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Two cases of telangiectatic hamartoma of the iris and ciliary body are reported, one of them with its histopathological study. No previous cases have been found, as such, in the current literature. The most important descriptions of primary vascular lesions of the iris and ciliary body are briefly reviewed.
A neoplasm of the ciliary body extending from the equator to the iris and the anterior chamber was observed in an 18-year old girl. Because the visual acuity was lost and the clinical diagnosis was uncertain the eye was enucleated. Both optic and electron microscopic investigations allowed the diagnosis of the mesectodermal type of leiomyoma of the ciliary body.
PURPOSE: To investigate the density, distribution and morphology of macrophages and MHC class II-positive dendritic cells in the iris and ciliary body of lewis rats. METHODS: Immunohistochemistry was performed using monoclonal antibodies specific to monocytes and macrophages (ED1, ED2) and MHC class II-positive cells (OX6) on wholemounts of the iris-ciliary body complex isolated form normal lewis rats. RESULTS: A well developed network of macrophages was present in the iris and ciliary body of normal lewis rats. These cells, morphologically displaying dendritiform or pleiomorphic appearance, were more densely arranged in mid-iris (950 +/- 189 cells/mm2) than in iris base (482 +/- 78 cells/mm2) and pupil margin (595 +/- 92 cells/mm2). A similar network of MHC class II-positive cells with a cell density 452 +/- 78 cells/mm2 was almost uniformly distributed in the iris of normal lewis rats. CONCLUSIONS: A network of macrophages and MHC class II-positive cells was established in the iris and ciliary body of normal lewis rats. These cells may be involved biologically in the generation of anterior chamber associated immune deviation-inducing signal within the anterior chamber and the transferring of this signal from the eye to the spleen, and pathophysiologically in the pathogenesis of uveoretinitis.
AIMS: To investigate the usefulness of ultrasound biomicroscopy for evaluating changes in the ciliary body in patients with Vogt-Koyanagi-Harada disease. METHODS: Ultrasound biomicroscopy was used to evaluate 14 eyes of seven patients diagnosed with Vogt-Koyanagi-Harada disease. Cross sectional images of the ciliary body and thickness of the pars plana 3.0 mm posterior to the scleral spur were examined. Predicted thickness of the pars plana was obtained by multiple linear regression analysis of thickness in the acute phase and in the remission phase. RESULTS: In the active phase, the cross sectional images showed a shallow anterior chamber in eight of the 14 eyes, ciliochoroidal detachment in five eyes, and a thickened ciliary body in all 14 eyes. Internal reflectivity of the ciliary stroma was low, with ciliary processes being unclear in 13 eyes. One month after steroid treatment, slit lamp examination findings were normal in 14 eyes. 10 eyes of five patients were examined by ultrasound biomicroscopy at this stage. Ciliochoroidal detachment was no longer seen in any eye. Internal reflection of the ciliary stroma became relatively homogeneous, and the ciliary processes were seen, though not clearly. However, the pars plana remained thickened. The actual thickness was greater at 1 month after steroid treatment than the predicted thickness for the remission phase. In the remission phase, the internal reflection was homogeneous and the ciliary processes were delineated clearly in all 14 eyes. CONCLUSION: Objective, quantitative evaluation of the ciliary body is possible with ultrasound biomicroscopy during the course of Vogt-Koyanagi-Harada disease. Ultrasound biomicroscopy is useful in determining disease activity in the anterior segment and in monitoring the clinical course, and it may improve evaluation of the efficacy of treatment.
Melanoma involving the ciliary body is a rare tumor which carries a poor prognosis when compared to all uveal melanoma. We have treated 54 patients with ciliary body melanoma using helium ions from 1978 to 1985. Because of the high rate of metastatic disease, the 5-year disease specific survival rate is only 59% despite a 5-year local control rate of 98%. The greatest diameter of the tumor was predictive of loss of vision and enucleation (p = .05, p = .04, respectively). Multivariate analysis showed that the greatest diameter of the tumor was the most important predictor of death from metastases. The incidence of neovascular glaucoma at 5 years is 43%. The 5-year actuarial rate of enucleation is 26%. Enucleation was done for pain and/or neovascular glaucoma. Univariate analysis showed treatment volume to be a statistically significant predictor for the development of neovascular glaucoma (p = .0017) and enucleation (p = .0078). Seventy percent of neovascular glaucoma occurred in patients with treatment volume greater than 5.5 cc. Seventy-four percent occurred in patients with an initial ultrasound height greater than 9.2 mm. Using this information, patients at high risk for neovascular glaucoma could be considered for prophylactic treatment with panretinal photocoagulation.
