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Choroid plexus cysts: infant and early childhood developmental outcome.

OBJECTIVE: To determine the infant and early childhood developmental outcome associated with choroid plexus cysts diagnosed prenatally. METHODS: Between January 1990 and August 1995, 8270 women underwent second-trimester ultrasound examinations. All women whose fetuses were diagnosed as having choroid plexus cyst(s) underwent ultrasonographic detailed anatomy survey, were offered fetal karyotyping, and were followed with serial ultrasounds. Fetal karyotype, associated structural anomalies, maternal serum triple analyte screen, neonatal outcomes, and infant and early childhood developmental milestones were recorded. The children were followed subsequently, and developmental assessment was performed with a modified Denver II Developmental Screening Test. RESULTS: A diagnosis of choroid plexus cyst was made in 89 fetuses (1.1%). The mean (+/-standard deviation [SD]) gestational age at diagnosis was 18.2 +/- 1.9 weeks (range 15-22). The cysts varied in size and laterality, with a mean (+/-SD) size of 5.9 +/- 3.3 mm (range 1-23). Three of the 61 women who underwent testing for fetal karyotype (4.9%) had abnormal karyotypes identified. All three karyotypes were trisomy 18, and all three trisomy 18 fetuses had additional sonographic abnormalities. All 28 women who chose not to undergo fetal karyotypic analysis delivered phenotypically normal infants. Infant and childhood developmental follow-up was performed on 76 children with cysts diagnosed prenatally. The mean (+/-SD) length of childhood follow-up was 35.5 +/- 16.2 months (range 12-82). All 76 children were found to be developmentally normal by the Denver II Developmental Screening Test. CONCLUSION: These observations suggest that the finding of isolated choroid plexus cysts is not associated with delayed infant and early childhood development or an increased risk of abnormal karyotype. The presence of isolated choroid plexus cysts does not warrant intensive infant and early childhood follow-up.

Brain Diseases↗

Two discrete choroidal melanomas in an eye with ocular melanocytosis.

We report a case of multifocal choroidal melanoma arising in an eye with ocular melanocytosis and review the pertinent literature. A 63-year-old Caucasian male with ocular melanocytosis in the left eye was found to have two discrete choroidal melanomas in the same eye. Histopathology of the enucleated eye confirmed the diagnosis of two discrete choroidal melanomas of mixed cell type within a region of choroidal melanocytosis. It is estimated that 1 in 160,000 patients with unilateral ocular melanocytosis may develop two uveal melanomas, based on the reported data. On the basis of random chance, patients with two melanomas in the same eye would be expected to have approximately 1000-fold greater likelihood of underlying ocular melanocytosis than the general Caucasian population. In conclusion, multifocal choroidal melanoma is rare and may be related to underlying ocular melanocytosis.

Choroid Neoplasms↗

Immunohistochemical study of rabbit choroidal innervation.

Immunocytochemical methods with antibodies to the light (68 kDa), medium (160 kDa), and heavy (200 kDa) chain subunits of the neurofilament triplet have been used to visualize neuronal structures in rabbit choroids. Choroidal nerve fibers were present in the suprachoroid and vascular laminae and absent in the choriocapillary layer. These fibers may be classified as perivascular and intervascular. Perivascular fibers surround all arterial and venous blood vessels and form a network; these fibers were labeled with the three NF antibodies, although they were more easily visualized with anti NF-160 and anti NF-200 than anti NF-68. Intervascular fibers formed two groups. The first group consisted of fibers situated between the blood vessels and parallel to the blood vessel wall surface (paravascular fibers); these fibers were better observed using anti NF-160 and NF-200 than anti NF-68. The second group consisted of fibers which travel the entire length of the choroid until they reach the nerve plexus of the ciliary body (long tract fibers). The plexus was observed with anti NF-68, anti NF-160 and anti NF-200; however, the long tract fibers were more clearly visualized with anti NF-160 and anti NF-200 than with anti NF-68. Two types of choroidal cell were also labeled: ganglion cells and melanocytes. Ganglion cells are small, scarce neurons situated in the peripheral choroid; they were labeled with anti NF-160 and anti NF-200. The melanocytes were only labeled with anti NF-200 and they were the only non neuronal structure visualized using antibodies against neurofilaments.

