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...

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Main Authors: Karthik Hegde, R Gopakumar, Subhas Babu, S Giridhar Naidu, Vidya Ajila, R Kamala
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
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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.
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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
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