CONGENITAL COLOBOMA OF THE MACULA: TOGETHER WITH AN ACCOUNT OF THE FAMILIAL OCCURRENCE OF BILATERAL MACULAR COLOBOMA IN ASSOCIATION WITH APICAL DYSTROPHY OF HANDS AND FEET

Abstract

IN 1852 von Ammon described the pathological appearances of an eye with coloboma. of the iris and other changes suggestive of what now would be called coloboma of the macula. Though the fovea was present, the macular area had a defect in the choroid, 1 inch by 0.5 inch, partially closed by a tissue poorly supplied with vessels. Ophthalmoscopically the first record comes from Streatfeild in 1866, who reported a typical case of unilateral macular coloboma as an unusual variation of posterior staphyloma seen in myopia. The recognition that the defect is of congenital origin and not the result of myopia came independently from Reich and de Wecker in 1870-not without a controversy in 1872 as to priority. The term coloborna as applied to this lesion was intoduced by Reich; Hirschberg in 1875 used the name of Dictyoschisma centralis, whilst Wiethe in 1885 employed the term Staphyloma of the sclerotic in the macular area. The first bilateral