Animals↗

Effect of arginine vasopressin on blood vessels of the perfused choroid plexus of the sheep.

The perfused sheep choroid plexus was used to evaluate the response of the plexus blood vessels to systemically administered arginine vasopressin (AVP). AVP was found to decrease the diameter of the choroid plexus arterioles with a maximum change of 28 +/- 5% (mean +/- S.E.) at a plasma peptide concentration of 10(-7) M. This effect was blocked by the specific V1-vasopressinergic antagonist, d(CH2)5Tyr(Me)AVP. In contrast, venules were found not to show any appreciable response to AVP. Plasma AVP concentrations necessary to evoke a significant response of the choroid plexus arterioles are much higher than the highest plasma peptide levels observed in different physiological or pathophysiological situations. Some indirect evidence suggests, however, that AVP might be released within the choroid plexus from the vasopressinergic synaptic terminals, thus reaching a considerably high local concentration. It is possible then that the plexus vessels' tone could be controlled by the putative vasopressinergic neuronal fibers ending in the choroid plexus.

Animals↗

Lateral ventricle choroid plexus papilloma in childhood: management and complications.

BACKGROUND: A review of the choroid plexus papilloma of the lateral ventricle in the pediatric age group is presented. The characteristic clinical features, imaging studies, preoperative, and operative approaches, as well as complications, will be included. METHODS: Among a group of 24 patients with ventricular choroid plexus papilloma treated by the authors over a 12-year period, seven patients were selected for this study. The selection included patients under the age of 8 years at the time of their presentation with choroid plexus papilloma of the lateral ventricle. Adult patients and children with choroid plexus papilloma at other sites or choroid plexus carcinoma were excluded. At least a 30-month follow-up period was available for all patients except one. Preoperative and postoperative imaging studies confirming total tumor resection were available for all patients. RESULTS: Developmental delays and regression were the most consistent clinical features. Magnetic resonance imaging (MRI) proved to be the diagnostic test of choice. The addition of magnetic resonance angiogram (MRA) obviates the need for angiography. Our attempts at tumor embolization failed. CONCLUSION: An intersulcal splitting approach to the lateral ventricular trigone, combined with a perioperative external ventricular drainage, may be of value in the avoidance of symptomatic subdural effusions.

Cerebral Angiography↗

Effects of ischemia on prostaglandin H synthase-2 expression in piglet choroid plexus.

We examined effects of ischemia on expression of prostaglandin H synthase-1 (PGHS-1) and prostaglandin H synthase-2 (PGHS-2) in piglet choroid plexus. Ten minutes of ischemia was induced by increasing intracranial pressure. Whole choroid plexus was removed and fixed and/or frozen after 1, 2, 4, and 8 h of recovery from anoxic stress. In addition, tissues were obtained from untreated animals or from time control animals. Tissues were analyzed for mRNA, using RNase protection assays, and for proteins, using immunohistochemical approaches. Limited, but detectable PGHS-2 immunoreactivity was present in choroid plexus under normal conditions, and there was no difference between time-control and non-treated animals. Further, PGHS-2 mRNA increased by 2-4 h after ischemia, and enhanced immunoreactivity for PGHS-2 was present at 8 h after ischemia. Enhanced immunoreactivity for PGHS-2 was present in vascular endothelial cells as well as cuboidal epithelial cells and macrophages. In contrast, PGHS-1 mRNA did not increase following ischemia. We conclude that PGHS-2 is present in piglet choroid plexus under normal conditions and that ischemia increases levels of PGHS-2 in choroid plexus.

Animals↗

Adjuvant indocyanine green in transpupillary thermotherapy for choroidal melanoma.

