Idiopathic gingival fibromatosis.

Idiopathic gingival fibromatosis (IGF) is a rare hereditary condition characterized by slowly progressive, nonhemorrhagic, fibrous enlargement of maxillary and mandibular keratinized gingiva caused by increase in submucosal connective tissue elements, mostly associated with some syndrome. This case report describes a case of nonsyndromic generalized IGF in an 18-year-old male patient who presented with generalized gingival enlargement. The enlarged tissue was surgically removed by internal bevel gingivectomy and ledge and wedge procedure. The patient was regularly monitored clinically for improvement in his periodontal condition as well as for any recurrence of gingival overgrowth.

INTRODUCTION

Idiopathic gingival fibromatosis (IGF) is an uncommon, benign, hereditary, slowly progressive, nonhemorrhagic fibrous enlargement of keratinized gingiva. It usually begins at the time of eruption of permanent teeth, but can develop with the eruption of deciduous dentition and rarely present at birth.[1] The hyperplastic gingival tissue is pale-pink, firm; has leathery consistency and presents a characteristic pebbled surface. The enlarged tissues may partially or totally cover the dental crowns, can cause diastemas, delay or impede tooth eruption and periodontitis. In severe cases, it may lead to mastication and speech impediments or lip closure difficulties.[2]

CASE REPORT

An 18-year-old male, accompanied by his father reported to the department with the complaint of enlarged gums in upper and lower arches, which caused difficulties in speech, mastication and complete closure of lips, thereby leading to esthetic impairment. The patient presented with gradual and progressive enlargement of both upper and lower gingival tissues from the age of 6 years. The family and postnatal histories were noncontributory. The patient exhibited no signs of hypertrichosis, mental retardation, epilepsy or intake of medication known to cause gingival overgrowth.

On examination, patient had bilaterally symmetrical face with incompetent lips with the bimaxillary protrusion. An intraoral examination revealed generalized, enlargement of the gingiva. The gingiva was pink in color with melanin pigmentation, and consistency was firm and fibrous. Stippling was present. The enlarged gingiva covered the crowns of all the teeth till the incisal or occlusal third region [Figure 1]. Generalized pseudo-pockets were observed with no bleeding on probing. Dentition revealed all permanent teeth except mandibular premolar (35) and an over retained deciduous molar (75). Malpositioning of the teeth was noted.

Figure 1

Preoperative photographs showing generalized gingival enlargement

Investigations included orthopantomograph, in which no significant alveolar bone loss was observed [Figure 2]. Laboratory investigations which included complete hemogram, thyroid tests (T3, T4, and thyroid stimulating hormone), parathyroid test, calcium and alkaline phosphatase levels were made. All the reports except the levels of alkaline phosphatase (which was slightly increased) were within the physiological limits. Based on history and clinical features, a provisional diagnosis of IGF was made.

Figure 2

Panoramic radiograph showing presence of retained deciduous molar in mandibular left region

The treatment plan consisted of sextant wise surgical excision of the enlarged gingiva under local anesthesia. The treatment procedure was explained to the patient and parent, and written consent was obtained. Internal bevel gingivectomy was performed on the buccal/labial aspects of both arches. Ledge and wedge procedure was done to remove gingival overgrowth over the palatal aspect of the maxillary posterior region and mandibular posterior region [Figure 3]. After the surgery, the site was covered with periodontal dressing for 7 days. The patient was prescribed a 0.2% chlorhexidine mouth rinse for 1-week. The excised tissue was sent for histopathological examination.

Figure 3

Intrasurgical photographs

Histopathology showed parakeratinized stratified epithelium, which was acanthotic at places and elongated rete pegs. The bulk of connective tissue was composed of dense fibrous connective tissue and numerous fibroblasts. All these features are consistent with IGF [Figure 4].

Figure 4

Histologic section shows hyperparakeratinized hyperplastic squamous epithelium with dense fibrocollagenous tissue

The patient was recalled every week for 6 weeks when the periodontal pack was removed, and the next sextant was operated upon. The patient was then placed on a schedule of periodic recall visits for maintenance care. The oral hygiene maintenance was reinforced at every recall. No recurrence of gingival enlargement was observed 6 months after the surgery [Figure 5].

Figure 5

Postoperative photographs after 6 months of follow-up showing no recurrence of gingival enlargement

DISCUSSION

Gingival fibromatosis may occur as an inherited condition known as hereditary gingival fibromatosis, or it may be associated with inflammation, leukemic infiltration, and medications.

According to Gorlin et al. IGF is most commonly associated with hypertrichosis, also occasionally associated with mental retardation and epilepsy. It occurs either as an isolated disease or combined with some rare syndromes like Zimmerman-Laband syndrome (defects of bone, nail, ear, nose and splenomegaly), Murray-Puretic-Drescher syndrome (multiple dental hyaline tumors), Rutherford syndrome (corneal dystrophy), Cowden syndrome (multiple hamartomas), and Cross syndrome (hypopigmentation with athetosis).[3]

In the present case, family history was negative. The patient exhibited no signs of hypertrichosis, mental retardation and epilepsy or intake of medication known to cause enlargement. Hence, the case was diagnosed as a nonsyndromic case of IGF.

Idiopathic gingival fibromatosis affects the attached gingiva as well as gingival margin and interdental papillae. The cause is unknown, and thus the condition is designated as “idiopathic”. Some cases have a hereditary basis, but the genetic mechanisms involved are not well understood. A study of several families found the mode of inheritance to be autosomal recessive in some cases and autosomal dominant in others.[4]

Histologically, the gingival hyperplasia is mainly due to an increase and thickening of collagen bundles in connective tissue stroma. The nodular appearance can be attributed to the thickened hyperparakeratinized epithelium. The cellular and molecular mechanisms that lead to this condition are not well understood. IGF keratinocytes seem to have an important role in pathogenesis by inducing extracellular matrix accumulation by fibroblasts.[5] Furthermore, it has been reported that increased proliferation and elevated production of extracellular matrix molecules, fibronectin and type I collagen could lead to an increased bulk of gingiva.[6]

In the present case, the history revealed that the enlargement started in mixed dentition period. Emerson recommended that the best time for the excision of gingival enlargement is when all the permanent teeth have erupted.[7]

Among the suggested treatment protocols, ledge and wedge technique along with internal bevel gingivectomy was selected as it helped in the placement of primary incisions as opposed to the conventional external bevel gingivectomy procedure. Moreover, this procedure does not leave a large external bevel and, therefore, result in less postoperative pain and bleeding.[8]

After surgery, recurrence is expected within a few months after surgery and is most commonly seen in children and teenagers rather than adults.[9] Studies have demonstrated that recurrence is faster in areas with plaque accumulation.[10] Maintenance of good oral hygiene with professional cleaning and home care maintenance is necessary to prevent the recurrence.

CONCLUSION

The present case report highlights a nonsyndromic IGF and its management. Surgery significantly improved the patient's esthetics and masticatory functions. Maintenance of oral hygiene is important to prevent recurrence.

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Conflicts of interest

There are no conflicts of interest.