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[A choroid plexus papilloma of the third ventricle in the neonatal period--a case report].

The incidence of choroid plexus papilloma is about 0.5% of all intracranial tumors, but choroid plexus papilloma in the third ventricle is rare. Fortuna reported 56 cases of choroid plexus papilloma of the third ventricle in 1979. Among them, the neoplasm of the third ventricle in the neonatal period was not found. This report is a case of choroid plexus papilloma of the third ventricle in the neonatal period. A 34 day-old female was admitted to our service with complaints of head enlargement, vomiting, and convulsive seizures on January 8, 1981. Enlarged head had been noticed by her family within the first two weeks. On admission, a marked congenital hydrocephalus was diagnosed by CT scan with symmetrical dilated ventricles and no abnormal high or low density area, and V-P shunt was performed on the next day. But several days later, she suffered from progressive abdominal distension, which was disclosed due to CSF overproduction by a choroid plexus papilloma. When the tumor was recognised by enhanced CT scan, her general condition was too poor to attempt a surgical procedure, and she died on March, 19, 1981. Histologically the neoplasm was a typical choroid plexus papilloma of the third ventricle. So far as we know, this case is the first one of the choroid plexus papilloma of the third ventricle in the neonatal period.

Cerebral Ventricle Neoplasms↗

Choroid plexus hemorrhage in premature neonates: recognition by sonography.

In 34 consecutive infants admitted to the neonatal intensive care unit with birth weight of less than 1,500 g, 80 cranial real-time sonograms were obtained to determine the incidence of choroid plexus hemorrhage. Choroid plexus hemorrhage was diagnosed only in the absence of germinal matrix hemorrhage. Diagnostic criteria included choroid plexus nodularity, enlargement (greater than 12 mm in anteroposterior diameter), or asymmetry between right and left (greater than 5 mm). Ipsilateral intraventricular clots or occipital horn dilatation supported the diagnosis of choroid plexus hemorrhage in most cases. Choroid plexus hemorrhage appeared to be the sole bleeding site in 10 (59%) of the 17 patients with intracranial hemorrhage. Hemorrhage in the region of the caudate nucleus was seen in the other seven cases (41%). Ventricular dilatation and/or intraventricular hemorrhage accompanied nine (90%) of the 10 cases of choroid plexus hemorrhage. This study suggests that in very low-birth-weight premature neonates, the choroid plexus may be a more frequent site of intracranial hemorrhage than previously believed.

Cerebral Hemorrhage↗

Differential S100 beta expression in choroidal and skin melanomas: quantitation by the polymerase chain reaction.

PURPOSE: S100 beta, a member of a calcium-binding protein family (S100s), is an important clinical marker for skin melanoma. In contrast, uveal melanomas appeared to express S100 beta protein less frequently and to a lesser degree. This study was performed to verify and extend this finding to the mRNA level. METHODS: A quantitative polymerase chain reaction (PCR)-based method was used. A ratio, comparing the S100 beta PCR fragment to that of beta-actin (an internal reference gene), was generated to compare S100 beta mRNA expression among samples. RESULTS: The ratios for skin melanomas (1.2 to 3.9; three tissues and two cell lines) were significantly higher than that for choroidal melanomas (0.1 to 0.63; seven of eight primary tumors and four of four cell lines). Only one choroidal melanoma biopsy had a ratio greater than 1. The PCR products from choroidal melanoma were identical in size and sequence to the S100 beta, as determined by gel electrophoresis and RNA conformational polymorphism. Because the ratios were also low in choroidal melanoma cell lines, the S100 beta phenotype appears to be genetically stable. CONCLUSION: S100 beta is differentially expressed at the RNA and protein levels by skin and choroidal melanomas, which are derived from distinct populations of melanocytes. However, choroidal melanomas expressing little or no S100 beta were significantly stained by antiserum specific for the S100 protein family. Taken together, these data suggest that choroidal melanocytes express another, perhaps even novel, S100 protein(s).

Actins↗

Intracranial hypertension and the syndrome of acquired hyperopia with choroidal folds.