OBJECTIVE: To determine the effect of indocyanine green (ICG) injection on the regression pattern of choroidal melanomas treated with transpupillary thermotherapy (TTT). DESIGN: Prospective, randomized, controlled study. PARTICIPANTS: Of the 60 patients with posterior pole choroidal melanoma, 30 were managed with TTT alone (control group), and 30 received adjuvant ICG before TTT. METHODS: Subjects with selected choroidal melanoma were treated with TTT by using infrared radiation delivered from the diode laser. For those patients randomized to ICG administration, the start of TTT was based on the onset of fluorescence documented on ICG angiography. MAIN OUTCOME MEASURES: Final tumor thickness (1.5 mm) and temporal tumor thickness regression. RESULTS: The mean initial tumor basal diameter was 7.7 mm, and tumor thickness was 2.9 mm in the TTT treatment group. Tumor dimensions were initially 7.8 mm in the base and 3.1 mm in thickness in the TTT + ICG treatment group. During a median follow-up of 30 months (range, 6-49 months), the mean tumor thickness gradually decreased to 2.3 mm at month 3 and to 1.9 mm at month 6 after the initial session in the TTT treatment group and to 2.5 mm at month 3 and 2.2 mm at month 6 in the TTT + ICG treatment group. In the TTT treatment group, the reduction of tumor thickness was 19% at month 3 and 30% at month 6. In the TTT + ICG treatment group, the reduction of tumor thickness was 22% at month 3 and 31% at month 6. There were 12 tumors (40%) in the TTT treatment group and 15 (50%) in the TTT + ICG treatment group that measured 1.5 mm in thickness, with a flat ophthalmoscopic appearance. Tumor control was achieved in 55 tumors (92%). Two patients developed tumor recurrence in the TTT treatment group, and three developed recurrence in the TTT + ICG treatment group. A smaller initial tumor thickness, more TTT sessions, and a tumor location other than temporal were statistically predictive of a final tumor thickness of 1.5 mm. There was no apparent effect of ICG administration before TTT on the final tumor thickness and temporal tumor thickness regression. CONCLUSIONS: Despite the efficacy of TTT in the management of selected choroidal melanomas, adjuvant ICG administration before each TTT session does not seem to be beneficial in their regression pattern. Further research in vivo with a larger sample of patients and longer follow-up will be necessary to determine the role of ICG in TTT for choroidal melanomas.

Adult↗

Bilateral choroidal folds and optic neuropathy: a variant of the crowded disk syndrome?

PURPOSE: To describe the clinical features of the syndrome that includes bilateral choroidal folds and optic neuropathy and to propose a novel etiology to explain this presentation. DESIGN: Observational small case series. PARTICIPANTS: Three patients. METHODS: Three patients received an extensive clinical work-up including complete ocular examination, A and B scan ultrasonography, Humphrey visual field analysis, and imaging of the brain and orbit. Neurological consultation was obtained in each patient two of whom underwent lumbar puncture. The findings of this detailed investigation are reported. RESULTS: Each patient demonstrated bilateral choroidal folds with optic disk congestion and leakage in one eye and optic atrophy in the fellow eye. Extensive ocular and extraocular investigation failed to uncover a specific etiology. Pseudotumor cerebri, a well-documented cause of papilledema and choroidal folds, was ruled out in each case. Each patient did demonstrate variable degrees of hyperopia and shortened axial lengths. CONCLUSIONS: The syndrome of bilateral choroidal folds and optic neuropathy may have various etiologies. Pseudotumor cerebri needs to be definitively ruled out. Normal neuro-ophthalmological investigation including lumbar puncture may indicate an alternative cause. Idiopathic acquired hyperopia in middle-aged patients who are hyperopic with shortened axial lengths may be associated with choroidal folds and a constricted scleral canal causing optic disk congestion and complicated by nonarteritic anterior ischemic optic neuropathy due to a crowded disk.

Aged↗

Amelanotic choroidal nevi.

A review of 373 patients with presumed choroidal nevi revealed 19 (5.1%) to have largely or totally amelanotic nevi. All patients but one with amelanotic nevi were white, and all were 48 years of age or older. Each lesion was located posterior to the equator, had a mean diameter of 3.2 mm, and was less than or equal to 1 mm in elevation. Intravenous fluorescein angiography most often disclosed early and late hyperfluorescence, corresponding to the amelanotic areas of the nevus. No lesions were observed to grow during a mean 14-month follow-up period. The differential diagnosis of amelanotic nevi of the choroid is discussed and includes amelanotic malignant melanoma, cavernous hemangioma of the choroid, metastatic cancer to the choroid, posterior scleritis, choroidal osteoma, and hypopigmented congenital hypertrophy of the retinal pigment epithelium.

Aged↗

Visual loss associated with choroidal nevi.

