Sturge-Weber Syndrome: A Report of a Rare Case

Abstract

Sturge-Weber Syndrome (SWS) is a rare congenital developmental disorder that arises from the abnormal persistence of the embryonic vascular plexus. The syndrome encompasses hamartomatous malformations that can impact the skin, eyes, and nervous system. The broad array of clinical manifestations and potentially life-threatening complications underscores the crucial and imperative need for an accurate diagnosis. An effective treatment strategy for SWS patients involves a multidisciplinary approach. Dental procedures in these individuals pose challenges due to the potential risk for substantial bleeding during both intra- and postoperative phases. This article aims to document a rare case of Sturge Weber Syndrome in a 21-year-old female patient who presented with seizures, unilateral facial port wine stains, gingival hyperplasia, and intracranial calcifications.

Keywords: gingival hypertrophy; port wine stains; radiographic imaging; sturge-weber syndrome; tram track calcification.

Figure 1. Clinical picture showing a diffuse reddish-purplish patch (port-wine stain) on the left side of the face.

Figure 2. Intraoral picture showing port-wine stain involving the left buccal mucosa and lower labial mucosa.

Figure 3. Intraoral picture showing gingival enlargement.

Figure 4. Lateral skull radiograph showing mild eminence of vessels.

Figure 5. CT scan revealing gyriform calcifications in the left parieto-occipital region.

The red arrow depicting the typical intracranial calcifications (tram track appearance).

Figure 6. Post-treatment picture