LONG-TERM FOLLOW-UP OF PATIENTS WITH FAMILIAL SUBEPITHELIAL AMYLOIDOSIS OF THE CORNEA

Purpose: To investigate the clinical course of patients with familial subepithelial amyloidosis of the cornea (FSA). Methods: The authors re trospectively investigated the clinical course of seven Japanese patie nts with FSA. Corneal specimens obtained at the time of keratoplasty w ere examined histopathologically. Results: The mean follow-up period w as 20.6 years, including four patients followed for more than 25 years . In all patients, the initial symptoms of photophobia and epiphora st arted in the first decade of life. All except one patient had their fi rst keratoplasty before 30 years of age. The seven Japanese patients w ith FSA had a total of 35 keratoplasties, each of which was followed b y a severe recurrence of disease. In each patient, subepithelial hazin ess developed in the graft within 1 year of keratoplasty (mean, 7.9 mo nths). Amyloid deposition typically recurred within a few years (mean, 26.6 months) followed by a deterioration of vision. There was a high incidence of postkeratoplasty complications such as wound dehiscence, glaucoma, and cataract. Histopathologic findings demonstrated that, in the early phase of recurrence, amyloid was deposited between the basa l cell of the epithelium and Bowman's layer. Conclusion: Patients with FSA have ocular symptoms with a deterioration in vision from an early decade of life. Conventional surgical approaches were complicated by subepithelial haziness in the first postoperative year, which was foll owed by a severe recurrence in each patient. New surgical approaches m ay be indicated in FSA.