Keratoconus in Congenital Diffuse Tapetoretinal Degeneration

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Abstract
During long-termed observation of the homogeneous group of 42 pupils of the Prague schools for the blind, 22 females and 20 males, whose cause of blindness was congenital diffuse tapetoretinal degeneration (t.d.) [amaurosis congenita in t.d. (Leber), the syndrome of congenital blindness (Kurz), heredoretinopathia monohybrida recessiva autosomalis (Alstrom), dysgenesis neuroepithelialis retinae (Waarden-burg), aplasia neuroepithelialis retinae (Sorsby)], the development of bilateral keratoconus in 16 patients, 11 males and 5 females was observed. In 7 patients keratoconus developed at the age of 7-10 years, in 4 patients at the age of 11-15 years and in 5 the onset was unknown. The frequency of keratoconus increased with age and in the age-group over 15 years keratoconus was present in 57%. In 7 patients the course of keratoconus was complicated by acute reversals and ruptures of the Descemet''s membrane. Keratoconus as well as cateract is considered to be the typical and frequent eye complication of congenital diffuse t.d.

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