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Case Reports
. 2017 May;11(5):ZD12-ZD14.
doi: 10.7860/JCDR/2017/25593.9891. Epub 2017 May 1.

Sturge Weber Syndrome: A Case Study

Mahesh Neerupakam  1 , Podduturi Sanjay Reddy  2 , Beeraboina Anand Babu  3 , Guttikonda Vamsi Krishna  4
Affiliations
Case Reports

Sturge Weber Syndrome: A Case Study

Mahesh Neerupakam et al. J Clin Diagn Res. 2017 May.

Abstract

The aim of this case review was to touch upon the various clinical presentations and diagnostic features of Sturge-Weber syndrome (SWS) as seen in the dental/medical practice. Sturge-Weber syndrome is a rare congenital disorder that belongs to a group of disorders collectively known as the phakomatoses. The characteristic pathological elements of the disease include leptomeninges angioma extending out to cerebral cortex with angiomatous lesions on the same side and unilateral facial nevus that affects trigeminal nerve division. The classic oral lesions involve haemangiomatous gingival lesion limited on the same side of upper or lower jaw. An 18-year-old female patient reported to the Department of Oral Medicine and Radiology with oral manifestations of SWS. The evaluation of the patient, radiological findings as well as the management is discussed in this case report. It is important for the dental physician to be aware of the non-oral manifestations of SWS in order to identify and manage it appropriately. The challenge here is to see the oral manifestation as part of the syndrome and not as an isolated oral condition. Early diagnosis and appropriate treatment plan is imperative to prevent development of complications.

Keywords: Gingival hyperplasia; Phakomatoses; Port-wine stain.

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Figures

[Table/Fig-1]:
[Table/Fig-1]:
Port wine stain on the right side of face.
[Table/Fig-2]:
[Table/Fig-2]:
Enlarged right eye lobe with bluish discoloration of bulbar conjunctiva.
[Table/Fig-3]:
[Table/Fig-3]:
Gingival hyperplasia on the right side.
[Table/Fig-4]:
[Table/Fig-4]:
Palatal ecchymosis.
[Table/Fig-5]:
[Table/Fig-5]:
Lateral skull showing slight prominence of the vessels.
[Table/Fig-6,7]:
[Table/Fig-6,7]:
Prominent right optical lobe with no significant brain parenchymal abnormality. (Images from left to right)
[Table/Fig-8]:
[Table/Fig-8]:
MRI showing generalized gingival hyperplasia of the right side.
[Table/Fig-9]:
[Table/Fig-9]:
Bilateral mild choroid plexus enlargements.
[Table/Fig-10]:
[Table/Fig-10]:
Right buphthalamos. (Images from left to right)
See this image and copyright information in PMC

References

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