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Case Reports
. 2012 Aug;4(Suppl 2):S349-52.
doi: 10.4103/0975-7406.100304.

Sturge-Weber syndrome

Natarajan Manivannan  1 , Subramanium Gokulanathan, Ramakrishnan Swamy Ahathya, Gubernath, Rajkumar Daniel, Shanmugasundaram
Affiliations
Case Reports

Sturge-Weber syndrome

Natarajan Manivannan et al. J Pharm Bioallied Sci. 2012 Aug.

Abstract

Encephalotrigeminal angiomatosis (Sturge-Weber syndrome) is a rather uncommon congenital condition characterized by the combination of venous angioma of the leptomeninges over the cerebral cortex with ipsilateral angiomatous lesions of the face, and sometimes the skull, jaws, and oral soft tissues. A case of portwine stain with intraoral gingival hemangioma is presented. There were no other systemic manifestations. Patient reported with a complaint of localized tumor-like swelling in gums. Based on the presence of sharply demarcated vascular lesion unilaterally on the face and with ipsilateral intraoral vascular hyperplasia in the lip and gingiva, a variant of encephalotrigeminal angiomatosis was diagnosed. Ultrasound Doppler flowmetry was used to determine the blood flow. Dental management included plaque control instructions, scaling, root planning, and excision of the lesion done under general anesthesia. Close follow-up and meticulous plaque control have kept the oral condition under fairly good control.

Keywords: Hemangioma; Sturge–Weber syndrome; portwine stain; ultrasound doppler.

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Conflict of interest statement

Conflict of Interest: None declared.

Figures

Figure 1
Figure 1
Extraoral view
Figure 2
Figure 2
Intraoral view of gingival hemangioma
Figure 3
Figure 3
Intraoral occlusal view (mirror image)
Figure 4
Figure 4
Color Doppler of the intraoral lesion
Figure 5
Figure 5
Spectral Doppler flowmetry of the intraoral lesion
Figure 6
Figure 6
Electrosurgical excision under GA
Figure 7
Figure 7
Histopathologic view showing numerous dilated capillaries
Figure 8
Figure 8
One month postoperative view showing satisfactory healing
Figure 9
Figure 9
Sixth month postoperative view showing remission of the lesion
Figure 10
Figure 10
Sixth month postoperative view showing remission of the lesion
See this image and copyright information in PMC

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