An idiopathic syndrome of acquired hyperopia with choroidal folds has been characterized. Orbital imaging correlates of this syndrome include flattening of the posterior globe and distention of the perioptic subarachnoid space. The mechanism responsible for the clinical and radiographic findings of this syndrome is undefined. Two patients with unusual presentations of papilledema are reported whose clinical and radiographic findings were otherwise identical to those described in the idiopathic syndrome of acquired hyperopia with choroidal folds. One patient had unilateral disc edema and bilateral choroidal folds. The other patient had bilateral choroidal folds observed 2 years before he developed papilledema in both eyes. Both patients had intracranial hypertension, idiopathic in the first, and related to severe chronic obstructive pulmonary disease and cor pulmonale in the second. A third patient is also described who had typical clinical and orbital imaging findings of idiopathic unilateral acquired hyperopia with choroidal folds. He was also found to have mild intracranial hypertension. Intracranial hypertension can cause acquired hyperopia and choroidal folds and may be the underlying mechanism in some patients with what appears to be idiopathic acquired hyperopia with choroidal folds.

Adult↗

Choroid plexus size in young children with Sturge-Weber syndrome.

PURPOSE: To assess the size of the choroid plexus in young children with unilateral and bilateral Sturge-Weber syndrome. METHODS: Subjects included 15 children 4 years old or younger with Sturge-Weber syndrome. Eleven cases were unilateral and four were bilateral. Unilateral or bilateral involvement was determined by the distribution of abnormal leptomeningeal enhancement on MR images. The diameters of the choroid plexus were measured on contrast-enhanced axial MR images. The choroid plexus of the affected and unaffected sides in these cases were compared with those of 15 age-matched children without Sturge-Weber syndrome who were being examined for seizures. RESULTS: Our results show a wide variation in the size of the choroid plexus in children with Sturge-Weber syndrome; however, plexus associated with a hemisphere affected by Sturge-Weber syndrome were significantly larger than those on the unaffected side and in the age-matched control group. The size of the choroid plexus was positively correlated with the extent of leptomeningeal involvement as demonstrated by abnormal contrast enhancement. CONCLUSION: The choroid plexus is enlarged early in the course of Sturge-Weber syndrome in both unilateral and bilateral cases. There is a positive correlation between choroid plexus size and extent of leptomeningeal involvement in children with Sturge-Weber syndrome.

Child, Preschool↗

A fluorescent vesicle system for the measurement of blood velocity in the choroidal vessels.

BACKGROUND AND OBJECTIVE: Evaluation of the pathophysiologic mechanisms involved in the choroidal circulation is difficult because of the presence of the retinal pigment epithelium. The authors have developed a technique for measuring blood velocity in individual choroidal vessels using the scanning laser ophthalmoscope and intravenously injected indocyanine green encapsulated in multilamellar vesicles. In this report, the authors describe their attempts to measure choroidal blood velocity in three different regions of the choroid of the primate eye. MATERIALS AND METHODS: A video-to-digital processor was used to digitize the video images of the choroid of a monkey at a sample rate of 1/30 of a second. The blood velocity in the selected choroidal vessel segments was calculated by measuring the distance the liposome traveled in a given time. RESULTS: The average blood velocities were 5.16 mm/s under the macula, 4.04 mm/s in the vortex vein system, and 2.03 mm/s in the choroidal circulation nasal to the optic nerve. CONCLUSION: This technique is an effective method of measuring blood velocity in a primate model and has several important advantages compared with the dye dilution technique.

Animals↗

-Choroidal detachment associated with rhegmatogenous retinal detachment: a risk factor for postoperative PVR?.

