Stevens-Johnson Syndrome and Corneal Ectasia: Management and a Case for Association

• Published in: American journal of ophthalmology Vol. 169; pp. 276 - 281
• Main Authors: Saeed, Hajirah N, Kohanim, Sahar, Le, Hong-Gam, Chodosh, James, Jacobs, Deborah S
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.09.2016; Elsevier Limited
• Subjects: Adult; Astigmatism; Classification; Cohort Studies; Contact Lenses; Corneal Diseases - diagnosis; Corneal Diseases - physiopathology; Corneal Diseases - therapy; Corneal Topography; Dilatation, Pathologic - diagnosis; Dilatation, Pathologic - physiopathology; Dilatation, Pathologic - therapy; Ecosystems; Female; Humans; Male; Medical records; Myopia; Ophthalmology; Patients; Prostheses; Prostheses and Implants; Reading; Retrospective Studies; Skin diseases; Statistical analysis; Stevens-Johnson Syndrome - diagnosis; Stevens-Johnson Syndrome - physiopathology; Stevens-Johnson Syndrome - therapy; Studies; Visual Acuity - physiology; Young Adult; New York; United States > US; Massachusetts
• ISSN: 0002-9394; 1879-1891
• DOI: 10.1016/j.ajo.2016.06.039

Purpose To report the occurrence of corneal ectasia (ECT) in patients with history of Stevens-Johnson syndrome (SJS), and to make the case for an association between these 2 diagnoses. We also report the impact of prosthetic replacement of the ocular surface ecosystem (PROSE) treatment on visual acuity (VA) in these patients. Design Retrospective cohort study. Methods A manufacturing database of PROSE patients from 2002 to 2014 at Boston Foundation for Sight (BFS), a single-center clinical practice, was reviewed to identify patients with diagnoses of both SJS and ECT. Results Nine patients were identified with diagnoses of both SJS and ECT. In each case, review of the medical record revealed that diagnosis of SJS preceded that of ECT. The prevalence of ECT in this population exceeded that in the general population ( P < .0001). Videokeratography was available for 13 eyes in 7 patients; using Krumeich's classification of keratoconus, 3 eyes were found to be at stage 1, 3 at stage 2, 1 at stage 3, and 6 at stage 4. Sixteen of 18 eyes underwent PROSE treatment. Of these 16 eyes, initial median VA was 20/200 (range, count fingers to 20/20; logMAR 1.0). Median VA after PROSE customization was 20/30 (range, 20/60–20/15; logMAR 0.1761, P < .0025). Conclusions ECT occurs at a higher-than-expected rate in patients with a history of SJS. PROSE treatment improves VA in these patients. The basis of the association between SJS and ECT is considered, as well as the role of plausible contributory factors such as corneal microtrauma and matrix metalloproteinases.