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Distribution and organization of the nerve fiber and ganglion cells of the human choroid.

Antibodies to the 68, 160 and 200 kD of the neurofilament triplets were used to study the distribution and organization of neuronal structures in the human choroid. Choroidal axons were observed in the suprachoroid and vascular laminae but absent from the choriocapillary layer. Most axons were situated in the suprachoroid. In this layer, there were band-like bundles. The two thickest band-like bundles could constitute the long ciliary nerve, while the rest could constitute short ciliary nerves. These bundles ran through the suprachoroid, branching out on the suprachoroid and the vascular laminae until they reached the ciliary body. In the submacular area of the suprachoroid, the branches of the band-like bundles were so intermingled that they looked like a meshwork. In the vascular layer, the large vessels and their primary branches were accompanied by paravascular axons. Some paravascular axons penetrated the medium-caliber vessel layer and in the submacular area interwove to form a network parallel to the arteriole walls. In addition, perivascular axons were revealed by antibodies to neuropeptides. Choroidal ganglion cells were more numerous in the central choroid, specifically in a circumferential area corresponding to the entrance of the short posterior ciliary arteries and their primary branches, and in the vicinity of the submacular area. These cells presented bipolar and multipolar morphology. The high concentration of innervation in the central human choroid could be necessary to maintain strict blood flow in this zone; thus if required, these neuron structures could induce early vasodilation reflexes at the entrance of the choroidal blood vessels to increase the blood flow.

Adolescent↗

Choroid plexus papilloma of bilateral lateral ventricle.

BACKGROUND: Choroid plexus papillomas are rare, accounting for less than 1% of all intracranial tumours in adults. However, they are relatively more common in childhood and constitute 1.5 to 4% of intracranial tumours. They are most often located in the lateral ventricle, followed by the fourth and third ventricles and, rarely, in the cerebellopontine angle. Bilateral lateral ventricle choroid plexus papilloma is very rare and only a few cases has been reported. CLINICAL PRESENTATION: A 3-year-old boy was admitted to our hospital with a history of irritability and vomiting. Neurological examination on admission was normal. A head computed tomographic scan and magnetic resonance imaging showed tumours in both lateral ventricles, hydrocephalus and a left temporal arachnoid cyst. The bilateral intraventricular mass enhanced densely and homogeneously. A presumptive diagnosis of choroid plexus papillomas was made. INTERPRETATION: The initial surgery was performed for removal of the lesion in the right lateral ventricle, and 20 days later removal of the left lateral ventricle tumour was carried out. Bilateral temporoparietal craniotomy and total removal of tumours was performed. Hydrocephalus was controlled by total tumour resection from both sides. The histology of these tumours was the same and revealed choroid plexus papilloma. Interpretation: Bilateral choroid plexus papilloma is extremely rare and distinct from diffuse villous hypertrophy and their surgical approaches are different from each other. Differential diagnosis should be made by MRI preoperatively. If bilateral choroid plexus papilloma is detected, total surgical resection should be performed. Total surgical removal of the neoplasm not only cures the tumour but also may lead to complete resolution of the hydrocephalus.

Child, Preschool↗

Isolated choroid plexus cyst(s): an indication for amniocentesis.

OBJECTIVE: Our purpose was to prospectively evaluate the risk of chromosomal abnormalities associated with isolated choroid plexus cyst(s) in gravid women undergoing second-trimester ultrasonographic examination. STUDY DESIGN: During a 24-month period 9100 pregnant women underwent midtrimester ultrasonographic evaluation. Women with a fetal diagnosis of choroid plexus cyst(s) were offered amniocentesis and a repeat examination in 4 to 6 weeks. RESULTS: A diagnosis of choroid plexus cyst(s) was made in 102 fetuses (1.1%). In four of these fetuses multiple congenital anomalies were noted. Three of the four fetuses had a chromosomal abnormality, two trisomy 18 and one unbalanced translocation, t(3;13). In the remaining 98 fetuses the choroid plexus cysts were isolated findings, that is, there were no other ultrasonographically detected anomalies. Seventy-five of these 98 fetuses underwent amniocentesis. An abnormal karyotype was identified in four fetuses: three had Down syndrome (two trisomy 21 and one unbalanced translocation, t[14;21]), and one trisomy 18. The offspring of the 23 patients in which amniocentesis was declined were phenotypically normal. CONCLUSIONS: In our prospective study the risk of chromosomal abnormality with isolated choroid plexus cyst(s) was 1:25, a risk that exceeds the 1:200 risk of pregnancy loss after amniocentesis and the 1:126 and 1:260 risk for aneuploidy and Down syndrome, respectively, in a 35-year-old pregnant women during the midtrimester. These findings indicate that amniocentesis should be offered to pregnant women in the presence of isolated fetal choroid plexus cyst(s).

