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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 3  |  Issue : 1  |  Page : 53-55

Oral and extraoral manifestationse of sturge-Weber syndrome: A rare finding


1 Department of Periodontology, Career Postgraduate Institute of Dental Sciences and Hospital, Lucknow, Uttar Pradesh, India
2 Department of Prosthodontic, Career Postgraduate Institute of Dental Sciences and Hospital, Lucknow, Uttar Pradesh, India
3 Department of Public Health Dentistry, Career Postgraduate Institute of Dental Sciences and Hospital, Lucknow, Uttar Pradesh, India

Date of Web Publication18-Jan-2016

Correspondence Address:
Amitandra K Tripathi
Department of Periodontology, Career Postgraduate Institute of Dental Sciences and Hospital, Lucknow, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1658-6816.174338

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  Abstract 

Sturge-Weber syndrome (SWS) is a rare congenital developmental disorder characterized by unilateral cutaneous vascular malformation (nevus flammeus or port-wine stains) in association with ipsilateral leptomeningeal angiomatosis and glaucoma. This article presents a case of SWS associated with gingival hyperplasia and pyogenic granuloma.

Keywords: Congenital developmental disorder, neurocutaneous ocular disorder, pyogenic granuloma


How to cite this article:
Tripathi AK, Khan MA, Deo K, Tripathi RM. Oral and extraoral manifestationse of sturge-Weber syndrome: A rare finding. Saudi J Oral Sci 2016;3:53-5

How to cite this URL:
Tripathi AK, Khan MA, Deo K, Tripathi RM. Oral and extraoral manifestationse of sturge-Weber syndrome: A rare finding. Saudi J Oral Sci [serial online] 2016 [cited 2020 Jan 18];3:53-5. Available from: http://www.saudijos.org/text.asp?2016/3/1/53/174338


  Introduction Top


It was first described by Schimer in 1860, and Sturge in 1879 described more specific features of SWS. [1] SWS is a developmental defect that occurs in first month of gestation period. In this condition, vascular plexuses persist around the cephalic portion of neural tube. Normally, theses plexuses develop in the sixth week of intrauterine life and undergo regression during the ninth week. [2] These persistent vascular plexuses and form venous angioma affect leptomeninges, face, jaws, and soft tissues. [3]


  Case Report Top


A 17-year-old female patient reported to the Department of Periodontology with chief complaint of bleeding gums and reddish discoloration (port-wine stain) on the right side of the face [Figure 1]. This discoloration was present since birth and taking dark color with age. Past medical history revealed that she was suffering from convulsive disorder since the age of 3 years.
Figure 1: Port-wine stain on the right side of the face

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Extraoral examination revealed that port-wine stain had unilateral distribution involving the right side of the face and extended up to the middle of forehead, eye, half of the nose, and right side of the upper lip. Eye examination showed dilated blood vessels on the right side of eye and left eye appeared normal [Figure 2].
Figure 2: Dilated blood vessels in the right eye

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Intraoral examination showed gingival hyperplasia and soft tissue overgrowth on the right side [Figure 3], and theses hyperplastic tissues showed blanching on application of pressure.
Figure 3: Unilateral hyperplastic lesions and soft tissue overgrowth on the upper right side of the maxilla

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In treatment, oral prophylaxis was done and instruction for oral hygiene maintenance was given, and the patient was advised to use chlorhexidine mouthwash. The soft tissue overgrowth was surgically removed by neodymium-doped yttrium aluminum garnet (NdYag) laser and was sent for histopathological examination, which showed pyogenic granuloma [Figure 4]. NdYag laser was used for the purpose of achieving immediate hemostasis, minimal damage of surrounding tissues, and less postoperative pain.
Figure 4: Histopathological feature showed that the connective tissue was loose, fibrillar, and comprised of numerous proliferative capillaries with dense mixed inflammatory infiltrate

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On radiographic examination, a lateral cephalogram showed tram-line calcification [Figure 5]. Computed tomography (CT) of alveolar bone showed severe bone loss on the affected side of the oral cavity [Figure 6]. Blood investigations were normal. Based on the history and clinical and radiological findings, a diagnosis of SWS was made.
Figure 5: Computed tomography showing tram-line calcification

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Figure 6: Computed tomography showing severe alveolar bone loss on the affected side of the oral cavity

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  Discussion Top


It is an uncommon, nonfamilial, typical neurocutaneous ocular disorder. The etiology of this condition is remains unclear. According to some authors, the persistent vascular plexus and form venous angioma affect leptomeninges, face, jaws, and soft tissues.

Angioma of leptomeninges alternate the vascular flow causing seizers, mental retardation, and hemiplegia. [4] Facial angioma develops characteristic stains that usually occurs in area supplied by ophthalmic and maxillary division of trigeminal nerve. [4]

According to Bioxeda et al., the distribution of port-wine stains mainly unilateral follows the V2 branch of the trigeminal nerve. They observed that out of the total 121 patients, in 88% the distribution of port-wine stains mainly unilateral follows the area of V2 branch. [5]

SWS is referred to as "complete" when it affects both central nervous system (CNS) and facial angioma, and "incomplete" when only one of these areas is affected.

Roach classified SWS into the following four types on the basis of their clinical manifestation of affected patient [6] :

Type I-Both facial and leptomeningeal angiomas may have glaucoma.
Type II-Facial angioma and no CNS involvement.
Type III-Isolated leptomeningeal angioma usually no glaucoma.
Type IV-When SWS is associated with other disorders such as tuberculosis and sclerosis.

The clinical manifestation and various investigation differentiate SWS from other disorders such as Rendu-Oslar-Weber syndrome, angio-osteodystrophy, and Von Hippel-Lindau disease. [4],[7]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Caiazzo A, Mehra P, Papagearge MB. The use of preoperative percutaneous transcatheter vascular occlusive therapy in the management of Sturge-Weber syndrome: Report of a case. J Oral Maxillofac Surg 1998;56:775-8.  Back to cited text no. 1
    
2.
Neville BW, Damm DD, Allen CM, Bouquot JE, editors. Oral and Maxillofacial Pathology. 6 th ed. St. Louis: Elsevier; 2009. p. 515-6, 552-3.  Back to cited text no. 2
    
3.
Rajendra R. Benign and malignant tumors of oral cavity. In: Rajendra R, Sivapathasundharam B, editors. Shafer's Textbook of Oral Pathology. 6 th ed.India: Elsevier; 2009. p. 169-73.  Back to cited text no. 3
    
4.
Suprabha B. Baliga S. Total oral rehabilitation in a patient with port wine stains. J Indian Soc Pedod Prev Dent 2005;23:99-102.  Back to cited text no. 4
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5.
Bioxeda P, de Misa RF, Arrazola JM, Pérez B, Harto A, Ledo A. Facial angioma and the Sturge-weber syndrome: A study of 121 cases. Med Clin (Barc) 1993;101:1-4.  Back to cited text no. 5
    
6.
Roach ES, Bodensteiner JB. Neurological manifestations of Sturge-Weber syndrome. Mt Freedom, New Jersy: Turge-Weber Foundation; 1999. p. 27-38.  Back to cited text no. 6
    
7.
Mukhopadhyay S. Sturge-Weber syndrome: A case report. J Indian Soc Pedod Prev Dent 2008;26(Suppl 1):S29-31.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]


This article has been cited by
1 Pathophysiology, diagnosis, and management of glaucoma associated with Sturge–Weber syndrome
Usman Javaid,Muhammad Hassaan Ali,Samreen Jamal,Nadeem Hafeez Butt
International Ophthalmology. 2017;
[Pubmed] | [DOI]



 

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