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Síndrome de Sturge Weber: combinación de lesiones angiomatosas coroideas y orbitarias en un paciente Angiomatous choroidal and orbital lesions in a patient with Sturge Weber Syndrome  [cached]
J. Martínez-Gutiérrez,R. López-Lancho,E. Pérez-Blázquez
Archivos de la Sociedad Espa?ola de Oftalmología , 2008,
Abstract: Caso clínico: Se presenta el caso de un varón de 34 a os afecto de síndrome de Sturge-Weber que acude a urgencias por dolor ocular y exoftalmos izquierdo. Presentaba un glaucoma agudo por bloqueo pupilar debido a luxación anterior de cristalino. Se realiza resonancia nuclear magnética (RMN) con contraste y extracción intracapsular de cristalino. Discusión: La RMN muestra lesiones hipercaptantes en T2 e isocaptante en T1 en meninges, coroides y órbita izquierdas compatibles con hemangiomas cavernosos, así como dilatación de vena oftálmica superior izquierda. La presión intraocular tras la cirugía es de 10 mm Hg y la agudeza visual es menor de 20/200. Case report: We present the case of a 34-year-old man with Sturge-Weber syndrome, who presented to the emergency room with left ocular pain and left chronic exophthalmos. He suffered an acute glaucoma secondary to pupillary block consequent upon an anterior lens subluxation. Orbital contrast Magnetic Resonance Imaging (MRI) was performed and he underwent intracapsular lens extraction. Discussion: The MRI showed T2 enhanced lesions in the left meninges, choroid, and orbit, compatible with cavernous hemangiomas, as well as a dilated superior ophthalmic vein. Intraocular pressure after cataract surgery was 10 mm Hg, and visual acuity was less than 20/200.
Síndrome de Sturge-Weber: relato de caso dos achados da avalia??o fonoaudiológica
Santos, Sara Virgínia Paiva;Cavalheiro, Laura Giotto;
Revista CEFAC , 2010, DOI: 10.1590/S1516-18462010000100022
Abstract: background: phonoaudiological evaluation of rare occurrence syndrome. procedures: describe the performance shown in phonoaudiological clinical evaluation of a patient with genetic diagnosis of sturge-weber syndrome. evaluation of the drive and orofacial functions, semantic and pragmatic skills of oral language, perceptual visual and auditory processes, cognitive functions and test development of denver ii. results: in the assessment of orofacial drive the patient had abnormalities concerning bone structures of the face, inadequate as for the morphology, mobility and tone of all speech organs, presence of reflections of demand and sucking primitive. in the assessment of skills and semantic pragmatic of oral language and speech, perceptual processes visual and auditory, cognitive functions the patient had sensoriomotor exploitation, with little interaction and shared attention of the patient and difficulty of eye contact; predominantly gestural communication, with no records of verbal communicative acts. the communicative functions of a request for action, exclamatory, exploratory and protest, and great use of non-focused light. listening proved to be altered, representative gestures were sporadic, processes and perceptual functional laterality undefined. in the test development of denver ii, the patient failed in the areas: personal, social, language, motor fine and coarse. conclusion: the patient showed speech diagnosis of phonoaudiological language and speech disturbance and moderate oropharyngeal neurogenic dysphagia, being required to maximize communication, and adapt the structures and orofacial motor functions.
Sturge Weber Syndrome Overlapping with Klippel Trenaunay Syndrome  [PDF]
Shatanik Sarkar,Chaitali Patra,Chandrasekhar Dey,Malay Kumar Dasgupta,Tapan Kumar Kundu
Journal of Nepal Paediatric Society , 2013, DOI: 10.3126/jnps.v33i2.7704
Abstract: Sturge Weber syndrome (SWS) is a mesodermal phakomatosis characterized by meningo-facial angiomas with cerebral calcification. Klippel Trenaunay syndrome (KTS) is another very rare type of phakomatosis with cutaneous angiomas, varicose veins and enlargement of soft tissue or bones. Overlap between SWS & KTS is very rarely encountered. We report a three and half year old girl with overlapping features of both SWS and KTS. DOI: http://dx.doi.org/10.3126/jnps.v33i2.7704 J Nepal Paediatr Soc. 2013; 33(2):147-149
Sturge-Weber syndrome: a case report  [PDF]
Joanna G. da Concei??o,Luiz Felipe G. dos Santos,Thaís Pimentel de Sá Bahia,Vanessa de A. S. Silva
RSBO , 2011,
Abstract: Introduction: The Sturge-Weber Syndrome, also known as encephalotrigeminal angiomatosis, is a rare vascular neurocutaneous alteration. The main clinical features of this syndrome are facial vascular cutaneous naevus, usually unilateral, which often follows the outline distribution of trigeminal nerve. Objective: To report a clinical case of Sturge-Weber Syndrome in a 29-yeral-old male patient who presented oral manifestations related to the syndrome. Case report and conclusion: The patient reported that he had presented a cutaneous vascular nevus on the face during childhood as well as epileptic crisis episodes. However, he had no ophthalmic alterations. Sturge-Weber syndrome is a systemic condition commonly affecting oral cavity through vascular lesions, therefore, dentists’ knowledge is extremely important to provide an adequate dental treatment without complications.
