1/9. Encephalotrigeminal angiomatosis.We report an unusual case of encephalotrigeminal angiomatosis in which the facial and oral angioma was bilateral, and several teeth were congenitally absent. The developmental nature of the anomaly is reviewed. Encephalotrigeminal angiomatosis is commonly referred to as the sturge-weber syndrome, after Sturge and Weber who first described this affliction in 1879. The main clinical features of this syndrome are 1. venous angiomatosis of the leptomeninges of the cerebral-cortex, usually unilaterally 2. ipsilateral facial angiomatosis that often follows in outline the distribution of the trigeminal nerve (Fig 1) 3. ipsilateral gyriform calcification of the cerebral cortex 4. epileptic convulsions (contralateral focus) or other seizures 5. ocular defects (choroidal angioma, glaucoma hemianopia) 6. mental retardation 7. contralateral hemiplegia 8. obesity 9. oral mucosal and gingival involvement. Other less typical features are 1. association with hypomelanosis of 1 to 10 2. leptomeningeal angioma contralateral to the facial nevus 3. leptomeningeal angioma without facial vascular naevus 4. association with gastro intestinal hemorrhage 5. paranasal sinus enlargement. This syndrome that affects males and females equally, is a rare congenital disorder, apparently hamartomatous in nature, from persistence of a primitive embryonal vascular plexus. During the sixth week of intra-uterine life this plexus develops around the cephalic portion of the neural tube and under the ectoderm in the region destined to become facial skin. In the sturge-weber syndrome, the vascular plexus fails to regress, as is normal during the ninth week, resulting in angiomatosis of the related tissues. Variation in the degree of persistence or regression of the vascular plexus accounts for unilaterality or bilaterality of involvement, and also for an incomplete syndrome in which the leptomeninges, but not the facial tissues are affected. Leptomeningeal angiomatosis is the primary abnormality of encephalotrigeminal angiomatosis, all other features of the syndrome probably being secondary to this. Calcification of the cortex is a poorly understood phenomenon which may result from stasis of blood in the angioma, associated with altered local metabolism. epilepsy and other neurological seizures, and mental retardation are probably, in their turn, secondary to the cortical calcification. The most striking clinical feature of the sturge-weber syndrome is the facial vascular naevus which generally follows the distribution of innervation of one or more divisions of the trigeminal nerve, whence the term encephalotrigeminal angiomatosis. However, the naevus may be more extensive, down the neck and even onto the chest. The oral tissues underlying the affected facial tissues are invariably also angiomatous and may be considerably enlarged as a result. Alterations in eruption of teeth have also been noted. Histologically, affected soft tissues are very vascular, resembling a pyogenic granuloma or a capillary, or cavernous hemangioma. Yukna, Cassingham and Carr noted that affected bone was partially replaced by a delicate fibrous tissue containing thin-walled vascular spaces. Neither inflammatory cells, nor fatty or haemopoietic marrow was noted.- - - - - - - - - - ranking = 1keywords = naevus, cell (Clic here for more details about this article) |
2/9. Gyriform calcifications in tuberous sclerosis simulating the appearance of Sturge-Weber disease.Two cases of tuberous sclerosis are presented. Extensive superficial occipital calcifications were found as classically described in sturge-weber syndrome. Other radiologic signs of tuberous sclerosis, such as subependymal calcifications in both patients and surgically proved giant cell astrocytoma in one patient, were present. At pathologic examination, the calcifications appeared to be located in extensive cortical tubers.- - - - - - - - - - ranking = 6.0206932782386E-6keywords = cell (Clic here for more details about this article) |
3/9. Anesthetic management of a patient with sturge-weber syndrome undergoing oral surgery.This case involves a possible complication of excessive bleeding or rupture of hemangiomas. Problems and anesthetic management of the patient are discussed. A 35-year-old man with sturge-weber syndrome was to undergo teeth extraction and gingivectomy. Hemangiomas covered his face and the inside of the oral cavity. We used intravenous conscious sedation with propofol and N2O-O2 to reduce the patient's emotional stress. It was previously determined that stress caused marked expansion of this patient's hemangiomas. periodontal ligament injection was chosen as the local anesthesia technique. Teeth were extracted without excessive bleeding or rupture of hemangiomas, but the planned gingivectomies were cancelled. deep sedation requiring airway manipulation should be avoided because there are possible difficulties in airway maintenance. Because this was an outpatient procedure, propofol was selected as the sedative agent primarily because of its rapid onset and equally rapid recovery. periodontal ligament injection with 2% lidocaine containing 1: 80,000 epinephrine was chosen for local anesthesia. gingivectomy was cancelled because hemostasis was challenging. As part of preoperative preparation, equipment for prompt intubation was available in case of rupture of the hemangiomas. The typically seen elevation of blood pressure was suppressed under propofol sedation so that expansion of the hemangiomas and significant intraoperative bleeding was prevented. periodontal ligament injection as a local anesthetic also prevented bleeding from the injection site.