielle Princiotta
Images:

Figure 1: Large iris cyst of right eye (12:00 to 4:30)

 

Figure 2: Iris cyst of left eye (2:30 to 4:00), small cyst (7:00 to 9:00, outside view of photograph)

Keywords/Main Subjects: iris, anterior segment dysgenesis, iris cyst

Diagnosis: Anterior segment dysgenesis, iris stromal cyst

Case presentation: A 3-month old female patient was brought to clinic by her parents who had noticed frequent tearing, light sensitivity, abnormal tracking and pupil asymmetry. The patient was diagnosed with anterior segment dysgenesis, nasolacrimal duct obstruction and congenital cataract. At approximately three years of age, the patient was found to have developed a large multi-lobed cyst on the nasal aspect of her iris in the right eye (figure 1). She subsequently also developed two smaller cysts in the left eye (figure 2). At the age of six, her visually-significant congenital cataract was successfully removed. Recent vision was 20/50 in the right eye and 20/100 in the left eye.

Discussion: Often diagnosed in early childhood or infancy, primary iris stromal cysts likely occur due to aberrant embryologic development, but they can be acquired in some cases.1,2 Congenital iris stromal cysts compose 16% of iris cysts in children.2 If they become enlarged, these cysts often obstruct vision and can cause glaucoma, corneal problems and iritis; the visual prognosis for patients with iris stromal cysts is guarded.1

Other types of iris cysts include primary pigment epithelium cysts (much more common than stromal cysts) and a variety of secondary cysts.2 Primary pigment epithelium cysts are usually stable, not diagnosed before the teenage years and rarely have ocular complications, making the visual prognosis for these cysts better than that associated with primary stromal cysts.1 Secondary cysts have many causes including trauma, tumors and nevi of the iris.1 They are much more likely than primary iris cysts to lead to ocular complications including decreasing visual acuity.2 B-scan ultrasound and ultrasound biomicroscopy (UBM) are the gold standards for evaluation of large iris cysts; however, anterior chamber OCT can also be useful.3

Treatment of iris stromal cysts should be based on a conservative approach and, if indicated, a stepwise escalating pattern. They may be initially observed if not progressing or causing symptoms.2 Interventions include fine-needle aspiration, photocoagulation (with Nd:Yag or argon laser), or, as a last resort, excision.1,2 To decrease the likelihood of recurrence, the cyst cavity can be induced to undergo sclerosis by injection of ethanol following aspiration, a technique that is quite effective and has been shown to be safe even in infants.4,5 Alternatively, antimitotic agents can be infused to help prevent recurrence.2 Primary pigment epithelium iris cysts and secondary iris cysts are treated with the same techniques and based on similar symptom-based escalating patterns.

Although pediatric iris cysts may not be present at birth, they likely represent a congenital anomaly that becomes manifest through the development and growth of the eye. Small cysts may go undetected in newborns and become evident to practitioners only when they become large enough to be symptomatic in early childhood. The difficulty of diagnosing iris cysts in the neonatal period should not be used as grounds to discount the likely congenital etiology of this structural anomaly. A congenital etiology should especially be considered when pediatric iris cysts develop in patients with other congenital aberrancies of the eye such as anterior segment dysgenesis as was seen in the patient presented above.

References:

Faculty Approval: Mark Mifflin, MD and Griffin Jardine, MD

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