Ocular outcomes of pediatric patients with Stevens-Johnson Syndrome over a five year period

Purpose: To report the visual outcomes of children afficted by Stevens-Johnson Syndrome (SJS) and/or toxic epidermal necrolysis (TEN). To report the etiology, clinical course, management and long-term ocular sequelae of children afficted by SJS and/or TEN. Methods: We retrospectively reviewed medical records of pediatric patients with SJS or TEN admitted over a 5 year period (1/1/2008 to 12/31/2012) to Nationwide Children's Hospital (NCH) in Columbus, Ohio. Data collection included age, sex, etiology, clinical manifestations and long-term outcomes, particularly ocular complications and visual acuity. Results: A total of 20 patients aged 2.9 to 15.6 years underwent ophthalmic evaluation for manifestations of SJS over the 5 year study period. There were no cases of TEN. The most frequent underlying etiology of SJS in the patient population was mycoplasmal infection (35%), followed by antibiotics (25%), anticonvulsants (15%) and viral illness (10%). The most frequent ocular findings were mild, consisting of eyelid edema, conjunctivitis and/or chemosis (68%). The majority of patients were treated systemically with some combination of intravenous immunoglobulin (IVIG) and corticosteroids (75%). Some combination of topical steroids and antibiotics was used to treat ocular manifestations of SJS in 12 out of 20 cases (60%). One patient received bilateral ProKera® (Bio-Tissue) rings. Two of the 20 patients suffered longterm ocular complications, requiring surgical intervention. The first of these 2 underwent ocular surface mucous membrane grafting and entropion repair. The second required multiple procedures, including fornix reconstruction with buccal mucosal graft, ocular surface amniotic membrane graft, and ultimately a lamellar corneal transplant and limbal stem cell transplant. Final best corrected visual acuity (BCVA) was 20/20 in 78% of eyes. Conclusions: Our research adds to the relative paucity of studies and data on ocular outcomes of SJS/TEN in children. Further research is needed regarding etiology (specifically mycoplasmal infection) in children and its connection to ocular manifestations. Our study highlights the lack of consensus on a treatment regimen for SJS/TEN, both systemically and topically. We hope to draw attention to newer treatment options available (ProKera®, amniotic membrane grafting) and the importance of their utility in the acute disease stage of SJS/TEN.