Transmural electrical properties of the isolated rabbit iris-ciliary body (I-CB) were measured in Ussing-Zerahn-type chambers. Control p.d. across the preparation was -1.2 +/- 0.1 mV, with the ciliary process (aqueous)-side consistently negative with respect to the ciliary body (blood)-side and the short-circuit current (SCC) was 7.9 +/- 0.6 microA cm-2. Bilateral bathing solution substitutions demonstrated absolute requirements for the presence of Na+, K+ and HCO3-for the maintenance of the p.d. Addition of 5 X 10(-5) M ouabain to the aqueous-side chamber increased the p.d. and SCC initially, with a subsequent decline to zero. Only a declining phase was observed when ouabain was added to the blood-side. Ouabain inhibited oxygen consumption by 28% in Tyrode's solution. Respiratory rate was also approximately 28% lower in Na+-free and K+-free Tyrode's solution and ouabain had no additional inhibitory effect in either of these two solutions. Thus the biphasic effect of ouabain on the electrical parameters cannot be explained by a toxic effect but rather as a selective inhibition of the Na+-, K+-pump. Our results indicate that Na+-, K+-ATPase activity and the presence of HCO3- are required for active ion transport in this preparation.
OBJECTIVE: To correlate ultrasound biomicroscopic images of iris and ciliary body melanomas with their histopathologic features. METHODS: Ultrasound biomicroscopy was performed in 3 cases of iris melanoma and in 3 cases of ciliary body melanoma. Cross-sectional ultrasound biomicroscopic images were compared with findings from clinical examination and light microscopy to evaluate associations between their histopathologic, surface, and internal ultrasound characteristics. Unique images of intrastomal and obscured posterior tumor margins were visualized by ultrasound biomicroscopy. RESULTS: Results of this study revealed that ultrasound biomicroscopy offers an accurate method to evaluate tumor shape, reflectivity, and local invasion. Neoplastic tissue had only medium echogenicity. Enlarged vessels were correlated to echolucent spaces in the iris stroma. Anterior tumor margins were found within the iris stroma, within the anterior chamber angle, and on the endothelial surface of the cornea. Posterior tumor extension was noted to encroach onto the lens, into the sclera, and serous peripheral retinal detachments were associated with ciliary body tumors. CONCLUSION: Ultrasound biomicroscopic images correlated well with histopathologic features of anterior uveal melanomas including shape, reflectivity, and local extension. Arch Ophthalmol. 2000;118:1515-1521
PURPOSE: To present the case of a patient with leiomyoma of the ciliary body and discuss the histological features and treatment of this rare intraocular tumor. METHODS/CASE REPORT: A 13-year-old boy presented with an asymptomatic tumor of the right eye. Visual acuity was 20/20 in both eyes. Ophthalmoscopy revealed an amelanotic, vascularized ciliary body tumor with exudative retinal detachment and partial transillumination. On ultrasound examination the tumor height was 8 mm and a low internal reflectivity was found. T2-weighted MRI scans showed a hypointense and T1-weighted scans a hyperintense intraocular mass with significant Gd-TPA enhancement. On the assumption that the diagnosis was consistent with an amelanotic ciliary body melanoma, a transscleral resection with adjuvant ruthenium-106 brachytherapy was performed. Visual acuity was 20/40 at 6 months after the operation. RESULTS: Routine stains revealed a pleomorphic tumor composed mainly of spindle cells with palisading in some areas and a prominent intercellular fibrillary background. Immunohistochemistry showed positivity for desmin, vimentin and actin. No reactivity with S-100 and HMB-45 was seen. Intracytoplasmatic filaments and micropinocytotic vesicles were detected by transmission electron microscopy. These findings were consistent with the diagnosis of a ciliary body leiomyoma. CONCLUSION: Typical clinical features of leiomyoma include a dome-shaped configuration and translucency, but the final diagnosis can only be confirmed by histology with the aid of immunohistochemistry and electron microscopy. Though rare, leiomyoma should be considered in the differential diagnosis of amelanotic uveal tumors. Transscleral resection is the treatment of choice of anterior uveal leiomyomas, with a fairly good visual prognosis.
Microsomes were prepared from bovine ciliary bodies. The contents of cytochrome P-450 and related components of the microsomal electron transport system were determined. The cytochrome P-450 content was 32 pmoles/mg protein, which was about 4% that in rat liver. The cytochrome b5 content was 59 pmoles/mg protein. The NADH-cytochrome c reductase and NADPH-cytochrome c reductase activities were 268 and 18 nmoles/min/mg protein, respectively. The ethoxyresorufin deethylase activity was 2.1 pmoles product formed/min/mg protein.
