Atypical Corneal Phenotype in Patients With Trachoma and Secondary Amyloidosis

Purpose: To report clinical presentation, in vivo confocal microscopic features, and corneal phenotype in patients with trachomatous keratopathy (TK) and secondary amyloidosis. Methods: Histopathological records of all patients undergoing keratoplasty at the Dr. Rajendra Prasad Centre for Ophthalmic Sciences over a 3-year period were scanned retrospectively for a diagnosis of TK and amyloidosis. Demographic profile and details of preoperative comprehensive ophthalmic assessment were extracted. The histopathology was freshly reviewed. Results: Fifteen patients (29 eyes) with TK and atypical corneal involvement due to amyloid deposition were identified. Herbert's pits and upper palpebral conjunctival scarring were present in all cases. Central or total diffuse corneal scarring was present involving the anterior stroma in 5 (31%) and the full thickness of the cornea in 11 (69%) of the eyes. Eight (73%) of 11 patients with deep stromal amyloid deposits revealed bilateral, discrete, blue-white opacities at the level of deep stroma and Descemet membrane (DM). Endothelial cells were atrophic and flattened with gutta formation. Confoscans revealed hyperreflective, needle-shaped crystalline deposits of extracellular amyloid at various depths of the corneal stroma up to DM. All host corneal buttons demonstrated Congo red-positive amyloid deposits on histopathological examination. Conclusions: We describe a distinct form of TK unlike the usual presentation of dense, leucomatous, vascularized corneal scarring in trachoma. We believe that amyloid deposits in DM and the corneal endothelium have not previously been reported in patients with trachoma.

Automated summary

This study reported the clinical presentation, in vivo confocal microscopic features, and corneal phenotype in patients with trachomatous keratopathy (TK) and secondary amyloidosis. The study identified a distinct form of TK with atypical corneal involvement due to amyloid deposition, different from the usual presentation of dense, leucomatous, vascularized corneal scarring in trachoma. Amyloid deposits in Descemet membrane and the corneal endothelium were observed in these patients.