Congenital microcoria associated with late-onset developmental glaucoma.

Abstract

PURPOSE:

To demonstrate that developmental glaucoma (instead of the term goniodysgenetic glaucoma is used in this paper), defined as glaucoma with goniodysgenesis resulting from a fetal maldevelopment of the iridocorneal angle, develops in association with congenital microcoria.

METHODS:

Three subjects descended from a family with autosomal dominant congenital microcoria and goniodysgenesis were followed up for more than 25 years.

RESULTS:

The extended family consisted of 3 generations including 8 males and 10 females. In the second generation, 2 of 7 subjects who presented with a history of congenital microcoria had late-onset goniodysgenetic glaucoma. In the third generation, all 3 descendants of the second generation subjects with congenital microcoria had congenital microcoria with goniodysgenesis. Two of these subjects developed late-onset goniodysgenetic glaucoma in both eyes during the 25-years follow-up period. They were both treated with a trabeculectomy in both eyes to control the glaucoma. Histologically, the iridocorneal angle tissues from the patients showed thick juxtacanalicular connective tissue with accumulations of a basement membrane-like extracellular matrix.

CONCLUSION:

Congenital microcoria are considered to be frequently associated with the incidence of late-onset goniodysgenetic glaucoma.

• Authors: Akihiko Tawara, Ken Itou, Toshiaki Kubota, Yukinori Harada, Norihiko Tou, Naofumi Hirose
• Journal: Journal of glaucoma (J Glaucoma) Vol. 14 Issue 5 Pg. 409-13 (Oct 2005) ISSN: 1057-0829 [Print] United States
• PMID: 16148591 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Topics

• Adult
• Basement Membrane (ultrastructure)
• Connective Tissue (ultrastructure)
• Cornea (abnormalities)
• Extracellular Matrix (ultrastructure)
• Eye Abnormalities (genetics)
• Female
• Glaucoma (congenital, surgery)
• Humans
• Intraocular Pressure
• Iris (abnormalities)
• Male
• Pedigree
• Pupil Disorders (congenital)
• Trabecular Meshwork (abnormalities, ultrastructure)
• Trabeculectomy