Idiopathic Gingival Fibromatosis: Review of Literature and a Case Report

Journal of Oral Health CASE REPORT Community& Dentistry

Idiopathic Gingival Fibromatosis: Review of Literature and A Case Report

Singhal R1, Rozra S2, Bhagol A3

Abstract Idiopathic Gingival Fibromatosis (IGF) is a rare condition of gingival enlargement that is characterized by gingival enlargement of normal colour and firm consistency that is non-haemorrhagic and asymptomatic. The purpose of this article is to report a case of 6-year old male child and review the literature related to IGF.

Keywords: Idiopathic gingival fibromatosis, Gingival enlargement, Hereditary gingival fibromatosis, Gingivomatosis, familial elephantiasis CASE REPORT 1 MDS INTRODUCTION Demonstrator ingival enlargement is the A six year old male child reported with the Department of Pedodontics & Preventive Dentistry chief complaint of slow growing, non-ten- Postgraduate Institute of Dental Sciences, overgrowth of the gingiva char Rohtak, Haryana, INDIA G acterized by an expansion and der gingival enlargement since birth. Ac- accumulation of the connective tissue with cording to his parents the enlargement be- 2 MDS Dental Surgeon occasional presence of increased number came more pronounced at the time of Rohtak, Haryana, INDIA of cells (1). The most common forms of eruption of deciduous teeth. The major concern of the patient and family was 3 MDS gingival enlargement are induced by sev- Assistant Professor eral factors such as inflammation, leukae- esthetic and functional problems includ- Department of Oral and Maxillofacial Surgery ing mastication, speech, and oral hygiene. Postgraduate Institute of Dental Sciences, mia, drugs and inheritance (2). Idiopathic Rohtak, Haryana, INDIA Gingival Fibromatosis (IGF) is a rare con- There was no history of epilepsy or long dition of gingival enlargement that is char- term medication for any other disease. acterized by gingival enlargement of nor- Family and the prenatal-natal history was mal colour and firm consistency that is non- non contributory. Skeletal growth of the haemorrhagic and asymptomatic (3,4). child was normal.

The aim of this article is to highlight the The patient had convex facial profile with diagnosis, clinical features and management incompetent lips and the nasal bridge was of a rare case of idiopathic gingival fibroma- flat. Intraoral examination revealed pale- tosis in a 6 year old boy.

Contact Author

Dr Ruchi Singhal [email protected] Figure 2: Preoperative right lateral Figure 1. Preoperative frontal view view J Oral Health Comm Dent 2013;7(3)174-177

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Figure 3. Preoperative left lateral view Figure 4. Preoperative palatal view pink, firm generalized gingival swelling Figure 5. Preoperative lingual view covering almost all deciduous posterior investigations including blood sugar level teeth with minimal involvement on upper study-casts were prepared. left side (Figure 1-5). Gingival enlargement Orthopantomogram were taken to rule out enclosed the major surface of the teeth bony changes, displacement and resorption present except the incisal/occlusal of the teeth. The results of the surfaces.Because of the enlarged gingival hematological investigations showed no tissue, the patient was not able to occlude abnormality. As the medical, family, prena- properly on the right posterior region (Fig- tal and drug histories were non-contribu- ure 1,2). tory, other reasons of gingival hyperplasia Figure 6. Segmental gingivectomy were excluded and the case was diagnosed performed on lower right side. After investigations like as idiopathic gingival fibromatosis. orthopantomogram and routine blood The case was planned for segmental gingivectomy of the hyperplastic tissue. After gingival contour and patient was able to initial diagnosis and treatment planning, maintain oral hygiene and was well satis- routine oral hygiene instructions were given fied with results (Figure 7-10). Histopatho- and oral prophylaxis was completed. Con- logical examination showed sidering the size and extent of gingival hyperparakeratinized hyperplastic stratified enlargement, a quadrant-wise gingivectomy squamous epithelium and thick fibrous was performed under local anaesthesia (Fig- connective tissue with dense collagen fibre ure 6). An external bevel gingivectomy was bundles (Figure 7). done in three quadrants excluding upper left side with an interval of about one REVIEW OF LITERATURE month in between each surgery. Gingival fibromatosis (GF) is a heteroge- neous group of disorders characterized by Surgeries were followed by histopathologi- progressive enlargement of the gingiva Figure 7. Histopathological view cal examination of the excised tissue and caused by an increase in submucosal con- showing thick fibrous connective postoperative care with regular follow up. nective tissue elements (5). tissue with dense collagen fibre Final postoperative result was acceptable bundles

Figure 8. Postoperative frontal Figure 9. Postoperative palatal Figure 10. Postoperative lingual view view view

