Kor J Ophthalmol Vol.21, No.4, 2007 OD OS Fig. 7. Three months postoperatively, the cornea was clear and the anterior chamber had deepened. The visual acuity was 20/30 (OD) and 20/40 (OS) without correction, and best corrected visual acuity was 20/20 (+sph 0.50 -cyl 2.00 Ax 160 : OD) and 20/25 (+sph 1.50 -cyl 3.00 Ax 30 : OS). and Kleinfelter syndrome. From these findings, we diagnosed this patient with a sporadic case of WMS with inversion of chromosome 15, who also showed the corneal endothelial dysfunction due to corneolenticular contact. Since this patient showed impending corneal decompensation, surgical extraction of the crystalline lens was performed in the left eye. After 3.2 mm-sized scleral tunnel incision, phacoemulsification and irrigation/aspiration of the nucleus and cortex were performed in the capsular bag. The wound was enlarged to 7.0 mm for sclera-sutured sulcus fixation of a polymethyl methacrylate intraocular lens (CZ70BD, Alcon, Fort Worth, USA). The intraocular lens was sutured to the sclera 1.0 mm from the limbus with a 10-0 polypropylene suture. Two weeks later, the same procedure was performed in the right eye. One month postoperatively, the visual acuity was 20/40 without correction in both eyes. Three months after surgery, UCVA was 20/30 (OD) and 20/40 (OS), and BCVA was 20/20 (+sph 0.50 -cyl 2.00 Ax 160 : OD), 20/25 (+sph 1.50 -cyl 3.00 Ax 30 : OS). On slit lamp examination, the cornea was clear and the anterior chamber had deepened (Fig. 7). Endothelial cell counts had increased to 1774 / 2882 cells per mm2 on specular microscopy. Six months postoperatively, the same visual acuities and endothelial cell counts were maintained (Table 1). D iscussio n WMS is a rare connective tissue disorder characterized by Table 1. Preoperative and pachymetry short stature, brachydactyly, and spherophakia. Its mechanism is thought to be due to a developmental abnormality of mesodermal origin tissues (ciliary body, lens, and epiphysis of bones). It is speculated that in spherophakia, the fetal lens which is physiologically spherical, had never been subjected to the force of a properly acting ciliary body and zonules.6 Two authors have reported isolated spherophakia cases in Korea.7,8 In those cases, the cornea was clear and there were no other systemic abnormalities. Their main problems were visual disturbances due to lenticular myopia and IOP elevation. Those patients received bilateral extracapsular lens extraction with/without trabeculectomy due to IOP elevation, intraocular lenses were not implanted, and visual improvement was not remarkable postoperatively. In contrast with previous reports, we presented a typical WMS case with genetic abnormalities and characteristic external appearances, including short stature and brachydactyly. Our case showed that the zonules were extremely elongated enough to produce impending corneal decompensation due to corneolenticular contact. In addition, we achieved successful visual rehabilitation and prevented threatening visual conditions by prompt surgical interventions. We also demonstrated a hypoplastic ciliary body, elongated zonules, and a spherophakic crystalline lens with the aid of modern imaging devices such as ultrasound biomicroscopy and rotating Scheimpflug camera. Despite the disease's clinical homogeneity, autosomal recessive (AR) and autosomal dominant (AD) modes of inheritance have been reported.4,5 In a literature review of postoperative changes of visual acuities, specular microscopy, and ultrasonic UCVA BCVA coEunndtsoth(ceelilalsl/ mcemll2) OD OS OD OS OD OS Preoperative 2/200 2/200 20/50 20/40 743 675 Postoperative 3 months 20/40 20/40 20/20 20/25 1774 2882 Postoperative 6 months 20/30 20/40 20/20 20/25 1738 2235 UCVA : uncorrected visual acuity, BCVA : best corrected visual acuity 258 Hexagonality (%) OD 33 25 25 OS 0 0 18 Central corneal thickness (μm) OD OS 672 712 587 610 566 585