Purpose
Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis syndrome (TENS) and overlap syndrome (SJS/TENS) are rare but serious diseases with potentially devastating ocular sequelae. There is little information in the current literature about the ocular manifestations of these diseases in children. The purpose of this study is to describe the acute and long term clinical features and visual acuity outcomes in this group of patients.
Methods
This is a retrospective cohort study of all children admitted to a tertiary paediatric referral centre with a diagnosis of SJS, TENS, or SJS/TENS between June 1 2001 and June 1 2011. Demographic information, detailed clinical findings at every ocular examination during admission and after discharge were obtained from medical records.
Results
Thirty-six patients were identified for inclusion (20 SJS, 9 SJS/TENS, 7 TENS). Ocular involvement was acutely present in 29 (80.6%) patients. It was severe in 5 (13.9%), moderate in 13 (36.1%) and mild in 11 (30.6%). Conjunctivitis and conjunctival epithelial defects were common manifestations in the acute phase. Conjunctival membranes and corneal epithelial defects affected a third of all patients. Corneal melt and opacification were observed in one patient each and three required amniotic membrane grafts. Of these 36 patients, 17 had at least one clinical review after discharge from hospital. The mean duration of follow-up was 26.9 months.The most frequent clinical findings after discharge from hospital included episodic conjunctivitis, dry eye and meibomian gland dysfunction. Corneal opacification was seen in 4 (23.5%) and corneal vascularization in 3 (17.6%) patients after 2.3 months and 9 months respectively. Limbal stem cell failure developed in 3 and new symblephara in 6 patients after discharge. Four were managed with a Boston scleral lens and one required surgical correction of lid malposition. At final review, all had a Snellen visual acuity at least 20/40 in their better eye. Three had a visual acuity of less than 20/40 in their worse eye.
Conclusions
Most paediatric patients with SJS, SJS/TENS and TENS have acute ocular involvement, which is potentially vision threatening in 13.9%. In children, ocular sequelae may not develop until many months after discharge. This finding emphasizes the importance of long term ophthalmic follow-up for all children with SJS, SJS/TENS and TENS.
Keywords: 463 clinical (human) or epidemiologic studies: prevalence/incidence •
479 cornea: clinical science