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

Abstract: Purpose 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. Design Retrospective comparative case series. Methods 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 (n=363) or definitive management (n=342) including mucous membrane grafting (MMG) and 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. Results The treatment sub-groups were comparable at baseline in terms of BCVA and prior management (P>0.10). Over a period of 10 years, children and adults receiving 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 Conclusions Both MMG and PROSE changed the natural course and helped in preserving and improving vision in eyes with SJS-induced lid-related keratopathy. Irrespective of age, those receiving both MMG and PROSE had the best long-term visual outcomes.