Biometric and Corneal Topographic Characteristics in Patients with Weill-Marchesani Syndrome

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

Setting: 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).

Conclusion: 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.