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Choroidal neovascular membranes and senile macular choroidal degeneration: their association in disciform macular degeneration.

Senile macular choroidal degeneration (SMCD) is responsible for the majority of cases of new central vision loss in the over-fifty population. It is also the single largest cause of legal blindness in the United States. Choroidal neovascular membranes are frequently associated with SMCD and initiate a disciform process that may lead to profound central vision loss. Clinically, it is very difficult to visualize actual new vessel membranes without the aid of rapid sequence fluorescein angiography; various clinical signs, however, are indicative of their presence. Recognition of choroidal neovascular membranes is important; certain membranes lend themselves to photocoagulation by the retinal specialist. This treatment may be successful in preventing further severe and permanent visual loss in some individuals. The purpose of this paper is to discuss the mechanisms of disciform macular degeneration, as well as the clinical signs important to the recognition of choroidal neovascular membranes.

Choroid↗

Refractive error and choroidal perfusion characteristics in patients with choroidal neovascularization and age-related macular degeneration.

The refractive error of 186 eyes with choroidal neovascularization (CNV) secondary to age-related macular degeneration (ARMD) was determined. It was found that the risk for the development of this form of ARMD increased with increasing degrees of hyperopia. The 96 patients afflicted with bilateral CNV were significantly older than the unilaterally affected patients (P = 0.027). Abnormal choroidal perfusion was identified in 66.4% of the fluorescein angiograms. These findings support the concept that hyperopic shortening in eyes afflicted with ARMD might cause secondary changes in the choroidal vasculature, predisposing them to choroidal neovascularization.

Aged↗

[Experimental research on choroidal circulation. 2. Choroidal blood flow increase elicited by electrical stimulation of parasympathetic nerve behind the eyeball in the cat].

The present studies were performed to examine whether the nerves behind the eye ball contain parasympathetic fibers which dilate the choroidal blood vessels. Choroidal blood flow in the cat (20 animals) was measured trans-sclerally with a laser Doppler flowmeter. Cats were anesthetized with pentobarbital sodium (30 mg/kg, i.v.) and artificially respirated after injection of pancuronium bromide (0.2 mg/kg/hr, i.v.). Electrical stimulation of the short ciliary nerve as well as of the nerve between the ciliary ganglion and accessory ciliary ganglion, where the sensory nerve is absent, elicited an increase choroidal blood flow in an intensity-dependent manner. Application of capsaicin, which is known to desensitize the polymodal nociceptive C-fibers, to these nerves did not abolish the increase in blood flow in response to electrical stimulation of the short ciliary nerve. Atropine, a muscarinic receptor antagonist, and phentolamine, an alpha-adrenoceptor blocking agent, had no effect on these responses. The results suggest that the blood flow increase elicited by stimulation of these nerves is mediated via activation of the atropine-resistant parasympathetic vasodilator fibers supplying the choroid in the cat.

Animals↗

Immunohistochemical studies on choroidal epithelial cyst and neuroepithelial cyst: in comparison with normal choroid plexus and ependyma.

Immunohistochemical evaluations with antibodies against S-100 protein, glial fibrillary acidic protein, cytokeratin and prealbumin were applied to 2 intracranial cysts of choroidal epithelial and neuroepithelial origin, and to the normal choroid plexus and ependyma. All specimens showed various immunoreactivities with the 4 antigens: especially, prealbumin-positive cells were detected in normal choroid plexus, part of normal ependyma, and part of choroidal epithelial cyst. The epithelium of neuroepithelial cysts presumably has multipotentials in cellular differentiation.

Brain Diseases↗

Papillomas and carcinomas of the choroid plexus: histological and immunohistochemical studies and comparison with normal fetal choroid plexus.

