Bilateral en coup de sabre with bilateral ophthalmic involvement and hearing loss: A rare case report with review of literature

Purpose En coup de sabre (ECDS) is a rare craniofacial variant of linear scleroderma characterized by a band-like induration of the frontoparietal skin resembling the strike of a sabre. Bilateral involvement is exceptionally rare, and concurrent bilateral ophthalmic manifestations with mixed hearing loss (sensorineural and conductive) have not been previously described.Method We report a case of bilateral ECDS presenting with bilateral periorbital involvement including madarosis and adnexal skin atrophy, along with bilateral hearing loss and retinal ischemia resulting in extensive neovascularization.Result A structured review of reported ophthalmic manifestations in linear scleroderma and ECDS is presented. The simultaneous occurrence of bilateral ocular adnexal involvement, retinal ischemia causing tractional retinal detachment (TRD), secondary Coats-like features, and bilateral mixed hearing loss in bilateral ECDS is a previously unreported combination that widens the phenotypic spectrum of this condition.Conclusion This case underscores the need for comprehensive ophthalmic and audiological surveillance in all patients with ECDS, particularly those with bilateral or extensive craniofacial disease.