Bietti ' s tapetoretinal degeneration with marginal corneal dystrophy ( crystalline retinopathy ) : case report

A tapetoretinal degeneration combined with a marginal corneal dystrophy was described by Bietti in three patients (two of whom were brothers) in 1937.' The fundus picture was reported to be similar to that of retinitis punctata albescens, but the spots, located mainly at the posterior pole, were refractile, polygonal, and could be seen at all levels of the neuroretina. Pigment changes were seen at the fovea and the retinal periphery. The corneal deposits were numerous, very small, yellowish white, and concentrated in the superficial layers of the cornea near the limbus. Eight similar cases have since been reported,23 and the term crystalline retinopathy has been applied to the fundus appearance.3 Our case is the first to be reported in Britain.