Twenty-two of 206 patients (11%) with choroidal lesions diagnosed clinically as nevi of the posterior fundus had decreased visual acuity attributable to the lesion. The decreased visual acuity was secondary to serous foveal detachment (50%), presumed photoreceptor degeneration without serous detachment (42%), and subretinal choroidal neovascularization overlying the nevus (8%). Visual loss associated with a small melanotic choroidal tumor does not necessarily imply that the tumor is a choroidal malignant melanoma as 88% of the small melanotic choroidal tumors causing visual impairment did not grow during an average follow-up period of 25 months, and the great majority of these tumors were probably true spindle cell nevi. However, three tumors (12%), initially categorized as "suspicious nevi," enlarged during follow-up and were recategorized as malignant melanomas.

Adolescent↗

Computed tomography in choroidal detachment.

Computed tomography proved valuable in localizing and differentiating serous or hemorrhagic choroidal detachment and scleral infolding. The mean attenuation values (CT numbers) in the region of fresh hemorrhagic choroidal detachment were 74 Hounsfield units (HU). The 90% confirmation limit of the mean was 55 to 84 HU. For serosanguineous choroidal detachment, the mean was 50 HU, with a 90% confirmation limit of 46 to 54 HU. For serous choroidal detachment the mean was 46 HU, with a 90% confirmation limit of 39 to 53 HU. Inflammatory choroidal detachment showed uveoscleral enhancement, which in cross section appeared as a ring (ring sign), and subchoroidal or intrauveal accumulation of fluid. Ocular hypotony produced the characteristic CT "umbrella sign."

Adolescent↗

The treatment of serous macular detachment secondary to choroidal melanomas and nevi.

The authors successfully treated with laser photocoagulation five patients who had a serous macular detachment secondary to leakage from a pigmented choroidal tumor. Three patients had dye leakage on fluorescein angiography from a choroidal neovascular membrane. The subretinal fluid resolved in all three patients after the neovascular membrane was obliterated by either krypton red (2 patients) or argon green (1 patient) laser photocoagulation. The other two patients had prominent leakage from a localized choroidal neovascular membrane as well as mild diffuse leakage over the entire tumor. Focal argon laser treatment to the neovascular membrane alone did not cause permanent resolution of the subretinal fluid. The fluid did resolve, however, after the entire area of diffuse leakage was retreated with photocoagulation. In one of the patients who received both focal and confluent laser treatment, the choroidal tumor grew in a collar-button fashion through Bruch's membrane at the original site of focal treatment. This eye was enucleated, and results of histopathologic examination showed a choroidal melanoma.

Aged↗

Transpupillary thermotherapy in the management of choroidal melanoma.

BACKGROUND: Several methods of treatment have been used for choroidal melanoma. The purpose of this report is to evaluate the effectiveness of transpupillary thermotherapy using near-infrared radiation to treat choroidal melanoma. METHODS: A prospective study was conducted to evaluate the clinical features, treatment results, and complications of patients with choroidal melanoma who were treated with transpupillary thermotherapy delivered over one to four sessions and followed for at least a 6-month period. All treated tumors had either documentation of growth or ophthalmoscopic risk factors for future growth and/or metastasis. RESULTS: There were 17 patients with choroidal melanoma treated with transpupillary thermotherapy. The mean tumor size before treatment was 6.6 mm in base and 3.0 mm in thickness. The tumor margin was a mean of 2.3 mm from the optic disc and 2.7 mm from the foveola. Seven tumors (41%) touched the optic disc margin and three (18%) were under the fovea. The tumor responded to treatment in all patients, with a decrease in thickness and resolution of associated subretinal fluid. At a minimum of 6 months of follow-up, the mean tumor thickness was 1.7 mm, and the tumor site was a residual chorioretinal scar with partial visibility of the sclera in all patients. Despite the proximity to the optic disc and foveola, the final visual acuity was the same or improved in ten eyes (59%) and decreased in seven (41%). The improved vision was due to resolution of subfoveal fluid, whereas the decreased vision was primarily the result of treatment in the fovea with ultimate retinal vascular occlusion or preretinal traction. Although long-term follow-up is not yet available, there were no patients with tumor recurrence or tumor metastases. CONCLUSIONS: This preliminary study demonstrates that transpupillary the thermotherapy appears to be an effective treatment for selected small choroidal melanomas and may be a particularly useful modality for treating those tumors near the foveola and optic disc. Longer follow-up is necessary to assess for local recurrence and the impact of treatment on survival.

