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[Choroidal osteoma].

Choroidal osteoma is a benign, ossifying tumor, first described by Gass and Williams in 1978, typically found in healthy young females. Usually it is localized in the posterior pole of the eye, near the optic disc and the macula. Its aetiology is unknown: it may be caused by osseous metaplasia of the retinal pigment epithelium, or it may represent a kind of choristoma. The diagnosis of the choroidal osteoma is based mainly on its very characteristic clinical picture, ultrasonography and computed tomography--both demonstrating calcific plaque corresponding to the lesion. The most important complications of the tumor are: subretinal neovascularisation, subretinal and intraretinal haemorrhages, serous and haemorrhagic retinal detachments. Neovascularisation can be successfully treated by laser photocoagulation, which can even lead to decalcification and involution of the tumor. Choroidal osteoma must be differentiated mainly from amelanotic choroidal melanoma, choroidal nevus, choroidal haemangioma, choroidal metastasis, granuloma, organized subretinal haemorrhage, sclerochoroidal calcification and age-related macular degeneration. In this paper we present a case of a choroidal osteoma recognized in an 11-year-old girl. There have been no signs of tumor's growth or signs of subretinal neovascularisation for three years of follow-up. As far as we know, it is the first case of this disease described in Polish literature.

Child↗

Aetiology and management of choroidal folds.

Choroidal folds are known to be associated with a wide variety of pathological conditions, such as central serous retinopathy, choroidal naevi, tumours and papilloedema. They may also occur with surgical procedures, especially when hypotony of the globe occurs, such as following cataract surgery, laser therapy and from post-operative choroidal oedema or inflammation. However, choroidal folds are idiopathic in the majority of cases and can be associated with benign conditions, such as hypermetropia and optic disc drusen. Numerous patterns and orientations of choroidal folds are possible, with some authors suggesting that typical presentations are associated with particular pathological conditions. They may be seen as dark and light streaks on ophthalmoscopy (often more defined with red-free viewing) but are more apparent and differentiated from retinal folds by fluorescein angiography. The aetiology of choroidal folds appears to be linked to a combination of an anatomical attachment of Bruch's membrane to the underlying choriocapillaris and congestion of the choriocapillaris. This paper reviews aspects of the differential diagnosis of choroidal folds with guidelines for optometric management. Several case reports are presented to demonstrate some of the clinical features of choroidal folds.

Journal Article↗

A long-term follow-up of choroidal osteoma.

OBJECTIVE: To provide long-term follow-up information on a large series of patients with choroidal osteoma. METHODS: Review of patients with a diagnosis of choroidal osteoma who had been examined at the Bascom Palmer Eye Institute, Miami, Fla, or known to one of us (J.D.M.G.). Information was obtained from hospital medical records or by a questionnaire sent to referring ophthalmologists. Life-table analysis was used to study the loss of vision and development of choroidal neovascularization. RESULTS: We followed up 36 patients, 31 (89%) were female, mean age, 21 years (range, 5-54 years) for a mean of 10 years (range, 2-22 years). Growth was observed for 9 (41%) of 22 well-documented osteomas. The probability of loss of visual acuity to 20/200 or worse was 58% by 10 years and 62% by 20 years. The probability of developing choroidal neovascularization was 47% by 10 years and 56% by 20 years. Successful treatment of the choroidal neovascularization with laser photocoagulation was performed for 5 (25%) of 20 patients. CONCLUSIONS: Most patients with choroidal osteomas maintain good vision in at least 1 eye, but they have a high risk of developing choroidal neovascularization. When this occurs, only a minority can be successfully treated with laser photocoagulation.

Adolescent↗

A role for connective tissue growth factor in the pathogenesis of choroidal neovascularization.

