Gingival Fibromatosis Associated with Zimmermann-Laband Syndrome: A Case Report

病例報告：Zimmermann-Laband Syndrome相關之牙齦纖維瘤

林佩萱(Pei-Hsuan Lin) ； 洪祥熙(Hsiang-Hsi Hong)

《臺灣牙周病醫學會雜誌》 19卷3期 (2014/09) Pp. 238-247

https://doi.org/10.6121/TAP.2014.19.3.06

摘要

牙齦纖維瘤的特徵為牙齦纖維組織無痛、非出血性的緩慢增生，他可以為單一偶發的病症，也可以合併系統性症候群一起發生。Zimmermann-Laband syndrome是一種極為罕見的全身性症候群，主要的表徵有牙齦纖維瘤，合併有手指和／或腳趾末端指節和／或指甲的缺失或發育不全，臉部表徵有球狀鼻、鬆弛的耳朵及濃密的眉毛及睫毛，部分關節可過度伸展，多毛症，肝脾腫大及智能不足。每個病例呈現出來的表現型差異很大。這個症候群最早是由Zimmermann在1928年的兩個單獨病例中提出，後於1964年Laband在一篇報導了一位母親及其7位孩子中的5位的病例報告中，對此症候群的病徵有了更詳盡的描述。在本篇病例報告中我們報導了一位男孩因為廣泛的牙齦腫大於5歲時第一次求診，根據其他全身性的表徵，我們診斷為Zimmermann-Laband syndrome，並將增生的牙齦手術切除。然而四年後，牙齦增生又復發了，因此又做了第二次的牙齦切除手術。在術後一年的回診追蹤，手術結果良好，但牙齦還是呈現有復發的傾向。針對這類的病人，定期回診追蹤並維持良好的口腔衛生是很重要的。

關鍵字

濟─拉二氏綜合症候群 ； 牙齦纖維瘤 ； 牙齦增生 ； 牙齦肥大

並列摘要

Gingival fibromatosis is characterized by non-hemorrhagic, painless, slowly-progressive, fibrous enlargement of gingival tissue with normal color. It can be an isolated condition or associated with a syndrome. Zimmermann-Laband Syndrome is a rare disorder characterized by gingival fibromatosis and is associated with the absence and/or hypoplasia of the nails or terminal phalanges of the hands and feet, abnormality of the nose and ears, hyperextensibility of joints, hirsutism, hepatosplenomegaly and mental retardation. However, variability of the phenotype haa been reported. It was first described by Zimmermann in 1928 in two isolated cases. This syndrome was delineated better by Laband in a case report that included a mother and five of her seven children in 1964. Here we reported a boy who first came to our dental clinic due to profuse gingival overgrowth at the age of 5 year-old. Based on the child's displayed systemic characteristics, a diagnosis of Zimmermann-Laband syndrome was given. Gingivectomy of the lower anterior area was performed at that time. However, the gingival overgrowth had recurred 4 years later and therefore a second gingivectomy was performed. At the 1-year follow-up, the treatment outcome was fair, but the gingival overgrowth was still showing a tendency to recur. Periodic examination with professional cleaning and oral hygiene instruction is deemed necessary when treating patients with this syndrome.

並列關鍵字

Zimmermann-Laband syndrome ； gingival fibromatosis ； gingival overgrowth ； gingival enlargement