Adult↗

Tissue plasminogen activator-assisted surgical excision of subfoveal choroidal neovascularization in age-related macular degeneration: a randomized, double-masked trial.

OBJECTIVE: To determine whether lysing subretinal fibrin with tissue plasminogen activator (t-PA) before excising subfoveal choroidal neovascularization improves visual acuity in patients with age-related macular degeneration. DESIGN: Randomized, double-masked trial. PARTICIPANTS: Eighty eyes of 80 patients with subfoveal choroidal neovascularization secondary to age-related macular degeneration were studied. INTERVENTION: Each eye underwent pars plana vitrectomy and received a subretinal injection of t-PA or balanced salt solution (BSS) before the neovascular membrane was excised. Preoperative and postoperative protocol refraction, ophthalmic examination, color photography, and fluorescein angiography were performed in all 80 eyes. MAIN OUTCOME MEASURES: Visual acuity and fluorescein angiographic evidence of leakage after 1 year. RESULTS: Visual acuity did not differ between the t-PA group (n = 40) and the BSS group (n = 40), and median best-corrected visual acuity was 20/320 for both groups (P = 0.38). Changes in visual acuity from baseline were also equal, with a median loss of 1 line in each group (P = 0.78). Patients whose initial visual acuity was 20/250 or less were more likely to improve by 2 or more lines (P = 0.01) and less likely to lose 2 or more lines (P < 0.001). Patients with choroidal neovascularization of at least 4 disc areas were more likely to improve by 2 or more lines (P = 0.02) and less likely to lose 2 or more lines (P < 0.001). After 1 year, choroidal neovascularization was present in seven of the t-PA eyes and in eight of the BSS eyes (P = 0.78). CONCLUSIONS: With current surgical techniques, the use of t-PA before surgical excision of subfoveal choroidal neovascularization is of no visual or anatomic benefit to patients with age-related macular degeneration.

Aged↗

Annular peripheral choroidal detachment simulating aqueous misdirection after glaucoma surgery.

PURPOSE: The purpose of the study was to define a newly recognized complication after glaucoma surgery and to recommend a therapeutic regimen. METHODS: Eighteen patients diagnosed initially as having aqueous misdirection after glaucoma surgery, but who subsequently were found by ultrasonography to have an annular peripheral choroidal detachment that resulted in secondary angle closure glaucoma, were studied. Ten of these patients were treated with topical cycloplegics and corticosteroids, and 8 were treated with drainage of suprachoroidal fluid. Outcomes of these two treatment methods were compared. RESULTS: Annular peripheral choroidal detachment reliably was diagnosed with ultrasonography. Of the variables studied, time elapsed before resolution of the annular peripheral choroidal detachment was noted to be statistically significant (P < 0.00005). Immediate resolution followed drainage of suprachoroidal fluid, whereas a mean of 19.6 days was required for resolution after medical therapy. CONCLUSIONS: Annular peripheral choroidal detachment should be considered in the differential diagnosis of a flat or shallow anterior chamber with normal or high intraocular pressure after glaucoma surgery. The diagnosis of annular peripheral choroidal detachment can be confirmed most reliably by ultrasonography. Medical therapy is as effective as is surgery, although a significantly longer time to resolution is required.

Adult↗

The prognosis of idiopathic choroidal neovascularization in persons younger than 50 years of age.

