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Query: UMLS:C0027960 (mole)
 21,279 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Eleven cases of unilateral glaucoma are summarized. The typical presentations of these cases are (1) unilateral; (2) middle-aged women; (3) blurred vision; (4) corneal endothelial guttae (unilateral); (5) corneal edema; (6) stromal iris atrophy; (7) pupil abnormality; (8) peripheral anterior synechias;(9) elevated intraocular pressure; and (10) if previously diagnosed, the diagnosis is likely incorrect. Variations in these may occur. The disease can occur in men. Some patients may have nodular iris nevi. All of them represent the spectrum of Chandler's syndrome. Accurate diagnosis is important particularly in view of the optimistic prognostic implications for the fellow eye.
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PMID:The spectrum of Chandler's syndrome: an often overlooked cause of unilateral glaucoma. 30 13

Progressive essential iris atrophy, Chandler's syndrome, and the iris nevus (Cogan-Reese) syndrome are considered to be variations of a single disease process, which is characterized by abnormalities of the cornea, anterior chamber angle, and iris. In each variation, the typical patient is a white woman with unilateral disease, negative family history, and an onset of symptoms in early to middle adulthood. Since the membrane theory of Campbell suggests that the disease is a fundamental abnormality of the corneal endothelium, rather than the iris, the term "iridocorneal endothelial syndrome," as proposed by Yanoff, may be an appropriate inclusive term for the spectrum of disease, although further study of the pathogenesis is needed. For each variation of the disease, corneal edema and secondary glucoma are both treated primarily by medical or surgical reduction of the intraocular pressure, although penetrating keratoplasty is occasionally required for cases with advanced corneal edema.
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PMID:Progressive essential iris atrophy, Chandler's syndrome, and the iris nevus (Cogan-Reese) syndrome: a spectrum of disease. 48 59

Sixteen cases of essential iris atrophy and two of iris nevus (Cogan-Reese) syndrome were reviewed retrospectively by light and electron microscopy. Varying degrees of corneal endothelial proliferation onto the trabecular meshwork and anterior iris were seen in all cases. The demonstration of an endothelial-abnormal basement membrane complex on the anterior iridic surface, deep to synechias in over half the cases, indicates that endothelialization may be primary, since it precedes the formation of anterior synechias. Pathologic and clinical evidence suggests that the iris nevus syndrome, Chandler's syndrome, and essential iris atrophy represent a continuum of clinical manifestations of a single disease process involving proliferation of the corneal endothelial and characteristic iris abnormalities; the latter may be secondary to variations in the pattern, rate, and extent of endothelial proliferation. We suggest the term "proliferative endotheliopathy" to emphasize the common pathogenetic role of corneal endothelial proliferation in all three entities (iris nevus syndrome, Chandler's syndrome, and essential iris atrophy). A more succinct alternate term is "the iridocorneal endothelial (ICE) syndrome."
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PMID:Proliferative endotheliopathy with iris abnormalities. The iridocorneal endothelial syndrome. 50 77

The clinical and irisangiographical findings of 2 patients with essential progressive iris atrophy of the juvenile are reported. Histopathological examination was possible in one case. The differential diagnosis including to iridoschisis, Chandler's syndrome, Cogan-Reese (iris-naevus) syndrome is discussed. The aetiology of this rare disease remains obscure. Most authors believe that the iris stroma changes and pigment epithelial atrophy result from a vascular deficiency. Iris angiographical findings confirm this belief. The prognosis is poor, due to the therapy resistant secondary glaucoma. Surgery of the ciliary body appears to be the only possible procedure.
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PMID:[A contribution on the clinical pictures, fluorescence angiography and histology oe essential progressive iris atrophy (author's transl)]. 88 1

This paper reports on 9 cases of iris nevus syndrome diagnosed clinically, eight of which were confirmed by microscopy. The clinicopathologic features, etiology and pathogenesis of iris nevus syndrome are discussed. It was verified that iris nevus syndrome and other clinical variations of iridocorneal endothelial (ICE) syndrome had the same characteristic corneal endothelial defect. Also this syndrome was considered to be related to Chandler's syndrome Clinically.
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PMID:Iris nevus syndrome (report of 9 cases). 184 28

A case of iris nevus (Cogan-Reese) syndrome is presented. Our patient's condition was recognized clinically and followed for many years. The unusual amount of proliferated endothelium and abnormal basement membrane and the pedunculated pigment nodules and ectropion uvea were most interesting. If there is a spectrum of disease that includes essential iris atrophy, Chandler's syndrome, and iris nevus syndrome, one would have to say this case fits at the iris nevus end.
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PMID:Iris nevus syndrome. 319 Jan 9

Three iridocorneal endothelial syndromes are distinguished: essential progressive iris atrophy, iris nevus (Cogan-Reese) syndrome, and Chandler's syndrome. Obviously, there are patients in whom findings do not correspond fully to any one of the three syndromes; signs and symptoms which justify being considered as a new iridocorneal endothelial syndrome are described.
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PMID:[A new iridocorneal endothelial syndrome]. 362 10

Progressive iris atrophy, Chandler's syndrome and the iris naevus syndrome are recognised as subgroups of the irido-corneal endothelial syndrome but in some cases a confusing overlap of the clinical signs is found. The clinical features of five cases selected from 19 patients with this syndrome are described. The findings suggest that the disease can be better subdivided into two groups based on the morphology of the posterior corneal surface as seen with the slit lamp.
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PMID:Clinical features of the irido-corneal endothelial syndrome. 386 18

Irido-corneal endothelial syndrome regroups three disorders including: endotheliopathy, peripheral anterior synechiae and iris changes. According to the aspect of these abnormalities, three syndromes are described: essential iris atrophy, Chandler's syndrome and iris naevus (Cogan-Reese) syndrome. Endothelial changes studied by specular microscopy are typical: in early stages, a rounding off cell angles and intracellular blackout areas can be seen; in former stages, black out areas increase and there is a disruption of the regular mosaic. These features are a major point for differential diagnosis. We present six cases reports among which three where effectively diagnosed as iridocorneal endothelial syndrome and three were not.
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PMID:[Specular microscopy in the diagnosis of the iridocorneal endothelial syndrome]. 387 10

The clinical, histological and ultrastructural findings in a case of Cogan-Reese syndrome (iris nevus) are presented. The typical iris nodules first appeared 14 years after a unilateral, juvenile, open-angle glaucoma was diagnosed. No lasting regulation of the intermittently elevated pressure was possible either medically or surgically. The failure of two filtering operations was due to the histologically verified endothelialization of the blebs. Endothelialization and deposition of Descemet's membrane on the anterior surface of the lens and on the zonule is described for the first time in this syndrome. The histological findings support the view that the peripheral anterior synechiae and iris nodules are secondary to the endothelialization. Hence the diagnosis of Cogan-Reese syndrome cannot be made until relatively late in the course of this disease. Cytological similarities between the proliferating endothelium in this case and in Chandler syndrome support the recent hypothesis that the Cogan-Reese syndrome belongs to the irido-corneal-endothelial group of syndromes.
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PMID:[Cogan-Reese syndrome]. 713 78


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