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SYNONYMS pattern and experiences difficulty in speech type. There may be combination of both Synonyms of GF include gingivomatosis, and mastication (7). the types (8). diffuse fibroma, hereditary gingival fibromatosis, familial elephantiasis, Idi- ETIOLOGY HISTOLOGIC FEATURES opathic fibromatosis, elephantiasis Idiopathic gingival fibromatosis is a rare Histopathologically, the bulbous increased gingivae, congenital hypertrophy of the hereditary condition that has no definite connective tissue is relatively avascular and gingiva, fibromatosis gingivae, gigantism cause. The condition may manifest as an has densely arranged collagen-fibre bun- of the gingiva, symmetric fibroma of the autosomal-dominant or, less commonly, dles, numerous fibroblasts and mild palate, congenital macrogingivae, hereditary an autosomal-recessive mode of inherit- chronic inflammatory cells (6,24). The over- gingival hyperplasia, and hypertrophic ance, either as an isolated disorder or as lying epithelium is thickened and acan- gingival (2,6-8). part of a syndrome (3,6). Syndromic thotic, and has elongated rete ridges. The gingival fibromatosis has been associated nodular appearance can be attributed to the CLINICAL FEATURES with ancillary features such as hypertricho- thickened parahyperkeratinized epithelium Idiopathic gingival fibromatosis is a slowly sis, mental retardation, epilepsy, progres- (7). Unusual findings include the presence progressive benign enlargement that affects sive sensorineural hearing loss and abnor- of small calcified particles, amyloid depos- the marginal gingiva, attached gingiva and malities of the extremities, particularly of its, islands of odontogenic epithelium and interdental papilla (9). It varies from mini- the fingers and toes (11,21,22). Autosomal- osseous metaplasia in the connective tis- mal involvement of only the tuberosity dominant forms of gingival fibromato- sue, and ulcerations of the overlying mu- area and the buccal gingiva around the lower sis, which are usually nonsyndromic, have cosa (2,9). molars to generalized enlargement inhib- been genetically linked to the chromosome iting eruption of teeth (3,8,10,11). The 2p21-p222 and 5q13-q22 (23). Recently, a TREATMENT hyperplastic gingiva is pale-pink, firm, mutation in the son of sevenless-1 (SOS- Although there is a large consensus on the leathery in consistency that is non- 1) gene has been suggested as a possible modality of treatment to be performed in hemorrhagic and asymptomatic. Males and cause of isolated (nonsyndromic) gingival HGF patients, there are controversies females are equally affected at a phenotype fibromatosis, but no definite linkage has among the authors with regard to the ex- frequency of 1:175,000 (6,11). been established (24). Although over the act period in which it should be accom- past few years increasing efforts have been plished. According to several authors, the Although gingival tissue may appear nor- made to understand the genetic, molecular best time is when all of the permanent mal at birth, hyperplastic gingival fibroma- and cellular basis of gingival enlargement dentition has erupted, because the risk of tosis may become evident with the erup- of IGF patients, it has been impaired by recurrence is higher before it (6,10,15,26). tion of primary or permanent dentition, the intense clinical, genetic and biologic However, in some cases, a delay in the sur- suggesting a trauma -induced tissue reac- heterogeneity of the disease. gical treatment may cause significant con- tion during the eruption (4,6,7,12-14). sequences for the patients, such as primary Furthermore, the presence of teeth seems There are many syndromes associated with dentition retention with delay in the erup- to be necessary for HGF to occur because IGF like Zimmermann- Laband syn- tion of the permanent teeth, difficulties in the condition disappears or recedes with drome, Rutherfurd syndrome, Murray- mastication and phonation, the loss of the teeth (6,15). Puretic-Drescher syndrome, Cross syn- malpositioning of teeth, esthetic effects, drome, Prune belly syndrome, Ramon syn- and psychological problems for the patients COMPLICATIONS drome, Juvenile-hyaline syndrome and and relatives (4,10,17,18,20,27). The gingival enlargement results in both Jones syndrome (2,5,7,23,25). esthetic and functional problems for af- Treatment depends on the severity of en- fected individuals. The most common ef- INCIDENCE largement and shows varying degree of fects are diastemas, malpositioning of The number of patients with this oral le- success. When the enlargement is minimal, teeth, prolonged retention of primary den- sion has greatly increased in the last three thorough scaling of teeth and home care tition, delayed eruption, cross and open decades. Hereditary GF (HGF) is rare, af- may be all that are required to maintain bites, prominent lips and open lip posture fecting only one in 7,50,000 people (5,6). good appearance. On the other hand, (3,4,11-14,16-21). Although the gingival overgrowth tissues should be surgically enlargement does not directly affect the al- CLASSIFICATION removed (2). veolar bone, the gingival swelling may in- The condition has been classified into 2 crease the bacterial plaque accumulation, types; Nodular form characterized by pres- Although recurrence is unpredictable, it is inducing periodontitis, bone resorption ence of multiple tumors in the dental pa- most often seen in children and teenagers and halitosis (2). In extreme cases of mas- pillae and other form which is symmetric rather than adults (1,4). It has been dem- sive gingival enlargement, an affected child resulting in uniform enlargement of onstrated that recurrence is faster in areas usually develops abnormal swallowing gingival and represents the most common with dental plaque accumulation (18,27).

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