Corneal Limbal Stem Cell Deficiency in Children with Stevens-Johnson Syndrome

To determine the incidence of corneal limbal stem cell deficiency (LSCD) as chronic ocular sequelae in children with Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) and analyze the factors associated with LSCD development.

Retrospective case series.

Medical records were reviewed of pediatric patients who had been admitted to Seoul National University Hospital with SJS/TEN and who were diagnosed as having acute ocular involvement. For each record, the following data were collected: demographic information, underlying diseases, causative agents, acute systemic and ocular manifestations, systemic and ocular treatments, chronic ocular complications including LSCD, and visual acuities.

Of 19 children with SJS/TEN, LSCD developed in 6 (32%) patients at a mean of 12.3 ± 21.3 months after the onset of SJS/TEN (median 3.5 months). Severity of acute systemic involvement including elevation of liver enzyme levels and serum C-reactive protein levels was significantly correlated with the development of LSCD (P = .0038) and chronic ocular complications (P = .0044). The presence of corneal epithelial defect necessitating the use of therapeutic contact lenses in the acute phase was also associated significantly with LSCD development. Combined penetrating keratoplasty and limbal allograft were performed in 3 of 6 LSCD patients, and grafts failed in 2 (67%) patients because of infection. At the last follow-up, visual acuities were significantly poorer in patients with LSCD compared with those without LSCD (P = .0055).

Corneal LSCD occurred in 32% of pediatric patients with SJS/TEN, leading to poor visual outcome. Severity of acute systemic involvement was significantly associated with the development of LSCD and chronic ocular complications.