Adult↗

The long-term course of multifocal choroiditis (presumed ocular histoplasmosis).

We studied 40 patients with the presumed ocular histoplasmosis triad for ten to 15 years. Another patient developed the choroidal features of the syndrome in previously normal eyes. The basic lesion is a nodular choroidal infiltrate that evolves from clinically and angiographically normal retina. It is initially hypofluorescent and has an overlying serous detachment. It evolves through episodes of serous detachment and hyperfluorescence into a choroidal scar with breaks in Bruch's membrane and occasional subretinal neovascularization. Seven of 42 eyes (16.6%) developed new choroidal infiltrates in choroid and retina that were clinically and angiographically normal more than ten years ago. After ten years about 50% of eyes with central scars will have visual acuity changes of 3 or more lines because of progressive remodeling and extension of the disciform scar. Nine of 40 fellow eyes (22.5%) sustained a disciform process during the entire follow-up period. One patient developed typical choroidal lesions in previously normal fundi. Results of extensive physical and laboratory tests for histoplasmosis and other diseases were normal.

Adult↗

Acute sectorial choroidal ischemia.

Four eyes (of three patients) with sectorial choroidal ischemia by temporal arteritis or carotid obstruction underwent fluorescein angiography during and after the acute phase. In all cases hypoperfusion affected the whole temporal choroid; however, disturbance of retinal pigment epithelium was much less extensive. In two cases the retinal pigment epithelium became necrotic and scarred within a limited area that was typically triangular in shape; in the third case scarring was limited to some pigmented streaks, and in the fourth case, retinal pigment epithelium remained normal. The triangular scar that appeared after episodes of choroidal hypoperfusion did not seem to be a true reflection of the extent of the choroidal artery obstruction in the acute phase. Our observations suggest that the deficient choroidal perfusion involved more than the choroidal artery supplying the triangular area of pigment epithelium disturbance. Several mechanisms (for example, retrograde venous filling, restoration of perfusion) reduce the deleterious effects of ischemia. Thus the necrosis of the retinal pigment epithelium would appear in only those sectors where ischemia was particularly severe or prolonged.

Acute Disease↗

Differential glycosylation of the GLUT1 glucose transporter in brain capillaries and choroid plexus.

The sodium-independent GLUT1 glucose transporter is expressed in high density in human erythrocytes and in tissues which serve a barrier function. In the polarized endothelial cells of the brain capillaries, which comprise the blood-brain barrier (BBB), GLUT1 is expressed on both apical and basolateral membranes; however, in the epithelium of the choroid plexus, GLUT1 expression is restricted to the basolateral surface. The present study examined whether these differences in subcellular localization of GLUT1 at the BBB and choroid plexus could be correlated with differential N-linked or O-linked glycosylation of the protein. Western blot analysis of solubilized brain capillaries (BC) and choroid plexus (CP) revealed that while the BC GLUT1 had an average molecular mass identical to that of the purified human erythrocyte transporter (54 kDa), the CP GLUT1 was of lower molecular mass (47 kDa). Treatment of brain capillaries and choroid plexus with N-glycanase resulted in a shift in the mobility of the GLUT1 of both samples to a lower molecular mass of 42 kDa; however, in contrast, treatment with O-glycanase produced no change in the mobility patterns of GLUT1, but did result in O-linked deglycosylation of another BBB marker, gamma-glutamyl transpeptidase. In conclusion, BBB and choroid plexus GLUT1 are subject to differential N-linked glycosylation with the protein having an N-linked carbohydrate side chain of higher molecular mass at the BBB in comparison to the choroid plexus.

Amidohydrolases↗

Choroid plexus blood flow in the sheep.

