Kor J Ophthalmol Vol.21, No.4, 2007 Fig. 1. X-ray of the hand showing brachydactyly, short metacarpal bones, and delayed carpal ossification. brachydactyly and short metacarpal bones on X-ray images of the hand (Fig. 1). Initial Snellen uncorrected visual acuity (UCVA) was 2/200 in both eyes, and best corrected visual acuity (BCVA) was 20/50 (-sph 20.50 -cyl 3.00 Ax 180) in her right eye and 20/40 (-sph 16.00 -cyl 6.00 Ax 30) in her left eye. A-scan biometry revealed that the axial lengths of her eyeballs were 23.65/23.30 mm, suggesting a lenticular origin for her myopia. She had been treated with bilateral laser peripheral iridotomies at a local clinic two years ago. Despite this treatment, her initial intraocular pressure (IOP) was 26/22 mmHg by Goldmann applanation tonometry. Slit lamp examination of both eyes before pupillary dilation showed edematous corneas, extremely shallow anterior chambers, open peripheral iridectomy and iridocorneal/ corneolenticular contact. After pupillary dilation, IOP decreased to 22/19 mmHg, due to ciliary muscle relaxation. Slit lamp examination revealed bilateral superonasal subluxation of the crystalline lens with the lens equator and zonule visible within the pupil (Fig. 2). Central corneal edema was observed because the anteriorly dislocated lens was touching corneal endothelium. Due to corneal endothelial 2 dysfunction, endothelial cell counts (743/675 cells per mm ) and hexagonality (33/0%) were decreased on specular microscopy (Cell ChekⓇ, Konan, Tokyo, Japan) (Fig. 3), and Fig. 2. Slit lamp examination showing bilateral superonasal subluxation of the crystalline lens with the lens equator and zonule visible within the pupil. O S Fig. 3. Specular microscopic examination showing decreased endothelial cell counts (743/675 cells per mm2) and hexagonality (33/0%) due to corneal endothelial dysfunction. 256