Complete Tri Symptomatic Sturge Weber Syndrome - A case report with review of literature
Sturge-Weber syndrome (SWS) belongs to a group of disorders collectively known as the phakomatoses ("mother-spot" diseases). It consists of congenital hamartomatous malformations that may affect the eye, skin, and central nervous system at different times. Sturge-Weber syndrome is rare. It...
Main Authors: | , , , , , |
---|---|
Format: | Article |
Language: | English |
Published: |
Wolters Kluwer Medknow Publications
2007-01-01
|
Series: | Journal of Indian Academy of Oral Medicine and Radiology |
Subjects: | |
Online Access: | http://www.jiaomr.in/article.asp?issn=0972-1363;year=2007;volume=19;issue=4;spage=575;epage=584;aulast=Hegde;type=0 |
_version_ | 1818889571314696192 |
---|---|
author | Karthik Hegde R Gopakumar Subhas Babu S Giridhar Naidu Vidya Ajila R Kamala |
author_facet | Karthik Hegde R Gopakumar Subhas Babu S Giridhar Naidu Vidya Ajila R Kamala |
author_sort | Karthik Hegde |
collection | DOAJ |
description | Sturge-Weber syndrome (SWS) belongs to a group of disorders collectively known as the phakomatoses ("mother-spot" diseases). It consists of congenital hamartomatous malformations that may affect the eye, skin, and central nervous system at different times. Sturge-Weber syndrome is rare. It is present at birth in about 1 in every 50,000 babies.
SWS is classified into complete trisymptomatic SWS when all 3 organ systems are involved, incomplete bisymptomatic SWS when the involvement is either oculocutaneous or neurocutaneous, and incomplete monosymptomatic SWS when there is only neural or cutaneous involvement. Patients with no cutaneous involvement appear to be spared from the ocular manifestations of the syndrome.
Presenting a case report of this rare disorder in a 26 year old male, who presented with lip enlargement. |
first_indexed | 2024-12-19T17:11:08Z |
format | Article |
id | doaj.art-5479d2ed7e9f4e3eab6c414c9f1bdf4e |
institution | Directory Open Access Journal |
issn | 0972-1363 0975-1572 |
language | English |
last_indexed | 2024-12-19T17:11:08Z |
publishDate | 2007-01-01 |
publisher | Wolters Kluwer Medknow Publications |
record_format | Article |
series | Journal of Indian Academy of Oral Medicine and Radiology |
spelling | doaj.art-5479d2ed7e9f4e3eab6c414c9f1bdf4e2022-12-21T20:13:01ZengWolters Kluwer Medknow PublicationsJournal of Indian Academy of Oral Medicine and Radiology0972-13630975-15722007-01-01194575584Complete Tri Symptomatic Sturge Weber Syndrome - A case report with review of literatureKarthik HegdeR GopakumarSubhas BabuS Giridhar NaiduVidya AjilaR KamalaSturge-Weber syndrome (SWS) belongs to a group of disorders collectively known as the phakomatoses ("mother-spot" diseases). It consists of congenital hamartomatous malformations that may affect the eye, skin, and central nervous system at different times. Sturge-Weber syndrome is rare. It is present at birth in about 1 in every 50,000 babies. SWS is classified into complete trisymptomatic SWS when all 3 organ systems are involved, incomplete bisymptomatic SWS when the involvement is either oculocutaneous or neurocutaneous, and incomplete monosymptomatic SWS when there is only neural or cutaneous involvement. Patients with no cutaneous involvement appear to be spared from the ocular manifestations of the syndrome. Presenting a case report of this rare disorder in a 26 year old male, who presented with lip enlargement.http://www.jiaomr.in/article.asp?issn=0972-1363;year=2007;volume=19;issue=4;spage=575;epage=584;aulast=Hegde;type=0Sturge-Weber syndrome |
spellingShingle | Karthik Hegde R Gopakumar Subhas Babu S Giridhar Naidu Vidya Ajila R Kamala Complete Tri Symptomatic Sturge Weber Syndrome - A case report with review of literature Journal of Indian Academy of Oral Medicine and Radiology Sturge-Weber syndrome |
title | Complete Tri Symptomatic Sturge Weber Syndrome - A case report with review of literature |
title_full | Complete Tri Symptomatic Sturge Weber Syndrome - A case report with review of literature |
title_fullStr | Complete Tri Symptomatic Sturge Weber Syndrome - A case report with review of literature |
title_full_unstemmed | Complete Tri Symptomatic Sturge Weber Syndrome - A case report with review of literature |
title_short | Complete Tri Symptomatic Sturge Weber Syndrome - A case report with review of literature |
title_sort | complete tri symptomatic sturge weber syndrome a case report with review of literature |
topic | Sturge-Weber syndrome |
url | http://www.jiaomr.in/article.asp?issn=0972-1363;year=2007;volume=19;issue=4;spage=575;epage=584;aulast=Hegde;type=0 |
work_keys_str_mv | AT karthikhegde completetrisymptomaticsturgewebersyndromeacasereportwithreviewofliterature AT rgopakumar completetrisymptomaticsturgewebersyndromeacasereportwithreviewofliterature AT subhasbabu completetrisymptomaticsturgewebersyndromeacasereportwithreviewofliterature AT sgiridharnaidu completetrisymptomaticsturgewebersyndromeacasereportwithreviewofliterature AT vidyaajila completetrisymptomaticsturgewebersyndromeacasereportwithreviewofliterature AT rkamala completetrisymptomaticsturgewebersyndromeacasereportwithreviewofliterature |