Choroid plexus blood flow was measured in 29 adult female sheep using the radioactive microsphere technique. Basal blood flow, response to change in arterial carbon dioxide tension, and response to change in mean arterial blood pressure were determined. The results were compared to cerebral cortical blood flow values in the same sheep. Mean choroid plexus blood flow was 601 ml/100 g/min under basal conditions. Choroid plexus blood flow fell 31% with hypocarbia, a reduction comparable to that seen in cortex. With hypercarbia choroid plexus blood flow rose only 27% whereas cortical blood flow increased by 199%. Unlike cortical blood flow, choroid plexus blood flow fell significantly with pharmacogenic hypertension. The latter finding may reflect the absence of a blood-brain barrier in choroid plexus.

Animals↗

Uptake and concentrations of calcium in rat choroid plexus during chronic hypo- and hypercalcemia.

The choroid plexus has been implicated in the regulation of cerebrospinal fluid (CSF) [Ca], but little information is available concerning Ca transport by this epithelium. We determined the transfer coefficients for 45Ca uptake into choroid plexus from blood, as well as tissue [Ca], in weanling Fischer-344 rats fed low, normal, or high Ca diets for 8 weeks. Plasma [Ca] decreased by 45% with low Ca diet and increased by 25% with high Ca diet. Choroid plexus 45Ca uptake varied inversely with plasma [Ca]. This relation was due largely to changes in extracellular Ca binding rather than to entry from blood, as the transfer coefficient was independent of plasma [Ca]. The extracellular Ca distribution in choroid plexus, the intercept of a plot of tissue 45Ca distribution against time, was reciprocally related to plasma [Ca]. Changes in total cell [Ca] during hypercalcemia were equivalent to those in plasma, and in hypocalcemia were 70% of those in plasma. These findings indicate that regulation of CSF [Ca] does not involve saturable transport of Ca into the choroid epithelium from blood, and that the apical membrane of the choroid epithelium is involved in homeostasis of CSF [Ca].

Animals↗

Effect of serotonin on blood flow to the choroid plexus.

The choroid plexus contains a very high density of serotonin receptors and serotonin has been reported to influence the rate of formation of cerebrospinal fluid. The goal of this study was to examine effects of serotonin (5-hydroxytryptamine) on blood flow to the choroid plexus. Blood flow to the choroid plexus was measured in anesthetized cynomolgus monkeys and dogs using radioactive microspheres. Under control conditions, blood flow to choroid plexus was approximately 4 times greater than blood flow to the cerebrum in monkeys and approximately 7 times greater than blood flow to the cerebrum in dogs. Infusion of serotonin (40 micrograms.kg-1.min-1) into the left atrium increased blood flow to choroid plexus by 101 +/- 26% (mean +/- S.E.M.) in monkeys and by 201 +/- 45% in dogs. Serotonin did not affect cerebral blood flow. These findings suggest that serotonin may play an important role in regulation of blood flow to the choroid plexus.

Animals↗

Choroidal osteoma.

This review details the characteristic clinical features, diagnostic approaches, management, and prognosis of the choroidal osteoma. A comprehensive differential diagnosis is organized to help the ophthalmologist differentiate this tumor from conditions which can sometimes be clinically similar, such as amelanotic choroidal melanoma and nevus, metastatic choroidal carcinoma, choroidal hemangioma, metastatic and dystrophic intraocular calcification, and others. The pathology of the choroidal osteoma is illustrated and several theories of the pathogenesis of this tumor including the possibilities of a choristomatous, inflammatory, and hereditary etiology are discussed. The recently recommended therapeutic technique of photocoagulation of subretinal neovascularization associated with choroidal osteoma are discussed.

Choroid Neoplasms↗

Microsurgical anatomy of the choroidal fissure.

The microsurgical anatomy of the choroidal fissure was examined in 25 cadaveric heads. The choroidal fissure, the site of attachment of the choroid plexus in the lateral ventricle, is located between the fornix and thalamus in the medial part of the lateral ventricle. The choroidal fissure is divided into three parts: (a) a body portion situated in the body of the lateral ventricle between the body of the fornix and the thalamus, (b) an atrial part located in the atrium of the lateral ventricle between the crus of the fornix and the pulvinar, and (c) a temporal part situated in the temporal horn between the fimbria of the fornix and the lower surface of the thalamus. The three parts of the fissure are the thinnest sites in the wall of the lateral ventricle bordering the basal cisterns and the roof of the third ventricle. Opening through the body portion of the choroidal fissure from the lateral ventricle exposes the velum interpositum and third ventricle. Opening through the temporal portion of the choroidal fissure from the temporal horn exposes the structures in the ambient and crural cisterns. Opening through the atrial portion of the fissure from the atrium exposes the quadrigeminal cistern, the pineal region, and the posterior portion of the ambient cistern. The neural, arterial, and venous relationships of each part of the fissure are reviewed. The operative approaches directed through each part of the fissure are also reviewed.

