Neurotrophic corneal endothelial failure complicating acute Horner syndrome

Purpose: The authors report the clinical findings of a unique case of rapid corneal endothelial decompensation in association with acute Horner syndro me. Study Design: Case report and literature review. Methods: The authors followed a 38-year-old woman who developed Horner synd rome after right jugular vein catheterization during cardiac valvular surge ry. Shortly after the operation, Horner syndrome accompanied by conjunctiva l hyperemia and stromal corneal edema developed in the right eye. Over the course of 4 months, the eye became painful, the corneal endothelial cell co unt dropped precipitously, and the stromal edema worsened, causing a differ ence of 100 mu m in central corneal thickness compared to the unaffected ey e. Deep stromal vascularization started at the limbus, resembling interstit ial keratitis. Results: A 3-week course of topical steroid treatment resulted in a dramati c improvement in the stromal corneal edema and regression of the deep strom al vascularization. Ocular and right hemicranial pain subsided shortly ther eafter. Conclusion: The authors hypothesize that corneal endothelial failure in thi s unique case may have resulted from traumatic sympathectomy. According to experimental evidence in the reviewed ophthalmologic literature, sympatheti c innervation may have a neurotrophic role in the cornea. Corneal pathology similar to the authors' case has been described in hemifacial atrophy (Par ry-Robson syndrome), a disorder that is assumed to result from sympathetic denervation and that can be produced in animals by cervical sympathectomy. The authors therefore hypothesize that sympathetic denervation of the corne a may rarely cause endothelial decompensation and corneal edema. To the aut hors' knowledge, this is the first reported case of corneal endothelial fai lure in Horner syndrome.