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.