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¹Beyoğlu Göz Eğitim ve Araştırma Hastanesi, Göz Hastalıkları, İstanbul, Doç. Dr.
²Beyoğlu Göz Eğitim ve Araştırma Hastanesi, Göz Hastalıkları, İstanbul, Asist. Dr.
³Beyoğlu Göz Eğitim ve Araştırma Hastanesi, Göz Hastalıkları, İstanbul, Prof. Dr. A 20-year-old female presented to the clinic with the complaint of vision loss in her left eye. She was diagnosed with Weill-Marchesani syndrome according to skeletal (short build, broad thorax, brachydactylia and brachymetacarpia, and hypoextendable joints) and ocular findings (bilateral microspherophakia and lens dislocation). In her left eye, there was irreversible vision loss because of chronic angle-closure glaucoma secondary to pupil block. In order to prevent angle-closure glaucoma in the right eye, we performed extraction of the microspheric lens with irrigation-aspiration (I/A), and implantation of a Cionni capsular tension ring (Cionni Ring, Type 2L, double eyelet, MORCHER®, dimension: 11 mm, Cionni CTR) and a three-piece hydrophobic acrylic foldable intraocular lens (AcrySof MA30AC). Thus, pupillary block and secondary angle glaucoma risk was eliminated and high myopic refraction due to microspherophakia was corrected. Angle-closure glaucoma due to microspherophakia is seen in 80% of patients with Weill-Marchesani syndrome. In order to prevent angle-closure glaucoma in these patients, there are some methods such as yttrium-aluminum-garnet (YAG) laser peripheral iridectomy, and the use of miotic or mydriatic ophthalmic drugs. However, in spite of all these measures, angle-closure glaucoma can still be seen in Weill-Marchesani syndrome. Surgical methods are more difficult but more effective than the other mentioned measures. Moreover, it was very important to protect the right eye. To the best of our knowledge, this surgical approach in Weill-Marchesani syndrome is reported for the first time. Keywords : Microspherophakia, pupillary block, cionni ring