Sturge Weber syndrome: A case report

Authors

  • Pournima Godge Terna dental college
  • Shubhra Sharma Terna Dental college
  • Monica Yadav Terna dental college
  • Pallavi Patil VYWS Dental College and Hospital
  • Sandeep Kulkarni Terna dental college

Keywords:

Sturge-Weber Syndrome, Portwine stain, encephalotrigeminal syndrome, oral mucosa

Abstract

Purpose: The Sturge-Weber syndrome (SWS) is a rare congenital disorder that occurs sporadically and features lesions of both skin and nervous system. We report a case of 7 year-old girl with Sturge-Weber syndrome and discuss its clinicopathological features, differential diagnosis and also emphasize the importance of its diagnosis in the clinical oral practice. Case Description: The classic pathognomonic manifestations include angioma of the leptomeninges extending to cerebral cortex with ipsilateral angiomatous lesions, unilateral facial nevus affecting a division of trigeminal nerve, hemiparesis, intracranial calcification, mental retardation and refractory epilepsy. The most apparent indication of SWS is a facial birthmark or “Port Wine Stain” present at birth and typically involving at least one upper eyelid and the forehead. Management of a patient with Sturge-Weber Syndrome may be challenging due to risk of hemorrhage. Conclusion: The dentists should focus on comprehensive therapy, starting with behavior management and stress on preventive measures.

Author Biographies

Pournima Godge, Terna dental college

Prof. Dept Of Oral Pathology

Shubhra Sharma, Terna Dental college

Lecturer, Dept Of Oral Pathology

Monica Yadav, Terna dental college

Prof, Terna dental college

Pallavi Patil, VYWS Dental College and Hospital

Lecturer, VYWS Dental College and Hospital

Sandeep Kulkarni, Terna dental college

Lecturer, Terna dental college

Downloads

Published

2011-11-15

Issue

Section

Case Report