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[Problems in juvenile xanthogranuloma of the anterior uvea (author's transl)].

With reference to a case of juvenile xanthogranuloma localized in the anterior uvea the authors describe the clinical signs, differential diagnosis, morphology and treatment of this disease. If the juvenile xanthogranuloma is located in the anterior uvea, the eye may be severely affected. Spontaneous hyphema in infants is characteristic. At present radiation is the most successful treatment for preserving the bulbus and visual function. The earlier the disease is discovered the better the chances of successful treatment.

Diagnosis, Differential↗

[Metastatic carcinomata in the anterior uvea as the presenting clinical manifestation (author's transl)].

Metastatic carcinomata in the anterior uvea are rare. Some of them may be diagnosed before the primary tumor is clinically detected. We report on three such cases. The vast majority of secondaries in the anterior uvea have their primary in the bronchus. It is rather exceptional that a metastasis in the anterior chamber can be traced to an eosophageal carcinoma as in one of our cases.

Aged↗

The contribution of aqueous humor cytology in the differential diagnosis of anterior uvea inflammations.

BACKGROUND: Cytology of the aqueous humor can confirm the diagnosis of many intraocular conditions by revealing inflammatory cells, ghost red cells, lens material, and microorganisms. PURPOSE: The purpose of this study was to emphasize the contribution of aqueous humor fine needle aspiration cytology in cases where there is a clinical diagnostic dilemma whether an infectious or a noninfectious inflammatory process of the uvea is present. METHODS: Collection of 0.15-0.2 ml aqueous humor was carried out with an insulin needle. The aspirated aqueous was then prepared for examination using either the Cytospin or the Thin-Prep technique and smears were stained with modified Papanikolaou and May-Grünwald-Giemsa stains. RESULTS: Cytology of the aqueous humor essentially facilitated the differential diagnosis in 16 cases with an anterior uveal inflammation clinical picture. The cases included phacoanaphylactic endophthalmitis (n = 6), chronic postoperative bacterial endophthalmitis (n = 3), phacolytic glaucoma (n = 2), ghost cell glaucoma (n = 2), metastatic endophthalmitis (n = 1), post-traumatic lenticular abscess (n = 1), and iridocyclitis (n = 1). Treatment of the cases was successful. CONCLUSIONS: In selected cases, cytology of the aqueous humor reveals specific, with regard to the clinical entity, cell populations and other elements and thus contributes effectively to the differential diagnosis and therapeutic approach to some anterior uvea inflammations.

Adult↗

Primary pulmonary adenocarcinoma metastatic to the uvea, brain and adrenal gland in a dog.

A 10-year-old male Briard dog was presented because of listlessness, abnormal gait, fever, inappetence, and seizures. A non-pigmented growth was observed in the ventral quadrant of the left iris. Thoracic radiographs revealed multiple pulmonary metastases and the owner opted for killing. On necropsy, lung masses and nodules in left iris, right adrenal medulla, and brain were detected. Histologically the primary tumour was diagnosed as pulmonary adenocarcinoma with predominant solid pattern. Metastases to regional lymph nodes, uvea, adrenal medulla, and brain were recognized. The metastatic behaviour resembled that occurring in humans. To the authors' knowledge, this is the first report of a pulmonary adenocarcinoma with metastasis to the uvea in a dog.

Adenocarcinoma↗

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.

Adolescent↗

Presence of the R1748X mutation in the NF1 gene in a Brazilian patient with ectropion uveae.

Congenital ectropion uveae is a rare, nonprogressive anomaly characterized by the presence of iris pigment epithelium on the anterior surface of the iris stroma and is occasionally associated with Rieger's anomaly, Prader-Willi syndrome and neurofibromatosis type 1 (NF1). The most important complication of ectropion uveae is congenital or juvenile glaucoma. We described a patient with ectropion and the mutation R1748X in the NF1 gene. This is the third report in the literature describing ectropion associated with neurofibromatosis. If this association is confirmed by other authors, the NF1 patients should be examined for the presence of ectropion and, consequently, for the development of glaucoma.

DNA Mutational Analysis↗

Retinal pigment epithelial tear in reactive lymphoid hyperplasia of uvea.

A 76-year-old patient developed cystic exudative retinal detachment with many yellowish subretinal precipitates and a large retinal pigment epithelial (RPE) tear in midperiphery of the right eye in the course of polyclonal hypergammaglobulinemia. The left eye showed localized RPE detachment in the posterior pole which gradually extended. Systemic steroid administration reduced the subretinal precipitates to some extent, but suspicion of malignancy led to enucleation of the right eye after unsuccessful diagnostic vitrectomy. A histopathological study revealed massive infiltration of the uvea with plasma cells and small lymphocytes but no abnormal cells. This is the first report to show the association of an RPE tear with reactive lymphoid hyperplasia of the uvea.