PURPOSE: To determine whether preoperative choroidal detachment associated with primary rhegmatogenous retinal detachment is an independent risk factor for postoperative PVR. MATERIAL AND METHODS: One hundred eighty nine primary rhegmatogenous retinal detachments in 187 consecutive patients, initially operated on by the same surgeon, were enrolled in a prospective study. Ten eyes showed preoperative choroidal detachment associated with retinal detachment. Owing to the small number of eyes with preoperative choroidal detachment, we conducted the statistical analysis using the calculation of relative risk. RESULTS: The correlation between preoperative choroidal detachment and preoperative PVR grade C-D was statistically significant (RR: 9.03; confidence interval: 2.91-28). Postoperative PVR occurred, or recurred, in one of the 10 eyes with preoperative choroidal detachment (10%), and 10 of the 179 eyes with no choroidal detachment (5.58%). The difference between the two groups is not statistically significant (RR: 1.79; confidence interval: 0.25-12.64). All eyes which developed postoperative PVR showed one or two recognized independent risk factors for postoperative PVR (preoperative PVR grade C-D and/or retinal tears > or = 90 degrees). CONCLUSION: With the treatment modalities used in the present series (preoperative and postoperative steroids, and vitreoretinal microsurgery) preoperative choroidal detachment associated with primary rhegmatogenous retinal detachment no longer appears to be an independent risk factor for postoperative PVR.

Adult↗

Changes in apical organization of choroidal cells in rats adapted to spaceflight or head-down tilt.

Structural changes observed in choroid plexuses from rats dissected aboard a space shuttle, on day 13 of an orbital flight (NASA STS-58 mission, SLS-2 Experiments) demonstrated that choroidal epithelial cells display a modified organization in a microgravitational environment. Results were compared with ultrastructural observations of choroid plexus from rats maintained under anti-orthostatic restraint (head-down tilt) for 14 days. In both experiment types, the main alterations observed by transmission electron microscopy, at the level of choroidal epithelial cells from the third and fourth ventricles, concerned the formation and the organization of apical microvilli, whereas pseudopod-like structures appeared. Immunocytochemical distribution of ezrin, a cytoskeletal protein involved in apical cell differentiation in choroid plexus, confirmed the structural alteration of microvilli in head-down tilted rats, Kinocilia tended to disappear from the apical surface, suggesting a partial loss of cell polarization. In addition, large amounts of clear vesicles were gathered in the apical cytoplasm of choroidal epithelial cells. Disorganization of apical microvilli accumulations of apical vesicles and partial loss of cell polarity showed that long-stays in weightlessness induced alterations in the fine structure of choroid plexus, consistent with a marked reduction of cerebrospinal fluid production.

Adaptation, Physiological↗

Morphometric analysis of the choroid, Bruch's membrane, and retinal pigment epithelium in eyes with age-related macular degeneration.

PURPOSE: To quantify morphologic changes in the choroid, including choriocapillaris and larger choroidal vessels, Bruch's membrane, and retinal pigment epithelium in eyes with age-related macular degeneration (AMD) and late ARM (age-related maculopathy). METHODS: The authors analyzed 40 eye bank eyes with late ARM (21 with the neovascular AMD and 19 with nonneovascular AMD) and compared them with 40 age-matched eyes without signs of late ARM (AMD). The eyes were processed for light microscopy, and seven variables were measured in the macular and peripheral regions with a digital filar micrometer. RESULTS: There were no statistically significant differences observed between eyes with neovascular versus nonneovalcular AMD. The single most important difference between eyes with and without AMD was the amount of basal laminar deposit (P < 0.001). Eyes with AMD displayed fewer large choroidal vessels in the submacular choroid than eyes without AMD (3.5 +/- 1.5 mm-1 and 5.7 +/- 1.6 mm-1; P < 0.001). The submacular choriocapillaris density was higher in eyes with AMD (0.62 +/- 0.06) than in eyes without AMD (0.51 +/- 0.08; P < 0.001). The diameter of the large choroidal vessels in the peripheral choroid was increased in eyes with AMD (30 +/- 8 microns) compared to eyes without AMD (21.4 +/- 6.2 microns; P < 0.001). The peripheral choriocapillaris density displayed the same pattern as in the macular region in eyes with and without AMD. CONCLUSIONS: The amount of basal laminar deposit strongly correlates with the histologic presence of AMD. Eyes with AMD show differences of the density and diameter of choroidal blood vessels compared to eyes without AMD.

Aged↗

[Choroidal lesions and surgical treatment of bullous retinal detachment].

