Idiopathic gingival fibromatosis with asymmetrical presentation and electrosurgical management

Abstract

Idiopathic gingival fibromatosis is a rare genetically heterogeneous condition characterized by recurrent gingival enlargement without any identifiable cause. We report a case of 14-year-old female patient affected with sporadic, nonsyndromic, progressive gingival enlargement. It manifested more severely on the right side of the mouth with history of recurrence after prior conventional surgical excision. Electrosurgical resection of the enlargement was done, and overall patient management had a successful outcome along with achieving preservation of teeth with poor prognosis in the 2 years follow-up.

INTRODUCTION

Gingival fibromatosis (GF) is a slowly progressive, benign, nonbleeding, painless, localized or generalized overgrowth of the maxillary and mandibular keratinized gingiva.[1] It can lead to diastema, malocclusion, delayed eruption of permanent dentition or prolonged retention of primary dentition, causing aesthetic and functional problems. Hereditary GF (HGF) is a rare disorder; about one in 1,75,000 individuals transmitted either as an autosomal dominant or rarely, an autosomal recessive trait.[2] The onset usually coincides with the eruption of the permanent dentition though some cases have even been reported in the deciduous dentition. Different clinical variations are seen depending on the genetic heterogeneity.[3] The gingival tissues usually are pink, nonhemorrhagic with a firm, and fibrotic consistency.

CASE REPORT

A 14-year-old female patient reported to our department with complaint of swelling in the gums of the upper and lower right hand side quadrants of the mouth since a year. The patient first noticed the swelling 4 years before in the upper right side of the mouth - gradually and slowly increasing swelling.

The patient had delayed tooth eruption of the upper and lower right premolar teeth associated with gingival swelling. She underwent excisional surgery for the same 2 years before at a private dental clinic. Postsurgery within 6 months, the patient again noticed the swelling which gradually increased in size. The patient gave history of rapid increase in size since the preceding 6 months and spread toward the teeth of the left hand side of the mouth. Swelling was painless, but the patient complained that it interfered with chewing.

There was no history of epilepsy or major illness. She was undergoing treatment for anemia with iron supplement. Developmental milestones and other systems of the child were normal. Family and menstrual history was noncontributary. The patient has unilateral mastication habit with left side since childhood. The right hand used for brushing.

Extraoral examination

General physical evaluation was done. The patient had normal physical appearance and psychomotor skills. Normal bone development seen on the chest and extremity radiographs. The patient showed slight facial asymmetry with fullness of the right upper lip. The lips were competent [Figure 1].

Figure 1.

Extraoral photograph

Intraoral findings

Gingival enlargement was more predominant on the right hand side of the mouth, but mild involvement was present extending to the incisors as well as the lingual aspect of the left mandibular molar region [Figures 2 and 3]. Only the maxillary left posterior teeth were completely uninvolved. Both facial and palatal/lingual aspects were involved in the maxillary and mandibular right hand side quadrants covering almost the entire clinical crown. There was diffuse involvement of marginal, papillary, and attached gingiva. The swelling was irregular, largely pale pink, and firm devoid of stippling along with softened reddish pink areas toward the occlusal surface associated with calculus deposits.

Figure 2.

Preoperative right and left lateral intraoral photographs showing major right side involvement

Figure 3.

Preoperative intraoral maxillary and mandibular occlusal view

Bleeding on probing was present with 14–16 regions. Several teeth were clinically submerged including the maxillary canines (13, 23) and mandibular left canine (33) and both the mandibular second premolars (35, 45). Even, the maxillary and mandibular second molar of the right hand side (17, 47) were clinically submerged. Deep pseudopockets along with attachment loss of up to 13–15 mm were present with 14, 15, 16, and 46 which showed Grade II mobility. Grade I mobility was present with 11, 12, 21, 22, 31, 32, 41–44.