OBJECTIVE: To evaluate the expression of connective tissue growth factor (CTGF) in choroidal neovascular membranes from patients with age-related macular degeneration and the effect of CTGF on choroidal endothelial cell (CEC) function. METHODS: Using immunohistochemical methods, we analyzed CTGF expression in 13 surgically excised choroidal neovascular membranes related to age-related macular degeneration. The expression of CTGF in retinal pigment epithelial and CEC cultures was determined by means of reverse transcriptase polymerase chain reaction and Western blot, and its regulation by vascular endothelial growth factor and transforming growth factor beta was determined. The effects of CTGF on bovine CEC proliferation, attachment, migration, and tube formation were measured. RESULTS: Vascularized human choroidal neovascular membranes showed strong CTGF immunoreactivity. Double staining disclosed colocalization of CTGF with retinal pigment epithelial cells and CECs. The CTGF induced a significant increase in attachment and migration of CECs; however, it did not stimulate CEC proliferation. The CTGF protein was up-regulated in retinal pigment epithelial cells and CECs by stimulation with transforming growth factor beta and vascular endothelial growth factor, respectively. CONCLUSIONS: The expression of CTGF in choroidal neovascular membranes, its regulation by angiogenic growth factors, and its proangiogenic effects on CEC function suggest that CTGF may play a role in the pathogenesis of choroidal neovascularization. Clinical Relevance Multiple growth factors are involved in the pathogenesis of choroidal neovascularization in age-related macular degeneration.

Aged↗

Atypical serpiginous choroiditis.

Patients with serpiginous choroiditis may demonstrate atypical features. In this series 14 (56%) of 53 patients demonstrated either active choroidal neovascularization (13%) or disciform macular scarring (13%). Despite a slight excess of male subjects, five of seven patients with active choroidal neovascularization were female. Three had bilateral disciform disease. All patients with this complication had visual acuity of 6/60 or less. None were able to be successfully treated by photocoagulation. One patient had secondary branch venous obstruction peripherally, associated with retinal neovascularization. Patients with serpiginous choroiditis are at risk of losing central vision from either an acute active lesion in the macula or choroidal neovascularization occurring at the margin of an inactive chorioretinal scar. Serial examination at regular intervals is recommended to rule out the development of possibly treatable choroidal neovascularization in patients with serpiginous choroiditis.

Choroid↗

Limitations of imaging choroidal tumors in vivo by optical coherence tomography.

BACKGROUND: Optical coherence tomography (OCT) produces two-dimensional cross-sectional images with a longitudinal resolution of 10 microns. Its capacity for imaging retinal structure has been shown in a variety of diseases. There are no reports on its capacity and limitations in imaging choriocapillary and choroidal structures. METHODS: Twenty-two patients with the diagnosis of malignant melanoma of the choroid were submitted to OCT. We used a prototype and a commercial device, both with an 850-nm superluminescent diode with a band-width of 30 nm (reported longitudinal resolution 10 microns). The images were evaluated for retinal thickness, changes in retinal pigment epithelium, subretinal fluid accumulation and changes in choriocapillary or choroidal reflectivity. RESULTS: Retinal edema and detachment found on biomicroscopic examination for fluorescein angiography was detected by OCT in all such cases. In 2 of 22 cases small retinal detachments were detected only by OCT. Tumor extension through the retinal pigment epithelium was not seen in this series, either by biomicroscopy or by OCT. The pattern of choroidal or choriocapillary reflectivity was nonspecifically lower than that of normal choroid, but did not yield any additional information about tumor histology. When normal retina was present, the OCT appearance of a malignant melanoma resembled that of normal choroid. CONCLUSION: OCT may provide information about the retinal structure overlying prominent tumors and the extent of adjacent retinal detachment. In its present state of development, OCT is of little value in the differential diagnosis of choroidal tumors. Its potential value for the follow-up of shallow tumors needs further investigation.

Adult↗

Submacular surgery to remove choroidal neovascularization associated with central serous chorioretinopathy.

