Lattice corneal dystrophy

Lattice corneal dystrophy is a rare, inherited disorder of the cornea, the clear front window of the eye, in which abnormal protein fibers called amyloid deposits build up in the middle and front layers of the corneal stroma. Under examination the deposits appear as clear, comma-shaped overlapping dots and branching filaments that form a lattice pattern, which gives the condition its name. Over time the lattice lines grow opaque and converge, clouding the cornea and reducing vision. The disease affects both eyes and is usually noticed before the end of the first decade of life, most often in children between two and seven. Deposits can also lift the outer epithelium and cause recurrent erosions that expose corneal nerves and cause severe pain. Three types are described: type I, with no systemic involvement, caused by mutations in the TGFBI gene; type II, the Finnish form, tied to systemic amyloidosis and gelsolin; and a later-onset type III. Swiss ophthalmologist Hugo Biber is credited with first describing it in 1890. Care ranges from eye drops and ointments for erosions to phototherapeutic keratectomy or corneal transplantation, though the disease can recur in a donor cornea. This entry records what is held about the condition and is not medical advice.