BACKGROUND: Choroid plexus tumors are rare. Results on immunohistochemical features are scanty and controversial even regarding normal plexus. METHOD: Thirteen cases of choroid plexus tumors and five samples of normal fetal choroid plexus were submitted to immunohistochemical study using a panel of epithelial, neuronal and stromal markers. RESULTS/CONCLUSIONS: Relevant histological findings were presence of clear cells in 3/5 papillomas (PP) and 7/8 carcinomas (CA) and all 5 fetal plexuses; rhabdoid cells, desmoplasia and vascular proliferation were found respectively in 3, 4 and 5 cases out of 6 poorly differentiated CA and were absent in PP and well differentiated CA. Pancytokeratin AE1/AE3 was strongly positive in all 13 cases, even in the undifferentiated component of poorly differentiated CA, where reactivity was focal in 3 and diffuse in 3 cases. Low molecular weight cytokeratin (35betaH11) was not expressed in any of the 8 CA, but was present in all 5 PP. In 4 of 6 poorly differentiated CA there was reactivity for smooth muscle actin (1A4) in 10 to 30% of the cells. This was true also for one case lacking rhabdoid cells. Laminin was undetectable in all 6 cases of poorly differentiated CA but was present in 4 PP and 2 well differentiated CA. All 5 fetal plexuses expressed GFAP.

Adolescent↗

Polypoidal choroidal vasculopathy associated with Doyne's familial choroiditis: treatment with thermal laser.

PURPOSE: To report the unusual occurrence of polypoidal choroidal vasculopathy (PCV) in a patient with Doyne's familial honeycomb choroiditis (DFHC) and its course after laser treatment. DESIGN: Interventional case report. METHODS: Indocyanine green (ICG) angiography guided laser was performed on active polypoidal lesions. RESULTS: A 45-year-old man with a 15-year history of bilateral DFCH and a scarred macular choroidal neovascularization in the right eye (RE) was referred to us with exudative maculopathy in the left eye (LE). His best-corrected visual acuity (BCVA) was 20/800 in the RE and 20/40 in the LE. ICG angiography revealed a picture that was characteristic for PCV in both eyes. ICG guided argon green laser was performed on the active parapapillary and perifoveal polypoidal lesions of the LE. Eight months after the laser photocoagulation treatment, the macular exudative lesions had subsided and the BCVA improved to 20/20. The favorable anatomic and functional results have remained stable over 3 years. CONCLUSIONS: This is, to our knowledge, the first case of a PCV that occurred secondary to DFHC.

Choroid↗

Angiographic and flow patterns of retinal choroidal anastomoses in age-related macular degeneration with occult choroidal neovascularization.

OBJECTIVE: To identify the angiographic features of retinal choroidal anastomoses (RCAs) in patients with newly diagnosed occult choroidal neovascularization (CNV) in the setting of age-related macular degeneration (AMD) and to determine the sequence of flow between the RCA and the CNV. DESIGN: Retrospective cross-sectional study. PARTICIPANTS: The angiograms of 205 eyes of 153 consecutive patients with occult CNV on fluorescein angiography (FA) and focal hot spots on indocyanine green angiography (ICGA) were evaluated retrospectively. METHODS OF TESTING: Red-free photographs and sequential digital fluorescein and indocyanine green angiograms obtained by confocal scanning laser ophthalmoscope (the Heidelberg Retina Angiograph; Heidelberg Engineering GmbH, Dossenheim, Germany). MAIN OUTCOME MEASURES: The angiograms were evaluated for the presence of RCA. The following angiographic characteristics were recorded: number and type of anastomoses, location, distance from fovea, area of CNV, presence of pigment epithelium detachment (PED), cystoid macular edema on FA, and intraretinal leakage on ICGA. The direction of flow between the RCA and the CNV was identified by analyzing high-speed angiograms. RESULTS: Retinal choroidal anastomoses were identified in 57 eyes (28%), in 49 of 154 eyes with PED (32%), and in 8 of 51 eyes without PED (16%). Of 109 anastomoses, 70% were venous and 30% were arterial. Ninety-six percent of the eyes had at least one venous anastomosis, 49% of the eyes had an arterial anastomosis, and 46% of the eyes had both. Cystoid macular edema was seen on FA in 37 eyes (65%), and intraretinal indocyanine green leakage was noted in 52 eyes (91%). Twenty-two eyes that underwent high-speed ICGA were analyzed for the direction of flow. All 15 eyes having arterial and venous anastomoses demonstrated a filling pattern from the retinal arteriole to the CNV, followed by the retinal venule. Seven eyes with venous RCA showed flow sequence from the CNV to the collecting retinal venule. CONCLUSIONS: Our study supports the presence of RCA in the early stages of acute exudative AMD with occult CNV, mainly with serous PED. High-speed angiography helps to identify the filling sequence of the RCA and the CNV, and therefore may guide the clinician in planning treatment strategies.

