출생 시 발생한 특발성 치은섬유종증 = IDIOPATHIC GINGIVAL FIBROMATOSIS AT BIRTH

국문 초록 (Abstract)

치은섬유종증(Gingival fibromaotsis)은 치은조직의 섬유성 증식을 나타내는 드문 구강질환이다. 원인은 명확히 규명되지 않았으나 상염색체 우성 또는 열성의 유전성이거나 특발성일수 있다. 주로 영구치 맹출 시기에 나타나기 시작하나, 드물게 유치열기나 출생시부터 이환되는 경우도 있다. 치은은 서서히 증식하여 치아의 해부학적 치관부위를 덮거나 구개 변이를 일으켜 혀 운동장애를 야기하거나 입술 폐쇄를 방해하기도 한다.
이 증례의 환아는 14개월 된 여아로 출생 시부터 계속딘 치은증식을 주소로 내원하였다. 특별한 가족력이나 의학적 병력은 없었고, 임상 유전검사 결과 알려진 어떤 증후군으로 진단되지 않았으나, 신체발달이 지연되어 있었다. 펀치 생검을 하였으며, 조직검사명은 치은섬유유종증이었다. 가족력이 없어 특발성 치은섬유종증으로 진단하였다. 환아의 연령 및 전신 상태를 고려해 치은 절제술 등의 외과적 치료는 연기하기로 하였다.

다국어 초록 (Multilingual Abstract)

Gingival fibromatosis is a rare condition characterized by varying degrees of gingival hyperplasia. Gingival fibromatosis usually occurs as an isolated disorder or can be associated with a variety of other syndromes. It usually appears at the time of eruption of permanent dentition but, can develop at the time of eruption of the primary dentition and rarely at birth. It may deform palatal contour and subsequently restrict the tongue movement, resulting in interference during speech and mastication. In addition, it incapacitates maintenance of normal lip closure.
A 14-month-old girl visited the department of pediatric dentistry, Yonsei University Dental Hospital, for the congenital gingival overgrowth. There was no one in the family, who showed similar pattern of gingival growth. The intraoral clinical examination revealed generalized severe enlargement throughout the maxillary and the mandibular arches. Enlarged gingival tissue was pink and had firm consistency. She was referred for chromosomal analysis, which confirmed absence of any known syndrome. Under local anesthesia, "Punch-biopsy" was performed on the labial area, and the specimen was histologically diagnosed as gingival fibromatosis. For she did not have any medical problem nor familiar history, she was diagnosed as having idiopathic gingival fibromatosis. Regarding her age and behavior, close follow-up was decided.

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