PURPOSE: To report the results of submacular surgery for removal of choroidal neovascularization associated with central serous chorioretinopathy. METHODS: Ten eyes of nine consecutive patients with central serous chorioretinopathy and subfoveal or juxtafoveal choroidal neovascularization underwent pars plana vitrectomy with removal of the choroidal neovascular membrane between January 1994 and January 1999. RESULTS: All 10 eyes (nine patients) were followed postoperatively for at least 6 months. The mean postoperative follow-up was 23 months (range, 6 to 56.5 months). The patients were followed for an average of 6.2 months from the time of symptoms to the removal of the choroidal neovascularization. Preoperative mean best-corrected visual acuity was 20/100 (range, 20/25 to 20/400), and postoperative best-corrected mean visual acuity was 20/60 + 2 (range, 20/20 to 20/400). Seven eyes had improved postoperative visual acuity, with an average of 3.4 lines gained. Of the three eyes that had worse acuity, an average of 1.3 lines of visual acuity was lost; final mean postoperative visual acuity was 20/80 + 1 (range, 20/25 to 20/400). Of the six eyes with symptoms of less than 3 months' duration, four had a final visual acuity of 20/50 or better. All three eyes with 20/300 or worse final visual acuity had loss of foveal retinal pigment epithelium after surgery; the remaining eyes had preserved retinal pigment epithelium with a visual acuity of 20/70 or better. Two eyes had intraoperative peripheral retinal tears, and two eyes had recurrence of the choroidal neovascular complex. CONCLUSIONS: The anatomic and visual results in eyes with choroidal neovascularization associated with central serous chorioretinopathy are modestly encouraging and suggest that submacular surgery for choroidal neovascularization in patients with central serous chorioretinopathy is a treatment option that may salvage good macular function in some eyes.

Adult↗

Choroidal capillary and venous congestion in central serous chorioretinopathy.

PURPOSE: Abnormalities in choroidal perfusion have been hypothesized to be causative factors in central serous chorioretinopathy. This prospective study was performed to evaluate changes in the choroidal circulation in cases of central serous chorioretinopathy. METHODS: In 32 consecutive patients with acute or chronic recurrent central serous chorioretinopathy, complete clinical ophthalmologic examinations, fluorescein angiography, and indocyanine green angiography with a scanning laser ophthalmoscope and a digital imaging system were performed. RESULTS: All patients with acute and chronic recurrent central serous chorioretinopathy demonstrated a localized delay in arterial filling followed by choroidal hyperperfusion in the area of the damaged retinal pigment epithelium, frequently associated with dilated capillaries and dilated draining venules in one or more choroidal lobules. These changes corresponded to areas with pigment epithelial detachment or focal leakage from the retinal pigment epithelium found in fluorescein angiography. Furthermore, in some patients, localized choroidal ischemia could be observed in additional areas throughout the central fundus in both diseased eyes and normal fellow eyes. CONCLUSIONS: Delayed arterial filling followed by capillary and venous hyperemia, angiographically appearing as capillary and venous congestion, can be observed frequently in eyes with central serous chorioretinopathy. The results suggested that capillary or venous congestion after ischemia in one or more choroidal lobules might be the reason for the choroidal hyperpermeability associated with central serous chorioretinopathy.

Acute Disease↗

Comparison of fluorescein angiography and indocyanine green angiography for imaging of choroidal neovascularization in hemorrhagic age-related macular degeneration.

PURPOSE: To compare fluorescein angiography and indocyanine green angiography for imaging of choroidal neovascularization located under subretinal hemorrhage, in age-related macular degeneration. METHODS: In a retrospective study, 30 eyes of 30 consecutive patients with hemorrhagic age-related macular degeneration were evaluated with fluorescein angiography and indocyanine green angiography. In each eye, areas of macular hemorrhage, choroidal neovascularization, and total lesion (hemorrhage plus choroidal neovascularization) were measured, and the choroidal neovascularization was categorized as classic, occult, or mixed on fluorescein angiography, and as hot spot, plaque, or combined lesion on indocyanine green angiography. Results of the two techniques were compared for the ability to identify a laser-treatable lesion. RESULTS: Fluorescein angiography showed fluorescence in most cases (28, 93.3%). In 23 cases (76.6%), the fluorescence pattern was compatible with occult choroidal neovascularization. Indocyanine green angiography revealed defined patterns of fluorescence in 27 cases (90%): 12 hot spots, seven plaques, and eight combined hot spots and plaques. Twenty lesions (66.6%) detected by indocyanine green angiography were considered eligible for laser therapy. Nine of them (45%) were extrafoveal. CONCLUSIONS: Indocyanine green angiography is beneficial for imaging choroidal neovascularization located under subretinal hemorrhage. Choroidal neovascularization demonstrated on indocyanine green angiography may be considered eligible for laser treatment. Therefore, indocyanine green angiography should be considered in cases of hemorrhagic age-related macular degeneration.

