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Sturge-Weber syndrome - A case report.

Shahid M Shaikh1, Mousumi Goswami2, Sanjay Singh3

  • 1Post Graduate Student, Department of Pedodontics & Preventive Dentistry, I.T.S. Dental College, Hospital & Research Center, Greater Noida, India.

Journal of Oral Biology and Craniofacial Research
|April 9, 2015
PubMed
Summary

Sturge-Weber syndrome (SWS) is a rare neurological disorder causing facial and brain abnormalities. This case highlights SWS with significant oral, ocular, and neurological symptoms.

Keywords:
Gingival enlargementPort-wine stainsSturge–Weber syndrome

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Area of Science:

  • Neurology
  • Genetics
  • Dermatology

Background:

  • Sturge-Weber syndrome (SWS), or encephalotrigeminal angiomatosis, is a rare congenital neurocutaneous disorder.
  • It is characterized by facial capillary malformations (port-wine stains) and neurological complications.
  • Vascular anomalies are a key feature, often affecting the brain and skin.

Purpose of the Study:

  • To report a case of Sturge-Weber syndrome.
  • To detail the oral, ocular, and neurological manifestations observed in this patient.
  • To contribute to the understanding of SWS presentation.

Main Methods:

  • Case report.
  • Clinical examination of oral, ocular, and neurological features.
  • Review of relevant literature.

Main Results:

  • The patient presented with characteristic facial capillary malformations.
  • Oral manifestations included gingival hemangiomatosis affecting the upper and lower jaws.
  • Associated ocular and neurological symptoms were noted.

Conclusions:

  • Sturge-Weber syndrome requires comprehensive evaluation due to its multi-systemic involvement.
  • Oral manifestations like gingival hemangiomatosis can be significant in SWS.
  • Early recognition and management are crucial for patients with SWS.