Medically reversible limbal stem cell disease: clinical features and management strategies

Abstract

Purpose: To describe the clinical features and management strategies in patients whose limbal stem cell (LSC) disease reversed with medical therapy.

Design: Retrospective case series.

Participants: Twenty-two eyes of 15 patients seen at 3 tertiary referral centers between 2007 and 2011 with 3 months or more of follow-up.

Methods: Medical records of patients with medically reversible LSC disease were reviewed. Demographic data, causes, location and duration of disease, and medical inventions were analyzed.

Main outcome measures: Primary outcomes assessed included resolution of signs of LSC disease and improvement in visual acuity.

Results: Causes of the LSC disease included contact lens wear only (13 eyes), contact lens wear in the setting of ocular rosacea (3 eyes), benzalkonium chloride toxicity (2 eyes), and idiopathic (4 eyes). Ophthalmologic findings included loss of limbal architecture, a whorl-like epitheliopathy, or an opaque epithelium arising from the limbus with late fluorescein staining. The superior limbus was the most common site of involvement (95%). The corneal epithelial phenotype returned to normal with only conservative measures, including lubrication and discontinuing contact lens wear in 4 patients (4 eyes), whereas in 11 patients (18 eyes), additional interventions were required after at least 3 months of conservative therapy. Medical interventions included topical corticosteroids, topical cyclosporine, topical vitamin A, oral doxycycline, punctal occlusion, or a combination thereof. All eyes achieved a stable ocular surface over a mean follow-up of 15 months (range, 4-60 months). Visual acuity improved from a mean of 20/42 to 20/26 (P < 0.0184).

Conclusions: Disturbances to the LSC function, niche, or both may be reversible with medical therapy. These cases, which represent a subset of patients with LSC deficiency, may be considered to have LSC niche dysfunction.

Figure 1

(A) Slit-lamp photograph of a 39 year old male (patient #9 in table 1) with LSC disease demonstrating a demarcation line between healthy and unhealthy epithelium (arrow). (B) Fluorescein staining was used to further highlight these differences (arrow). (C) A 57 year-old female (patient #12 in table 1) with history of dry eyes and glaucoma for 10 years presented with red and irritated eyes with progressive decrease in visual acuity. Initial examination revealed an opaque epithelial growth superiorly with late fluorescein staining. (D) With continued treatment, the LSC disease resolved with significant improvement in symptoms and visual acuity from 20/50 to 20/25. (E) A 40 year-old male (patient #14 in table 1) with a 23 year history of soft contact lens wear presented with LSC disease. Initial examination revealed an irregular opaque corneal epithelium extending from superior limbus. (F) Late fluorescein staining was present. (G) Following medical treatment, the conjunctival type epithelium completely regressed with improvement of visual acuity to from 20/60 to 20/25.

Figure 2

Visual acuity before and after treatment for LSC disease.