Indian Journal of Radiology Indian Journal of Radiology  

   Login   | Users online: 3804

Home Bookmark this page Print this page Email this page Small font sizeDefault font size Increase font size     

 

HEAD AND NECK Table of Contents   
Year : 2002  |  Volume : 12  |  Issue : 3  |  Page : 335-338
Idiopathic gingival fibromatosis : A neglected case


VSS Medical College Hospital, At /P O Burla, Sambalpur. Orissa, India

Click here for correspondence address and email
 

Keywords: Gingival Fibromatosis, Gingival hypertrophy

How to cite this article:
Pappachan B, Narayan J, Nayak A. Idiopathic gingival fibromatosis : A neglected case. Indian J Radiol Imaging 2002;12:335-8

How to cite this URL:
Pappachan B, Narayan J, Nayak A. Idiopathic gingival fibromatosis : A neglected case. Indian J Radiol Imaging [serial online] 2002 [cited 2019 Dec 6];12:335-8. Available from: http://www.ijri.org/text.asp?2002/12/3/335/28474
Gingival fibromatosis is a heterogenous group of disorders characterized by progressive enlargement of the gingivae caused by an increase in submucosal connective tissue elements. Many cases are iatrogenic and some are inherited or idiopathic. We present a case of idiopathic gingival fibromatosis.


   Case Report Top


A thirty-year old male presented with gradual progressive enlargement of both gums since childhood with recent spurt in growth since two years which prevented proper speech articulation, mastication and caused inadequate lip apposition. There was no history of fever, prolonged medications, anorexia, weight loss, seizures or hearing loss. Examination revealed gross nodular enlargement of the gingivae (upper more than lower) and the hard palate, which were pink in colour with firm consistency. The teeth were barely visible as they were buried deep within the growth. There was no hypertrichosis. Routine laboratory investigations didn't reveal any abnormality. Since there was no family or relevant medication history, a clinical diagnosis of idiopathic gingival fibromatosis was made.

Anteroposterior and lateral radiographs of the skull and oblique view of the mandible revealed a soft tissue shadow superimposed on the maxilla and mandible with malalignment of the teeth, widening of the interdental space and distortion of anterior aspect of the maxillary arch. There was no evidence of any bone erosion. Few supernumerary teeth were also visible [Figure - 1].

Non-contrast axial CT slices showed a lobulated soft tissue mass of variable attenuation involving both the alveolar arches and the hard palate extending anteriorly and laterally beyond the alveolar margins and bulging posteriorly into the oropharynx [Figure - 2]. Small air bubbles were seen within the mass which partly represented the groove between the enlarged gingivae extending from both palatal surfaces of the teeth and was partly due to air introduced during the preceding aspiration cytology procedure [Figure - 3]. Contrast study shows the lesion to be faintly enhancing in nature as suggested by increased attenuation [Figure - 4]. Bone window showed distortion of the maxillary arch without any bony erosion and with malalignment of teeth and widening of the interdental space [Figure - 5]. Few supernumerary teeth were also visible within the mass [Figure - 6].

Surgical treatment in the form of simple gingivectomy was carried out. Histopathological study of the growth showed lining of stratified epithelium focally keratinized with elongated rete ridges showing tubular pattern and arborization. The subepithelial tissue is composed of interlacing bundles of dense collagen intervening with loose fibrovascular connective tissue.


   Discussion Top


Gingival Fibromatosis (GF) may occur as an inherited condition - Hereditary GF (HGF) or in association with medications like nifedipine, phenytoin, verapamil and cyclosporin (Iatrogenic GF) or may be Idiopathic (IGF). It can also be caused by inflammation and leukemic infiltration [1].

Synonyms of GF include Elephantiasis gingivae, Congenital hypertrophy of gingiva, Fibromatosis gingivae, Gigantism of gingiva, Symmetric fibroma of palate, Congenital macrogingivae, Hereditary gingiva hyperplasia and Hypertrophic gingiva [2],[3]. HGF is rare, affecting only 1 in 7,50,000 people [4]. It can be inherited as an autosomal dominant or recessive condition [2]. Members of the family across generations are affected and a positive family history is always present. In IGF no causative agent can be identified and a family history is always lacking.

GF can occur as an isolated condition or be associated with other diseases and syndromes and the involvement can be generalized or localized. Accordingly they are classified [2]:

  1. Isolated Hereditary GF:a) Generalized b) Localized
  2. Isolated Idiopathic GF:a) Generalized b) Localized
  3. GF with hypertrichosis
  4. GF with hypertrichosis and mental retardation and/or epilepsy.
  5. GF with mental retardation and/or epilepsy.
  6. GF associated with other diseases with formation of syndromes.


Isolated HGF occurs more often in the younger age groups than isolated IGF. HGF has a tendency to occur more frequently as a generalized type in comparison to IGF. The ratio of generalized-to-localized types in HGF and IGF was 15.2:1 and 1.6:1 respectively [2].

