Journal of the korean academy of Pediatric Dentistry (대한소아치과학회지)

Korean Academy of Pediatric Dentistry (대한소아치과학회)
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STURGE-WEBER SYNDROME : A CASE REPORT

Sturge-Weber Syndrome 환아의 치험례

  • Shin, Hye-Sung (Department of Pediatric Dentistry, Chonnam National University School of Dentistry, Dental Research Institute and Second stage of BK21) ;
  • Yang, Kyu-Ho (Department of Pediatric Dentistry, Chonnam National University School of Dentistry, Dental Research Institute and Second stage of BK21) ;
  • Choi, Nam-Ki (Department of Pediatric Dentistry, Chonnam National University School of Dentistry, Dental Research Institute and Second stage of BK21) ;
  • Kim, Seon-Mi (Department of Pediatric Dentistry, Chonnam National University School of Dentistry, Dental Research Institute and Second stage of BK21)
  • 신혜성 (전남대학교 치의학전문대학원 소아치과학교실 및 치의학연구소 및 2단계 BK21) ;
  • 양규호 (전남대학교 치의학전문대학원 소아치과학교실 및 치의학연구소 및 2단계 BK21) ;
  • 최남기 (전남대학교 치의학전문대학원 소아치과학교실 및 치의학연구소 및 2단계 BK21) ;
  • 김선미 (전남대학교 치의학전문대학원 소아치과학교실 및 치의학연구소 및 2단계 BK21)
  • Published : 2009.02.27
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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.

Sturge-Weber Syndrome은 드문 선천성 질환으로 안면의 삼차신경 분포영역에 포도주양 반점(port wine nevus)을 나타내고, 녹내장 등의 안구 증상과 간질, 편측마비 등의 신경학적 증상 등을 동반한다. 또한 구강 내 증상으로 구강 점막의 편측성 혈관 증식, 치은의 혈관 증식, 치은 비대, 거대치, 편측성 거대설, 상악 또는 하악의 혈관 이상, 치아 맹출 이상 등을 나타낸다. 본 증례는 Sturge-Weber Syndrome으로 진단된 8세 남아의 구강 내 증상과 설강직증의 치료를 위해 설소대 절제술을 시행하여 양호한 결과를 보여 이를 보고하고자 한다.

Keywords

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