Oral Surgery in Patients With Sturge-Weber Syndrome

Abstract

The Sturge-Weber syndrome (SSW) is a congenital neurocutaneous malformation, with angiomas involving the leptomeningea and facial skin. This syndrome is characterized by corticocerebral angiomatosis, cerebral calcifications, ocular affections, mental retardation, increased risk of stroke, counterlateral hemiplegia, and seizures. Another important feature of SSW is the flameus nevus on the face. In the oral cavity, SSW appears as hemangiomatous lesions affecting the mucous membranes and occasionally the dental pulp. Gingival hyperplasia may be present due to the use of anticonvulsant drugs. The present article reports the management of 2 female patients with Sturge-Weber syndrome who required oral surgery in regions affected by hemangiomatous lesions. In the first case, no hemostatic agents were necessary. On the other hand, the second case required the use of several hemostatic agents to control hemorrhage during surgery. Both patients recovered uneventfully without episodes of bleeding or infection.

Non-surgical treatment of idiopathic gingival enlargement: A case report

“…In the oral cavity, SWS manifested as a hemangiomatous lesion involving the mucosa. [ 19 ] Based on the patient’s clinical examination, imaging, comparison of the healthy and affected sides of the gingiva, and general condition, we diagnosed the patient with idiopathic GE, which may be associated with facial capillary malformations.…”

Sturge-Weber Syndrome: A Review of Pathophysiology, Genetics, Clinical Features, and Current Management Approache

“…For this reason, careful pre-operative planning should be carefully done prior to any dental surgery and a multidisciplinary approach is recommended in the hands of an experienced pediatric dentist and maxillofacial surgeon at tertiary centers rather than outpatient dental/ surgical offices, which may offer limited support. 64 Some additional features are to be considered and screened in patients with SWS (Table 3). Hyper and undergrowth have been reported in previous studies.…”

Undiagnosed Epileptic Case Since Childhood of Sturge-Weber Syndrome: First Case Report from Somalia

“… 4 However the feature which is commonly found in the oral cavity is gingival hemangioma, which mostly affects the ipsilateral area of maxilla, mandible, mouth’s floor, lips, plate, tongue and jugal mucosa. 5 Radiological investigations are the most useful, with standard skull radiography, computed tomography, and magnetic resonance imaging all playing important roles in revealing brain changes. 6 Other investigative techniques include electroencephalography (EEG) to assess brain function and ocular ultrasonography to discover ocular abnormalities.…”