• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.1
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• pp.145-149
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• 2009

Sturge-Weber Syndrome is a rare congenital disorder and is characterized by port wine nevus following one or more divisions of trigeminal nerve, ocular involvement(eg, glaucoma) and neurologic involvement(eg, epilepsy, mental retardation). Oral menifestations include unilateral blood vessel expansion of the oral mucosa and gingiva, gingival hyperplasia, giant tooth, ipsilateral large tongue, blood vessel anomaly of maxilla or mandible and abnormal eruption sequence. This case report is about 8-year-old Sturge-Weber Syndrome patient presented violet discoloration on upper gingiva and buccal mucosa, gingival hyperplasia and abnormal eruption sequence. In this case, we performed lingual frenectomy and periodic oral hygiene management, and obtained satisfactory result.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.6 no.1
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• pp.15-18
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• 2010

Sturge-Weber syndrome is a rare nonhereditary developmental condition that is characterized by a hamartomatous vascular proliferation involving the tissue of brain and face. The clinical features are characterized by port wine nevus following one or more divisions of trigeminal nerve, ocular involvement and neurologic involvement such as epilepsy, mental retardation, and contralateral hemiplegia. Oral manifestations include unilateral blood vessel expansion of the oral mucosa, vascular hyperplasia of gingiva, pyogenic granuloma-like massive hemangiomatous proliferation of oral mucosa, macrodontia, ipsilateral macroglossia, blood vessel anomaly of maxilla or mandible and abnormal tooth eruption sequence. This case report is about 11-year-old Sturge-Weber syndrome patient presented port wine nevus on the face, venous malformation on soft plate and buccal mucosa. In this case we performed simple extraction of several deciduous teeth and periodic oral hygiene management. If a patient with Sturge-Weber syndrome has to undergo dental surgery in affected areas of the mouth, great care must be taken to prevent severe hemorrhage.

• Clinical and Experimental Pediatrics
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• v.46 no.9
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• pp.909-912
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• 2003

Klippel-Trenaunay syndrome is a rare mesodermal phakomatosis characterized by cutaneous haemangiomata( usually unilateral and involving an extremity), venous varicosities and osseous and soft tissue hypertrophy, of the affected limb. Sturge-Weber syndrome, also a mesodermal pharkomatosis, is characterized by a port-wine nevus, which is present from birth and covers the face and cranium in the territory of the first division of the trigeminal nerve. Homolateral to the skin lesion, there is atrophy and calcification of the cerebral hemisphere. We experienced an unusual 26-months-old female, who had features of both Klippel-Trenaunay syndrome and Sturge-Weber syndrome. She had an extensive nevus flammeus which extended primarily over both sides of her face and the right side of the trunk and extremities, hypertrophy of the right extremity without evidence of arterovenous fistula, right glaucoma, choroidal hemangioma and leptomeningeal hemangioma, which combined Klippel-Trenaunay syndrome and Sturge-Weber syndrome. We reported this rare case with a brief review of some related literatures.

• Journal of Dental Anesthesia and Pain Medicine
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• v.15 no.3
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• pp.173-179
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• 2015

Issues related to the control of seizures and bleeding, as well as behavioral management due to mental retardation, render dental treatment less accessible or impossible for patients with Sturge-Weber syndrome (SWS). A 41-year-old man with SWS visited a dental clinic for rehabilitation of missing dentition. A bilateral port-wine facial nevus and intraoral hemangiomatous swollen lesion of the left maxillary and mandibular gingivae, mucosa, and lips were noted. The patient exhibited extreme anxiety immediately after injection of a local anesthetic and required various dental treatments to be performed over multiple visits. Therefore, full-mouth rehabilitation over two visits with general anesthesia and two visits with target-controlled intravenous infusion of a sedative anesthesia were planned. Despite concerns regarding seizure control, bleeding control, and airway management, no specific complications occurred during the treatments, and the patient was satisfied with the results.