Kor J Ophthalmol Vol.21, No.4, 2007 because the immersion probe may compress the cornea, and the dislocated spherophakic lens may be posteriorly displaced in supine position. A rotating Scheimpflug camera can be measured without corneal contact, with the patient in the sitting position, and requires less time overall. Interestingly, by comparing two imaging devices, we found that the rotating Scheimpflug camera showed bilateral corneoleticular contact in sitting position (Fig. 4), but in ultrasound biomicroscopy, the left eye showed corneolenticular contact, and the right eye showed only iridocorneal contact without corneolenticular touch, by the posterior shifting mechanism of the lens in supine position (Fig. 5). This result means that continuous corneolenticular contact was present in her left eye, while only intermittent contact occurred in her right eye. In her left eye, the corneal endothelial count was less(743/675 cells/mm2) and the central corneal thickness was thicker (672/712 μm) than those of her right eye. For that reason we performed the operation on the left eye first. Consequently, utilizing these modern imaging devices can be helpful in accurately diagnosing the morphologic status of WMS, which cannot be identified on slit lamp examination only and also in making a decision regarding the optimal surgical intervention method and timing. In conclusion, WMS with chromosomal abnormality may cause severe corneal endothelial dysfunction due to a dislocated spherophakic lens. Prompt lensectomy is thus important in preventing such complications. References 1. Weill G. Ectopie du cristallin et malformations générales. Ann Ocul 1932;169:21-44. 2. Marchesani O. Brachydaktylie und angeborene kugellines als systemerkrankung. Klin Mibl Augenheilkd 1939;103: 392-406. 3. Faivre L, Dollfus H, Lyonnet S, et al. Clinical homogeneity and genetic heterogeneity in Weill-Marchesani syndrome. Am J Med Genet A 2003;123:204-7. 4. Dagoneau N, Benoist-Lasselin C, Huber C, et al. ADAMTS10 mutations in autosomal recessive Weill- Marchesani syndrome. Am J Hum Genet 2004;75:801-6. 260 5. Wirtz MK, Samples JR, Kramer PL, et al. Weill- Marchesani syndrome--possible linkage of the autosomal dominant form to 15q21.1. Am J Med Genet 1996;65:68-75. 6. Dietlein TS, Mietz H, Jacobi PC, Krieglstein GK. Spherophakia, nanophthalmia, hypoplastic ciliary body and glaucoma in brachydactyly-associated syndromes. Graefes Arch Clin Exp Ophthalmol 1996;234 Suppl 1:S187-92. 7. Lee WR, Lee HB. A Case of Spherophakia. J Korean Ophthalmol Soc 1982;23:761-5. 8. Park SC, Lee KH. Two Cases of Spherophakia in Sister and Brother. J Korean Ophthalmol Soc 1986;27:429-35. 9. Kloepfer HW, Rosenthal JW. Possible genetic carriers in the spherophakia-brachymorphia syndrome. Am J Hum Genet 1955;7:398-425. 10. Faivre L, Gorlin RJ, Wirtz MK, et al. In frame fibrillin-1 gene deletion in autosomal dominant Weill-Marchesani syndrome. J Med Genet 2003;40:34-6. 11. Apte SS. A disintegrin-like and metalloprotease (reprolysin type) with thrombospondin type 1 motifs: the ADAMTS family. Int J Biochem Cell Biol 2004;36:981-5. 12. Porter S, Clark IM, Kevorkian L, Edwards DR. The ADAMTS metalloproteinases. Biochem J 2005;386:15-27. 13. Nicholson AC, Malik SB, Logsdon JM, Van Meir EG. Functional evolution of ADAMTS genes: evidence from analyses of phylogeny and gene organization. BMC Evol Biol 2005;5:11. 14. Hayward C, Brock DJ. Fibrillin-1 mutations in Marfan syndrome and other type-1 fibrillinopathies. Hum Mutat 1997;10:415-23. 15. Sakai LY, Keene DR, Engvall E. Fibrillin, a new 350-kD glycoprotein, is a component of extracellular microfibrils. J Cell Biol 1986;103:2499-509. 16. Willi M, Kut L, Cotlier E. Pupillary-block glaucoma in the Marchesani syndrome. Arch Ophthal 1973;90:504-8. 17. Ritch R, Wand M. Treatment of the Weill-Marchesani syndrome. Ann Ophthalmol 1981;13:665-7. 18. Macken PL, Pavlin CJ, Tuli R, Trope GE. Ultrasound biomicroscopic features of spherophakia. Aust N Z J Ophthalmol 1995;23:217-20. 19. Urbanek J. Glaucoma juvenile inversum. Z Augenheilkd 1930;71:171-2. 20. Pavlin CJ, Buys YM, Pathmanathan T. Imaging zonular abnormalities using ultrasound biomicroscopy. Arch Ophthal 1998;116:854-7.