Tapetochoroidal degeneration combined with cataract and lentodonesis

Presumably the combination of regional choroidal atrophy, cataract and lentodonesis is a new genetic entity. The hereditary transmission seems to be autosomal-recessive. As the first visual impairment, hemeralopia is noted during the second decade of life. In these early stages patches of choroidal atrophy are limited to the intermediate and perimacular zone. ERGs are not known from these early stages. Later on, no scotopic and only subnormal photopic reactions are registered. The patients can do their work relatively well until the end of the sixth decade, when a cataract develops and the choroidal atrophy gradually extends to the posterior pole. Together with the cataract, a lentodonesis is developing. The lentodonesis is caused by degeneration and elongation of the zonular fibres. Therefore, in order to escape the danger of a lens-subluxation or a lens-luxation into the anterior chamber, an intracapsular extraction should be done at an early stage of lentodonesis. In spite of this, the visual prognosis is poor, since the areas of choroidal atrophy are gradually enlarging. In these late stages the macula seems to be involved in all cases.