PURPOSE: To evaluate the gonioscopic signs indicative of underdevelopment of the iridocorneal angle and thereby obtain a correct diagnosis of goniodysgenetic glaucoma. METHODS: We examined morphologically the iridocorneal angle of 28 enucleated human eyes, including those from premature infants as well as from older subjects. The ratio of the ciliary body band width to the width of the posterior limbus (the distance between the termination of Descemet's membrane and the iris root), termed the theoretic C/L ratio, was calculated by drawing on light micrographs. Data were compared for three age groups: premature infants, children, and subjects older than 9 years. We also examined clinically the appearance of the ciliary body band on gonioscopy in 135 eyes of 69 younger patients having primary glaucoma with open iridocorneal angles and in 94 eyes of 47 age-matched control subjects. RESULTS: The theoretic C/L ratio +/-SD was 1.4% +/- 2.7% in premature infants, 24.0% +/- 5.4% in children, and 36.7% +/- 8.0% in the older subjects. There was a statistically significant difference between each of the three groups (between premature infants and children, P < .001; between children and older subjects, P < .005; between premature infants and older subjects, P < .001). On gonioscopy, 105 of 135 glaucomatous eyes (77.8%) and 13 of 94 control eyes (13.8%) showed either an invisible or very narrow ciliary body band (P < .0001). CONCLUSION: The ciliary body band seen on gonioscopy provides an indicator of the development of the iridocorneal angle. An invisible or very narrow ciliary body band represents an underdevelopment of the angle.
PURPOSE: The effects of subconjunctival injection of mitomycin C and different concentrations of 5-fluorouracil on the epithelium of the ciliary body of twenty Sprague Dawley SD rats was studied. METHODS: Twenty rats were divided into four treatment groups. The first three groups received 0.2 ml of 5, 10, and 30 mg of 5-fluorouracil subconjunctivally respectively, and the fourth group 0.2 ml of 0.4 mg/ml mitomycin C subconjunctivally. The right eye received 0.2 ml of the antimetabolite, while the left eye was injected with 0.2 ml of saline subconjunctivally, as a control. The eyes were examined histologically, in a masked fashion, by light and by transmission electron microscopy. In each treatment group, two eyes were examined after one week, and three eyes were examined one month after the subconjunctival injection. RESULTS: Electron microscopy revealed toxic effects in the epithelium of the ciliary body of all treatment groups. The 5-fluorouracil group revealed focal mitochondrial edema, enlargement of intercellular spaces, and dilatation of intracellular spaces. The mitomycin C group showed pyknotic nuclei, enlargement of intercellular spaces, and irregular flattened epithelial cells. The severity of changes correlated with concentration and length of exposure. No pathology was found by light microscopy in all groups. CONCLUSIONS: This study demonstrates that subconjunctival antimetabolites mitomycin C and 5-fluorouracil can penetrate the sclera and exert toxic effects on the epithelium of the ciliary body, even in low doses. These changes were only apparent by electron microscopy and were still present one month after the injection. These findings may contribute to the theory that the application of antimetabolites during or after surgery has a direct effect on the epithelium of the ciliary body, besides its known effect on the conjunctiva. Further studies are needed to evaluate its effect on intraocular pressure.
A ciliary body tumor that was believed clinically to be a malignant melanoma developed in a 28-year-old woman and led to enucleation of her eye. The light microscopic appearance of the tumor was interpreted by light microscopy as a neurogenic neoplasm, possibly a choristomatous astrocytoma. Electron microscopic studies disclosed the smooth muscle origin of the tumor. Results of experimental embryologic studies performed on lower animals have proved that the neural crest contributes most of the connective tissues, "mesectoderm," of the globe and orbit in these species. The occurrence of hybrid neurogenic-myogenic tumors in the human eye supports the applicability of these embryologic discoveries to man. The association of a pigmented nevus with the present tumor is additional evidence of the neural crest origin of mesectodermal leiomyomas, since melanocytes are well-recognized descendants from the neural crest.
We describe a new technique for the separation and isolation of nonpigmented ciliary body epithelial cells from rabbit. Excised ciliary body is incubated in a medium buffered with EGTA to a free-Ca2+ concentration of 10(-8) M, and the nonpigmented cell layer is separated from the pigmented layer by microdissection under direct visualization. This technique yields intact cells which are greater than 99% nonpigmented. It can be used to produce preparations of nonpigmented cell plasma membrane and of viable whole cells, which may be useful for biochemistry, pharmacology, cell transport studies and tissue culture.
The trigeminal nerve fibers in the ciliary body and the iris have been identified electron microscopically by using intra-axonally transported horseradish peroxidase injected into the trigeminal ganglion. The tracer revealed unmyelinated fiber profiles in the stroma of the ciliary body and the iris. These were not found to be associated with any particular organ but rather may terminate as free endings. Large profiles of varicosities contained mitochondria. Thin profiles, containing neurotubules, probably represent axons between the varicosities or endings. An interaction between the sensory and the autonomic peripheral fibers in the anterior uvea is suggested.
