Chronic Ocular Sequelae of Stevens-Johnson Syndrome in Children: Long-term Impact of Appropriate Therapy on Natural History of Disease.

PURPOSE: To describe the long-term ocular and visual morbidity in children with chronic sequelae of Stevens-Johnson syndrome (SJS) and visual outcomes of various management strategies. DESIGN: Retrospective comparative case series. METHODS: This study included 568 eyes of 284 children with SJS who presented between 1990 and 2015. Affected eyes received either conservative therapy (n = 440) or definitive management (n = 128), including lid margin mucous membrane grafting (MMG), prosthetic replacement of the ocular surface ecosystem (PROSE) contact lenses, allogeneic limbal transplantation, or keratoprosthesis using an algorithmic approach based on the severity of dryness and cause and extent of corneal damage. The primary outcome measure was best-corrected visual acuity (BCVA). RESULTS: Two thirds of patients presented more than a year after acute SJS, 99% without prior amniotic membrane grafting, with low vision or blindness in 60% of eyes. Children 8 years or younger in age had significantly worse ocular and visual morbidity (P ≤ .037). At 5 years of follow-up, definitive therapy significantly altered the natural history of the disease by improving BCVA and preventing the development or progression of keratopathy, as compared to conservative therapy (P ≤ .002). In eyes with lid-related keratopathy, MMG was significantly more effective than PROSE, although both were significantly better than conservative therapy and the combination of MMG followed by PROSE provided the best results (P < .0001). CONCLUSION: Children receiving suboptimal care during acute SJS presented later with severe ocular and visual morbidity. Timely therapy, particularly with PROSE and MMG in eyes with lid-related keratopathy, changed the natural course and helped in preserving and improving vision.

Limbal epithelial stem-microenvironmental alteration leads to pterygium development.

Maintenance of tissue homeostasis relies on the accurate regulation of tissue specific stem cell activity which is governed by the dynamic interaction between the positive and negative feedback modulating mechanism of stem cell microenvironmental niche. Alteration or deregulation of the "stem-microenvironmental networking" provokes disease development. Limbal epithelial stem cells (LESC) are the initiator hierarchy that maintains corneal integrity. Compartmentalization of LESC within the limbal vicinity provides an opportunity to understand the stem-microenvironmental relationship. The purpose of this study was to determine the microenvironmental alteration associated with LESCs fate in pterygium condition in comparison with healthy state. Clinical observations evaluated the ocular surface disorder with respect to corneal vascularization, tear film abnormality, and thickening of limbal area in pterygium patients. Structural alteration of limbal stem/progenitor cells and its neighboring niche components were observed using histology and scanning electron microscopy. Receptor overexpression of TGFß-R1, EGF-R1, and IL6-Rα and alteration of IL2-Rα expression pointed toward aberration of "stem-microenvironmental networking" in the limbal vicinity during disease development. Increased cell proliferation index along with TERT, Cyclin-D1, and PCNA over-expression in limbal part of pterygium epithelial cells indicated increased cellular proliferation and disturbed homeostatic equilibrium. We postulate that pterygium is associated with limbal microenvironmental anomaly where the resident epithelial cells became hyperproliferative.