To define choroidal lesions of bullous retinal detachment, we performed indocyanine green angiography (IA) on 20 eyes of 10 patients with bullous retinal detachment. Four of these eyes were treated with sclerectomy and sclerostomy (Gass), and the benefit was evaluated. IA showed the following choroidal abnormalities: choroidal filling delay in the macular region (6 of 18 eyes, 33%) and underneath the leaking sites (9 of 18 eyes, 50%), localized (1 eye, 5%) and extended (18 eyes, 90%) choroidal venous dilatation, and intrachoroidal hyperfluorescence (all eyes, 100%). After the surgical treatment, retinal detachments resolved within 9 weeks (mean 5.3 weeks). Since choroidal filling delay, choroidal venous dilatation and intrachoroidal hyperfluorescence were observed, it is suggested that choroidal congestion plays a causative role in bullous retinal detachment. Sclerectomy and sclerostomy seemed to be beneficial in treatment of this condition.

Adult↗

Nonvascular contractile cells in sclera and choroid of humans and monkeys.

PURPOSE: To investigate by histochemistry and immunohistochemistry the distribution and innervation of nonvascular contractile cells in the sclera and choroid of humans and monkeys. METHODS: Globes were obtained from 2 macaque monkeys and 19 human cadavers that ranged in age from fetal life to 94 years. Immunohistochemistry was performed using monoclonal antibody against human smooth muscle (SM) alpha-actin and tyrosine hydroxylase (TH). The nicotinamide-adenine dinucleotide phosphate (NADPH)- diaphorase reaction was used as a marker for nitric oxide synthase. RESULTS: The scleras of all but fetal, newborn, and infant globes exhibited myofibroblasts, amelanotic, fibroblastlike cells having SM alpha-actin immunoreactivity. In the choroid of all but fetal eyes, SM cells were present in the suprachoroidal layer, forming a reticulum of flattened laminae, and in the choriocapillaris where ovoid-to-spindle-shaped SM cells were arrayed in parallel layers immediately adjacent to Bruch's membrane. Contractile cells in the sclera and choroid were most concentrated subfoveally and were sparse anteriorly. Nerve terminals positive for NADPH- diaphorase were colocalized with SM alpha-actin-positive cells in the sclera and choroid, whereas TH-positive nerve terminals colocalized with SM cells in the choroid. Clusters of ganglion cells were present on the posterior surface of globes near SM cells. CONCLUSIONS: The posterior choroid and sclera of humans and monkeys contain nonvascular contractile cells. The presence of nerve terminals and adjacent ganglion cells suggests neural control of these contractile cells. The absence of such contractile cells in fetal, newborn, and infant eyes is an argument against a major role of these cells in promoting ocular enlargement. These contractile cells may instead participate in regulation of refractive state by maintenance of ocular size in the face of intraocular pressure or in intermediate-term regulation of choroidal thickness.

Actins↗

Expression of extradomain-B-containing fibronectin in subretinal choroidal neovascular membranes.

PURPOSE: To investigate the presence of the fibronectin isoform containing the extradomain B (B-FN), a marker-protein of angiogenesis, in surgically excised human choroidal neovascular membranes (CNVM) to evaluate whether B-FN could be used as a therapeutic target for specific antibody-photosensitizer immunoconjugates. DESIGN: Laboratory investigation. METHODS: The setting was an institutional practice. The study population consisted of 15 eyes (15 patients) with CNVM undergoing membrane excision (four eyes with age-related macular degeneration, seven with pathologic myopia and four with multifocal choroiditis). The control group consisted of eight eye bank eyes (four subjects) without choroidal neovascularization. Light microscopic immunohistochemistry on cryostat sections of tissues was obtained. B-FN was detected by a human recombinant antibody, CGS-1, and compared with immunostaining for endothelial cells with factor VIII-related antigen. The main outcome measure was the presence of CGS-1 positively stained cells or areas of the extracellular matrix. Staining of CGS-1 was scored on a scale from 0 to 3. RESULTS: Fourteen of 15 neovascular membranes stained strongly with CGS-1 (score 2 or 3). One membrane from a patient with pathologic myopia was negatively stained (score 0). CGS-1 positive staining was detected around endothelial cells and in the extracellular matrix of CNVMs. The retina of eyes without choroidal neovascularization was negative with CGS-1 in all eight donor eyes, while the choroid contained some weakly CGS-1 positive cells (score 0 and 1, respectively). CONCLUSIONS: The extradomain B is abundantly expressed in CNVMs, but its expression is more restricted in eyes harboring no apparent choroidal neovascularization. In the future, B-FN might serve as a target for the delivery of antibody-photosensitizer immunoconjugates to newly developed vessels to enhance the selectivity of photodynamic therapy.