Iris neovascularization in Sturge-Weber syndrome
Verma Lalit,Kumar Atul,Garg S,Khosla P
Indian Journal of Ophthalmology , 1991,
Abstract: 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.
Sturge Weber syndrome: a case report
Godge, Pournima;Sharma, Shubhra;Yadav, Monica;Patil, Pallavi;Kulkarni, Sandeep;
Revista Odonto Ciência , 2011, DOI: 10.1590/S1980-65232011000400016
Abstract: purpose: the sturge-weber syndrome (sws) is a rare congenital disorder that occurs sporadically and features lesions of both skin and nervous system. we report a case of 7 year-old girl with sturge-weber syndrome and discuss its clinicopathological features, differential diagnosis and also emphasize the importance of its diagnosis in the clinical oral practice. case description: the classic pathognomonic manifestations include angioma of the leptomeninges extending to cerebral cortex with ipsilateral angiomatous lesions, unilateral facial nevus affecting a division of trigeminal nerve, hemiparesis, intracranial calcification, mental retardation and refractory epilepsy. the most apparent indication of sws is a facial birthmark or "port wine stain" present at birth and typically involving at least one upper eyelid and the forehead. management of a patient with sturge-weber syndrome may be challenging due to risk of hemorrhage. conclusion: the dentists should focus on comprehensive therapy, starting with behavior management and stress on preventive measures.
Sturge-Weber Syndrome with Osteohypertrophy of Maxilla  [PDF]
Prashant Babaji,Anju Bansal,Gopal Krishna Choudhury,Rashmita Nayak,Ashok Kodangala Prabhakar,Nagarathna Suratkal,Veena Raju,Suresh S. Kamble
Case Reports in Pediatrics , 2013, DOI: 10.1155/2013/964596
Abstract: Sturge-Weber syndrome is a rare nonhereditary developmental condition with neurological and skin disorder, characterized by presence of port wine stain on the face along with ocular disorders, oral manifestations and leptomeningeal angiomas. Here we present an unusual case of Sturge-Weber syndrome with osseous hypertrophy of maxilla. 1. Introduction Sturge-Weber syndrome (SWS) or encephalotrigeminal angiomatosis belongs to group of disorders collectively called as phakomatoses (“mother-spot” disease). This rare congenital neurocutaneous syndrome is characterized by unilateral facial cutaneous vascular malformations affecting the eye and skin in association with ipsilateral leptomeningeal angiomatosis [1, 2]. In 1860, Schirmer first identified this syndrome, and Sturge in 1879 described it in detail; later Frederick Parkes Weber in 1992 demonstrated intracranial calcification [1, 2]. The prevalence is 1?:?50,000 live births. It is equally affected in males and females with no racial predilection [2]. The incidence of osseous involvement in the cutaneous capillary angioma associated with SWS is unknown; however, only few cases have been reported with osseous abnormalities [3–13]. Neoplastic occurrence with vascular malformation is extremely rare but has been reported [3]. Etiology is still unclear [2]. SWS is considered sporadic without genetic abnormalities [3]. It was thought that SWS is caused by persistence of vascular plexus around the cephalic portion of the neural tube, which develops during the sixth week of I.U. life and undergoes regression during the ninth week [1]. Here we report an interesting unusual case of SWS with osseous hypertrophy of maxilla. 2. Case Report An 8-year-old female patient reported with osseous abnormalities in the oral cavity. Her history revealed reddish discoloration (port wine stain) on the face since birth and also history of enlarging right maxilla. Medical history revealed that the patient was under medication for convulsion (carbamazepine). There was no visible sign of mental retardation. Family history was noncontributory. Extraoral examination revealed, port wine stain with unilateral (right side) distribution involving forehead, eyelids, cheek, philtrum, upper lip, half of nose, neck, chest, abdomen, and hand. The lower lip and jaw were unaffected (Figure 1). Both eyes appeared normal. Blanching of port wine stains was observed on digital pressure. Figure 1: Extraoral unilateral involvement of port wine stain on face, neck, chest, abdomen, and hand. Intraoral examination of maxilla on the right side revealed