Radiographic finding

Orthopantomogram (OPG) and three dimensional cone beam computed tomography evaluation was done. The radiographs revealed that the submerged teeth had bony impaction except for the maxillary and mandibular right second molars which appeared to be still erupting. Severe bone loss was present with 14, 15, and 16 teeth [Figure 4]. In this region, there was evidence of increased spacing and further bone loss compared to an OPG taken 3 years prior and the current radiograph. In addition, generalized crestal bone loss was present except with the left maxillary posterior teeth.

Figure 4.

Radiograph (orthopantomogram) - showing severe bone loss with 14, 15, 16 and impacted teeth

Blood investigations revealed the normal blood profile and hormonal levels. Hemoglobin level was low 10.3 mg%.

Treatment

After obtaining written consent, thorough scaling, root planing, and curettage were done and anti-microbial rinse chlorhexidine gluconate 0.2% prescribed and in addition used for irrigation of the pockets. This regime was repeated weekly for 3 weeks as it was difficult for the patient to maintain adequate plaque control. An incisional biopsy was done and 8 mm × 10 mm tissue specimen was sent for histopathological evaluation.

Postphase I appearance of the tissues showed overall superficial reduction in inflammation. Following this phase, electrosurgical gingivectomy was performed. First, the maxillary surgery was performed. After administering nerve block anesthesia to the patient, the area of tissue to be excised was demarcated with a probe. The electrosurgical unit (megasurg - high frequency radiosurgery unit) was set up and the passive electrode – the patient plate tied to the patient's calf. The needle electrode (active electrode) at 8 MHz was used to outline the external bevel incision. The tissues were further undermined with a bar electrode and removed in bulk. The bar electrode was then used to plane the tissues and achieve a favorable tissue contour. Following excision, thorough irrigation of the site was done. In some sites, additional curettage was done along and closure of the area with sutures. The operative procedures for Maxilla are shown in Figure 5. Periodontal dressing was placed, and the patient was prescribed anti-biotic and analgesic medication.

Figure 5.

Intraoperative procedures for maxillary arch - incision, electrosurgical ablation, and suturing

The mandibular surgery was performed 2 weeks later similarly [Figure 6]. Postoperative healing a week later was satisfactory. The case was followed-up for 8 weeks postoperatively, and then every 3 months for 2 years. Favorable healing of the sites were seen postoperatively at 1 week, 1 month, and 3 months intervals. The mobility of teeth had significantly decreased from Grade II mobility to physiologic by 3 months postoperatively. The patient was able to maintain good oral hygiene. The patient has visited for recall follow-up since the past 2 years with minimal evidence of recurrence [Figure 7].

Figure 6.

Immediate postoperative photograph following electrosurgical excision of enlargement in the mandibular arch

Figure 7.

Intraoral photographs at the 2 years postoperative recall

Histopathologic report

Evaluation was performed using routine H and E staining as well as polarized microscopy with specific connective tissue staining to verify the nature of the fibrosis. Abundant collagen with hyalinization of the collagen fibers was seen. Congested blood vessels with mixed inflammatory infiltrate focally transgressing the overlying epithelium were observed. There was no evidence of dysplasia or malignancy [Figure 8].

Figure 8.

Microscopic picture showing epithelial and connective tissue hyperplasia with dense fibrosis and areas of hyalinization and inflammation

DISCUSSION

Massive GF causes difficulty in function and unaesthetic appearance. In early childhood, it may also result in an abnormal swallowing pattern. It is painless except if it gets traumatized during mastication. HGF is also known as congenital hypertrophy of the gums, gingival elephantiasis, gingival gigantism, symmetrical fibroma of the palate, gingival hyperplasia, gingival overgrowth (GO), and congenital macrogengiva.[4]

HGF and sporadic, idiopathic cases cannot be completely eliminated because of the inherent tendency to recur postsurgery as long as the teeth are present. It is important to rule out other conditions manifesting with gingival enlargement such as drug induced gingival enlargements, endocrine disorders, leukemia, Vitamin C deficiency, allergic, familial or syndromic gingival enlargement before terming the condition as idiopathic. It is also very essential to rule out dysplastic and neoplastic gingival growths.