OBJECTIVE: To examine the prognosis of idiopathic choroidal neovascular membranes in patients younger than 50 years of age at the time of diagnosis. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Eighteen consecutive patients from a university clinic (20 eyes) with idiopathic choroidal neovascularization in the macular area. All patients diagnosed between 1979 and 1996 were included; follow-up time varied from 5 to 187 months (mean, 55 months). INTERVENTION: Treatment was conservative. All patients were informed about argon laser photocoagulation, but in the majority of cases, patients were not encouraged to undergo such treatment. Treatment was given on patient's request. MAIN OUTCOME MEASURES: The dimensions of the choroidal neovascular membrane and its distance to the foveola were determined by computerized image analysis and their correlation with final visual acuity determined. RESULTS: Fifteen (75%) of the 20 eyes retained a visual acuity of 20/60 or better. In ten eyes (50%), acuity was 20/25 or better. Six eyes had been treated with laser photocoagulation; only one had a membrane located 200 microns or more from the foveola at the time of treatment. The outcome for treated eyes was no better than for untreated eyes, although the two groups were not directly comparable. In no eye did an initially juxtafoveal or extrafoveal membrane continue to grow under the foveola. CONCLUSIONS: The prognosis of idiopathic choroidal neovascularization was much better than reported previously for eyes with choroidal membranes associated with age-related macular degeneration. A conservative treatment regimen should be considered. The authors have no reason to believe that laser photocoagulation would have improved the outcome in the nontreated eyes.

Adult↗

Primary vitrectomy for rhegmatogenous retinal detachment associated with choroidal detachment.

OBJECTIVE: This study aimed to report the success rate of primary vitrectomy, scleral buckling, and oral steroids in eyes with combined rhegmatogenous retinal detachment (RRD) and choroidal detachment (CD) and to compare these results with those reported in the literature for similar cases managed by scleral buckling alone. DESIGN: Consecutive case series. PARTICIPANTS: Twenty-one eyes of 21 consecutive patients with primary RRD associated with CD were examined. INTERVENTION: All patients were treated with systemic corticosteroids before surgery. Pars plana vitrectomy with scleral buckling or encircling or both were performed. MAIN OUTCOME MEASURES: Retinal reattachment rate and resolution of choroidal detachment were measured. RESULTS: At mean follow-up of 11.4 months, retinal reattachment was attained in 19 eyes (90.5%) compared to less than 53% reported in the literature for scleral buckling alone. With preoperative oral steroids, choroidal detachment regressed completely in 13 eyes (61.9%). In the remaining eyes with persistent choroidal detachment, suprachoroidal fluid was drained during vitrectomy. CONCLUSION: Aggressive treatment with oral steroids followed by pars plana vitrectomy and scleral buckling or encircling is recommended instead of scleral buckling alone in the management of combined primary rhegmatogenous retinal detachment and choroidal detachment.

Administration, Oral↗

Stereotactic radiation therapy for malignant choroidal tumors: preliminary, short-term results.

PURPOSE: To evaluate the role of stereotactic radiation therapy (SRT) in the treatment of malignant choroidal tumors. DESIGN: Prospective, noncomparative case series. PARTICIPANTS: Ten patients with unifocal choroidal metastasis (three lung carcinoma, three breast carcinoma, three colon carcinoma, one cutaneous melanoma) and five patients with primary choroidal melanoma underwent single-dose or fractionated SRT. METHODS: Before treatment, computed tomography (CT) scans of the orbit were obtained with the patient wearing an individualized immobilization mask. An integrated macro-CCD-camera system viewed the eye for detection of movements. Three-dimensional computer-based treatment planning was carried out. Dose distribution was calculated and displayed in isodose lines on the CT data set. For SRT, a dedicated stereotactic linear accelerator (6 MV) was used. Total doses for choroidal metastases were 12 to 20 Gy in a single dose or 30 Gy over 10 days (3 Gy each session), and total doses for choroidal melanoma were 50 Gy over 5 or 10 days (10 or 5 Gy each session). MAIN OUTCOME MEASURES: Best corrected visual acuity (ETDRS-chart), biomicroscopy, ultrasound examination, fluorescein angiography, and magnetic resonance imaging (MRI) were performed before treatment and at regular intervals after completion of SRT. RESULTS: During a follow-up period from 1 to 34 months (median, 6.5 months), local tumor control was achieved in all eyes. A decrease in tumor size on ultrasonography or MRI was noted in eight patients. No persistent side effects were observed during follow-up. CONCLUSIONS: Stereotactic radiation therapy allows steep dose gradients outside the target volume by minimizing the field of exposure. Thus only low radiation doses affect surrounding radiosensitive ocular structures. Our initial findings suggest that this technique may be effective in controlling tumor growth. Further studies are needed to compare treatment efficacy and safety with conventional treatment methods.

Adult↗