Adult↗

Indocyanine green videoangiography of idiopathic polypoidal choroidal vasculopathy.

PURPOSE: To identify the precise choroidal abnormalities associated with idiopathic polypoidal choroidal vasculopathy (IPCV), patients with IPCV were examined with indocyanine green (ICG) videoangiography. METHODS: Twelve patients with IPCV were examined using standard clinical, fluorescein, and ICG videoangiographic techniques. RESULTS: Indocyanine green videoangiography showed two basic choroidal vascular changes: a branching network of vessels in the inner choroid, and vascular dilations at the border of the network of vessels. The vascular dilations appeared to be associated with the exudative and hemorrhagic manifestations of IPCV. CONCLUSION: The choroidal vasculopathy seen in IPCV is distinct from the changes seen in other choroidal abnormalities. Recognition of these changes aids in diagnosis and patient management, since the clinical implications of IPCV differ from those of other similar entities.

Adult↗

Clinicopathological correlation of polypoidal choroidal vasculopathy revealed by ultrastructural study.

AIMS: To describe the clinical and histopathological findings in a patient with polypoidal choroidal vasculopathy. METHODS: A 76 year old Japanese man had a discrete, orange-red lesion of 1 disc diameter in the macula, with the fluorescein and indocyanine green angiographic and optical coherence tomographic findings compatible with polypoidal choroidal vasculopathy. He underwent a surgical removal of the macular lesion, followed by light and electron microscopic examinations. RESULTS: The histopathological examination revealed that the specimen consisted of degenerated retinal pigment epithelium-Bruch's membrane-choriocapillaris complex and inner choroid. A tortuous, unusually dilated venule was present adjacent to an arteriole with marked sclerotic changes, appearing to form arteriovenous crossing. These vessels seemed to represent native inner choroidal vessels, and had haemorrhage per diapedesis. Blood cells and fibrin filled the lumina of the vessels and accumulated in the extravascular spaces, indicating vascular stasis. CONCLUSION: Hyperpermeability and haemorrhage due to stasis of a dilated venule and an arteriole involved by sclerosis at the site where they cross in the inner choroid might cause oedema and degeneration of the tissue. Voluminous accumulation of blood cells and fibrin might generate elevation of tissue pressure sufficient to displace the weakened lesion anteriorly. The result suggests that the polypoidal vessels in this case represent abnormality in the inner choroidal vasculature.

Aged↗

Idiopathic polypoidal choroidal vasculopathy in Japanese patients.

OBJECTIVE: To describe the vascular nature and clinical features of idiopathic polypoidal choroidal vasculopathy in Japanese patients. METHODS: Patients thought to have idiopathic polypoidal choroidal vasculopathy were examined with binocular ophthalmoscopy, slitlamp biomicroscopy with a contact lens, fluorescein angiography, and indocyanine green angiography. RESULTS: From January 1993 to December 1997, 35 eyes in 32 patients were diagnosed as having idiopathic polypoidal choroidal vasculopathy. Men were predominantly affected (22 patients [69%]). Most patients were unilaterally involved (29 patients [91%]) and elderly, with a mean age of 65.7 years (range, 44-82 years). Ocular manifestations were relatively mild, with serous or hemorrhagic detachments of the retinal pigment epithelium and neurosensory retina in the posterior pole. Most patients had a favorable course, although some experienced recurrence, and a few eyes developed disciform scarring. In all patients, indocyanine green angiograms demonstrated branching vascular networks with polypoidal dilations at terminals of the network beneath the retinal pigment epithelium. These lesions were mostly in the macula (33 eyes [94%]), with a few in the peripapillary area. CONCLUSIONS: Idiopathic polypoidal choroidal vasculopathy in Japanese patients differs from that in American patients. It seems that this disorder occurs in elderly Japanese patients and should be treated as a distinct clinical entity. It is probably a peculiar form of choroidal neovascularization beneath the retinal pigment epithelium. We propose the term "polypoidal choroidal neovascularization" for this disorder.

