Medically Reversible Limbal Stem Cell Disease: Clinical Features and 1 Management Strategies

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. Subjects: 22 eyes of 15 patients seen at 3 tertiary referral centers between 2007 and 2011 with greater than 3 months follow-up. Methods: Medical records of patients with medically reversible LSC disease were reviewed. Demographic data, etiologies, location and duration of disease and medical inventions were analyzed. Main Outcome Measures: Primary outcomes assessed included resolution of 62 signs of LSC disease and improvement in visual acuity. Results: Etiologies 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) while 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, and/or punctal occlusion. 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 and/or niche may be potentially reversible by medical therapy. These cases, which represent a subset of patients with LSC deficiency, may be considered to have LSC niche dysfunction. PRECIS We demonstrate the reversibility of limbal stem cell disease through medical treatment and withdrawal of toxic and traumatic insults. This reversibility suggests the limbal disease may result from dysfunction of the limbal stem niche.