Purchase this article with an account.
Sahar Kohanim, Hong-Gam Le, Deborah S. Jacobs; When Lightening Strikes Twice: Seven cases of Stevens-Johnson Syndrome and Corneal Ectasia. Invest. Ophthalmol. Vis. Sci. 2012;53(14):4708.
Download citation file:
© ARVO (1962-2015); The Authors (2016-present)
We report seven cases of reduced vision and visual function related to corneal ectasia and history of Stevens-Johnson syndrome (SJS) that were referred for prosthetic replacement of the ocular surface ecosystem (PROSE). PROSE treatment uses FDA approved prosthetic devices to replace or enhance the functions of the ocular surface system.
Retrospective medical record review of cases extracted by search on diagnoses of Stevens-Johnson syndrome and corneal ectasia conducted on a manufacturing database of devices fabricated from 2002-2011. Review of literature on causal vs. coincidental relationship between these syndromes was conducted.
Seven patients 5F:2M with corneal ectasia and Stevens Johnson syndrome were identified. Age at diagnosis of SJS, age at diagnosis of ectasia, and age at presentation are reported. All patients received PROSE treatment for reduced vision and visual function. Of 3 patients presenting after 2006, best corrected visual acuity on presentation ranged from 20/25 to counting fingers with various modes of correction, including specialty contact lens. There was a significant improvement in the visual acuity with PROSE treatment to 20/20-20/60 range, with 6/6 of eyes obtaining an improvement in best-corrected visual acuity (defined as a gain of 2 or more Snellen lines or 20/20 in the eye 20/25 at baseline). Average wear time of PROSE device ranged from 8-16 hours in these 3 patients who had improvement in visual function subjectively and as measured by NEI VFQ-25. The natural history of patients fitted prior to 2007 is reported, with range of follow-up from 1 month to 27 years.
Patients with corneal ectasia and concomitant ocular surface disease from Stevens-Johnson syndrome pose a special challenge for visual rehabilitation. Contact lens may be poorly tolerated and penetrating keratoplasty is high risk. PROSE treatment is a useful option for patients such as these with complex corneal disease. Causal vs. coincidental relationship between SJS and corneal ectasia is considered.
This PDF is available to Subscribers Only