PURPOSE: To evaluate the distribution of different alpha- and beta-type connexins (Cx) present in the dual layered ciliary body epithelia (CBE) of both rabbit and rat. METHODS: Immunocytochemical detection of Cx26, Cx32, Cx43, and Cx50 was performed on frozen sections of rabbit and rat ciliary body using indirect immunofluorescent methods. The identity of the antigens recognized by the monoclonal primary antibodies was further confirmed by Western immunoblots. Double labeling experiments based on either conventional or confocal microscopy were carried out to establish the exact spatial relationship between different connexins. RESULTS: Connexin 50 was found only in the nonpigmented epithelium (NPE) at apical and basolateral membranes, whereas Cx43 was observed exclusively and at a very high concentration in the pigmented epithelium (PE), primarily in the apical cell membrane, with minimal extension to the proximal lateral zone. The correct antigenicity of the antibodies was confirmed by Western blots of rabbit ciliary body membranes. In rabbit, the Cx26 antibody detected an antigen that was abundant in the NPE and was weakly expressed in the PE. In rat, however, the Cx26 staining was confined to capillary wall endothelia. Western blots of ciliary body and liver membranes and liver immunohistology indicated that the Cx26 antibody used does not recognize rabbit Cx26. Cx32 did not yield any substantial epithelial labeling in either species. CONCLUSIONS: The distribution of Cx50 around the entire NPE cell perimeter suggests its involvement in NPE-NPE cell homotypic gap junctions. The concentration of Cx43 and Cx50 at the apical membranes of the PE and NPE cells, respectively, and their complete absence from the opposite cell suggest that these connexins may participate in the formation of heterotypic gap junctions, either with each other or with other yet unidentified connexins.
PURPOSE: The purpose of the study was to use ultrasound biomicroscopy to identify and quantity changes in anterior segment parameters after scleral buckling procedures. METHODS: Ultrasound biomicroscopy was used to examine 15 patients with retinal detachment within 1 week before and after surgery. Quantitative measurements were performed of anterior chamber depth, supraciliary effusion depth, ciliary body thickness, and angle opening. RESULTS: Supraciliary fluid was present after surgery in 12 patients (80%). Average supraciliary fluid depth was 0.16 +/- 0.13 mm. Ciliary body thickness measurements at a point 2-mm posterior to the scleral spur increased after surgery in all patients an average of 0.15 +/- 0.10 mm. There was a strong correlation between ciliary fluid levels and change in ciliary body thickness (r = 0.742, P < 0.01). Anterior chamber depth decreased after surgery in 14 patients (93%). A decrease of angle opening of greater than 5 degrees was noted in 11 patients (73%). In all of these 11 patients, the ciliary body and iris root were considered to be rotated anteriorly. Six (55%) of 11 of these patients showed anterior bowing of the iris, indicating pupillary block. Complete angle closure occurred over one to three quadrants in three patients, but none of these patients had complete angle closure or glaucoma. CONCLUSIONS: Supraciliary effusions and ciliary body thickening are common after scleral buckling procedures and can produce conditions conducive to angle closure. Angle narrowing occurs through a combination of direct anterior iris rotation and induced pupillary block.
The enzymatic activity of adenylate cyclase in homogenates and membrane-rich fractions prepared from rabbit iris-ciliary bodies and bovine trabecular meshwork was found to be inhibited by timolol. Treatment of iris-ciliary body homogenates with Triton X-305 resulted in abolition of the inhibitory effect of the drug on the activity of the enzyme. The stimulatory effect of salbutamol on the enzyme was also susceptible to blockade by timolol. It is suggested that the hypotensive action of timolol on intraocular pressure results from structural and functional changes induced on the plasma membranes of the iris-ciliary body and trabecular meshwork by the thiadiazole group of the molecule, and, also, from the occupation of the adrenergic receptors of the iris-ciliary body by the tert-butylamino-2-hydroxypropoxy part of the compound.
Unlike smooth muscle elsewhere in the body, the smooth muscle of the iris and ciliary body is derived from neuroectoderm (mesectoderm). Leiomyomas that arise from the ciliary body, and therefore are of mesectodermal origin, may resemble spindle cell neurogenic tumors by light microscopy. They show positive immunostaining for smooth muscle actin but negative staining for neural markers. Ultrastructurally, the cells have the features of smooth muscle cells. The authors report a typical case of mesectodermal leiomyoma in a 47-year-old woman in which skeinoid fibers, considered to be an ultrastructural marker of neurogenic spindle cell tumors, were frequent together with other ultrastructural features often seen in neuroglial cell tumors. The findings indicate that mesectodermal leiomyoma is unique in its histogenesis as well as in its morphology.