1/12. Clinical and histopathologic findings of iris nevus (Cogan-Reese) syndrome.PURPOSE: To report a case of Cogan-Reese syndrome. METHOD: Case report. A 37-year-old man presented with Cogan-Reese syndrome. RESULTS: visual acuity was 0.5 in the right eye and 1.0 in the left eye. There were corneal edema and pigmented nodules on the anterior surface of the iris, iris atrophy and ectropion uvea in the right eye. The intraocular pressure was 42 mmHg in the right eye and there was glaucomatous optic atrophy of the optic disk. trabeculectomy with mitomycin C has been performed as the intraocular pressure did not decrease with the maximum medical treatment. Electron microscopic examination of the trabeculum and the iris tissue revealed a lot of melanocytic cells in the stroma. CONCLUSION: trabeculectomy with mitomycin C might be effective in Cogan-Reese cases with glaucoma resistant to medical treatment.- - - - - - - - - - ranking = 1keywords = ectropion (Clic here for more details about this article) |
2/12. Late-onset unilateral primary developmental glaucoma associated with iridotrabecular dysgenesis, congenital ectropion uveae and thickened corneal nerves: a new neural crest syndrome?The association of unilateral primary developmental glaucoma with iridotrabecular dysgenesis and congenital ectropion uveae has been well documented in the literature. The glaucoma in this entity may present at birth, infancy or may develop at a later stage in life. I report the case of a child with late-onset unilateral primary developmental glaucoma due to iridotrabecular dysgenesis, congenital ectropion uveae, and who had a previously undescribed association with ipsilateral thickened corneal nerves in the stroma.- - - - - - - - - - ranking = 6keywords = ectropion (Clic here for more details about this article) |
3/12. Choroidal neurofibromatosis with congenital iris ectropion and buphthalmos: relationship and significance.We report a case of unilateral buphthalmos in neurofibromatosis-1 without the other classical characteristics of the Francois syndrome (triad of unilateral buphthalmos, homolateral eyelid plexiform neuroma, and homolateral facial hemihypertrophy) and emphasize the difficulties in early diagnosis. The painful buphthalmic right eye was enucleated at the age of 13 months. Histopathology demonstrated diffuse choroidal neurofibromatosis in association with congenital iris ectropion syndrome. Cutaneous manifestations of neurofibromatosis subsequently developed in the patient and stigmata of the disease were later identified in other asymptomatic family members.- - - - - - - - - - ranking = 5keywords = ectropion (Clic here for more details about this article) |
4/12. Coexistence of prader-willi syndrome, congenital ectropion uveae with glaucoma, and factor xi deficiency.A patient with prader-willi syndrome and unilateral congenital ectropion uveae with glaucoma was found to have factor xi deficiency and reduced levels of serum luteinizing hormone, follicle-stimulating hormone, and testosterone. Administration of gonadorelin (LH-RH) increased serum levels of luteinizing hormone and follicle-stimulating hormone, while clomiphene citrate had no effect, suggesting a primary hypothalamic defect. patients with congenital ectropion uveae should be followed up for the development of both glaucoma and neural crest disorders.- - - - - - - - - - ranking = 6keywords = ectropion (Clic here for more details about this article) |
5/12. Infantile glaucoma in unilateral uveal ectropion.Two case reports are presented where a unilateral uveal ectropion was associated with congenital and late infantile glaucoma. Ipsilateral to the anterior segment anomaly a dysgenetic angle was found to be the basis of the glaucomatous process. The clinical implication that when such an iris malformation is found in a young child the possibility of glaucoma must be considered is discussed in detail.- - - - - - - - - - ranking = 5keywords = ectropion (Clic here for more details about this article) |
6/12. aniridia, congenital glaucoma, and hydrocephalus in a male infant with ring chromosome 6.A premature infant with unilateral aniridia and congenital ectropion uveae, contralateral Rieger anomaly, bilateral congenital glaucoma, and hydrocephalus was found to have ring chromosome 6. The findings are consistent with multiple manifestations of a neural crest-derived maldevelopment of the anterior segment and central nervous system. Comparison with the 14 previously reported cases of ring chromosome 6 illustrates the phenotypic variability of this syndrome.- - - - - - - - - - ranking = 1keywords = ectropion (Clic here for more details about this article) |
7/12. Congenital ectropion uveae and glaucoma.Congenital ectropion uveae is a rare condition which may be present in one or both eyes. If the patient is followed glaucoma will always be found to be present. Associated features which have been described are ptosis, Rieger's anomaly, Prader Willi syndrome, facial hemiatrophy and neurofibromatosis. This paper describes a patient followed for 18 years who had bilateral congenital ectropion uveae, bilateral ptosis, asthma and late onset of a dental defect.- - - - - - - - - - ranking = 6keywords = ectropion (Clic here for more details about this article) |
8/12. Corneal endothelial overgrowth of angle and iris. Evidence of myoblastic differentiation in three cases.Corneal endothelialization of the anterior chamber angle was observed in three cases of iris neovascularization and one case of post-contusion deformity. Scanning electron microscopy in two cases revealed a sheet composed of confluent fusiform cells forming a regular mosaic, growing in a tissue culture-like fashion, and extending over the anterior iris surface. Electron microscopy revealed an endothelial lining continuous with the corneal endothelium extending over the fibrovascular membrane which covered the anterior iris surface in the cases of iris neovascularization and partially extended over the trabecular meshwork in an eye with post-contusion deformity. These endothelial cells possess junctional complexes, apical villi, and prominent basement membrane. In addition, myoblastic differentiation with intracytoplasmic microfilaments, measuring 5 to 6 nm in diameter and often marginally located with fusiform densities, were observed. The presence of myoblastic features may play a role in the ability of corneal endothelial cells to migrate and participate in production of peripheral anterior synechia and ectropion of the iris.- - - - - - - - - - ranking = 1keywords = ectropion (Clic here for more details about this article) |
9/12. Congenital ectropion uveae with glaucoma.Congenital ectropion uveae (CEU) is a rare, non progressive anomaly characterised by the presence of iris pigment epithelium on the anterior surface, an anterior iris insertion, dysgenesis of the drainage angle and glaucoma. The condition is characteristically unilateral and rarely hereditary. The current theory of development arrest is discussed. We report three cases with CEU of which two already developed glaucoma. All patients with CEU should be carefully examined periodically to detect glaucoma.- - - - - - - - - - ranking = 5keywords = ectropion (Clic here for more details about this article) |
10/12. Cornea and iris changes in congenital ectropion uvea after trabeculotomy.This article details a previously undescribed complication of trabeculotomy occurring in a patient with congenital ectropion uvea and discusses its possible association with an anterior chamber membrane. A 6-year-old boy with congenital ectropion uvea underwent trabeculotomy. After the trabeculotome was swept into the anterior chamber, multiple ovoid and round endothelial and posterior stromal opacities developed in the cornea, and new areas of iris surface irregularities appeared. This is the first reported case of corneal endothelial and iris changes occurring in a patient with congenital ectropion uvea after trabeculotomy without direct endothelial or iris contact from the trabeculotome. The exact cause of these changes is unknown.- - - - - - - - - - ranking = 7keywords = ectropion (Clic here for more details about this article) |
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