Aged↗

Indocyanine green-mediated photothrombosis combined with intravitreal triamcinolone for the treatment of choroidal neovascularization in serpiginous choroiditis.

PURPOSE: To report a case of peripapillary choroidal neovascularization (CNV) complicating serpiginous choroiditis that was treated by a single indocyanine green (ICG)-mediated photothrombosis session combined to intravitreous triamcinolone acetonide (TA) injection. METHODS: Interventional case report. A 48-year-old patient with peripapillary CNV was submitted to a laser-dye-mediated technique that uses ICG and low-intensity 810-nm light for continuous laser application; TA was then injected into the vitreous cavity 1 hour later, and prospective evaluation with fluorescein and ICG angiography as well as optical coherence tomography (OCT) was performed. RESULTS: At 2 weeks after treatment, best-corrected visual acuity improved from 20/200 to 20/50, with further improvement to 20/20-1 in the subsequent 10 weeks. Absence of fluorescein leakage from the CNV and OCT evidence of resolved retinal oedema was observed at that time. Clinical stabilization was maintained up to 1 year of follow-up. There was no significant complication related to the procedure. CONCLUSION: Combined ICG-mediated photothrombosis and intravitreous TA induced rapid and significant visual acuity recovery in this particular case of peripapillary CNV complicating serpiginous choroiditis. Accordingly, angiographic and OCT findings demonstrated neovascular lesion regression and restoration of the macular architecture.

Anti-Inflammatory Agents↗

Variable outcome of photodynamic therapy for choroidal neovascularization associated with choroidal nevus.

PURPOSE: To report five cases of classic choroidal neovascularization (CNV) associated with choroidal nevus treated with photodynamic therapy (PDT) with verteporfin. METHODS: The patients underwent an ophthalmologic evaluation, including fluorescein angiography and indocyanine green angiography. Clinical and angiographic data were retrospectively analyzed to evaluate visual acuity outcomes and both clinical evolution and angiographic evolution. RESULTS: Two patients presented with subfoveal CNV, and three had juxtafoveal CNV. The mean follow-up was 25.8 months. Visual outcomes were extremely variable. Indeed, best-corrected visual acuity decreased in three eyes, stabilized in one case, and improved in the other case. The number of PDT sessions necessary to obtain CNV stabilization with cessation of fluorescein leakage varied from one to six. CONCLUSION: Bearing in mind that both the natural history and the post-PDT outcome may be extremely variable, further studies are needed to assess the real benefit of PDT for classic CNV secondary to choroidal nevus.

Aged↗

Subretinal choroidal neovascularization associated with choroidal nevus.

