Lid-Related Keratopathy in Stevens-Johnson Syndrome: Natural Course and Impact of Therapeutic Interventions in Children and Adults.

Abstract

To compare the long-term visual outcomes of different management strategies in children and adults with Stevens-Johnson Syndrome (SJS)-induced chronic lid-related keratopathy. Retrospective comparative case series. This study included 705 eyes of 401 patients (81 children and 320 adults) with SJS who presented with chronic lid-related keratopathy between 1990 and 2015. Affected eyes received either conservative therapy [topical medications (n = 363)] or definitive management (n = 342) that included mucous membrane grafting (MMG), prosthetic replacement of the ocular surface ecosystem (PROSE) contact lenses, or both. The primary outcome measure was change in best corrected visual acuity (BCVA) over time. The secondary outcome measure was the odds of developing corneal ulceration or perforation in the first year. The treatment subgroups were comparable at baseline in terms of BCVA and previous management (P > .10). Over 10 years, children and adults who received conservative therapy lost at least 5 lines of median BCVA and carried a 3 times higher risk of developing corneal ulceration in the first year. Conversely, definitive therapy provided significant benefit by improving median BCVA (P < .0001). In children, MMG was more effective than PROSE (P = .009), whereas PROSE was more effective than MMG in adults (P = .028). However, the combination of MMG followed by PROSE provided the best results in both children and adults (P < .036). Both MMG and PROSE changed the natural course and helped in preserving and improving vision in eyes with SJS-induced lid-related keratopathy. Regardless of age, those who received both MMG and PROSE had the best long-term visual outcomes.