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First Author: I.Almeida PORTUGAL
Co Author(s): I. Lopes-Cardoso F. Esteves M. Amorim J. Salgado-Borges
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To describe a case of an iridocorneal endothelial (ICE) syndrome.
Iridocorneal endothelial (ICE) syndrome is a progressive, usually unilateral, and non-familial disorder with female predominance. Its clinical spectrum includes essencial iris atrophy, Chandler syndrome and Cogan-Reese and can progress to corneal failure, iris destruction and glaucoma. It is the result of a transformation of corneal endothelial cells leading to characteristic changes in their morphology and biological behavior, (the ICE cells), which are pathognomonic and demonstrated in corneal specular microscopy.
Clinical case report
A 53-year-old female complained of decreased visual acuity (VA) in the right eye (RE), pain and photophobia. She described similar episodes in the past 10 years and she was medicated with timoptol 0.5% twice daily for the RE. Best corrected visual acuity (BCVA) was 4/10 (+2,0+0,50x95Ḟ) RE and 10/10 (+2,50+0,50x95Ḟ) left eye (LE). Slit lamp examination of the RE revealed corneal edema with a silver hammered appearance of the endothelium, slight inferior corectopy , sectorial superior and inferior iris atrophy, with no keratic precipitates and no cellular reaction on the anterior chamber. Gonioscopy revealed a 360 ẃ open iridocorneal angle and a peripheral anterior synechiae at the 6 oṀclock position. LE slit lamp examination and fundoscopy (both eyes) were normal The intraocular pressure was 12 mm Hg on both eyes (with timoptol RE). Ultrasonic corneal thickness was 688 ?m on the RE and 581 ?m on the LE. Corneal specular microscopy showed abnormal endothelial cells with dark/light reversal pattern and central bright reflex on the RE. She was treated with dexamethasone for one month and hypertonic artificial tears. After 4 months she presented a 10/10 BCVA and no corneal edema besides the abnormal endothelium.
The diagnosis ICE syndrome is suggested by the typical biomicroscopic alterations that can be quite impressive. This case represents a subtle case of Chandler Syndrome whose clinical signs are so subtle that it could be misdiagnosed as other disease. Besides other and more common diagnostic possibilities, the hammered endothelial appearance was the key to the correct diagnosis. Abnormal endothelial cells with dark/light reversal found with specular microscopy confirmed the diagnosis of ICE syndrome. This diagnosis should be considered in all cases of corneal edema and ocular hypertension.