Congenital microcoria associated with late-onset developmental glaucoma.
OBJECTIVE
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.


CONCLUSIONS
Congenital microcoria are considered to be frequently associated with the incidence of late-onset goniodysgenetic glaucoma.
Tawara
Akihiko
A
Department of Ophthalmology, School of Medicine, University of Occupational and Environmental Health, Japan. Tawara-a@med.uoeh-u.ac.jp
Itou
Ken
K
Kubota
Toshiaki
T
Harada
Yukinori
Y
Tou
Norihiko
N
Hirose
Naofumi
N
D000328:Adult; D001485:Basement Membrane / Q000648:ultrastructure; D003238:Connective Tissue / Q000648:ultrastructure; D003315:Cornea / Q000002:abnormalities*; D005109:Extracellular Matrix / Q000648:ultrastructure; D005124:Eye Abnormalities / Q000235:genetics*; D005260:Female; D005901:Glaucoma / Q000151:congenital* / Q000601:surgery; D006801:Humans; D007429:Intraocular Pressure; D007498:Iris / Q000002:abnormalities*; D008297:Male; D010375:Pedigree; D011681:Pupil Disorders / Q000151:congenital*; D014129:Trabecular Meshwork / Q000002:abnormalities* / Q000648:ultrastructure; D014130:Trabeculectomy