Ocular manifestations of Stevens-Johnson syndrome and toxic epidermal necrolysis in children

Abstract Purpose To describe the acute and chronic ocular manifestations of Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN) and SJS/TEN Overlap syndrome (Overlap syndrome) in children. Design Retrospective case series. Methods Medical records of children admitted to the Hospital for Sick Children between 2001 and 2011 with SJS, TEN and Overlap syndrome were reviewed. Demographic information, all abnormal ophthalmic findings (and median time to first diagnosis), visual acuities and ophthalmic treatments prescribed were collected for each eye for every patient. Results Thirty-six children were identified for inclusion in the study. Twenty-nine (81%) had acute ocular involvement including all patients with TEN (n=7). Conjunctivitis was the most common (78%) clinical sign. This, together with conjunctival membranes and subconjunctival hemorrhage were the earliest signs, presenting by a median of 1 day. The percentage of patients and median day to occurrence of complications were as follows for lid margin ulceration and corneal epithelial defects (25%, 3 days), conjunctival ulceration (39%, 3.5 days), symblepharon (28%, 4 weeks), corneal opacification (11%, 4 months), limbal stem cell failure (8%, 7 months) and corneal vascularization (8%, 10 months) after admission. Over 90% of children maintain a visual acuity of 20/40 or better in each eye at a mean follow-up of 1.4 years. Conclusions Ocular involvement in SJS, TEN and Overlap syndrome is common and the ocular manifestations may develop many months after the initial presentation mandating the need for long term follow-up of these children. Despite the high frequency of sight-threatening disease, most children maintain good vision in the long term.