Choroid↗

Subfoveal choroidal neovascularization in punctate inner choroidopathy. Surgical management and pathologic findings.

PURPOSES: To evaluate submacular surgery for the management of subfoveal choroidal neovascularization in punctate inner choroidopathy, to describe the histopathology and ultrastructure of the excised subretinal tissue, and to propose a staging system that characterizes the development of choroidal neovascularization with associated subretinal fibrosis. METHODS: The authors reviewed the records of five patients (6 eyes) with punctate inner choroidopathy who underwent submacular surgery for subfoveal choroidal neovascularization. Surgical specimens were examined using light and transmission electron microscopy. RESULTS: Visual improvement was noted postoperatively in all six eyes, with follow-up ranging from 8 to 36 months (median, 14 months). Recurrences (6 in 4 eyes) were common. Five of the six recurrences required additional procedures: three were managed surgically, two with laser photocoagulation, and one with observation. "Bridging" of separate foci of choroidal neovascularization resulted in stellate or "dumbbell-shaped" areas of subretinal fibrosis in four of six eyes. Histopathologic evaluation of the excised tissue showed endothelial-lined vascular channels, retinal pigment epithelium, lymphocytes, plasma cells, fibrocytes, collagen fragments, and rarely, outer retinal elements. CONCLUSIONS: Subfoveal choroidal neovascularization in punctate inner choroidopathy may be managed with submacular surgery. Recurrences are common and may result in substantial loss of vision. Choroidal neovascular membranes with an accompanying fibrotic reaction are responsible for the stellate or dumbbell-shaped areas of subretinal fibrosis. No beneficial effect was demonstrated using corticosteroid treatment of the choroidal neovascularization.

Adult↗

Radiation choroidal vasculopathy: an indocyanine green angiography study.

An indocyanine green angiography (ICG-A) study of choroidal vasculature in the irradiated peritumoral zone in 16 eyes treated with 106Ru/106Rh brachytherapy for choroidal melanoma was performed between 4 and 72 months following treatment. The earliest changes observed were peritumoral atrophy of the retinal pigment epithelium and choriocapillaries. With time, the larger choroidal vessels progressively became non-perfused. There was only minimal staining of the smaller vessels. However, at 2 years or more after radiotherapy the larger choroidal vessels showed progressive vascular wall staining with ICG ('perivasculitis') associated with continuing closure. Previous histopathological studies of radiation damage to the choroid have demonstrated atrophy and occlusion of the vasculature but have shown no evidence of vasculitis. It is suggested that ICG staining of the choroidal vascular wall in radiation choroidopathy is due to radiation-induced endothelial cell loss or dysfunction rather than a true vasculitis. Subsequent progression results in vascular occlusion, and development of new clusters of abnormal choroidal vessels.

Angiography↗

Silent occult choroidal vascular abnormalities.