- - - - - - - - - - ranking = 1.2041386556477E-5keywords = cell (Clic here for more details about this article) |
4/9. Regional cerebral blood flow characteristics of the sturge-weber syndrome.Four patients with the sturge-weber syndrome were studied using the non-invasive xenon-133 inhalation technique. All four patients had decreased regional cerebral blood flow in the area of their lesion, and in two patients who were subsequently tested with 5% carbon dioxide inhalation, impaired vasomotor reactivity was documented. Diminished regional cerebral blood flow is consistent with previously described nuclide flow studies which demonstrated a delay in the initial perfusion blush in the region of the abnormal vasculature. The focal decrease in blood flow was greatest in the most severely affected patient, but was also prominent in the two younger patients, both of whom have excellent neurologic function. These studies suggest that localized decrease in blood flow and vasomotor dysfunction in sturge-weber syndrome can precede the occurrence of severe neurologic impairment and extensive cerebral atrophy and possibly be a major contributing factor in progressive dysfunction. A secondary observation was that the blood flow in the unaffected hemisphere was significantly greater in two children compared to the two adults and was similar to the age-related differences reported for normal children and adults.- - - - - - - - - - ranking = 6.0206932782386E-6keywords = cell (Clic here for more details about this article) |
5/9. Basal cell carcinoma developing in a nevus flammeus.A 44-year-old man with sturge-weber syndrome developed a basal cell carcinoma at the site of a nevus flammeus on his right cheek, which had been treated with a course of grenz rays at six years of age without obvious improvement. He also exhibited erythematous papulovesicular lesions on the upper trunk, which histologically proved to be transient acantholytic dermatosis. The association of nevus flammeus and basal cell carcinoma is extremely rare; to the best of our knowledge, only four similar cases have been previously reported.- - - - - - - - - - ranking = 3.6124159669432E-5keywords = cell (Clic here for more details about this article) |
6/9. 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 = 1.2041386556477E-5keywords = cell (Clic here for more details about this article) |
7/9. 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 = 1.8062079834716E-5keywords = cell (Clic here for more details about this article) |
8/9. sturge-weber syndrome associated with naevus of Ota.The association of sturge-weber syndrome with naevus of Ota is an infrequently reported phenomenon and there are only four previously described cases in the literature. In this paper we briefly review the literature regarding the coexistence of vascular and pigmentary naevi and present an additional patient with the association of the sturge-weber syndrome and naevus of Ota.- - - - - - - - - - ranking = 1.9999879586134keywords = naevus (Clic here for more details about this article) |
9/9. Phacomatosis pigmentovascularis type IIb associated with sturge-weber syndrome and pyogenic granuloma.A case of phacomatosis pigmentovascularis (PPV) in a 6-year-old girl with sturge-weber syndrome, pyogenic granuloma, and other complications is described. It is relatively rare that a complete form of sturge-weber syndrome was associated with PPV. A review of the literature on PPV, focusing on total number of reported cases and etiological speculations, is presented. To our knowledge, a total of 118 cases of PPV, including the present one, have been reported to date. Regardless of many speculations, the true etiology remains unknown. The average "density" of mast cells (MCs) per mm2 appearing in the central region of the pyogenic granuloma was calculated to be 86.3/mm2 and that in the adjacent nevus flammeus was 37.9/mm2. The "density" of mast cells in pyogenic granuloma separately calculated from ten other cases was 105.5 /- 28.6/mm2 (mean /- SD), compared with that in normal skin, 6.85 /- 4.9/mm2 (n = 20). There was a significant difference between the two, indicating that MCs are closely associated with angiogenesis in pyogenic granuloma.- - - - - - - - - - ranking = 1.2041386556477E-5keywords = cell (Clic here for more details about this article) |