Cerebral Arteries↗

Choroidal responses in microgravity. (SLS-1, SLS-2 and hindlimb-suspension experiments).

Fluid and electrolyte shifts occurring during human spaceflight have been reported and investigated at the level of blood, cardiovascular and renal responses. Very few data were available concerning the cerebral fluid and electrolyte adaptation to microgravity, even in animal models. It is the reason why we developed several studies focused on the effects of spaceflight (SLS-1 and SLS-2 programs, carried on NASA STS 40 and 56 missions, which were 9- and 14-day flights, respectively), on structural and functional features of choroid plexuses, organs which secrete 70-90% of cerebrospinal fluid (CSF) and which are involved in brain homeostasis. Rats flown aboard space shuttles were sacrificed either in space (SLS-2 experiment, on flight day 13) or 4-8 hours after landing (SLS-1 and SLS-2 experiments). Quantitative autoradiography performed by microdensitometry and image analysis, showed that lateral and third ventricle choroid plexuses from rats flown for SLS-1 experiment demonstrated an increased number (about x 2) of binding sites to natriuretic peptides (which are known to be involved in mechanisms regulating CSF production). Using electron microscopy and immunocytochemistry, we studied the cellular response of choroid plexuses, which produce cerebrospinal fluid (CSF) in brain lateral, third and fourth ventricles. We demonstrated that spaceflight (SLS-2 experiment, inflight samples) induces changes in the choroidal cell structure (apical microvilli, kinocilia organization, vesicle accumulation) and protein distribution or expression (carbonic anhydrase II, water channels,...). These observations suggested a loss of choroidal cell polarity and a decrease in CSF secretion. Hindlimb-suspended rats displayed similar choroidal changes. All together, these results support the hypothesis of a modified CSF production in rats during long-term (9, 13 or 14 days) adaptations to microgravity.

Adaptation, Physiological↗

Expression of the genes for transthyretin, cystatin C and beta A4 amyloid precursor protein in sheep choroid plexus during development.

The levels of mRNA for transthyretin, cystatin C, and beta A4 amyloid precursor protein were measured in the choroid plexus of sheep embryos during different stages of development, using specific cDNA probes and Northern blot analysis. The 3 different mRNAs were detectable in the brain of very young embryos with a crown-rump length of 1 cm, corresponding to only a few days of gestation. The choroid plexus increased in weight very rapidly in the first half of gestation and much more slowly in the second half. The level of transthyretin mRNA in choroid plexus increased during the first half of gestation and stayed constant thereafter until birth, at a level of about 70% of that in choroid plexus of adult sheep. The proportion of mRNA for the proteinase inhibitor cystatin C in total RNA from choroid plexus increased throughout gestation to adult levels at birth. The concentration of the mRNA for beta A4 amyloid precursor protein in choroid plexus early in development was already as high as in adults and remained at this level throughout gestation. Messenger RNA for cystatin C or mRNA for beta A4 amyloid precursor protein was not detected in adult sheep liver.

Amyloid beta-Peptides↗

NADPH-diaphorase histochemistry of rat choroid plexus blood vessels and epithelium.

Choroid plexus is the major source of cerebrospinal fluid. The hemodynamics and secretory function of this tissue are controlled by multiple endocrine and neural mechanisms. Nitric oxide (NO) has been demonstrated to play an important role in regulating choroidal blood flow. In the present study, performed on adult male Sprague-Dawley rats, we employed a NADPH-diaphorase (NADPH-d) histochemical method to localize nitrergic innervation of choroidal blood vessels. This approach was based on previous observations that NADPH-d colocalizes with NO synthase, a synthetic enzyme for NO, in the central and peripheral nervous systems. NADPH-d-positive nerve fibers were found to accompany both large arteries and veins and blood microvessels (possibly arterioles) located in choroidal stroma. NADPH-d reaction product was also localized to the vascular endothelial lining and choroidal epithelial cells. All the above sources of NO may play important roles in the regulation of secretory and hemodynamic functions of the choroid plexus.

Animals↗

Choroidal ependymocytes in culture: expression of markers of polarity and function.

