Weill-Marchesani syndrome and secondary glaucoma associated with ectopia lentis.
Weill-Marchesani syndrome (WMS) is a rare systemic connective tissue disorder with the systemic features of short stature, short and stubby hands and feet and stiff joints, especially in the hands. Occasionally, it is associated with heart defects and mental retardation. The main ocular features of WMS are microspherophakia (small and spherical crystalline lens), ectopia lentis (a displaced or malpositioned lens), severe myopia and glaucoma. Rare findings include asymmetric axial lengths associated with presenile vitreous liquefaction. A 14-year-old patient with WMS, who developed a secondary glaucoma and suffered visual loss from the ocular features of WMS, is described. The clinical findings and its successful management are also reported.
School of Optometry and Vision Science, University of New South Wales, Australia. chujang1973@yahoo.com
Byoung Sun
BS
Chu
D000293:Adolescent; D003937:Diagnosis, Differential; D004479:Ectopia Lentis / Q000150:complications* / Q000601:surgery; D005901:Glaucoma / Q000150:complications* / Q000601:surgery; D006130:Growth Disorders / Q000150:complications*; D006801:Humans; D007906:Lens Subluxation / Q000150:complications* / Q000601:surgery; D008297:Male; D013508:Ophthalmologic Surgical Procedures; D013577:Syndrome; D014792:Visual Acuity