Dental School, Aristotle University of Thessaloniki, 54124, Thessaloniki, Greece.
Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece.
Clin Oral Investig. 2021 Jun;25(6):3599-3607. doi: 10.1007/s00784-020-03682-x. Epub 2020 Nov 13.
Hereditary gingival fibromatosis (HGF) is an uncommon, inherited condition with slow and progressive fibrous hyperplasia of the gingiva. Due to its association with mastication, speech, and occlusion problems, early diagnosis is important. We sought to summarize the available data regarding the epidemiology, clinical characteristics, and outcomes of children with HGF (< 18 years).
A systematic literature review of the MEDLINE and Cochrane Library databases was conducted with respect to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement (end-of-search date: March 1, 2019).
A total of 99 articles reporting on 146 patients were included. The mean age was 10.82 ± 3.93 years, and generalized gingival enlargement was seen in 97.16% (95% CI 92.69 to 99.14). Jaw, gingival, and teeth abnormalities; poor oral hygiene; eating; or speech difficulties were typical HGF-induced, while 60.90% had extraoral manifestations (95% CI 52.41 to 68.78). The disease was most commonly inherited in an autosomal dominant manner (88.41%, 95% CI 78.5 to 94.26), and about one-third of the patients had syndromic HGF (33.85%, 95% CI 23.50 to 46.00). Gingivectomy was performed in the majority of cases (91.15%, 95% CI 84.31 to 95.29), and recurrence was seen in 33.85% (95% CI 23.50 to 46.00).
HGF should be suspected in children with nodularity and gingival fibrosis, teeth abnormalities, or jaw distortion. Family history can help to establish the diagnosis.
More cases should focus on longer-term follow-up after gingivectomy as disease recurrence is not uncommon.
遗传性牙龈纤维瘤病(HGF)是一种罕见的遗传性疾病,表现为牙龈的纤维组织缓慢、进行性增生。由于其与咀嚼、言语和咬合问题有关,早期诊断很重要。我们旨在总结有关儿童 HGF(<18 岁)的流行病学、临床特征和结局的现有数据。
根据系统评价和荟萃分析的首选报告项目声明,对 MEDLINE 和 Cochrane 图书馆数据库进行了系统文献检索(搜索截止日期:2019 年 3 月 1 日)。
共纳入 99 篇报道 146 例患者的文章。平均年龄为 10.82±3.93 岁,97.16%(95%置信区间 92.69 至 99.14)的患者表现为牙龈弥漫性肿大。颌骨、牙龈和牙齿异常、口腔卫生差、咀嚼或言语困难是典型的 HGF 引起的症状,而 60.90%(95%置信区间 52.41 至 68.78)的患者有颌外表现。该病最常以常染色体显性遗传方式遗传(88.41%,95%置信区间 78.5 至 94.26),约三分之一的患者为综合征性 HGF(33.85%,95%置信区间 23.50 至 46.00)。大多数患者接受了牙龈切除术(91.15%,95%置信区间 84.31 至 95.29),复发率为 33.85%(95%置信区间 23.50 至 46.00)。
儿童出现结节性和牙龈纤维瘤、牙齿异常或颌骨畸形时,应怀疑为 HGF。家族史有助于确诊。
鉴于疾病复发并不少见,更多的病例应关注牙龈切除术后的长期随访。