PURPOSE: Evaluation of a large series of choroidal nevi inducing the formation of a neovascular membrane in order to more clearly define the clinical presentation and to evaluate the efficacy of various treatment options. METHOD: Retrospective study of 22 clinical cases. RESULTS: All nevi were situated in the posterior choroid. They had a mean diameter of 3.8 mm and a mean thickness of 1.4 mm. Neovascular membranes were classic in all cases, extrafoveal in 13 cases (59%), and subfoveal in 9 cases (41%). A serous retinal detachment was present in every case, hemorrhages were present in 13 cases (59%), and lipid deposits were present in 16 cases (73%). All extrafoveal neovascular membranes were successfully treated by thermal laser photocoagulation. Initial visual acuity was 0.1 in three cases, 0.2-0.4 in five cases, 0.5-0.8 in four cases, and 1.0 or more in two cases. Final visual acuity was 0.1 in one case, 0.2-0.4 in one case, 0.5-0.8 in four cases, and 1.0 or more in seven cases. Five subfoveal neovascular membranes were treated either by thermal laser, photodynamic therapy, or irradiation. No treatment was applied in four cases and in one of these cases, spontaneous resolution of the neovascular membrane was observed. No growth of the pigmented tumor was observed with a mean follow-up of 4.8 years. CONCLUSIONS: Proliferation of a neovascular membrane on the surface of a pigmented choroidal tumor is a rare complication and is considered to be a relative indicator of a benign nature of the lesion. In the authors' experience, neovascular membranes are extrafoveal in more than half of cases and are accessible to laser photocoagulation. In contrast, the various modalities used to treat subfoveal neovascular membrane were ineffective and functional prognosis was unfavorable in these cases.

Adult↗

Photodynamic therapy for choroidal neovascularization in patients with multifocal choroiditis and panuveitis.

PURPOSE: To evaluate the visual benefit of photodynamic therapy (PDT) with verteporfin in patients with choroidal neovascularization (CNV) secondary to multifocal choroiditis and panuveitis over a longer follow-up period. METHODS: A total of 14 eyes of 12 patients (mean age 34 years) with a classic subfoveal CNV (13/14) or juxtafoveal CNV (1/14) were treated with PDT. Visual outcome was assessed by best-corrected visual acuity (VA). Morphologic characteristics of CNVs such as localization, size, and activity were monitored by fluorescein angiography. RESULTS: Patients were followed for 3 to 45 months (mean 23 months). During this period, one to six PDTs (mean 2.4) were performed. At the time of the first PDT no acute inflammation was seen in the affected eyes. Improved or stabilized visual function (VA loss < or = 2 lines in the Early Treatment Diabetic Retinopathy Study chart) was observed in 71.4% of the eyes. A total of 78% of the eyes showed an inactive scar in the area of CNV after PDT. Treatment failure after PDT occurred due to uninfluenced CNV growth. No further complications were observed. CONCLUSIONS: PDT in subfoveal or juxtafoveal classic CNV secondary to multifocal choroiditis and panuveitis stabilized or improved VA in the majority of patients over a longer follow-up period. No risk factor for failed VA rehabilitation could be defined.

Adolescent↗

[Sub-tenon steroid treatment in choroidal neovascularization caused by serpiginous choroiditis. A case report].

CASE REPORT: We present a report of a patient suffering from serpiginous choroiditis complicated by choroidal neovascularization (CNV). Sub-tenon steroid treatment, added to the usual immunosuppressive therapy, resolved the CNV, improving visual acuity in one eye. DISCUSSION: Choroidal neovascularization is a sight-threatening complication of posterior uveitis. Our case suggests that sub-tenon steroid treatment, added to systemic immunosuppressive therapy, may accelerate the CNV resolution in posterior uveitis.

Azathioprine↗

Treatment of subfoveal choroidal neovascularization secondary to choroidal nevus using photodynamic therapy.

A 52-year-old man sought treatment for decreased vision in his right eye for 3 weeks. Visual acuity was 20/120. Examination revealed an elevated choroidal nevus at the posterior pole. Fluorescein and indocyanine green angiographies disclosed fibrovascular pigment epithelial detachment extending under the fovea. The patient was treated by two sessions of photodynamic therapy with verteporfin. Visual acuity improved to 20/40. The fibrovascular pigment epithelial detachment area diminished and the fovea is clear of lesion. Seven months after the last treatment, visual acuity remains stable. Photodynamic therapy with verteporfin could be an effective option for treating subfoveal choroidal neovascularization secondary to choroidal nevus.

Choroid Neoplasms↗

[Surgical treatment of retrofoveal choroid neovascularization in multifocal choroiditis].

