1/14. A case of glaucoma associated with sturge-weber syndrome and nevus of ota.The sturge-weber syndrome consists of a unilateral port-wine hemangioma of the skin along the trigeminal distribution and is accompanied by an ipsilateral leptomeningeal angioma. glaucoma is present in approximately half of the cases. The nevus of ota is a melanocytic pigmentary disorder, most commonly involving the area innervated by the trigeminal nerve. Elevated intraocular pressure, with or without glaucomatous damage, is observed in 10% of the cases. We report the first case of glaucoma associated with sturge-weber syndrome and nevus of ota in korea.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
2/14. A case of acute angle-closure glaucoma secondary to posterior scleritis in patient with sturge-weber syndrome.BACKGROUND: sturge-weber syndrome has been known to be frequently associated with facial cutaneous angioma and ipsilateral glaucoma. However, as far as we know, no cases accompanied by acute angle-closure glaucoma have been reported in patients with sturge-weber syndrome. CASE: A 14-year-old boy with unilateral acute angle-closure glaucoma secondary to posterior scleritis associated with sturge-weber syndrome is described. OBSERVATIONS: Slit-lamp examination revealed diffuse episcleral venous hemangioma in the right eye. With ultrasound biomicroscopy, a forward shift of the lens-iris diaphragm, a swelling of the ciliary body, and an anterior rotation of the ciliary processes with annular choroidal effusion were detected. The patient responded well to treatment with systemic corticosteroids and cycloplegics. CONCLUSIONS: In our patient, inflammatory changes of the sclera, including swelling of the ciliary body, choroidal effusion, an anterior rotation of the ciliary processes at the scleral spur, and swelling of the lens, leading to closure of the anterior chamber angle, were suggested to be the major mechanisms of intraocular pressure elevation.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
3/14. Ciliochoroidal effusion induced by topical latanoprost in a patient with sturge-weber syndrome.BACKGROUND: To report drug-induced ciliochoroidal effusion in a patient with sturge-weber syndrome.CASE: A 17-year-old man presented with unilateral glaucoma associated with sturge-weber syndrome.OBSERVATIONS: His corrected visual acuity was RE 20/20 and LE 40/60. intraocular pressure readings by Goldmann applanation tonometry were RE 32 mm Hg and LE 12 mm Hg. Fundus examination showed marked glaucomatous disc cupping in his right eye and normal finding in his left. The patient had a port-wine stain on his right upper eyelid ipsilateral to the glaucomatous eye. Antiglaucomatous medications were begun, including topical latanoprost, with a diagnosis of juvenile onset glaucoma associated with sturge-weber syndrome. Ultrasound biomicroscopy showed a 360 degrees circumference ciliochoroidal effusion. Forty days after starting medication, latanoprost treatment was discontinued. Ten days later, ultrasound biomicroscopy showed a total disappearance of the ciliochoroidal effusion.CONCLUSION: Interaction of the enhanced uveoscleral outflow with latanoprost in conjunction with elevated episcleral venous pressure may have caused the congestion of the aqueous humor in the supraciliary-choroidal space, resulting in the ciliochoroidal effusion.- - - - - - - - - - ranking = 2keywords = pressure (Clic here for more details about this article) |
4/14. Histopathological study of a case with glaucoma due to sturge-weber syndrome.PURPOSE: To investigate the cause of the secondary glaucoma in a case of sturge-weber syndrome by histopathology. CASE: A 10-year-old boy with sturge-weber syndrome and glaucoma in the right eye was studied. trabeculectomy was performed because of uncontrolled intraocular pressure, and the trabeculectomy specimen was examined histologically by both light and electron microscopy. RESULTS: Histological examination of the trabeculectomy specimen showed that the ciliary muscle was dislocated anteriorly, and the Schlemm canal was not present. The spaces in the juxtacanalicular connective tissue (JCT) were replaced by vascular structures and connective tissue. There were two kinds of vascular structures: in one, the endothelium was surrounded by pericytes; and in the other, the endothelium was not surrounded by pericytes. CONCLUSIONS: Developmental abnormalities of the Schlemm canal and the JCT may have caused the glaucoma. These observations suggest that the developmental abnormalities of both the mesoderm and the neural crest might be involved in the pathogenesis of the glaucoma in cases of sturge-weber syndrome.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
5/14. 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 = 2keywords = pressure (Clic here for more details about this article) |
6/14. 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 = 1keywords = pressure (Clic here for more details about this article) |
7/14. Anesthesia for encephalo-trigeminal angiomatosis (sturge-weber syndrome).A case report of rare congenital disease of encephalo-trigeminal angiomatosis (sturge-weber syndrome) (SWS), is presented to alert the anesthesiologist of its potential risk. The case was anesthetized for emergency orthopedic surgery. Anesthesia management is directed towards observing the clinical manifestations of localized superficial skin lesions, extensive systemic involvement, evaluating its associated anomalies and anticipating difficult intubation due to angiomas of the mouth and upper airway and be a ware of concurrent therapy. The authors reviewed the English-language literature and reported findings regarding the SWS pathophysiologic features, interventions, and outcomes, with emphasis on issues relevant to anesthesiologists. This patient tolerated balanced anesthesia well. Anesthesia should be planned to avoid trauma to the hemangiomata, increases in intraocular and intracranial pressure, and be cognizent of current anticonvulsant therapy.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
8/14. iris neovascularization in sturge-weber syndrome.An unusual case of sturge-weber syndrome with raised intraocular pressure is presented. The slit lamp examination and anterior segment fluorescein angiography were consistent with iris neovascularization. Cause and effect relationship of iris neovascularization with glaucoma in this syndrome is discussed.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
9/14. Ahmed glaucoma valve implant for childhood glaucoma in sturge-weber syndrome with choroidal hemangioma.We present our experience with implantation of an Ahmed glaucoma valve in a 9-year-old girl with sturge-weber syndrome with choroidal hemangioma. In this case of childhood glaucoma, the choice of surgical procedure should be based on the efficacy and elimination of intra-and post-operative complications. An Ahmed glaucoma valve offered safety and efficacy in controlling glaucoma. This technique avoided sudden drops in intraocular pressure and decreases the risk of intraoperative and postoperative choroidal effusion and hemorrhage. The surgical procedure was not complicated: a one-staged implant was satisfactory and a prophylactic sclerostomy was not required.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
10/14. Megalencephaly due to impaired cerebral venous return in a Sturge-Weber variant syndrome.An infant with a Sturge-Weber variant syndrome developed progressive megalencephaly and eventual hydrocephalus, which required shunting. cerebral angiography revealed absence of the deep cerebral venous system and the development of abnormal drainage channels via the periorbital veins. It is postulated that the abnormal enlargement of the brain was due to the impaired venous return. Resistance of the brain to continued expansion may have caused an increase in hydrostatic pressure and the development of hydrocephalus.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
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