Recurrent Amyloid Material in Grafts Used in Patients with Lattice Corneal Dystrophy 2 (Meretoja’s Syndrome)

This is an open-access article distributed under the terms of the Creative Commons Attribution NonCommercial 3.0 License (CC BY-NC 3.0), which allows users to read, copy, distribute and make derivative works for non-commercial purposes from the material, as long as the author of the original work is cited properly. Dear Editor The lattice corneal dystrophies (LCD) are characterized by an accumulation of amyloid within the cornea. There are several types of LCD, and in the LCD2 type the corneal features are subtly unique. The lattice lines are finely arranged and tend to be denser in the periphery (1,2). Corneal erosions occur less frequently, and visual acuity is preserved until the sixth decade (2). Lattice corneal dystrophie 2 is associated with systemic amyloidosis type V (Meretoja syndrome/Finnish type) (1-4). This is a systemic autosomal dominant disease that arises in early adulthood and predominantly affects the cornea, skin, and cranial nerves (2,3). Amyloid deposition corresponds to a degradation product of gelsolin. Amyloidosis type V was first described in Finland (3) where it occurs with high frequency (1). New cases have been reported in several other countries (5). Regular biomicroscopic examination and intraocular pressure measurements are recommended since the disease is incurable and amyloid deposition will continue. When significant central corneal haze is present, only keratoplasty can clarify the cornea and restore vision. However, the graft could accumulates new amyloid. Slight-documentation of these grafts has been reported to survive. We recently reoperated on a Spanish patient with Meretoja syndrome (5). The patient suffered a graft rejection two years after a corneal transplant in the left eye. Months later a neurotrophic central defect was present in the cornea with progressive accumulation of a white substance suspected to be amyloid material (Fig. 1).