Long-term primary cultures derived from fetal mouse or rat choroid plexus were obtained in serum-supplemented media. Monoclonal and polyclonal antibodies to basolateral and apical membrane components were used to observe the expression of specific markers of polarity and function. Choroid plexus cultures and thin frozen sections of adult tissues were compared by immunocytochemistry. Two polyclonal antibodies directed against laminin and fibronectin were used on cultured choroid plexus and sectioned tissues, showing that fibronectin and laminin are located on the basolateral membrane domain in ependymocytes in vitro, as well as in vivo. Na(+)-K(+) ATPase was apically detected by light and electron microscopy with a monoclonal antibody (Mab H30) in both cultured cells and sectioned tissues. Double immunofluorescent staining with Mab H30 and affinity-purified polyclonal antibodies to the alpha subunit of G0 protein (G0 alpha) demonstrated the relatively similar distribution of the two antigens on the apical face of the choroidal tissue, both in vivo and in vitro. The distribution of these markers shows a typical differentiation with maintenance of polarized features in choroidal ependymocytes in culture, testifying that this cell culture system constitutes an interesting model for studying the functional characteristics of ependymal cells of the choroid plexus.

Animals↗

Macular burn after transpupillary thermotherapy for occult choroidal neovascularization.

PURPOSE: To report a case of severe macular burn as a complication of transpupillary thermotherapy treatment for occult choroidal neovascularization. DESIGN: Interventional case report. METHODS: A 65-year-old man developed a severe macular burn following transpupillary thermotherapy treatment. RESULTS: Before treatment, fluorescein angiography and indocyanine green angiography showed a progressive, ill-defined leakage corresponding to the presence of occult choroidal neovascularization. One month after treatment, fundus examination disclosed macular atrophy. The early phases of fluorescein angiography and indocyanine green angiography showed that the macular choroidal filling time had worsened dramatically. At the late phase of indocyanine green angiography, the initial hyperfluorescence of choroidal neovascularization was replaced by a persistent, markedly hypofluorescent area. CONCLUSION: Prolonged choroidal filling may be a risk factor for macular burn and choroidal occlusion after transpupillary thermotherapy. In such cases, we suggest that transpupillary thermotherapy should be considered with caution and, when applied, that its intensity should be reduced.

Aged↗

Dorzolamide-induced choroidal detachment in a surgically untreated eye.

PURPOSE: Choroidal detachment is a known complication of topical hypotensive agents when used to treat eyes sensitized by prior surgery. We document the abrupt development of an extensive choroidal detachment after initiation of dorzolamide therapy in a surgically untreated eye with primary open-angle glaucoma. DESIGN: Observational case report. METHODS: A 76-year-old woman with primary open-angle glaucoma and no history of ocular surgery developed a choroidal detachment 12 hours after initiation of therapy with dorzolamide eye drops. Choroidal detachment was diagnosed clinically and confirmed by echography. RESULTS: Withdrawal of the drug and initiation of corticosteroid drops resulted in prompt resolution of the choroidal detachment. CONCLUSIONS: Choroidal detachment can occur in surgically untreated eyes after use of a topical carbonic anhydrase inhibitor.

Aged↗

Intravitreal triamcinolone acetonide for choroidal granuloma in sarcoidosis.

PURPOSE: To report the clinical course of intravitreal triamcinolone acetonide on choroidal granuloma secondary to sarcoidosis that was refractory to systemic prednisolone DESIGN: Interventional case report. METHODS: A 29-year-old Chinese man with sarcoidosis presented with isolated posterior segment involvement in his left eye as retinal periphlebitis, retinal granulomas, and choroidal granuloma. Retinal periphlebitis and retinal granulomas responded well to oral corticosteroid but not the choroidal granuloma. It gradually grew in size and caused a drop in left-eye vision to 20/70. Intravitreal triamcinolone acetonide (4 mg) was injected three times in total. RESULTS: The choroidal mass began to shrink in size after the second injection and completely regressed after the third. The choroidal granuloma became a scar, with no angiographic leakage. The patient's visual acuity improved and remained stable at 20/40 at the last follow-up, 6 months after the last injection. CONCLUSIONS: Given the encouraging results, further study on intravitreal triamcinolone acetonide for macular and peripapillary choroidal granulomas secondary to systemic sarcoidosis is warranted.

Adult↗