Syndromes with gingival fibromatosis: A systematic review

Abstract

Objective The aim of systematic review was to describe the phenotypes and molecular profiles of syndromes with gingival fibromatosis (GF). Methods A comprehensive search of PubMed, LILACS, Livivo, Scopus, and Web of Science was conducted using key terms relevant to the research questions and supplemented by a gray literature search. The Methodological Quality and Synthesis of Case Series and Case Reports in association with the Case Series and Prevalence Studies from the Joanna Briggs Institute critical appraisal tools were used for the risk of bias. We followed the PRISMA checklist guidelines. Results Eighty‐four studies reporting GF as an oral manifestation of a syndrome were identified in this review. Enamel renal syndrome was the most frequently reported syndrome with GF, represented by 54 individuals in 19 studies, followed by Zimmermann–Laband syndrome with 24 individuals in 15 studies and Costello syndrome, which was presented in a case series study with 41 individuals. Among reported cases, other clinical manifestations such as hypertrichosis, ectopic gingival calcification, and cherubism were described. Conclusions The results emphasize the need of systematic oro‐dental‐facial phenotyping for future descriptions as well as further molecular analysis in order to better understand the occurrence of syndromic GF.

Pathogenesis of Enamel-Renal Syndrome Associated Gingival Fibromatosis: A Proteomic Approach

“…Consistent with the ERS gingival phenotype, characterized by ECM accumulation and calcium deposition, most of the dysregulated proteins have biomineralization functions (49) and were previously associated to ectopic calcification and/or fibrosis (21,(50)(51)(52)(53).…”

High‐power laser as a treatment of recurrent gingival fibromatosis in a patient with a rare syndrome: A case report

“…In addition, there is a consensus that GF must be surgically removed conservatively, with excision of the enlarged gingival tissues and preservation of the tooth. 8 Although different techniques are possibly employed (scalpels, electrosurgery, and lasers), HPLs seem to be equally efficient or superior to scalpels. 7 Many studies reported the use of HPL in oral soft tissues, with good results, and this is the first report of this technique in these patients.…”

Periodontal Disease Associated with Genetic Disorders

“…The prevalence is unknown, the incidence is 1:175,000 according to phenotype and 1:350,000 according to genotype, and equal between males and females. HGF is commonly described as an isolated disorder (non-syndromic), or it can develop as a part of a syndrome (syndromic) like Cowden's syndrome, Zimmermann-laband syndrome, Cross syndrome, Rutherford syndrome, Ramon syndrome, Jones syndrome, Costello syndrome, Ectro-amelia syndrome, and hyaline fibromatosis syndrome [15]. In this part, we mainly discuss non-syndromic HGF.…”

“…In severe cases, the teeth are almost completely covered. The firm yet painless enlargement of the gingiva does not commonly affect the alveolar bone but can lead to the development of pseudo-pockets, which facilitate plaque accumulation due to suboptimal daily oral hygiene [13][14][15][16].…”

“…Common clinical complications secondary to gingival enlargement including but not limited to difficulties in speech, mastication, and occlusion, as well as changes in facial features. Interestingly, the condition may disappear or regress with the loss of teeth, thus suggesting that the presence of teeth might serve as one condition for the development of HGF [13][14][15][16].…”