Sturge Weber Syndrome An Unusual Case with Multisystem Manifestations

Abstract

BACKGROUND: Sturge-Weber syndrome (SWS) is a rare congenital neurocutaneous disorder. It ischaracterized by the presence of facial port wine stains, neurological abnormalities like seizures andmental retardation, ocular disorders, oral involvement and leptomeningeal angiomas.CASE REPORT: A 13-year-old boy presented with the chief complaint of swollen, bleeding gums anddeposits on the teeth. Detailed medical and dental history, clinical examination and investigationsconfirmed the diagnosis of Sturge-Weber syndrome. The treatment comprised of a thorough plaquecontrol regimen to reduce the gingival enlargement, and it included oral hygiene instructions, thoroughscaling, root planing at regular intervals and plaque index scoring which motivated the patient at eachvisit.CONCLUSION: This case illustrates that early intervention in a patient with Sturge-Weber syndrome isquintessential because of its associated gingival vascular features and their complicating manifestations.Furthermore, the need for periodic oral examinations and maintenance of good oral hygiene to preventany complications from the oral vascular lesions has been highlighted.