Central corneal thickness in patients with congenital aniridia.
OBJECTIVE
To compare the mean central corneal thickness (CCT) in patients with congenital aniridia to that of a group of age-matched control subjects. The findings of specular and confocal microscopy in a patient with aniridia are discussed.


METHODS
The mean values of five consecutive pachymetry measurements of patients with aniridia and control subjects were used for analysis. Statistical analysis was performed with a Mann-Whitney rank sum test. Specular microscopy was performed on one patient with aniridia using a Konan Specular Microscope Noncon ROBO CA (Hyogo, Japan). Confocal microscopy through focusing was performed with the Tandem Scanning Confocal Microscope (Reston, VA).


RESULTS
Mean CCT measured 691.8 +/- 75.4 mum for patients with aniridia (16 eyes of 10 patients) and 548.2 +/- 21.2 mum for control subjects (P < 0.001). Specular microscopy in a patient with aniridia showed normal endothelial cell counts and structure. Confocal microscopy through focusing of this patient showed normal-appearing keratocytes and a thick corneal stroma.


CONCLUSIONS
Patients with congenital aniridia have significantly thicker corneas than do age-matched control subjects. This difference can have important implications for the treatment of those patients who develop secondary glaucoma. The increased CCT in patients with aniridia is not a result of endothelial dysfunction but appears to be the result of the production of a thickened but otherwise healthy cornea by the mutated PAX6 gene.
Whitson
Jess T
JT
Department of Ophthalmology, University of Texas Southwestern Medical Center at Dallas, Dallas, TX 75390-9057, USA. Jess.Whitson@UTSouthwestern.edu
Liang
Chanping
C
Godfrey
David G
DG
Petroll
W Matthew
WM
Cavanagh
H Dwight
HD
Patel
Dharmendra
D
Fellman
Ronald L
RL
Starita
Richard J
RJ
D000293:Adolescent; D000328:Adult; D015783:Aniridia / Q000150:complications* / Q000235:genetics; D000886:Anthropometry; D001837:Body Weights and Measures; D016022:Case-Control Studies; D002452:Cell Count; D003315:Cornea / Q000000981:diagnostic imaging / Q000473:pathology*; D004728:Endothelium, Corneal / Q000473:pathology; D005136:Eye Proteins / Q000235:genetics; D005260:Female; D018398:Homeodomain Proteins / Q000235:genetics; D006801:Humans; D008297:Male; D018613:Microscopy, Confocal; D008875:Middle Aged; D009154:Mutation; D000071841:PAX6 Transcription Factor; D051761:Paired Box Transcription Factors / Q000235:genetics; D011446:Prospective Studies; D012097:Repressor Proteins / Q000235:genetics; D014463:Ultrasonography
D005136:Eye Proteins; D018398:Homeodomain Proteins; D000071841:PAX6 Transcription Factor; C000605730:PAX6 protein, human; D051761:Paired Box Transcription Factors; D012097:Repressor Proteins