Abstract
Purpose :
Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) can lead to chronic ocular surface disease (OSD). There is little outcomes data in the pediatric SJS/TEN population. We performed a retrospective cohort study to better assess prosthetic replacement of the ocular surface ecosystem (PROSE) treatment in pediatric patients with a history of SJS/TEN.
Methods :
Through review of a manufacturing database from 2006-2011 at Boston Foundation for Sight (BFS), we reviewed medical records of patients who underwent PROSE treatment and had SJS/TEN. Age at consultation, time from consultation to PROSE fitting, etiology of SJS/TEN, change in visual acuity (VA) with treatment, and change in visual functioning (VF) at 6 months using the National Eye Institute 25-item Visual Functioning Questionnaire (NEI-VFQ25) was recorded.
Results :
236 patients with SJS/TEN underwent PROSE treatment. 40 patients were under 18 years old (YO) at the time of presentation to BFS, with a range of 4-17 YO (median 9 YO, 23 females, 17 males). Of these pediatric patients, time from presentation to dispense of prosthetic device varied from .04-5.41 years. There was a trend toward longer customization and training time for those ≤7 YO compared to those 8-18 YO (1.28 years vs. 0.73 years, p=0.452).
Of the 40 pediatric patients, 18 patients (32 eyes) had complete VA and NEI-VFQ data (9 females, 9 males). Age at PROSE treatment ranged from 4-16 YO (median 8.5 YO). Suspected etiologies included antibiotics (n=6, amoxicillin=5/6), ibuprofen (2), quinine (1), phenytoin (1), lamotrigine (1), phenobarbital (1), viral infection (2), unknown (4). Median VA at presentation was 20/100 (range, LP-20/25). Median VA after PROSE treatment was 20/30 (range, HM-20/20, p<0.0001). Follow-up ranged from 6-42 months (mean 13.9 months). Mean NEI-VFQ25 score prior to PROSE treatment was 65.57. Mean score 6 months after was 78, p=0.016.
Conclusions :
PROSE treatment can improve VA and VF in pediatric patients with chronic OSD from SJS/TEN, with significant positive impact in these patients who might otherwise be poor contact lens candidates. Time from consultation to dispense of prosthetic device may be longer in the youngest of pediatric patients but our data show that children with a history of SJS/TEN, including as young as 4 YO, are good candidates for PROSE treatment.
This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.