Dr.Nida Raza, N21009
A 25 year old female, presented with pain, redness and visual disturbance in the right eye. Visual acuity was finger counting close to face (FCCTF) improving to 6/36. Slit lamp examination revealed dilated fixed pupil with microspherophakic lens dislocated in the anterior chamber. Anterior chamber was shallow, intraocular pressure was raised. She was high myopic. Systemic examination showed brachydactyly, brachymorphia, patient was diagnosed as Weill-Marchesani Syndrome. Systemic workup revealed valvular pulmonary stenosis on 2-D echocardiography.
Lens extraction of the dislocated lens was planned to prevent corneal endothelial damage and to lower intraocular pressure.
Weill-Marchesani Syndrome can present with microspherophakia, ectopia lentis with secondary glaucoma, high myopia, brachydactyly, brachymorphia, valvular heart disease. Removal of microspherophakic lens is recommended to control intraocular pressure and improve the visual outcome.
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