alexa Sturge-Weber Syndrome Associated with Maxillofacial Osteohypertrophy | Open Access Journals
ISSN: 2471-8726
Oral Health Case Reports
Make the best use of Scientific Research and information from our 700+ peer reviewed, Open Access Journals that operates with the help of 50,000+ Editorial Board Members and esteemed reviewers and 1000+ Scientific associations in Medical, Clinical, Pharmaceutical, Engineering, Technology and Management Fields.
Meet Inspiring Speakers and Experts at our 3000+ Global Conferenceseries Events with over 600+ Conferences, 1200+ Symposiums and 1200+ Workshops on
Medical, Pharma, Engineering, Science, Technology and Business

Sturge-Weber Syndrome Associated with Maxillofacial Osteohypertrophy

Payal Saxena1, Sandhya Jain2, Saurabh Kumar Gupta1* and Deshraj Jain3

1Department of Conservative Dentistry, Government College of Dentistry, Indore, Madhya Pradesh, India

2Department of Orthodontics, Government College of Dentistry, Indore, Madhya Pradesh, India

3Department of Prosthodontics, Government College of Dentistry, Indore, Madhya Pradesh, India

*Corresponding Author:
Saurabh Kumar Gupta
Department of Conservative Dentistry
Government College of Dentistry
Sardar Patel Marg, Indore, Madhya Pradesh, India
Tel: 918103078123
E-mail: [email protected]

Received Date: December 21, 2016; Accepted Date: January 30, 2017; Published Date: February 06, 2017

Citation: Saxena P, Jain S, Gupta SK, Jain D (2017) Sturge-Weber Syndrome Associated with Maxillofacial Osteohypertrophy. Oral health case Rep 3:130. doi: 10.4172/2471-8726.1000130

Copyright: © 2017 Saxena P, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Visit for more related articles at Oral Health Case Reports


This case report presents a rare case of an adult female patient of Sturge-Weber Syndrome with unilateral maxillofacial osteohypertrophy. Incidence and exact cause of association of Sturge-Weber Syndrome with osseous hypertrophy is not known and there are very few similar cases that have been reported in literature. Patient visited to the dental department with the chief complaint of malalignment of teeth for which orthodontic treatment plan was done.


Soft tissue vascular malformations may rarely be associated with osteohypertrophies. There are only a few case reports showing association of Sturge-Weber syndrome (SWS) with osteohypertrophy of maxillofacial region [1-3]. SWS is a rare congenital neurocutaneous syndrome with a prevalence of 1:50,000 live births with equal sex distribution and no racial predilection [4]. This syndrome has variable phenotypic expression usually manifestated as cutaneous capillary angioma, congenital glaucoma with ipsilateral leptomeningeal angiomatosis. Here we report an unusual case showing the association of SWS with maxillofacial osteohypertrophy in an adult patient.

Case Presentation

A 23 year old female patient reported with the complaint of malalignment of teeth. Her medical history revealed reddish discoloration on left side of face since birth. She also gave histoty of abnormal growth pattern of left maxillofacial region. The patient was mentally normal with no previous medication and non-conbtributory family history. On extraoral examination, port-wine stains were present unilaterally on left side of face extending from forehead to eyelids, cheek, nose, philtrum and upper lip (Figures 1a and 1b). On applying digital pressure, blanching of port wine stains was noticed. Facial asymmetry due to hypertrophy of left side of face was evident resulting in deviated plane of occlusion (Figure 1c). Intraoral examination showed reddish discoloration of gingiva in premolar-molar region (Figure 2). Dental malocclusion including deep overbite was present which might be due to regional osseous overgrowth. Clinical signs of port wine angioma were present in the eye of affected side.


Figure 1: Extraoral examination showing portwine stain present unilaterally on left side of face (a) front view, (b) lateral view and (c) deviated plane of occlusion due to facial hypertrophy of left side.


Figure 2: Intraoral view showing unilateral reddish discoloration of gingiva on left side at premolar-molar region.


Figure 3: (a) Submentovertex radiograph showing more anteriorly placed zygomatic prominence and zygomatic arch on the left side. (b) Posterio-Anterior cephalogram showing increased vertical maxillary height on the left side.

Submentovertex radiograph revealed more anteriorly placed zygomatic prominence and zygomatic arch on the affected (left) side than normal side. The condyles and pterygomaxillary fissure were found to be bilaterally symmetrical in both sagittal and transverse direction. However, in the cranial base, foramen spinosum, on the left side, was placed more anteriorly than its counterpart (Figure 3).

