The Wrath Of Steven Johnson Syndrome: A Continuum Of Ocular Complications

Ocular Stevens-Johnson Syndrome is a rare, severe inflammatory disorder with widespread mucocutaneous involvement due to delayed hypersensitivity. Corneal keratinization often necessitates keratoprosthesis & glaucoma drainage device implantation. Ocular complications include anterior segment inflammation, tissue disruption, scleral rigidity, optic neuritis, & uveitis from biomechanical and autoimmune damage. Flares may be triggered by infection, medications, or vaccines. This paper aims to present a complex case of ocular SJS who underwent Boston KPro and GDD implantation, then developed optic neuritis and uveitis following COVID-19 infection, for which she is recalcitrant to steroid therapy, & discuss the approach to diagnosis & management

This case report is set in Manila, Philippines, featuring a 55-year-old hypertensive female with bilateral ocular Stevens-Johnson Syndrome. She underwent Boston Keratoprosthesis (KPro) and glaucoma drainage device (GDD) implantation. Following a COVID-19 infection, she developed optic neuritis and uveitis, proving recalcitrant to steroid therapy. This report highlights the diagnostic and management challenges in a complex ocular SJS case within a resource-limited setting.

A 55-year-old female with bilateral ocular SJS underwent Boston KPro and GDD implantation of the right eye. She developed COVID-19 infection, then noted gradual progressive blurring of vision on the right from 20/70 to 20/150. Optic neuritis was noted. 5-day pulse IV methylprednisolone was given, improving BCVA to 20/40. Gradual taper of 1 mg/kg/day prednisone was done. While off steroids, she had an episode of eye redness with blurring of vision and flare of optic neuritis. Vision on the right dropped to 20/100, with increased floaters and scleritis. High dose oral prednisone was started, where improvement was noted. On slow taper to 60 mg/day, vision worsened to light perception, and was assessed with anterior and intermediate uveitis, both eyes with optic neuritis recurrence, right eye. Rheumatologic work-ups are negative. Subtenon injection of steroids done. Methotrexate and higher dose of steroids were started. Improvement to 20/40 was noted. However, the left eye had a sudden episode of uveitis with an increase in intraocular pressure. Implantation of GDD was done on the left eye, with stabilization of intraocular pressure.

This case highlights the complexity of managing ocular Stevens-Johnson Syndrome, particularly in patients with recurrent inflammation and steroid dependency. Despite initial improvement with high-dose steroids and immunosuppressive therapy, multiple relapses of optic neuritis and uveitis posed significant challenges. The need for long-term immunomodulation became evident. Ultimately, the patient achieved visual stability with methotrexate and GDD implantation, underscoring the importance of individualized, multidisciplinary management in severe ocular SJS cases.