Biometric and corneal topographic characteristics in patients with Weill-Marchesani syndrome.
OBJECTIVE
To determine the biometry of ocular structures and corneal topographic characteristics in patients with Weill-Marchesani syndrome.


METHODS
Department of Ophthalmology, Shiraz University of Medical Sciences, Shiraz, Iran.


METHODS
Patients with Weill-Marchesani syndrome (syndrome group) and age-matched normal subjects (control group) were evaluated. All had a complete ocular examination including refraction and analysis using Fourier transformation, slitlamp biomicroscopy, pachymetry, keratometry, and ocular biometry. The following A-scan parameters were measured: anterior chamber depth (ACD), lens thickness, axial length (AL), lens-AL factor, and relative lens position (RLP). Corneal topography examinations were performed in the syndrome group only.


RESULTS
The syndrome group comprised 7 female patients and the control group, 20 female subjects. The syndrome group had statistically significantly higher myopia and corneal astigmatism than the control group (P<.0001). Oblique astigmatism was common in the syndrome group, showing a strong right-left specificity (right eyes' axes in the 135-degree meridian, left eyes' axes in the 45-degree meridian). The syndrome group had a statistically significantly shorter AL, shallower ACD, thicker lens, higher calculated lens power, and a higher lens-AL factor (P<.0001). However, there was no statistically significant difference in RLP (P = .32). The syndrome group also had statistically significantly thicker corneas and higher keratometry values (P<.0001).


CONCLUSIONS
Characteristic ocular findings in patients with Weill-Marchesani syndrome were a steep, thick cornea with a strong right-left specificity of corneal astigmatism; a thick lens; a short AL; and a shallow ACD.
Razeghinejad
Mohammad Reza
MR
Department of Ophthalmology, Khalili Hospital, Shiraz University of Medical Sciences, Shiraz, Iran. razeghinejad@gmail.com
Hosseini
Hamid
H
Namazi
Niloofar
N
D000015:Abnormalities, Multiple; D000328:Adult; D000869:Anterior Eye Segment / Q000000981:diagnostic imaging / Q000473:pathology*; D001699:Biometry; D003240:Connective Tissue Diseases / Q000175:diagnosis* / Q000235:genetics; D003316:Corneal Diseases / Q000175:diagnosis*; D019781:Corneal Topography; D005124:Eye Abnormalities / Q000175:diagnosis*; D005260:Female; D006801:Humans; D007429:Intraocular Pressure; D011446:Prospective Studies; D012029:Refraction, Ocular; D012030:Refractive Errors / Q000175:diagnosis*; D013577:Syndrome; D014463:Ultrasonography; D055815:Young Adult