Sturge Weber Syndrome- A Rare Congenital Neuro-cutaneous Disease

Authors

  • Wahida Sultana Resident, Dept. of Oral & Maxillofacial Surgery, Dhaka Dental College Hospital, Dhaka
  • Sufia Nasrin Rita Associate Professor and Head, Dept of Orthodontics, Sapporo Dental College Hospital, Dhaka
  • SM Anwar Sadat Lecturer, Dept. of Oral & Maxillofacial Surgery, Dhaka Dental College, Dhaka

DOI:

https://doi.org/10.3329/updcj.v5i2.27275

Keywords:

Sturge Weber syndrome, congenital, encephalotrigeminal angiomatosis, vascular lesions, oral and limb manifestations

Abstract

Sturge Weber Syndrome (SWS) or Encephalotrigeminal Angiomatosis is specifically a rare non hereditary congenital sporadic disorder of elusive etiology. It belongs to a group of disorders collectively known as the phakomatoses (motherspot diseases). It has a vast continuum of cutaneous, neurologic, ophthalmic and oral manifestations. It consists of congenital hamartomatous malformations that may affect the eye, skin, and central nervous system (CNS) at different times, characterized by the combination of venous angiomas of leptomeninges, face, jaws and oral soft tissues. The classic pathognomonic features of disease include angioma of the leptomeninges extending to cerebral cortex with ipsilateral angiomatous lesions, unilateral facial nevus after one division of trigeminal nerve and epileptic convulsions. The most characteristic oral manifestation is represented by gingival hemangiomatous lesion usually restricted to ipsilateral maxilla or mandible. We report a case of Sturge Weber Syndrome with its characteristic oral manifestations and review of relevant prevailing literature.

Update Dent. Coll. j: 2015; 5 (2): 47-51

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Author Biography

Wahida Sultana, Resident, Dept. of Oral & Maxillofacial Surgery, Dhaka Dental College Hospital, Dhaka



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Published

2016-04-07

How to Cite

Sultana, W., Rita, S. N., & Sadat, S. A. (2016). Sturge Weber Syndrome- A Rare Congenital Neuro-cutaneous Disease. Update Dental College Journal, 5(2), 41–51. https://doi.org/10.3329/updcj.v5i2.27275

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Section

Case Reports