Abstract

P 124

Association of unilateral progressive iridoschisis and Chandler syndrome with glaucoma

Heidi Maria Riederle, Eugen Gramer
Universitäts-Augenklinik Würzburg

Objective
Iridocorneal endothelial syndrome are progressive iris atrophy, Candler syndrome or Cogan-Reese syndrome. These are unilateral, not congenital, and manifest by middle adulthood. Another rare, but bilateral decribed disease is progressive iridoschisis (IS). An unilateral association of both diseases was not described to date. Both are complicated by glaucoma in 50%.
Methods
Purpose/Methodology: 1. Presentation of a patient with unilateral IS associated with Chandler syndrome. 2. Retrospective evaluation of frequency of unilateral IS in 13 consecutively examined and photo-documented patients with IS.
Results
1. Case report: A 46-year-old patient, presenting healthy eyes seven years ago, had an intraocular pressure (IOP) in the right eye of 60mmHg and classic IS with splitting of the anterior leaf of the iris disintegrated into fibrils, goniosynechiae, anterior synechiae, a discrete nasal iris atrophy without pupillary distortion and without glaucoma signs of the optic disc. The unilateral specular microscopy of the corneal endothelial cells with pleomorphism in size and shape and loss of the hexagonal margins resulted in a diagnosis of Chandler syndrome. Left eye: normal. Previous perceptions of coloured halos with the right eye in patients history. Topical IOP lowering therapy and YAG-iridotomy on the right eye resulted in a temporary reduction of IOP. Three months later, a progression of the IS emerged on the entire circumference of the iris with intraocular pressure of up to 40 mmHg. Only a therapy using miotics achieved a regulation of intraocular pressure.
2. The retrospective evaluation of the records of 13 patients with IS showed bilateral IS in 6 and unilateral IS in 7 patients, respectively.
Conclusions
Absence of hole formation of the iris, and absence of pupillary distortion leads to the diagnosis Chandler syndrome and not to the diagnosis progressive iris atrophy. With regard to the pathogenesis of the atypical unilateral, progressive IS, we suggest, that due to high IOP an iris ischemia resulted in a classic, but unilateral IS. Thus, there is an association of the two disorders and no comorbidity. Our retrospective IS-evaluation shows, that IS can frequently occur unilaterally. Consequently bilaterality is not mandatory for diagnosis of IS.

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