New aspects on the heredity of open angle glaucoma

Oen angle glaucoma is known to be inherited, but detailed knowledge of the primary genetic factor and its mode of inheritance are lacking. By studying both the glaucomatous and non-glaucomatous members in afflicted pedigrees, it has been established that premorbid goniodysgenesis, inherited in an autosomal dominant mode, plays a decisive role for the eventual development of glaucoma. Goniodysgenesis has been documented not only in infantile congenital glaucoma but also in late congenital glaucoma (often misinterpreted as glaucoma simplex) and in families with pigmentary glaucoma and exfoliation glaucoma. In addition, there is evidence that goniodysgenesis is the morphological prerequisite of the high elevation of the IOP on provocation with topical steroids. The article contains a short survey of the literature and presents three families with intrafamilial variations of open angle glaucoma based on a dominant genetic trait, viz. goniodysgenesis.