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Goniodysgenesis in familial primary open-angle glaucoma

F D Verbraak¹, W vd Berg, J W Delleman

¹Ophthalmogenetic Department, The Netherlands Ophthalmic Research Institute, Amsterdam.

Acta Ophthalmologica

|February 1, 1994

Summary

Goniodysgenesis does not explain familial open-angle glaucoma in this study. However, goniodysgenesis may be inherited independently of glaucoma within these families.

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Area of Science:

Ophthalmology
Genetics

Background:

Familial open-angle glaucoma (FOAG) is a significant cause of vision loss.
Goniodysgenesis, an abnormality of the eye's drainage angle, is a potential contributing factor to glaucoma.

Purpose of the Study:

To investigate goniodysgenesis as a cause of familial open-angle glaucoma.
To compare goniodysgenesis scores in patients with FOAG, their relatives, and a normal population.

Main Methods:

A pilot study compared goniodysgenesis scores in 11 patients with FOAG, 12 relatives with glaucoma, 26 relatives without glaucoma, and 120 controls.
Eight characteristics of goniodysgenesis were scored to determine a total goniodysgenesis score for each individual.

Main Results:

A highly significant difference in goniodysgenesis scores was observed between FOAG patients and the normal population (p < 0.001).

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No significant difference in goniodysgenesis scores was found between relatives with glaucoma and relatives without glaucoma.

A significant difference was found between the normal population and all relatives combined, with higher scores in the relatives group.

Conclusions:

Goniodysgenesis does not appear to be the primary cause of open-angle glaucoma in the studied families.
Goniodysgenesis may be inherited independently of glaucoma, potentially via an autosomal dominant pattern within these families.