Harrison M, Odell EW, Agrawal M Gingival fibromatosis with prune-belly syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1998; 86:304-307 https://doi.org/10.1016/s1079-2104(98)90176-7
Beaumont J, Chesterman J, Kellett M, Durey K. Gingival overgrowth: Part 1: aetiology and clinical diagnosis. Br Dent J. 2017; 222:85-91 https://doi.org/10.1038/sj.bdj.2017.71
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Hereditary gingival fibromatosis (HGF) is a benign, non-haemorrhagic, fibrous gingival overgrowth of unknown cause. Clinically, gingivae can be seen to partially or fully cover teeth, and can limit oral function, for example eating, speaking, and maintaining oral hygiene. HGF normally starts in the transition between the primary and secondary dentition. Surgical excision is often required. This case describes the management of a 37-year-old male with generalized HGF with extensive bilateral palatal growths extending into the oropharynx; not a reported presentation of HGF. Awareness of the condition and familial history could aid management at an earlier stage.
CPD/Clinical Relevance: This article describes the management of an uncommon presentation of hereditary gingival fibromatosis in the palate that affected the patient's oral function.
Article
Hereditary gingival fibromatosis (HGF), also known as hereditary gingival hyperplasia, is a benign, non-haemorrhagic gingival overgrowth. It can make the maxilla and mandible appear bulky, fully or partially cover teeth, and alter the function of the oral cavity.1,2 Its prevalence is relatively low (1:175,000) and the condition can present with or without syndromic associations.1,3 There is no evidence that it affects the alveolus; however, when tissues become plaque retentive, a secondary periodontal disease can occur.
HGF normally appears with the eruption of the permanent dentition.1 Classically, it presents with no inflammation, a fibrous appearance, and marked stippling. HGF can be generalized or localized to specific sites, typically the maxillary tuberosities or the labial gingivae around the mandibular molars.1
The aetiopathogenesis of HGF is unknown, although it is generally considered to have an autosomal dominant pattern with 20% of cases presenting with no familial association.4 There are changes in cell size and subepithelial fibroblast proliferation, with modifications in the extra-cellular matrix whereby there is an increase in collagen and fibronectin synthesis, and a reduction in matrix metalloproteinases.1,2,5
Syndromes that may be associated with HGF include craniofacial dysmorphism, infantile systemic hyalinosis, Murray–Puretic–Drescher syndrome, amelogenesis imperfecta, Ramon syndrome, and rarer conditions such as Prune-belly syndrome.3,4 It has been documented that squamous cell carcinoma can develop from the tissue.6 Other causes of gingival enlargement include drug-induced hyperplasia (by phenytoin, nifedipine and cyclosporine); however, this would be excluded prior to diagnosing HGF.1,7
Treatment involves surgical removal of the excess tissue; the timing of surgery can be controversial. HGF undergoes most changes during the eruption of teeth, and removing it once the permanent dentition has been established will reduce the risk of recurrence. However, when function is compromised with for example, difficulties with speech, swallowing, and mastication, alongside unsatisfactory aesthetics and retention of primary teeth, there is an argument for early intervention.1
The literature describes generalized hyperplasia affecting the attached gingiva.1,2,3,4,5,7,8 Rare cases document complete dental arch coverage;9 however, there are no alternative reported presentations.
Case report
A 37-year-old, fit and well male was referred to the department of oral surgery by his general dental practitioner (GDP) for excessive gingival overgrowth. This had been present for over 20 years and gradually increased in size. He reported a change in his speech and difficulty eating due to the palatal overgrowths. He was keen for treatment because it had become a potential risk for airway obstruction. His mother and maternal aunts had also exhibited similar features of gingival overgrowth, which had required surgical excision.
Examination revealed extensive bilateral palatal gingival overgrowth, pedunculated posteriorly, extending into the oro-pharynx; the soft palate was not visible. There was mild to moderate buccal gingival overgrowth, which did not cover his teeth (Figure 1).
Radiographic imaging ruled out any underlying hard tissue changes, and, as a result, a suspected diagnosis of HFG was made.
Elective surgical excision of the excessive palatal gingival overgrowths was carried out under general anaesthetic using monopolar electrosurgery (Figure 2). In order to intubate, a nasal tube was required owing to the minimal oral airway access, safety in maintaining a patent airway on induction, and the need for procedural access. The masses removed each measured 6 x 3 x 2 cm (Figure 3). The hard palate was sound, although high vaulted, and the attachment of the tissue was from the maxillary premolars to the tuberosities.
Despite this case having a rare severe palatal involvement extending towards the pharyngeal space, the histopathology demonstrated features typical of HGF – an increased amount of collagen fibres that were haphazardly distributed, a sparse number of cells distributed among the collagen fibres, and clusters of chronic inflammatory cells in the subepithelial tissue.
There was no recurrence of the overgrowth after the surgical excision (Figures 4 and 5). The patient is now under the care of a periodontal specialist for maintenance.
Conclusion
HGF is an uncommon disease that normally forms during the eruption of primary teeth. The atypical presentation in this case, owing to the presence of excess fibrous gingivae in the palate, contributed to limited oral function and potential airway obstruction. A multidisciplinary team approach was essential to manage this patient's needs in the peri-operative stages – airway management, surgical planning, and maintenance. In such cases, patients should be warned that there is a risk of recurrence and that they should maintain good oral hygiene. Furthermore early presentation could help prevent gingival overgrowth becoming potentially life threatening.