1. 17278 Prosthetic Replacement in Steven-Johnson Syndrome Varsha Rathi Somasheila I Murthy L V Prasad Eye Institute, Hyderabad, India The authors have no financial interest to disclose

2. Introduction Stevens Johnson disease (SJS) is assumed to be an autoimmune response to an environmental factor such as a drug or virus Conjunctival involvement and corneal involvement with dry eye and reduced visual acuity Purpose: To study the role of scleral contact lens (Prosthetic replacement of the ocular surface ecosystem [PROSE]) in Stevens-Johnson Syndrome (SJS)

3. Scleral Contact lens Prosthetic replacement of the ocular surface ecosystem PROSE, Boston Foundation for Sight, Needham Heights USA How PROSE works! The fluid reservoir conserves moisture Reduces pain Reduces photophobia Prevents rubbing of tarsus on cornea Aids in epithelization Masks irregular astigmatism 1.Romero-Rangel T, Stavrou P, Cotter J, Rosenthal P, Baltatzis S, Foster CS. Gas-permeable scleral contact lens therapy in ocular surface disease. Am J Ophthalmol 2000;130:25-32. 2.Rosenthal P, Croteau A. Fluid-ventilated, gas-permeable scleral contact lens is an effective option for managing severe ocular surface disease and many corneal disorders that would otherwise require penetrating keratoplasty. Eye Contact Lens 2005;31:130-134.

4. Material and Methods Patients having SJS who were dispensed with scleral contact lens (PROSE, prosthetic replacement of the ocular surface ecosystem, Boston Foundation for Sight, MA, USA) were analyzed retrospectively Goal of Scleral lens fitting was to improve comfort and vision SJS was diagnosed by the presence of history of allergic drug reaction All patients underwent comprehensive eye examination including Slit lamp biomicroscopy Visual acuity was noted with and without PROSE lens wear in eye

5. Statistical analysis Microsoft excel Windows version 7 was used Visual acuity was converted to LogMAR for statistical analysis Paired t test was used to find significance p <0.05 was termed statistically significant

6. Results Mean age, 28.86 years n= 38 were <16 years Gender: 116 M, 91 F Mean follow up = 18.9 months 12 lenses were dispensed within 6 weeks of having SJS Diameter -<18 mm -69 lenses Remaining were > 18 mm lenses – most being spherical lenses Average daily lens wear = 9h Average training sessions = 3.89 (3 sessions to 11 sessions) were required to teach lens insertion and removal Visual acuity Average Uncorrected visual acuity - 20/100 Average best corrected visual acuity without PROSE– 20/80 Average Prose wear visual acuity – 20/50 The p value was statistically significant (p<0.05) All these patients had photophobia and difficulty in day light with reduced visual acuity and the symptoms reduced and visual acuity improved significantly

7. SJS in acute phase PROSE was dispensed within 6 weeks of presentation of SJS

8. Complications Microbial keratitis – 1 eye – healed with scarring and medical treatment Infiltrative keratitis – 2 eyes of one patient ( changed to new lens) Second order of lens was required in 26 eyes – either for lens breakage or changed fitting over time ( third order was required for 3 eyes – broken lens, changed fitting) Lens breakage - 15 patients Increased photophobia with PROSE wear = 2 eyes ( discontinued lens wear) Deposits on lens surface 4 lenses ( 2 lenses grew fungus) Lens intolerance 4 eyes ( underwent Mucus membrane graft) Deep vessels – 2 eyes of one patient – whether it is due to SJS per say or overuse? The deep vessels regressed with discontinuation of PROSE for a while and then rewear with a high Dk material lens

9. SJS Front surface drying Debris collection in the fluid This required replacing the fluid daily in between to improve vision

10. Challenges Shortened fornix Symblepharon Mucus discharge Pediatric patients Small apertures Presence of tarsorrhaphy Small diameter lenses Creates more suction Close to limbus – More focal scleral compression – blanching of conjunctiva

11. Discussion All these patients had photophobia and difficulty in day light with reduced visual acuity – symptoms reduced and visual acuity improved significantly Lens breakage was noted. The newer lenses were ordered only if lens broke or deposits developed over lens over follow up. The mean follow up was 18.9 months and patient continued to use the same lens over follow-up. This indicates that though the cost of the lens is high, use of the same lens over follow-up is cost effective provided the condition of PROSE is good

12. Conclusion South east Asia – first series Statistically significant improvement in visual acuity was noted with PROSE wear Using a small inventory of lenses, it is possible to fit most of the patients with less reorder rate. Cost effective