Polz S., Clemens S.

Clinic for Ophthalmology of the Ernst- Moritz- Arndt- University, Rubenowstr. 2,17487 Greifswald

Background: The Weill-Marchesani-Syndrome is a rare disorder with an autosomal recessive trait. A defect in the insertion of zonula fibrilles at the unpigmented ciliary epithelium of the pars plana causes a progressive subluxation of a sphaeric lens. One consequence is the development of a hardly controllable secondary glaucoma.
Case report: A 63 year old patient introduced himself to the clinic with a limited retinal detachment in the left eye. A total luxation of the lens into the vitreous appeared fundoscopic. A mydriasis could achieved neglibly. The tension was about 13 mm Hg by the local treatment with dorzolamid, timolol and clonidin. The patients body showed the special small stature of the Weill-Marchesani-Syndrome including short ton upper torso and stubby fingers. In the history retinal detachment and recurrent iridocyclitis were peculiar on both eyes. The retina of the right eye had been reapplied by Cerclage and plomb three times. In 1966 a retinal detachment without foramen was completely reattached under strict bed rest and stenopeic spectacles. The bilateral secondary glaucoma (pressure peaks of 60 mm Hg) was treated with a maximum local and systemic therapy as well as laser iridotomy. We performed a pars plana vitrectomy in the left eye. The luxated lens was removed from the vitreous compartment and the retinal detachment was reapplied with a gasair- mixture. In order to regulate the secondary glaucoma a deep sclerectomy was performed. In the end the visus amounted to 0,4. The intraocular pressure was under control.
Conclusion: Patients with a peculiar brachymorphy and lens luxation point out a Weill-Marchesani-Syndrome. Secondary complications like iridocyclitis, secondary glaucoma and retinal detachment should be expected in the further course.

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