Clinical features of Sturge-Weber syndrome
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. Objecti...
| Main Authors: | , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Thieme Revinter Publicações Ltda.
2008-12-01
|
| Series: | International Archives of Otorhinolaryngology |
| Subjects: | |
| Online Access: | http://www.arquivosdeorl.org.br/conteudo/acervo_eng.asp?Id=573 |
| _version_ | 1819227671544987648 |
|---|---|
| author | Palheta Neto, Francisco Xavier Vieira Junior, Milton Alencar Ximenes, Lorena Souza Jacob, Celidia Cristina de Souza Rodrigues Junior, Adilson Góes Palheta, Angélica Cristina Pezzin |
| author_facet | Palheta Neto, Francisco Xavier Vieira Junior, Milton Alencar Ximenes, Lorena Souza Jacob, Celidia Cristina de Souza Rodrigues Junior, Adilson Góes Palheta, Angélica Cristina Pezzin |
| author_sort | Palheta Neto, Francisco Xavier |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-23T10:45:06Z |
| format | Article |
| id | doaj.art-4d9a97cc2b5b40a6ab8a6d583ac23405 |
| institution | Directory Open Access Journal |
| issn | 1809-9777 1809-4864 |
| language | English |
| last_indexed | 2024-12-23T10:45:06Z |
| publishDate | 2008-12-01 |
| publisher | Thieme Revinter Publicações Ltda. |
| record_format | Article |
| series | International Archives of Otorhinolaryngology |
| spelling | doaj.art-4d9a97cc2b5b40a6ab8a6d583ac234052022-12-21T17:50:02ZengThieme Revinter Publicações Ltda.International Archives of Otorhinolaryngology1809-97771809-48642008-12-01124565570Clinical features of Sturge-Weber syndromePalheta Neto, Francisco XavierVieira Junior, Milton AlencarXimenes, Lorena SouzaJacob, Celidia Cristina de SouzaRodrigues Junior, Adilson GóesPalheta, Angélica Cristina PezzinIntroduction: 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.http://www.arquivosdeorl.org.br/conteudo/acervo_eng.asp?Id=573Sturge-Weber syndromeCongenitalAngiomatosisOtorhinolaryngology |
| spellingShingle | Palheta Neto, Francisco Xavier Vieira Junior, Milton Alencar Ximenes, Lorena Souza Jacob, Celidia Cristina de Souza Rodrigues Junior, Adilson Góes Palheta, Angélica Cristina Pezzin Clinical features of Sturge-Weber syndrome International Archives of Otorhinolaryngology Sturge-Weber syndrome Congenital Angiomatosis Otorhinolaryngology |
| title | Clinical features of Sturge-Weber syndrome |
| title_full | Clinical features of Sturge-Weber syndrome |
| title_fullStr | Clinical features of Sturge-Weber syndrome |
| title_full_unstemmed | Clinical features of Sturge-Weber syndrome |
| title_short | Clinical features of Sturge-Weber syndrome |
| title_sort | clinical features of sturge weber syndrome |
| topic | Sturge-Weber syndrome Congenital Angiomatosis Otorhinolaryngology |
| url | http://www.arquivosdeorl.org.br/conteudo/acervo_eng.asp?Id=573 |
| work_keys_str_mv | AT palhetanetofranciscoxavier clinicalfeaturesofsturgewebersyndrome AT vieirajuniormiltonalencar clinicalfeaturesofsturgewebersyndrome AT ximeneslorenasouza clinicalfeaturesofsturgewebersyndrome AT jacobcelidiacristinadesouza clinicalfeaturesofsturgewebersyndrome AT rodriguesjunioradilsongoes clinicalfeaturesofsturgewebersyndrome AT palhetaangelicacristinapezzin clinicalfeaturesofsturgewebersyndrome |