Anterior chamber angle anomalies associated with signs of pigment dispersion in a group of black probands and their first-degree relatives.
OBJECTIVE
To evaluate the presence of iridocorneal angle anomalies associated with signs of pigment dispersion among a group of black probands and their first-degree relatives.


METHODS
Eleven blacks who exhibited pigment dispersion syndrome (PDS) signs, including Krukenberg's spindles, moderate-to-heavy trabecular meshwork pigmentation, and peripheral lenticular/zonular pigmentation, received a complete eye examination that included gonioscopy. Overt iridocorneal angle abnormalities other than pigment deposition were photo-documented. Twenty-two first-degree relatives (6 siblings, 15 children, and 1 parent) of these probands underwent similar examination.


RESULTS
Among the subjects, 5 of 11 probands (age range 13 to 59 years) and 4 of 22 relatives (age range 14 to 51 years) exhibited definite angle anomalies. The nine subjects with angle anomalies belonged to 7 of 11 separate families. The four relatives with angle anomalies did not have PDS according to our criteria, but two other relatives from two additional families did have signs consistent with clinical PDS. Types of angle anomalies included (1) peripheral anterior synechia-like lesions; (2) a prominent concavity of the peripheral iris at the 6-o'clock meridian; (3) a whitish, thread-like band of tissue near the iris base; (4) a prominent, externally-visible Schwalbe's line at the 6-o'clock meridian; and (5) a prominent, web-like confluence of exaggerated iris processes 360 degrees along the angle wall. Iris processes and prominent Schwalbe's lines were not considered abnormal or anomalous unless they were clearly atypical.


CONCLUSIONS
Iridocorneal angle anomalies, other than pigment deposition, were common among our black group who exhibited signs of pigment dispersion as well as among their first-degree relatives. Further consideration should be given to the possibility that among some patient groups, overt goniodysgenesis could be part of a constellation of signs indicative of a pigment dispersion syndrome. Potentially, clinically detectable goniodysgenesis could prove to be a useful marker to identify certain subjects who carry PDS genetic traits but who have not expressed complete phenotypical disease.
Roberts
D K
DK
Illinois Eye Institute, Illinois College of Optometry, Chicago 60616, USA. droberts@eyecare.ico.edu
Flynn
M F
MF
Gable
E M
EM
D000293:Adolescent; D000328:Adult; D000867:Anterior Chamber / Q000002:abnormalities*; D044383:Black People; D017889:Exfoliation Syndrome / Q000175:diagnosis / Q000208:ethnology* / Q000235:genetics; D005124:Eye Abnormalities / Q000175:diagnosis / Q000208:ethnology* / Q000235:genetics; D005260:Female; D006068:Gonioscopy; D006801:Humans; D008297:Male; D008875:Middle Aged; D014129:Trabecular Meshwork / Q000473:pathology