Srijana Adhikari,¹ Pravin Nepal,² Badri Prasad Badhu,¹ Sudesh Kumar Arya^1
1Department of Ophthalmology, and ²Department of Orthopedics, BP Koirala Institute of Health Sciences, Ghopa Dharan, Nepal

Weill-Marchesani syndrome is a rare systemic connective tissue disorder with features of short stature, short stubby hands and feet, and stiff joints, and may be associated with heart defects and mental retardation. The main ocular features are microspherophakia, ectopia lentis, severe myopia, and angle closure glaucoma. This report is of an 18-year-old woman who presented with short stature, short stubby fingers and toes, coarse skin, and pansystolic murmur. Ocular examination revealed microspherophakia with high myopia and open angle glaucoma in both eyes. Trabeculectomy with 5-fluorouracil was performed for both eyes. The intraocular pressure normalised in both eyes after 3 months. The association of open angle glaucoma with Weill-Marchesani syndrome has not been mentioned previously in the literature.

Key words: Glaucoma, open-angle, Microspherophakia, Weill-Marchesani syndrome

Asian J Ophthalmol. 2008;10:230-232.