Posterio-Anterior cephalogram clearly showed increased vertical maxillary height on the left side which is in agreement with the transverse cant in the occlusal plane (Figure 1c). The ramus height on the affected side was marginally higher as compared to the opposite side as a compensation of the increased vertical maxillary height on the affected side which led to facial asymmetry.

Diagnosis of Sturge-Weber Syndrome associated with maxillofacial osteohypertrophy was made on the basis of clinical and radiographic examination.


Osseous involvement in form of any hypertrophy, lysis, hypoplastic changes or other abnormality has been illustrated in relation to different vascular malformations but its incidence in association with SWS is not known [5,6]. Osteohypertrophy is benign osseous overgrowth that can be described as angiodysplasia that entail some vascular abnormality which is related to secondary changes including further vascular malformations and bony overgrowth [3]. There is scanty literature available and only a few number of cases [1-3,6] reported for SWS associated with maxillofacial hypertrophy as seen in this case. Facial bone overgrowth may lead to severe facial disfigurement with facial and dental asymmetry, occlusal canting and malocclusion. Gingival enlargement may also be present that can be due to hypertrophy, antiepileptic medications such as phenytoin sodium, or a combination of both [7].

A vascular malformation–induced osseous change can be a strong consideration by virtue of close association with the distribution of capillary angioma, the underlying mechanism may be varied and remains elusive [1]. Boyd et al. [5] has suggested possible mechanisms including mechanical, physiologic, and developmental processes that could explain for the alteration of skeletal growth pattern in various vascular malformations.

Treatment rendered depends on the severity of symptoms. Osseous overgrowth can be corrected by surgical intervention [6]. Laser therapy, dermaabrasion or tattoing can be done for port wine stain as these can cause psychological trauma to the patient. Further medication can be given on basis of any symptom such as seizure, headache, glaucoma or complaints of bleeding.

Conflict of Interest



Select your language of interest to view the total content in your interested language
Post your comment

Share This Article

Relevant Topics

Recommended Conferences

  • Asia-Pacific Dental and Oral Care Congress
    October 26-28, 2017 Osaka, Japan
  • American World Dentistry
    November 13-14, 2017 San Antonio, USA
  • 39th World Dental Congress Summit
    Dec 4-6,2017 Sao Paulo,Brazil

Article Usage

  • Total views: 272
  • [From(publication date):
    April-2017 - Aug 24, 2017]
  • Breakdown by view type
  • HTML page views : 243
  • PDF downloads :29

Post your comment

captcha   Reload  Can't read the image? click here to refresh

Peer Reviewed Journals
Make the best use of Scientific Research and information from our 700 + peer reviewed, Open Access Journals
International Conferences 2017-18
Meet Inspiring Speakers and Experts at our 3000+ Global Annual Meetings

Contact Us

Agri, Food, Aqua and Veterinary Science Journals

Dr. Krish

[email protected]

1-702-714-7001 Extn: 9040

Clinical and Biochemistry Journals

Datta A

[email protected]

1-702-714-7001Extn: 9037

Business & Management Journals


[email protected]

1-702-714-7001Extn: 9042

Chemical Engineering and Chemistry Journals

Gabriel Shaw

[email protected]

1-702-714-7001 Extn: 9040

Earth & Environmental Sciences

Katie Wilson

[email protected]

1-702-714-7001Extn: 9042

Engineering Journals

James Franklin

[email protected]

1-702-714-7001Extn: 9042

General Science and Health care Journals

Andrea Jason

[email protected]

1-702-714-7001Extn: 9043

Genetics and Molecular Biology Journals

Anna Melissa

[email protected]

1-702-714-7001 Extn: 9006

Immunology & Microbiology Journals

David Gorantl

[email protected]

1-702-714-7001Extn: 9014

Informatics Journals

Stephanie Skinner

[email protected]

1-702-714-7001Extn: 9039

Material Sciences Journals

Rachle Green

[email protected]

1-702-714-7001Extn: 9039

Mathematics and Physics Journals

Jim Willison

[email protected]

1-702-714-7001 Extn: 9042

Medical Journals

Nimmi Anna

[email protected]

1-702-714-7001 Extn: 9038

Neuroscience & Psychology Journals

Nathan T

[email protected]

1-702-714-7001Extn: 9041

Pharmaceutical Sciences Journals

John Behannon

[email protected]

1-702-714-7001Extn: 9007

Social & Political Science Journals

Steve Harry

[email protected]

1-702-714-7001 Extn: 9042

© 2008-2017 OMICS International - Open Access Publisher. Best viewed in Mozilla Firefox | Google Chrome | Above IE 7.0 version