PURPOSE: To describe clinically occult choroidal vascular abnormalities that can be revealed by indocyanine green (ICG) angiography. METHODS: Out of approximately 2,700 patients who underwent ICG angiography, a lesion was incidentally observed in eight eyes of eight patients. In five patients, the ICG study included a second examination taken during artificially induced intraocular hypertension. Examinations were repeated in six patients over a follow-up period ranging from 4 months to 3 years. RESULTS: On ICG angiogram, the choroidal vasculopathy appeared as a round-oval hyperfluorescent area 2-4 disk diameters in size that was located at the temporal vascular arcades in six eyes, at the inferomacular region in one eye, and above the optic disk in one eye. The lesions were not identifiable with funduscopic, fluorescein angiographic, or ultrasonographic examination. The lesions filled at the same time as the choroidal arteries and lost fluorescence in mid-late phase of the ICG angiogram. The ICG series taken during induced intraocular hypertension showed the hyperfluorescent areas originated from choroidal arterial abnormalities giving rise to confluent hyperfluorescent patches. Draining vessels connecting the choroidal vasculopathy with a vortex vein were evidenced in three eyes. A sector of apparent choroidal hypoperfusion downstream from the lesion was present in three eyes. During the follow-up period, the lesions remained occult and with an unchanged ICG angiographic pattern in all patients. CONCLUSION: Some silent occult choroidal vascular abnormalities may be incidentally revealed by ICG angiography. These must be distinguished from ICG imaging of concomitant chorioretinal disorders.

Aged↗

Possibilities and limitations of radioimmunoscintigraphy and conventional diagnostic modalities in choroidal melanoma.

A prospective clinical study to assess the value of immunoscintigraphy with a monoclonal antibody (MoAb) against high molecular weight melanoma associated antigen (225.28S) was performed in 43 patients with choroidal melanoma; in six patients with a lesion suspected of being choroidal melanoma, and in seven patients with a benign lesion simulating a choroidal melanoma. The results of immunoscintigraphy in choroidal melanoma were compared with results of conventional diagnostic modalities like ultrasonography and fluorescein angiography. Planar scintigraphy showed a detection rate of 49% which is comparable with other studies. The detection with scintigraphy was correlated to the size of the choroidal melanoma. The use of single photon computed tomography did not increase the sensitivity of immunoscintigraphy. Ultrasonography yielded a correct diagnosis in 37 of 42 melanomas (88%). With fluorescein angiography a correct diagnosis was obtained in 11 of 30 melanomas (36.6%). The value of immunoscintigraphy with MoAb 225.28S in small choroidal melanomas is limited; its reliability increases in large tumours. Immunohistochemistry with MoAb 225.28S showed antigen expression in 95% of the stained tissue specimens of choroidal melanoma.

Adult↗

Features of abnormal choroidal circulation in central serous chorioretinopathy.

AIMS: To evaluate abnormalities in the choroidal circulation in cases of central serous chorioretinopathy (CSC). METHODS: A complete clinical ophthalmological examination was performed using simultaneous fluorescein and indocyanine green (ICG) angiography with a confocal scanning laser ophthalmoscopy and the digital images analysed in 36 consecutive patients with acute CSC. To quantify the choroidal circulation, the foveal choroidal blood flow was measured in 11 patients using laser Doppler flowmetry. RESULTS: Fluorescein angiography showed focal leakage from the retinal pigment epithelium in all patients. ICG angiography revealed delays in arterial filling in 27 eyes (75%), and fluorescein angiography showed small hypofluorescent points around the leakage in 27 eyes (75%). Abnormal choroidal hyperfluorescence was observed in 30 eyes (83%). The choroidal blood flow in eyes with CSC was 45% lower than in fellow eyes (p<0.01). CONCLUSION: Decreased choroidal blood flow in CSC was demonstrated for the first time. The decreased choroidal blood flow might be correlated with the small, localised hypofluorescent areas, which may indicate non-perfused areas of the choriocapillaris that are frequently seen during ICG angiography.

Adult↗

Drusen and 'choroidal filling defects': a cross-sectional survey.