Sturge-Weber syndrome presenting with intractable seizures  [PDF]
YB Roka,PR Puri,WS Ahmad,BK Bista
Journal of Institute of Medicine , 2010, DOI: 10.3126/joim.v32i1.4002
Abstract: Sturge-Weber syndrome (SWS) also known as encephalotrigeminal angiomatosis is one of the many neurocutaneous syndromes consisting of port wine stain (facial nevus flammeus), congenital glaucoma and cortical calcification with anomalous leptomeningeal venous plexus. A child with intractable seizures, facial port wine stain and occipital cortical calcification is presented in this case report. DOI: http://dx.doi.org/10.3126/joim.v32i1.4002 Journal of Institute of Medicine, April, 2010; 32:1 pp.40-42
Clinical features of Sturge-Weber syndrome  [cached]
Palheta Neto, Francisco Xavier,Vieira Junior, Milton Alencar,Ximenes, Lorena Souza,Jacob, Celidia Cristina de Souza
International Archives of Otorhinolaryngology , 2008,
Abstract: Introduction: The Syndrome of Sturge-Weber is a rare condition of congenital development, and is characterized by a neurocutaneous disorder with angiomas wrapping the leptomeninges and the face skin, mainly in the course of ophthalmic (V1) and maxillary (V2) branches of the trigeminal nerve. Objective: To review the literature about the Sturge-Weber Syndrome with emphasis on the current aspects. Method: The following databases were searched: EMedicine, Encyclopedia of Medicine, FindArticles, LILACS, MEDLINE, Merck Manuals On-Line Medical Library and Scielo, and the searches applied the terms: Sturge-Weber Syndrome, neurocutaneous syndromes, encephalo-trigeminal angiomatosis, nevus flammeus, in articles published between 1991 and 2007. Literature's Review: The most characteristic clinical statement is the presence, since the birth, of nevus flammeus, that generally reaches one half of the face and may stretch out up to the neck; in addition, other clinical manifestations may be present, like the corticocerebral angiomatosis, cerebral calcifications, epilepsy, ocular and buccal affections and mental retardation. The diagnosis is established by means of the inquiry of neurological and ophthalmic alterations in patients with a characteristic nevus flammeus, allied to the clinical data of complementary exams such as Computerized Tomography. The treatment consists basically of controlling the already confirmed clinical manifestations and preventing from the appearing of other alterations, mainly buccal and ocular. Conclusion: This syndrome is not much frequent, but it needs to be early diagnosed, since it brings a series of complications to its carriers when not treated, specially because of reaching the Nervous Central System. The health professionals have to be suitably able to recognize its characteristic signs and symptoms, and so improve the quality of life of these patients.
Síndrome de sturge weber, revisión de la literatura y presentación de un caso con maloclusión severa
Prato G,Rafael J; Peraza H,Raiza Y;
Acta Odontológica Venezolana , 2006,
Abstract: el síndrome de sturge weber es una rara alteración vascular con componentes neurocutaneos, en el que se presentan manifestaciones sistémicas y bucales. entre sus características se incluyen; el nevo cutaneofacial, angioma venoso en las leptomeninges, calcificaciones intracraneales, hemangiomas intrabucales e hiperplasia gingival. el caso presentado se refiere a un paciente que acude a consulta para realizar tratamiento ortodóntico por presentar una maloclusión severa concomitante con asimetría facial. en este trabajo se realizó una extensa revisión de la literatura en relación al síndrome de sturge weber y se se?alan las alteraciones intra y extraorales que presentó el paciente al realizarse el diagnóstico ortodóntico. las múltiples sinónimias se deben a la alusión de participaciones descriptivas del síndrome y se refleja ampliamente en la literatura. cada caso del síndrome de sturge-weber es único, sin embargo son escasas las descripciones odontológicas y más aún las ortodónticas, que hayan sido citadas en la literatura revisada. se rese?a a continuación una descripción del síndrome, si se quiere con tendencias estomatognáticas y énfasis en las implicaciones ortodónticas.
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