Gene/Protein Disease Symptom Drug Enzyme Compound
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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

The technique and results of 30 iridocyclectomies performed by the senior author are reviewed. Although the total number of cases is small for statistical purposes, its main value is that all the operations were performed by one surgeon, which means a uniform criteria for the indications, surgical technique, management of complications, immediate and postoperative decisions, follow-up control, and direct discussion of the histopathology with the pathologist. Three techniques were used: (1) routine excision only of uveal tissue in 15 cases, (2) cyclectomy, ie, plus excision of deep scleral wall in four cases, and (3) block excisions of cornea, sclera, affected iris, and ciliary body followed by a reconstructive penetrating graft in 11 cases. Twenty-one cases were melanocytoma; one, metastatic tumor; one, nevus; two, cysts; and three, foreign body granulomas. Prognostic features such as cell type, largest diameter above 10 mm, tumor thickness, tumor at edge, iris or ciliary body origin, location, and preoperative glaucoma are discussed, and some practical conclusions are advanced. The follow-up control for 21 melanoma cases in the series is five to ten years or more in 11 cases and up to four years in ten cases. The visual results are also analyzed.
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PMID:Iridocyclectomy technique and results. 53 91

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

A 53-year-old white man was noted to have bilateral retinitis pigmentosa associated with a left nevus flammeus, a left choroidal hemangioma and unilateral glaucoma. No central nervous system manifestations of Sturge-Weber syndrome were noted. The association of retinitis pigmentosa and the nevus flammeus is probably a chance occurrence.
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PMID:Retinitis pigmentosa associated with nevus flammeus and unilateral glaucoma. 90 Jul 9

Case reports of oculodermal melanocytosis (nevus of Ota) and ipsilateral glaucoma often have other ocular disorders which could account for the glaucoma. It has not been clearly shown that the oculodermal melanocytosis alone can cause secondary glaucoma. In the present case report, optic nerve head and visual field changes, suggestive of a long-standing unilateral elevation of intraocular pressure, occurred in an eye with oculodermal melanocytosis and no other apparent ocular disorder to account for the chronic glaucoma.
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PMID:Glaucoma in oculodermal melanocytosis. 92 Nov 51

From 1969-1974 1000 unselected enucleated globes have been examined histopathologically. 277 derive from the University Eye Hospital in Hamburg, 723 from various Eye Hospitals in northern and southern Germany. They originate from 589 men and 408 women, three times the sex was unknown. 86 globes had to be removed from children less than 15 years old. 6 groups of etiologies have been distinguished: trauma (308), histologically confirmed neoplastic disease (281), ocular manifestations of systemic diseases (diabetes mellitus, occlusions of central retinal vessels presumably following generalized vascular disease etc.: 128), "operative ocular disease" (164), primary inflammatory disease (71), miscellaneous (malformations, high myopia, pseudo-glioma and pseudo-melanoma: 48). The etiology "operative ocular disease" consists of 67 primary glaucomas (57 adults, 10 buphthalmus), 41 idiopathic cataracts (7 of these congenital) and 3 primary corneal dystrophies, as well as 53 cases of primary retinal detachment. Among the 281 neoplastic diseases, there are 238 primary intraocular malignant melanomas of the uvea, 18 retinoblastomas, 4 primary reticulumcellsarcomas of the retina, 2 choroidal nevi, 10 intraocular metastases and 9 orbital tumors. 16 enucleations among the 1000 enucleations have been performed for pseudo-gliomas (5 x Coats disease, 5 x persistent primary hyperplastic vitreous, 2 x retrolental fibroplasia, others 4 x). The manifestations of systemic disease are consisting of 68 central retinal vein-occlusions, 30 complications of diabetes mellitus and 10 central retinal artery occlusions as well as 20 other generalized diseases. A primary inflammatory disease led to enucleation 50 times due to an intraocular process, 5 times due to scleritis and 18 times as a consequence of keratitis (including 13 times herpes simplex). As the final clinical cause for enucleation the following categories have been elaborated: secondary glaucomas (416), clinical diagnosis of "tumor" (275), atrophy and phthisis bulbi (118), inflammation (112), acute trauma to 4 weeks after the accident (72), others (7). In conclusion the central role of rubeosis iridis leading to secondary angle closure glaucoma is emphasized. This process presents a challenge to ophthalmologic research. Finally the significance of early surgery for primary angle closure glaucomas and for complete restoration of the anterior chamber after trauma and any intraocular procedure is stressed.
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PMID:[Etiology and final clinical cause for 1000 enucleations. (A clinico-pathologic study) (author's transl)]. 95 59

