1/11. sturge-weber syndrome with bilateral congenital syndactyly: a previously undescribed association.The sturge-weber syndrome consists of unilateral port-wine haemangioma of the face which may be associated with an ipsilateral intracranial haemangioma and choroidal angioma. The common derivation of the meningeal, choroidal and facial vessels may explain a congenital malformation of all three areas. I report the case of a child with typical sturge-weber syndrome who had a previously undescribed association with bilateral congenital syndactyly.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/11. sturge-weber syndrome in a 56 year old woman: a case report.BACKGROUND: sturge-weber syndrome is a sporadic phacomatoses with angiomas involving the leptomeninges and skin of the face, typically in the ophthalmic and maxillary distributions of the trigeminal nerve. glaucoma is a common ocular manifestation. Presentation is typically at birth with a facial angioma. METHOD: Case note of a patient with a diagnosis of sturge-weber syndrome was used and the relevant literature reviewed. RESULT: A 56 year old woman with pain, photophobia in the left eye of 6 months duration. She had lost vision in the eye about 20 years earlier and was born with a dark patch on the left side of her face. She had no previous history of convulsion. On examination, she had a port-wine stain involving the left side of her face. Examination of the left eye revealed a visual acuity of no light perception with episcleral haemangioma. There was a relative afferent pupillary defect and fundoscopy revealed a pale pathologically cupped disc with tortuous retinal vessels. The intraocularpressure was elevated. CONCLUSION: sturge-weber syndrome is a rare phacomatoses which may present with ocular complications such as glaucoma. If glaucoma is left untreated decreased vision and blindness result. People of any age therefore with port-wine stain in the ophthalmic distribution of the trigeminal nerve should have yearly eye examination and measurement of intraocular pressure, regardless of whether they have symptoms or not.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/11. Extensive mixed vascular malformation clinically imitating multiple sclerosis--case report.vascular malformations usually develop as a result of influence of teratogenic factor(s) acting in the defined embryonic/fetal period. However, in the case examined by us, various types of vascular malformations formed in different periods of the ontogenic development were found. They were seen in all parts of the central nervous system and clinically mimicked multiple sclerosis. On the background of generalized ischemic lesions of the CNS, certain kinds of vascular malformations were seen: cavernous or fetallike vessels within meninges, superficially located capillary angioma penetrating into the brain and spinal cord white matter, and arterio-venous pathological conglomerates forming meningeal angiomatosis. In pathological vessels, immunocytochemical assessment of vascular endothelium with antibodies against antigens CD31, CD34, von Willebrand factor and lectin ulex europaeus was normal but examination of the vascular basal membrane compounds revealed poor immunoreactivity to laminin and fibronectin. There were no disturbances in expression of angiopoietin, platelet-derived growth factor, transforming growth factor beta and vascular endothelial growth factor receptors Tie-1/2, PDGFR-alpha/beta, endoglin and Flk-1, respectively. The presence of various types of pathological vessels originating from different ontogenic periods indicates remittent or prolonged influence of teratogenic factor(s) in all periods of fetal vessel development.- - - - - - - - - - ranking = 4keywords = vessel (Clic here for more details about this article) |
4/11. Sturge-Weber-Dimitri disease without facial nevus.A patient with Sturge-Weber-Dimitri disease presented with intractable seizures and progressive intellectual deterioration. There was no facial nevus or focal neurologic abnormality. CT disclosed bilateral calcification in a parieto-occipital gyral pattern. Histopathology of the brain revealed extensive calcification of vessel wall in parieto-occipital cortices.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/11. Choroidal effusion during glaucoma surgery in patients with prominent episcleral vessels.Rapid intraoperative choroidal effusion and flattening of the anterior chamber occurred during glaucoma filtering surgery in four young patients with prominent episcleral vessels, elevated episcleral venous pressure, and advanced open-angle glaucoma. Two of these four patients had sturge-weber syndrome. Intraoperative release of suprachoroidal fluid (SCF) through a posterior sclerotomy facilitated reformation of the anterior chamber and repositioning of the iris and ciliary body. Posterior sclerostomy performed prior to opening the anterior chamber minimized the above-mentioned untoward series of events. Analysis of SCF and serum demonstrated considerable differences in total protein and individual immunoglobulin levels; this appears to be a manifestation of molecular sieving at the level of the choriocapillaris. Choroidal detachment and postoperative serous retinal detachment are manifestation of this phenomenon.- - - - - - - - - - ranking = 5keywords = vessel (Clic here for more details about this article) |
6/11. Cilio-optic vein associated with phakomatosis.This presentation demonstrates the fluorescein angiographic characteristics of the cilio-optic vein. These congenitally enlarged vessels appear at the disc edge and dip into the optic nerve to anastomose with branches of the central retinal vein. fluorescein angiography shows lamellar filling of the vessels in the early choroidal phase. In one patient, these findings are demonstrated bilaterally with the use of bilateral simultaneous angiography. A second type of communication is presented, a retinociliary vein which drains the retina into the choroidal circulation. In both types presented, a branching hypofluorescent pattern extends from the disc vessel into the choroid. Our patients have evidence of a phakomatosis: neurofibromatosis or sturge-weber syndrome. This anomalous disc vessel should not be confused with optociliary shunt, disc neovascularization, cilioretinal artery, or arteriovenous shunt.- - - - - - - - - - ranking = 4keywords = vessel (Clic here for more details about this article) |