Aged↗

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.

Abnormalities, Multiple↗

Current alternatives in the management of posterior uvea melanomas.

The management of malignant melanomas of the posterior uvea recently has become a topic of great controversy. The traditional treatment by enucleation of the tumor-containing eye has recently been challenged by a number of authorities, and clinicians more frequently are using alternative methods of management when possible. Current management can range from periodic observation and fundus photography of selected small lesions that appear dormant, to photocoagulation, radiotherapy, or local resection in the case of growing tumors in eyes with useful or salvageable vision. In cases where the tumor is far advanced and there is no hope of useful vision, enucleation is often inevitable. The choice of therapy is a complex issue and each case must be individualized. In selecting a therapeutic approach certain factors must be carefully weighed. These include the size of the melanoma, its extent and location, its apparent activity, the condition of the opposite eye and the age, general health, and psychological status of the patient. Based on personal experience with approximately 3000 cases, the authors discuss their current indications for the various therapeutic techniques for posterior uvea melanomas.

Brachytherapy↗

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.

Adolescent↗

[Fluorescent angiography in different diagnosis of intra-ocular tumours/melanomas, metastatic tumours and haemangiomas of the uvea (author's transl)].

The angiographic pictures of 155 certified malignant melanomas, 15 metastatic intraocular tumours and 16 haemangiomas of the uvea are discussed. Eight angiographic signs are differentiated which can be observed in different combinations by different types of intraocular tumours. Correlation between the number of the angiographic signs by which the tumour is presented and its prominence, calculated by means of ultrasound biometry is proved. A malignant melanoma with high prominence is demonstrated by 3 signs, while for a certain diagnosis of tumours with low prominence, at least 6 angiographic signs should be observed. by means of methods of statistics, such as correlation and variation diagnostic differences in the angiographic pictures of the discussed 3 types of tumours are found, having compared tumours with equal prominence. It turns out that malignant melanomas with low prominence present a marked fluorescence (6 signs at least), while the haemangiomas of the uvea, with the same prominence are presented with less fluorescentic signs (maximum 4), and the metastatic tumours with even less (2-3 signs).

Adult↗

Sensory supply of the anterior uvea: a light and electron microscope study.

We have studied the ultrastructural features of sensory nerve fibers in the ciliary body and the iris and their parent trigeminal ganglion cells, using intra-axonally transported horseradish peroxidase as a tracer. Unmyelinated nerve fibers of ipsilateral trigeminal origin were found in stroma of both the ciliary body and the iris. Most appeared in bundles of nerve fibers; but some were found singly. Two distinct types of varicosities were found, one containing both a few irregularly shaped vesicles and mitochondria, the other containing mitochondria only. The thin profiles of the axons between these varicosities or endings contained neurotubules and filaments. The trigeminal ganglion cells supplying the anterior eye were of a relatively small size (15-50 micron in diameter) and confined to the anteromedial part of the ipsilateral ganglion. These cells could not be classified according to the arrangement of their subcellular organelles. The morphological characteristics of the sensory nerve fibers and ganglion cells supplying the anterior uvea are consistent with the view that pain may be the predominant sensation mediated by these neurons.

Animals↗

Structural analysis of potential barriers to bulk-flow exchanges between uvea and sclera in eyes of Macaque monkeys.

The uveal tract of the eyes of monkeys was examined by electron microscopy using both thin sections and freeze-fracture replicas. The ultrastructural features of the lamina fusca in the monkey resembled those previously described for rabbit. The lamina fusca was composed of numerous interleaved processes of fibroblastic and pigmented cells and contained tight junctions between fibroblastic cell processes that were predominantly discontinuous, as well as numerous fenestrations through the attenuated cell processes. There was no regional compaction of cellular processes traversing the entire uvea at the level of the ora serrata as reported previously in hamster eyes.

Animals↗

Mapping, quantitative distribution and origin of substance p- and VIP-containing nerves in the uvea of guinea pig eye.

VIP- and substance P-like immunoreactivities were found in considerable concentrations (VIP: 17.3 +/- 4.8 pmol/g, mean +/- SEM; substance P:11.1 +/- 1.8 pmol/g) in the uveal portion of the guinea pig eye. Immunocytochemistry localised these two regulatory peptides to nerve fibres found principally in a plexus in the iris (substance P) and in an extensive network surrounding the blood vessels of the choroid (VIP). A remarkable anatomical demarcation of the two types of peptide-containing nerves was established by the staining of substance P-containing nerves, which stops at the level of the ciliary body. This uveal area is known to be involved in the ocular responses to nociceptive stimuli. At the ultrastructural level, immunoreactivity for both peptides was localised to distinct subpopulations of p-type nerves, distinguishable by the size of their large dense-cored vesicles. Those immunoreactive for VIP were significantly larger (p less than 0.0005) than those immunoreactive for substance P (95 +/- 7 nm and 82 +/- 9 nm respectively; mean +/- SD). Interruption of the trigeminal pathway produced a remarkable decrease of substance P immunoreactivity in the anterior portion of the uvea (9.1 +/- 1.5 pmol/g, mean +/- SEM, control; 5.3 +/- 1.3 pmol/g, denervated), but not of VIP immunoreactivity in the choroid. Following colchicine treatment, VIP-immunoreactive neuronal cell bodies were localised in the choroid. The separate anatomical localisations and distributions of the two uveal peptides appear to be related to their different origins and functional roles in the response of the eye to noxious stimuli.