The present study was designed as a cross-sectional survey to assess the association between soft drusen and 'choroidal filling defects'. Sixty-eight eyes presenting hard drusen and 58 eyes with soft drusen of 126 subjects with an age range of 45-83 years were examined in the present study. Choroidal filling defects were present in 13 out of 68 (19.1%) patients with hard drusen and 29 out of 58 (50%) with soft drusen (chi 2 square = 13.4; p < 0.0001 and an odds ratio = 4.2 with 95% CI 1.9-9.3). Age, ocular and systemic hypertension, and diabetes did not influence the results. The association between soft drusen and choroidal filling defects, found in our study, suggests that these abnormalities are possibly due to changes in the staining or permeability properties of Bruch's membrane rather than to a defect in the choroidal blood supply. Soft drusen and choroidal filling defects may both be caused by an accumulation of hydrophobic material within the Bruch's membrane, which is discrete in the case of drusen and diffuse in the case of choroidal filling defects. Choroidal filling defects and soft drusen may represent useful clinical sign of hydrophobicity of Bruch's membrane.

Aged↗

Duplication of 9P and hyperplasia of the choroid plexus: a pathologic, radiologic, and molecular cytogenetics study.

Duplication of the short arm of chromosome 9 is a rare constitutional abnormality, and the presence of pathologically confirmed hyperplasia of the choroid plexus in one of two cases, and a choroid plexus papilloma in another, raises the possibility of a relationship between the 9p abnormality and abnormal growth of the choroid plexus. Molecular cytogenetic analysis using fluorescence in situ hybridization was used for detection of chromosome 9-derived material in various formalin-fixed choroid plexus abnormalities. Extra copies of chromosome 9-derived material was found in the hyperplastic choroid plexus and in a choroid plexus carcinoma. These findings suggest that there may be an association between duplication of chromosome 9 material and abnormal development of the choroid plexus.

Carcinoma↗

The potential for adjuvant radiotherapy in choroidal melanoma.

Controversy exists regarding the efficacy of enucleation in the management of choroidal melanoma. The effect of any form of ocular therapy on the prevalence of choroidal melanoma metastases is unknown. In a number of other systemic tumors, preoperative irradiation may lessen the occurrence of widespread disease. This type of therapeutic approach might be useful in the management of high-risk choroidal melanoma. In preliminary studies using 2,000 rad of preenucleation irradiation in patients with choroidal melanoma who have a poor prognosis, we have not observed notable morbidity. There are a number of limitations to the use of this adjunct therapy in choroidal melanoma management; however, a prospective national randomized trial of preenucleation irradiation in patients with high-risk choroidal melanoma might allow us to indirectly determine the effect of enucleation on the prevalence of choroidal melanoma metastases as well as delineate the potential role of this type of adjunct therapy in ocular melanoma.

Choroid Neoplasms↗

Is genetic amniocentesis warranted when isolated choroid plexus cysts are found?

Our aim was to evaluate the prevalence of trisomy 18 in the setting of isolated fetal choroid plexus cysts and then to consider the risk of trisomy 18 versus the risks of genetic amniocentesis. Fetuses with choroid plexus cysts were prospectively obtained from a total mid-trimester population of 18861 fetuses with known outcomes. If the fetuses had trisomy 18, they were part of the study group and part of the control group if they had normal karyotypes. Scans were retrospectively reviewed for the characterization of cysts according to size, laterality, and appearance (simple or complex echo patterns). Chi-square analysis of contingency tables of results was performed. 208/18861 (1.1 per cent) fetuses had choroid plexus cysts. 201/208 (96.6 per cent) were normal fetuses or newborns, while 7/208 (3.4 per cent) of the fetuses with choroid plexus cysts had trisomy 18. Overall, 16 fetuses had trisomy 18 and seven (44 per cent) of these had choroid plexus cysts. 0/16 fetuses had choroid plexus cysts as the only sonographic finding. Although laterality or complexity of the cysts did not correlate with the presence or absence of a cytogenetic abnormality, cysts > or = 10 mm were more often associated with trisomy 18 than with a normal karyotype (P < 0.01). We conclude that the discovery of choroid plexus cysts in otherwise normal fetuses in the late second trimester does not by itself justify the risks of genetic amniocentesis.

Adult↗