7/11. anterior chamber angle vascularization in sturge-weber syndrome. Report of a case.The case of a 20-year-old woman with a left-sided facial hemangioma and a homolateral glaucoma is reported, complete with the histology of a trabeculectomy specimen. Her left eye had an episcleral hemangioma and goniodysgenetic features in the anterior chamber angle, while the intraocular pressure was measured to be 45 mmHg. The left optic disc showed a large cupping and the left visual field was constricted. The right eye had no glaucomatous changes. Histological examination of the trabeculectomy specimen by both light and electron microscopy showed multiple congenital anomalies. There was a cluster of blood vessels in the trabecular meshwork. Abnormal accumulations of fine granular extracellular matrixes were observed in both the juxtacanalicular connective tissue and around the vascular structures. The lumen of Schlemm's canal was subdivided into three or four parts with few giant vacuole structures. The endothelial cells lining the inner wall of Schlemm's canal contained a well-formed basal lamina with many villi projecting into the lumen. These findings suggest that the multiple anomalies observed in the trabecular tissue may contribute to the manifestation of glaucoma in sturge-weber syndrome.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/11. Leukocyte adhesion molecules and x-ray energy dispersive spectroscopy in Sturge-Weber disease.We examined the light microscopic and ultrastructural features associated with Sturge-Weber disease, including x-ray energy dispersive spectroscopy to evaluate the chemical composition of the mineralized deposits and immunofluorescence microscopy with leukocyte adhesion molecules to examine the blood vessel proliferation further. Two patients (a 17-year-old girl and a 9-month-old boy) with Sturge-Weber disease comprise this series. Mineralized deposits stained strongly positive with von Kossa and negative with Prussian blue. Transmission electron microscopy of tissue removed during a functional hemispherectomy procedure in both cases indicated that most concretions were adjacent to or in the basal lamina of parenchymal vessels; no deposits were observed in leptomeningeal vessels. Energy dispersive spectroscopy of the deposits showed emission peaks corresponding predominantly to calcium, with lesser amounts of phosphorus. Fluorescent monoclonal antibodies to leukocyte adhesion molecules (endothelial cell, vascular cell, and intercellular: ELAM-1, VCAM-1, and ICAM-1) demonstrated strong positive staining of the meningeal vessels with all three antibodies. Cortical vessels were positive only for ICAM-1. Findings based on routine staining and energy dispersive spectroscopy indicate that the mineralized deposits detected in Sturge-Weber disease are composed primarily of calcium phosphate and are located primarily in and adjacent to the vascular basal lamina. There is an aberrant expression of ELAM-1 and VCAM-1 in the meningeal vascular proliferation similar to what is observed with other vascular malformations and tumors. Parenchymal vessel changes may be secondary to the meningeal vascular proliferation.- - - - - - - - - - ranking = 6keywords = vessel (Clic here for more details about this article) |
9/11. Innervation pattern of malformative cortical vessels in Sturge-Weber disease: an histochemical, immunohistochemical, and ultrastructural study.OBJECTIVE: This study was undertaken to elucidate the pattern of vascular innervation in areas of pial angiomatosis in Sturge-Weber disease (SWD) and eventually correlating it with the pathophysiology of the disease, namely its chronic ischemic changes. methods: We processed part of a surgical specimen resected from a 3-year-old female patient who underwent functional hemispherectomy for SWD and characterized the pattern of innervation of the malformative cortical vessels using histochemical, immunohistochemical, and ultrastructural techniques. RESULTS: Cortical vessels were observed to be supplied with numerous varicose nerve fibers containing immunoreactivity for neuropeptide tyrosine and the catecholamine-synthesizing enzyme, tyrosine, tyrosine hydroxylase. In contrast, no nerve fibers containing acetylcholinesterase activity and immunoreactivity for substance p, a calcitonin gene-related peptide and vasoactive intestinal peptide, were detected. Ultrastructural studies revealed the presence of numerous axon varicosities at the adventitial-medial border. Neuropeptide tyrosine immunoreactivity was localized in large granular vesicles in nerve varicosities that also contained numerous small granular vesicles. CONCLUSION: These results demonstrate that nerve supplying cortical vessels in SWD are arranged in a distribution pattern similar to the one observed in human normal cortical veins and suggest that these abnormal vessels are innervated only with noradrenergic sympathetic nerve fibers. This represents a clear difference from the pattern of innervation observed in both normal cortical arteries and veins, and is the consequence of the anatomic and functional dysangiogenic process characteristic of the affected cortical areas in SWD.- - - - - - - - - - ranking = 8keywords = vessel (Clic here for more details about this article) |
10/11. Ultrasound biomicroscopy in Sturge-Weber-associated glaucoma.PURPOSE: To evaluate Sturge-Weber-associated glaucoma using ultrasound biomicroscopy. METHOD: Case report. Clinical examination combined with ultrasound biomicroscopy was performed in a patient with Sturge-Weber-associated glaucoma. RESULTS: In the patient's left eye, which had Sturge-Weber-associated glaucoma, a 360-degree supraciliary effusion, dilated superficial and intrascleral vessels, and an open angle were detected by ultrasound biomicroscopy. CONCLUSION: The presence of dilated intrascleral vessels and supraciliary fluid support the hypothesis of increased episcleral venous pressure as the cause of elevated intraocular pressure in this syndrome.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
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