Recurrence of lattice corneal dystrophy caused by incomplete removal of stroma after deep lamellar keratoplasty

Purpose: To report clinical and histopathological characteristics of postoperative amyloidosis recurrence in a patient with lattice corneal dystrophy (LCD) type I. Methods: The clinical manifestation of recurrent amyloidosis in the residual stroma was delineated in a patient with LCD type I after deep lamellar keratoplasty (DLKP) for 6.5 years. Complete removal of the residual recipient stroma and regrafting of a new cryopreserved donor button were accomplished by a secondary DLKP. The primary DLKP donor graft and the underlying residual stroma of the recipient obtained by the secondary DLKP were examined for analysis of histopathologic and ultrastructural changes. Results: A tongue-shaped retained stroma with linear opacity was observed underneah the primary DLKP donor graft. The retained stromal layer was thoroughly detached from Descemet membrane, removed, and followed by grafting a new cryopreserved button. The primary donor button exhibited a normal epithelium, fewer keratocytes, an intact Descemet membrane, and mild positive Congo red staining in the middle layer of the stroma. The total retained recipient stroma removed by the secondary DLKP measured approximately 20 mu m in thickness, showing thick and massive amyloid accumulation. The surface of the removed residual stroma toward Descemet membrane showed collagen fibers in an interwoven fashion without bundle structure under a scanning electron microscope. Conclusion: Incomplete removal of the recipient stroma by DLKP can lead to the recurrence of amyloidosis in the residual stroma in patients with LCD Clinical and histologic findings in the primary graft and in the residual recipient stroma implicate stromal genesis of recurrence of LCD after DLKP.