Isolated IGF generalized type occurs more often in male patients as was in our case, Localized type occurs in female patients. GF with mental retardation and/or epilepsy with/without hypertrichosis occurs more frequently before 12 years of age and the latter is seen more often in female patients [2].

The syndromes associated with GF include Murray-Puretic Drescher Syndrome (multiple hyaline fibromas); Rutherford's Syndrome (corneal dystrophy); Laband Syndrome (ear, nose, bone and nail defects with hepatosplenomegaly); Jones' Syndrome (progressive deafness); Cross Syndrome (microphthalmia, mental retardation, athetosis and hypopigmentation) [5]; Cornelia de Lange Syndrome (primordial growth deficiency, severe mental retardation, anomalies of the extremities and a characteristic face) [2] and Ramon's Syndrome (association with cherubism). A syndrome associated with hearing deficiencies, hypertelorism and supernumerary teeth has been reported by Wynne and colleagues [3]. Other associations include hypothyroidism, chondrodystrophia and diffuse osteofibromatosis (GF with osteofibrosis) [2].

Enlargement usually begins with the eruption of the permanent dentition but can develop with the eruption of the deciduous dentition; rarely it may present at birth or arise in adulthood [1]. The age at onset is divided into pre-eruptive period (< 6 months), deciduous dentition period (6 months to 6 years), mixed dentition period (6 to 12 years), permanent dentition period before adolescence (12 to 20 years) and permanent dentition period after adolescence (20 years of age or older) [2]. Maximal enlargement occurs either during loss of deciduous teeth or in the early stages of eruption of permanent teeth and progresses rapidly during "active" eruption and decreases with the end of this stage [4].

The hyperplastic tissue is usually of normal pink colour. Enlargement may be generalized or localized to specific areas of the mouth, typically the maxillary tuberosities and the labial gingiva around the lower molars. Severity may vary from mild involvement of one quadrant to severe involvement of all four quadrants.


   Remarks Top


Although this condition has got more surgical (dental) than radiological significance and hence reported on several occasions in dental literature, it is reported here for sake of the radiological appearances which are hardly found in books and journals. Moreover association of GF with supernumerary teeth without hypertrichosis, mental retardation and/or epilepsy as seen in our case could be part of a new syndrome and further observations in this regard is warranted.

 
   References Top

1.Anderson J, Cunliffe W.J. Roberts DF, Close H. Gingival Fibromatosis. Br. Med J. 1969; 3:218-9.  Back to cited text no. 1    
2.Takagi M, Yamamoto H, Mega H, Hsieh KJ, Shioda S, Enomotos S. Heterogeneity in the gingival fibromatoses. Cancer 1991; 68: 2202-2212.  Back to cited text no. 2    
3.Wynne SE, Aldred MJ, Bartold PM. Hereditary gingival fibromatosis with hearing loss and supernumerary teeth - a new syndrome. J Periodontol 1995;66(1):75-9.  Back to cited text no. 3    
4.Fletcher JP. Gingival abnormalities of genetic origin. A preliminary communication with special reference to hereditay generalized gingival fibromatosis. J Dent Res 1966;45:597-612.  Back to cited text no. 4    
5.Ramer M, Marrone J, Stahl B, Burakoff R. Hereditary gingival fibromatosis: identification, treatment, control. J Am Dent Assoc 1996; 127:493-5.  Back to cited text no. 5    

Top
Correspondence Address:
B Pappachan
VSS Medical College Hospital, At /P O Burla, Sambalpur. Orissa
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


Rights and PermissionsRights and Permissions


    Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6]

This article has been cited by
1 Unusual clinical presentation of generalized gingival enlargement - A report of 3 cases
Thada, S.R. and Vineetha, R. and Pai, K.M.
International Journal of Collaborative Research on Internal Medicine and Public Health. 2012; 4(4): 240-245
[Pubmed]
2 Idiopathic gingival fibromatosis: A case report [Idiyopatik gingival fibromatozis: Olgu sunumu]
Bayar, G.R. and Özkan, A. and Şençimen, M. and Altuǧ, H.A. and Gülses, A. and Günhan, O.
Gulhane Medical Journal. 2011; 53(4): 294-296
[Pubmed]
3 Management of idiopathic gingival fibromatosis: Report of a case and literature review
Ramnarayan, B.K. and Sowmya, K. and Rema, J.
Pediatric Dentistry. 2011; 33(5): 431-436
[Pubmed]
4 Histopathological evaluation of five unusual gingival enlargement cases
Akca, A.E., Ortakoǧlu, K., Pikdöken, L., Deveci, S.
Military Medicine. 2005; 170(11): 986-990
[Pubmed]



 

Top
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  


    Case Report
    Discussion
    Remarks
    References
    Article Figures

 Article Access Statistics
    Viewed4984    
    Printed162    
    Emailed6    
    PDF Downloaded0    
    Comments [Add]    
    Cited by others 4    

Recommend this journal