Sturge Weber Syndrome: A Case Study

Abstract

The aim of this case review was to touch upon the various clinical presentations and diagnostic features of Sturge-Weber syndrome (SWS) as seen in the dental/medical practice. Sturge-Weber syndrome is a rare congenital disorder that belongs to a group of disorders collectively known as the phakomatoses. The characteristic pathological elements of the disease include leptomeninges angioma extending out to cerebral cortex with angiomatous lesions on the same side and unilateral facial nevus that affects trigeminal nerve division. The classic oral lesions involve haemangiomatous gingival lesion limited on the same side of upper or lower jaw. An 18-year-old female patient reported to the Department of Oral Medicine and Radiology with oral manifestations of SWS. The evaluation of the patient, radiological findings as well as the management is discussed in this case report. It is important for the dental physician to be aware of the non-oral manifestations of SWS in order to identify and manage it appropriately. The challenge here is to see the oral manifestation as part of the syndrome and not as an isolated oral condition. Early diagnosis and appropriate treatment plan is imperative to prevent development of complications.

Keywords

• gingival hyperplasia
• phakomatoses
• port-wine stain