Acetylcholinesterase↗

Mast cell heterogeneity in the human uvea.

To identify chymase- and tryptase-positive mast cells in the human uvea, and to study their associations with different types of resident uveal cells, uveal specimens from 24 human donor eyes were cryosectioned in sagittal and tangential planes. Enzyme histochemical staining of chymase was combined with immunohistochemical staining for tryptase, detected with the APAAP method. Fluorescence immunohistochemistry was performed with antibodies against c-kit, alpha smooth muscle actin, protein gene product (PGP) 9.5, CD45, and HLA-DR. In different uveal compartments, the total amounts of mast cells were calculated and the distributions of chymase and tryptase were quantified. All uveal mast cells were c-kit and CD45 positive and HLA-DR negative. No association existed between mast cells and actin-containing cells. Only a few mast cells were in close association with PGP 9.5-labeled nerve fibers. In the choroid, most mast cells were located in the inner central part (mean density = 48.9/mm(2)), and contained both chymase and tryptase (96%). The ciliary muscle contained numerous mast cells (mean density = 33.7/mm(2)), many of them tryptase positive but chymase negative (63%). In the pars plana, a high number of chymase-positive, tryptase-negative mast cells were found (20%). In the iris only a few mast cells were present. Although the choroid contains the most common subtype of mast cells, a unique situation concerning the distribution of chymase and tryptase is present in the anterior uveal tissues. A possible role for these cells in the special immunological situation of the anterior eye chamber merits further investigation.

Actins↗

Calcitonin gene-related peptide (CGRP) immunoreactive sensory nerves in the human and guinea pig uvea and cornea.

The presence of calcitonin gene-related peptide (CGRP) immunoreactive nerves in the uvea and cornea of human and guinea pig eyes was evaluated using immunohistochemical techniques. CGRP immunoreactivity was found in thin, varicose nerve fibers in both species. Most of the fibres were localized in the ciliary body, and were mainly associated with blood vessels. In the human ciliary body, a moderate number of CGRP immunoreactive nerves were also seen in the ciliary muscle. In the iris and cornea, CGRP immunoreactive fibres were relatively uncommon. In the iris, they were mostly found associated with blood vessels, while in the cornea they were seen sub-epithelially or as free nerve endings in the epithelium. In the trigeminal ganglion, small sized ganglion cells displayed CGRP immunoreactivity. About 40% of all ganglion cells were immunoreactive nerves in the guinea pig, while sympathetic denervation did not change the staining pattern of CGRP immunoreactivity. The present findings, together with previous physiological data, suggest that CGRP might play a role in the regulation of the blood flow, aqueous humour dynamics, and neurogenic inflammation, not only in experimental animals but also in man.

Animals↗

Does virus persist in the uvea in multiple sclerosis, as in canine distemper encephalomyelitis?

Epidemiological studies suggest that multiple sclerosis (MS) might be triggered by an infectious agent. Uveitis has been observed in a small percentage of MS patients. Dogs with canine distemper encephalomyelitis, another demyelinating disease of the central nervous system, have an anterior uveitis which is usually mild and asymptomatic, and dogs with persistent CNS infection and chronic distemper encephalomyelitis harbour virus persistently in the uvea. These observations in dogs suggest that pathological and virological studies of the uveitis associated with MS would be worth while.

Animals↗

Congenital ectropion uveae with glaucoma.

Congenital ectropion uveae (CEU) is a rare, nonprogressive anomaly characterized by the presence of iris pigment epithelium on the anterior surface of the iris stroma, often associated with neurofibromatosis and occasionally with other ocular anomalies. We present eight patients with unilateral CEU. Seven patients had glaucoma in the involved eye, while the eighth was a 10-week-old infant. In the two patients with bilateral glaucoma, the second eye was similar to the first, but without CEU. Three patients had neurofibromatosis, two had facial hemihypertrophy, one had Rieger's anomaly, one had Prader-Willi syndrome, and one had no systemic anomalies. Two had initially been misdiagnosed as having a large pupil in the involved eye and one as having a Horner's syndrome in the uninvolved eye. The finding of CEU in an infant warrants continued observation for the development of glaucoma and disorders of neural crest origin.

Abnormalities, Multiple↗