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Focal Limbal Stem Cell Deficiency in Turner Syndrome: Report of Two Patients and Review of the Literature

Strungaru, M. Hermina MD, PhD; Mah, Dean MD, FRCSC; Chan, Clara C. MD, FRCSC

doi: 10.1097/ICO.0000000000000040
Case Report

Purpose: The aim of this study was to report the presence of focal limbal stem cell deficiency (LSCD) in 2 cases of Turner syndrome.

Methods: This is a case review.

Results: A 25-year-old woman with a history of Turner syndrome diagnosed at 15 years of age was referred for “advanced chronic bilateral phlyctenular disease.” The patient's visual acuity at presentation was 20/25-2 in the right eye and 20/30-2 in the left eye. Slit-lamp examination of both eyes showed inferior conjunctivalization of the corneal epithelium consistent with LSCD. The patient was started on topical cyclosporine 0.05%. A 23-year-old woman with the Turner syndrome was referred for rosacea keratitis. An ocular assessment revealed the visual acuity at presentation to be 20/25 in the right eye and 20/30 in the left eye. Slit-lamp examination of both eyes showed significant conjunctivalization with epithelial scarring and opacity. The patient was started on erythromycin ointment before bedtime, artificial tears, and warm compresses.

Conclusions: This study reports the first cases in the literature describing the coexistence of LSCD and Turner syndrome. Management of any ocular surface inflammation is important to prevent the progression of LSCD.

*Department of Ophthalmology and Vision Sciences, University of Toronto, Toronto, Canada; and

Department of Ophthalmology and Vision Sciences, University of Alberta, Edmonton, Canada.

Reprints: Clara C. Chan, Department of Ophthalmology and Vision Sciences, University of Toronto, 399 Bathurst St, 6th Floor East Wing, Reception 1, Toronto, Ontario M5T 2S8, Canada (e-mail: clarachanmd@gmail.com).

Dr. C.C. Chan has received prior honoraria from Allergan, Alcon Labs Inc, and Bausch & Lomb. Dr. D. Mah has received prior honoraria from Allergan.

The authors have no other funding or conflicts of interest to disclose.

Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal’s Web site (www.corneajrnl.com).

Received August 26, 2013

Accepted October 23, 2013

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