STURGE-WEBER SYNDROME : A CASE REPORT

Shin, Hye-Sung; Yang, Kyu-ho; Choi, Nam-ki; Kim, Seon-mi

Journal of the Korean Academy of Pediatric Dentistry 2009;36(1):145-149.
Published online February 28, 2009.

STURGE-WEBER SYNDROME : A CASE REPORT

Hye-Sung Shin, Kyu-ho Yang, Nam-ki Choi, Seon-mi Kim

Department of Pediatric Dentistry, Chonnam National University School of Dentistry, Dental Research Institute and Second stage of BK21

Sturge-Weber Syndrome 환아의 치험례

신혜성, 양규호, 최남기, 김선미

전남대학교 치의학전문대학원 소아치과학교실 및 치의학연구소 및 2단계 BK21

Correspondence: Seon-mi Kim, Tel: 062-530-5668, Email: hellopedo@hanmail.net

Received: 16 May 2008 • Accepted: 12 December 2008

Abstract

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.

Key Words: Sturge-Weber Syndrome, Lingual frenectomy, Port wine nevus