We describe a family that shows autosomal dominant pattern dystrophy of the retinal pigment epithelium. There were 10 affected family members encompassing three generations. The various manifestations of these dystrophies, their inheritance characteristics, affects on vision function and their pathogenesis are discussed.

Psychogenic amblyopia and Stargardt's disease are visual loss in children; however, their prognoses and management differ greatly. Stargardt's disease is a progressive bilateral macular dystrophy, with autosomal recessive inheritance. In time, visual acuity may decrease to 6/60. Stargardt's disease is characterised by retinal pigment epithelial defects on fluorescein angiography, and prolonged photostress recovery time. The ophthalmoscopic appearance is of macular atrophy and yellow flecks surrounding the macula (fundus flavimaculatus), although initially die macular appearance may be unremarkable. Psychogenic amblyopia is a juvenile functional bilateral amblyopia. Alternative labels include Streff syndrome, functional visual loss, visual conversion reaction, and hysterical amblyopia. Patients with psychogenic amblyopia often have multiple symptoms, as well as a reduced academic achievement. It is linked to visual or emotional stress and the prognosis is usually good. Visual acuity measures are variable, kinetic visual fields may spiral, and an accommodative/vergence disorder is often present. Two patients are presented to illustrate the differential diagnosis and management of the two conditions.

A number of visual acuity tests are available for both the literate and the preliterate child. This paper compares the results obtained with five different visual acuity tests; Illiterate E, Landolt C, Bailey‐Hall Cereal test, Lighthouse Flash Cards and Stycar Five Letter test, for 43 children between the ages of three and five years. The linear Illiterate E test appears to be the test of choice; it will detect amblyopia, and the results are not significantly different from those obtained for the single optotype tests. No significant differences were found between the results for the different single optotype tests used in this study. The Landolt C test results are significantly different from those for the other tests and we do not recommend this test for young children.