Case report

Iridoschisis and cataract in a juvenile patient with periocular eczema

Aaberg, Thomas M. Jr. MD*; Nelson, Matthew Patrick

Author Information
Journal of Cataract and Refractive Surgery Online Case Reports: July 2017 - Volume 5 - Issue 3 - p 54-57
doi: 10.1016/j.jcro.2017.06.001
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Abstract

Iridoschisis is a rare ocular condition defined as separation of the iris stroma into 2 layers along the frontal plane and potentially results in ruptured fibers separating from the anterior iris and floating freely in the anterior chamber.1–5 Uncommon and first identified by Schmitt in 1922,6 iridoschisis had been reported in approximately 100 cases by 1996.7 We have identified at least 26 cases that occurred since then.2,8–28 Visual loss is atypical of this condition.

Iridoschisis is usually bilateral and accompanied by angle-closure glaucoma in 50% of cases; it generally presents in older patients.29 It is not uncommon for cataracts to present with iridoschisis. Reports of an association with congenital syphilis also exist.19

CASE REPORT

An 11-year-old white girl was referred for painless blurred vision in her right eye progressing over a few months. The medical history was pertinent for severe facial and periocular eczema due to atopic dermatitis, for which the patient used oral diphenhydramine (Benadryl) and topical triamcinolone acetonide, desoximetasone, pimecrolimus, and desonide. The patient denied any use of topical eye medications. She denied a history of facial or ocular trauma, and she and her father denied a family history of eczema.

External examination showed diffuse eczematous dermatologic changes on her face, including erythema, scaling, and oozing. The Snellen corrected distance visual acuity (CDVA) was hand motion in the right eye and 20/25 in the left eye. The intraocular pressure by applanation tonometry was 19 mm Hg and 18 mm Hg, respectively. Iridoschisis and its associated feathery changes in the iris were observed from 3 o'clock to 10 o'clock in the right eye (Figure 1). The right eye also had a milky white cataractous lens that obscured a view of the posterior segment. No cells were observed in the anterior chamber. Examination of the left eye showed a normal anterior segment, with no iris alterations and a clear lens (Figure 2).

Figure 1.
Figure 1.:
Iridoschisis from 3 o'clock to 10 o'clock in the right eye.
Figure 2.
Figure 2.:
The left and right eyes with unilateral cataract and iridoschisis.

Ocular echography of the anterior and posterior segments was performed. Ultrasound (US) biomicroscopy showed typical iridoschisis changes, with splitting of the iris stromal layers and focal areas of contact between the anterior schisis layer and the corneal endothelium (Figure 3). Anterior segment ocular coherence tomography (AS-OCT) was also performed and showed features similar to those seen on US (Figure 4). No zonular abnormalities were noted on the US, AS-OCT, or slitlamp examinations; specifically, there did not appear to be zonular loss or laxity. Iris plateau formation was not present. Posterior segment US showed normal anatomy with a fully attached retina, minimal vitreous opacities, and a normal optic disc.

Figure 3.
Figure 3.:
Ultrasound biomicroscopy of the anterior segment taken tangentially through the inferonasal quadrant of the right eye.
Figure 4.
Figure 4.:
Anterior segment OCT of the right eye with vertical roster through the inferonasal quadrant.

Laboratory testing for treponemal and syphilis immunoglobulin G antibodies was performed and was negative for both. The chemistry panel showed nothing unusual; the complete blood count, while otherwise normal, found eosinophils elevated at 0.73 absolute/9.9%.

Uneventful cataract surgery was performed with a temporal limbus-based approach, and a posterior chamber intraocular lens (IOL) was implanted. There were no unusual intraoperative findings. The zonular fibers appeared to be normal with no laxity or loss. The lens capsule was intact. The iridoschisis changes did not complicate the surgery, probably because of the inferior and nasal locations of the changes.

Postoperatively, the Snellen CDVA in the right eye improved to 20/20. At the 6-month follow-up, the extent of the iridoschisis in the right eye remained stable (Figure 5). The IOL remained well centered. There were equivocal iris changes in the left eye. The left lens remained clear.

Figure 5.
Figure 5.:
Anterior segment OCT of the right eye following cataract surgery with horizontal roster through the inferonasal quadrant.

DISCUSSION

The definitive feature of iridoschisis is detachment of the anterior and posterior iris stroma, which begin to float in the anterior chamber.19 The superior quadrants typically undergo less disintegration than the inferior quadrants,30 and synechiae are often present in the affected eye.15

Approximately half the eyes with iridoschisis have concurrent glaucoma; most of the other cases are associated with senescence.19 Long-lasting glaucoma is postulated to lead to iris atrophy4 and vice versa,31 although the precise etiology of iridoschisis is unknown. Fluorescein angiography of eyes with iridoschisis supports the hypothesis that the condition is not ischemic (normal levels of perfusion occurred in the affected areas),32 and although glaucoma and iridoschisis are strongly associated, it is not known whether 1 condition causes the other.19

Cataracts are also associated with iridoschisis.1,5,15 However, iridoschisis is typically considered to be age-related29,33; some studies have postulated a genetic component,7 others suggest trauma as a potential cause,3 and some report an association with syphilis, particularly syphilitic interstitial keratitis.19

Juvenile iridoschisis is atypical. We have identified 4 previous cases: 2 in microphthalmic eyes,34,35 1 in a glaucomatous individual,36 and 1 presenting with incomplete plateau iris.11 Adler and Weinberg8 report the only previous case of iridoschisis and eczema and discuss the possibility of an oculodermal syndrome characterized by iridoschisis and lens subluxation. Our patient had periocular eczema due to atopic dermatitis, which is known to be associated with cataract formation. However, the iridoschisis developed in the eye with the cataractous lens. These findings may be independent of each other, but we believe a potential common pathophysiologic mechanism may exist. It is important to note that the patient and parents denied a history of ocular trauma, but patients' recollections can often be inaccurate, particularly relative to injuries. That the patient was using several steroid creams around the eyes is also important; the medications, rather than the dermatologic condition, may be the true association. Nevertheless, we feel this case is unusual enough to warrant publication in the event other clinicians see similar clinical features in periocular eczema patients.

Iridoschisis is a rare condition typically caused by aging mechanisms; however, other etiologies may exist. Reports have identified both infectious (eg, syphilis) and inflammatory (eg, eczema) associations, although much about iridoschisis is still unknown. Further evidence is necessary to link the condition with a specific cause. Our patient will require long-term follow-up for potential changes in the fellow eye and glaucoma development. We will report any significant future findings and hope to collaborate with physicians who have patients with this condition.

Disclosure:

Neither author has a financial or proprietary interest in any material or method mentioned.

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