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
Highlights
Sturge Weber syndrome (SWS) was first described by Schirmer in 1860 and later by Sturge in 1879, who associated dermatological and ophthalmic changes of the disease to neurologic manifestations
Sturge-Weber syndrome is a sporadic neurocutaneous disease characterized by facial port-wine stain, ocular abnormalities and leptomeningeal angioma 1,2
Sturge Weber Syndrome which is known as Sturge Weber Disease, Leptomeningofacial Angiomatosis is characterized by facial port wine stains, ocular abnormalities and leptomeningial angiomas
Summary
Sturge Weber syndrome (SWS) was first described by Schirmer in 1860 and later by Sturge in 1879, who associated dermatological and ophthalmic changes of the disease to neurologic manifestations. It is believed to be caused by the persistence of vascular plexus around the cephalic portion of the neural tube This plexus develops during the sixth week of I.U. development but normally undergoes regression during ninth week[4]. The cutaneous angiomas are called port wine stains, which usually occur unilaterally along dermatomes supplied by the ophthalmic and maxillary division of trigeminal nerve. It may Case Report A 12 years old Bangladeshi girl reported to us with a complaint of red pigmentation on her left face and oral cavity. Angiomatosis may involve lips, buccal mucosa, palate, gingiva, and floor of mouth This syndrome is of rare occurrence and management becomes complicated due to risk of hemorrhage. As there was no complications, it was decided to give follow up of the patient to see the progression and any complications
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