Late failure of a filtering cicatrix occurred in a patient who had had surgery to control the glaucoma associated with the iris nevus syndrome (Cogan-Reese). The internal surface of the filtration site, as well as the anterior chamber angle, proved to be lined by corneal endothelium that had laid down a new, thick basement membrane (Descemet). The new endothelium-basement membrane probably obstructed the flow of aqueous into the bleb. Endothelialization of a filtering bleb is not unique to the iris nevus syndrome; other causes are discussed.
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PMID:Endothelialization of filtering bleb in iris nevus syndrome. 98 72

We examined the eyes of 50 patients with nevus flammeus of the face (port wine stain). Of 26 patients with nevus flammeus involving the area of both the first and second sensory branches of the trigeminal nerve, 4 had glaucoma and 8 were glaucoma suspects. Because the age at which glaucoma develops is uncertain, these patients require periodic ocular assessment. When the nevus flammeus involved the area of V1 alone or V2 alone, there was no associated glaucoma.
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PMID:Ocular findings in nevus flammeus. 112 38

Fourteen patients (ten women, four men) with iris nevus (Cogan-Reese) syndrome, all having unilateral glaucoma, were studied clinically. Many had corneal edema, and all had changes in the iris consisting of one or more of the following: iris whorls or nodules or both, atrophy of iris stroma, heterochromia (the darker iris usually involved), or ectropion uveae. All had peripheral anterior synechias. Material for histological study was available from ten patients and showed a nonmalignant diffuse nevus of the anterior surface of the iris. An overgrowth of endothelium and Descemet membrane extending onto the iris surface may be a characteristic part of the process. Patients tended to be women in middle age. Conservative treatment avoiding enucleation is advised. Whenever a patient with suspected iris nevus syndrome or essential iris atrophy is subjected to glaucoma surgery, a biopsy specimen of the iris should be obtained for histological study.
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PMID:Iris nevus (Cogan-Reese) syndrome. A cause of unilateral glaucoma. 118 Jul 55

In two patients suffering from congenital oculodermal melanocytosis (nevus of Ota) orbital, osseous, meningeal, and cerebral manifestations are documented. In a 38-year-old European with a nevus of Ota on the right side an ipsilateral orbital tumor was diagnosed and surgically removed. The histological examination revealed a primary spindle shaped malignant melanoma of the orbit. In addition, many benign melanocytes were found in the optic nerve, in the extraocular muscles, in the orbital fat tissue, and in the dura of the sphenoid. In a 17-year-old Ethiopian with a nevus of Ota of the right side an increase in size of the pigmented process was observed during puberty in the orbital fat tissue, in the extraocular muscles, in the optic nerve, in the periorbital bones, in the temporal muscle, and in the cortex of the frontal brain lobe. A deep excision from the lower eye lid revealed a histologically benign oculo-orbito-dermal melanocytosis. An irradiation was performed because of growth again after segmental excision, however without success. In the ipsilateral eye a secondary open-angle glaucoma was diagnosed. In the nevus of Ota an involvement of the orbit and of the meningo-cerebral tissue must be considered.
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PMID:[Orbital, osseous, meningeal and cerebral findings in oculodermal melanocytosis (nevus of Ota). Clinico-histopathologic correlation in 2 patients]. 150 91


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