BACKGROUND: Surgical removal of subfoveal choroidal neovascularization allows visual improvement, especially in young patients. METHODS: Three eyes of 3 patients were prospectively studied. Subfoveal choroidal neovascularization was related to presumed ocular histoplasmosis syndrome. The surgical procedure included vitrectomy, surgical excision of the neovascular membrane, and air tamponnade. RESULTS: Follow-up was 6, 12 and 20 months. Vision improved in 2 eyes. In one case, recurrent extrafoveal neovascularization was treated with laser photocoagulation. DISCUSSION: Surgery seems to be an alternative to photocoagulation in subfoveal choroidal neovascularization in presumed ocular histoplasmosis.

Adult↗

Osseous choristoma of the choroid simulating a choroidal melanoma. Association with a positive 32P test.

We report the clinical and histopathologic features of an osseous choristoma of the choroid that appeared in a 26-year-old woman. Her diagnostic evaluation included a radioactive phosphorus (32P) uptake test, which was 270% positive and led us to believe that the lesion was an atypical malignant melanoma. The extent of true ossification within this tumor, seen microscopically, explains the false-positive uptake of radioactive phosphorus by this benign tumor; it is well known that bone avidly accumulates radioactive phosphorus. We recommend that all "atypical choroidal melanomas" be studied for the presence of bone, with bone-free roentgenograms, ultrasonography, and possibly, computerized tomographic scans before 32P testing or enucleation. This, to our knowledge, is the second histopathologically proved case of an osseous choristoma of the choroid to be reported in the literature.

Adult↗

[Clinical and histopathological aspects of 113 necrotizing malignant melanomas of the choroid. 1. Clinical and histological features of necrotizing choroidal melanomas].

BACKGROUND: Tumor necrosis may reflect a destructive immune reaction. Systematic and statistically significant comparative clinico-histopathologic studies have not yet been reported. PATIENTS AND METHODS: 113 necrotizing choroidal melanomas (NCM) recruited from 701 enucleated globes 1967-1988 were resectioned, stained and compared to 100 choroidal melanomas without necrosis (CM), and data of 74 patients with a follow-up of more than 10 years were evaluated. RESULTS: Statistically significant characteristics of NCM were: patient age < 60 yrs. for NCM 27.4%, CM 46%; patient age in men for NCM was 64 yrs on average (CM: 58 yrs.), in women for NCM 67 yrs. (CM: 59 yrs.). Time elapsed between first symptoms and enucleation was < 12 months in 15.9% of NCM (89% for CM), and > 12 months in 23.9% of NCM (11% in CM). Mixed or epitheloid cell tumors was seen in 54.9% of NCM and 49% of CM, spindle cell tumors in 36.3% of NCM and 51% in CM. Advanced tumor stages T3 and T4 were present in 45.1% resp. 36.3% of NCM compared to 37% resp. 16% in CM. Scleral invasion was documented in 67.3% of NCM and 37% of CM, extrascleral dissemination in 43% of NCM and 16% of CM. Secondary glaucomas were seen in 62.2% of NCM and 6% CM, a penetration through Bruch's membrane in 61.0% of NCM and 46% of CM. Intratumoral hemorrhage was noted in 68.14% of NCM and 24% of CM, extratumoral bleeding in 23.9% of NCM and 0% CM. Inflammatory reactions in tumors were observed in 96.7% of NCM harboring > 30% necrosis compared to 5% in CM, and extratumoral in 94.5% of NCM and 0% of CM. Intraocular extratumoral necrosis was seen in 23.9% of NCM and 0% of CM. There were no significant differences in the degree of pigmentation of the 90.3% pigmented NCM or of the 94% pigmented CM, neither in the tumor localization, being constantly behind the equator in 87% of cases. Survival of patients with NCM patients was 5 years and 9 months on average (5-year mortality rate 41.9%), and 74.3% were deceased from metastatic spread. CONCLUSIONS: Significant clinical and histopathological differences between necrotizing and non-necrotizing malignant melanomas of the choroid can be identified. The inflammatory reaction of NCM must be further elucidated, particularly with respect to the nature of tumor-infiltrating lymphocytes.

Adult↗