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In most cases the iridocorneal endothelial (ICE) syndrome affects women unilaterally and shows endothelial degeneration, thickening of Descemet's membrane, iris atrophy, and glaucoma. The spectrum of the ICE syndrome includes the Chandler's syndrome, the essential iris atrophy, and the Cogan-Reese syndrome. We present a unique case of an ICE syndrome, which we would subclassify as Chandler's syndrome, with concomitant fingerprint dystrophy and multiple interepithelial bilaminar bodies within the fingerprint-like striae.
Ten years earlier a now 45 year old patient presented with a bilateral ICE syndrome and fingerprint dystrophy. On slit lamp microscopy the epithelial layer showed fingerprint lines, while the endothelial layer had a “hammered” appearance. Iris atrophy, corectopia, ectropium uveae and anterior synechiae were obvious (Fig 1A) and progressive in both eyes, nevertheless the intraocular pressure and the visual acuity were normal. Eight years later both corneas developed oedema (Fig 1B) and the left eye displayed cataract with reduction of visual acuity from 1.0 to 0.25 and 0.9 to 0.7 in the left and right eye, respectively. Combined perforating keratoplasty and extracapsular cataract extraction were performed on the left eye in June 1998. None of the patient's family members had a similar condition.
The histological examination of the paraffin embedded corneal specimen revealed fingerprint-like striae containing small basophilic deposits between the cells (Fig 2A). The van Kossa stain for calcium was negative and no hydroxyapatite crystals were seen. At the ultrastructural level the deposits were found to be round and bilaminar showing an electron dense core in the centre (Fig 2B). Some of the deposits coalesced to form oval structures. The cytoplasm of the adjacent epithelial cells was normal without deposits in relation to the cell membranes. Descemet's membrane was not thickened, the endothelial cells possessed microvilli, their mitochondria were degenerated, and intercellular interdigitations were pronounced.
We consider our case as one of Chandler's syndrome, which is part of the spectrum of the ICE syndrome, owing to obvious morphological changes of the iris despite non-specific endothelial changes. The striking features of Chandler's syndrome are corneal endothelial dystrophy and subsequent oedema.1 Corectopia, glaucoma, and iris atrophy are less compared to the two other ICE syndromes—that is, the essential iris atrophy and the iris-naevus syndrome, possibly owing to differences in endothelial proliferative activity.2
Our case is unique in several aspects. Firstly, our patient is a man and is affected bilaterally. Most often women are affected unilaterally by ICE syndrome, nevertheless an exception of essential iris atrophy with bilateral changes have been described.3
Secondly, an epithelial dystrophy—namely, a fingerprint dystrophy, is present in addition to the ICE associated changes. To our knowledge this association has not been described in the literature before.
Thirdly, the fingerprint striae contain bilaminar deposits with an electron dense core. Several types of deposits have been described in association with epithelial dystrophies. Patches of “unknown substance” were noticed in fingerprint dystrophy along basal plasmalemmas of the basal epithelial cells with no similarity to the bilaminar structures described here.4 Broderick et al mentioned intercellular dense oblong bodies which were embedded in condensed matrix and were much smaller than the deposits in our case.5 In a cornea with a Meesmann dystrophy intraepithelial “peculiar electron dense substance” were shown, also with a totally different appearance compared to that seen in the fingerprint striae of the specimen we present here. In a patient with ocular tumoral calcinosis, nodules from the palpebral conjunctiva contained calcified “spherules,”6 which show some similarity to our—nevertheless, uncalcified—deposits. Our deposits were located within the fingerprint striae, close to the epithelium. It seems to be obvious that the epithelial cells must have produced those bilaminar bodies, since no other cell type was present nearby, but there was no evidence of extrusion from the basal cells.
Our case shows that ICE syndrome can occur bilaterally in men, and can be associated with a fingerprint-like dystrophy. Further studies should be carried out to confirm our electron microscopical observation of bilaminar deposits within fingerprint-like striae and directed towards an analysis of their chemical composition of the extracellular material and origin.
We thank Mrs Annegret Mattes and Mrs Dorothy A Aitken for providing electron microscopic assistance.
This case was presented as a poster at the meeting of the Association for Research and Vision in Ophthalmology in Fort Lauderdale in May 2001.
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