Adult↗

Consequences of verteporfin photodynamic therapy on choroidal neovascular membranes.

OBJECTIVE: To examine the impact of photodynamic therapy (PDT) on angiogenesis in human choroidal neovascular membranes with respect to vascular endothelial growth factor (VEGF) expression, proliferation, and vascularization. METHODS: Retrospective review of an interventional case series of 50 patients (50 eyes) who underwent removal of choroidal neovascular membranes. Choroidal neovascularization was secondary to age-related macular degeneration. Twenty patients were treated with PDT 3 to 655 days before surgery. Choroidal neovascular membranes were stained for CD34, CD105, Ki-67, cytokeratin 18, and VEGF. Thirty choroidal neovascular membranes secondary to age-related macular degeneration without previous treatment were used as controls. RESULTS: Specimens without pretreatment disclosed varying degrees of vascularization, proliferative activity, and VEGF expression by different cells. Specimens treated with PDT 3 days earlier showed mostly occluded vessels, damaged endothelial cells, and low proliferative activity. In contrast, specimens excised at later time points after PDT were highly vascularized and proliferating. This chronology was associated with an impressive VEGF immunoreactivity unique to retinal pigment epithelial cells shortly after PDT that also shifted to other cells at later time points. CONCLUSIONS: Photodynamic therapy induces selective vascular damage in choroidal neovascular membranes. The effectiveness and selectivity of this treatment, however, seem to be jeopardized by a rebound effect initiated by enhanced VEGF expression in retinal pigment epithelial cells.

Aged↗

The expanding clinical spectrum of idiopathic polypoidal choroidal vasculopathy.

OBJECTIVE: To expand the clinical spectrum of idiopathic polypoidal choroidal vasculopathy based on historical cases and newly recognized observations. METHODS: A review of the previously reported 45 cases was carried out. An additional 20 cases were retrospectively reviewed to examine the clinical nature and course of idiopathic polypoidal choroidal vasculopathy. RESULTS: New observations on the clinical spectrum of idiopathic polypoidal choroidal vasculopathy were noted for demographic features, the nature and course of the vascular lesion, the possible association with intraocular inflammation, and the indocyanine green angiographic characteristics. CONCLUSIONS: Idiopathic polypoidal choroidal vasculopathy seems to be a distinct clinical entity that has a predilection for individuals of pigmented races. The disorder should be differentiated from typical choroidal neovascularization and other known choroidal degenerative, inflammatory, and ischemic disorders because of differences in clinical course and treatment.

Aged↗

Implication of stem cell factor in the proliferation of choroidal melanocytes.

The tyrosine kinase receptor c-kit and its ligand stem cell factor exert a broad range of biological activities during organogenesis. It also improves normal cell development including complex biological responses involved in the differentiation and proliferation of the melanocytes. Diffuse uveal melanocytic proliferation is a rare paraneoplasic syndrome, resulting in rapid bilateral visual loss due to proliferation of melanocytes within the choroid. We have therefore investigated whether the c-kit/stem cell factor pathway regulates the proliferation of choroidal melanocytes and also if such pathway plays a role in bilateral uveal melanocytic proliferation. Normal cultured melanocytes of the choroid and paraffin-embedded sections of melanocytic proliferation were studied. C-kit expression and effects of stem cell factor were measured. Western blot assays of cell extracts demonstrated that c-kit was expressed in choroidal melanocytes. Immunocytochemical analysis on cultured melanocytes showed a cytoplasmic distribution. Immunohistochemical analysis on melanocytic proliferation showed a strong cytoplasmic distribution in the pigmented spindle-shaped melanocytes localized in the multiple focal areas of choroidal thickening. The addition of stem cell factor did not change melanocyte morphologies and was mitogenic in the presence of bFGF, isobutyl-1-methylxanthine and cholera toxin. In contrast, stem cell factor was not able to produce any significant melanin. Activation of c-kit by its ligand may contribute to the proliferation of choroidal melanocytes.

Adult↗