Idiopathic GF may result from a variety of genetic mutations, and therefore the clinical presentation of the condition differs. It can occur by either autosomal dominant or autosomal recessive inheritance or a new type of genetic mutations. To date, four loci for isolated HGF have been identified on chromosome 2 that is GINGF on 2p21-22 and GINGF3 on 2p22.3-p23.3; one on chromosome 5 that is GINGF2 on 5q13-5q22; and on chromosome 11, 11p15 specifically that is GINGF4.[5,6] Of these, only one gene mutation has been identified in the son-of-sevenless-1 (SOS-1) gene while sequencing of the 16 known genes, which mapped chromosome 2p21-22 GINGF. This heterozygous mutation in SOS-1 gene was co-segregated with the disease in a Brazilian family.[7] In our case, the genetic analysis was carried out testing chromosome 2 for SOS gene mutation, and it was found to be normal so signifies unknown genetic mutation.

In referral to the period of onset, the overgrowth can be classified as preeruptive (<6 months of age), during the deciduous dentition (from 6 months to 6 years), during the mixed dentition period (6–12 years), and during the permanent dentition periods before (12–20 years) and after adolescence (age 20 or later).[8] Our patient reported onset coinciding with permanent tooth eruption; very rarely, it is associated with deciduous tooth eruption.

The gingival enlargement covered exceeded the occlusal level of the teeth and could be classified as Grade III enlargement.[9] Syndromic GF has been associated with features such as hypertrichosis, mental retardation, epilepsy, progressive sensorineural hearing loss, and abnormalities of the extremities, particularly of the fingers and toes. It has shown association with syndromes such as Cross syndrome, Rutherford syndrome, Ramen syndrome, Zimmermann Laband syndrome, Murray–Puretic–Drescher syndrome, Jones syndrome, and Prune Belly syndrome. Some cases are also associated with aggressive perioodntitis.[6]

There is an excess of collagen fibers of the connective tissue thought to be due to excess fibroblast proliferation or disturbed collagen turnover. Altered levels of matrix metalloproteinase 1 (MMP-1), MMP-2, transforming growth factor beta 1 (TGF-beta-1), interleukin-6 (IL-6), connective tissue growth factor (CTGF), E-cadherin, fibroblast-specific protein-1, and connective tissue stain-Vimentin are some of the histochemical markers for GF. It has been reported that MMP-1 and MMP-2 expression and production are lower in fibroblasts from HGF patients. These enzymes are required for degrading collagen so their deficiency leads to collagen excess. Also increased cross-linking may be responsible. It has been postulated that a higher expression of prolyl 4-hydroxylase, a rate-limiting enzyme in the synthesis of collagens, and a higher exogenous or autocrine production of TGF-beta-1 and IL-6 production increases the synthesis and reduces the proteolytic activities of fibroblasts from patients with HGF, which may favor observed accumulation of the extracellular matrix.[6] The Ras-MAPK pathway may be affected in those individuals with SOS-1 gene mutation. The activation of this pathway increases the expression of Type IV collagen, the CTGF, and growth factor TGF-beta of the collagenase inhibitors of metalloproteinases and decreases the expression of metalloprotease MMP that degrades the extracellular matrix components.[10] There is also increased epithelial proliferation. Some newer research is also exploring the possible role of keratinocyes and myofibrobalst cells in GF.[11] In our case, histopathologic evaluation revealed hyalinization and collagen excess which was further confirmed by polarized microscopy and connective tissue staining.

It mainly involves the attached gingiva and is associated with the presence of teeth. It completely disappears postextraction. Second, attachment loss and bone loss occur around the teeth due to the inability to maintain hygiene and secondary inflammation. There was an aggravation of the swelling in areas with deposits, and the patient also gave history of unilateral mastication.

Treatment depends on the severity of the enlargement. Minimal enlargement is treated conservatively with routine prophylaxis, irrigation, and mouthwashes. When there is an interference with chewing or unsatisfactory appearance, treatment includes gingivectomy with internal or external bevel incision was done conventionally or by using electrosurgery or lasers.[12] Electrosurgery was preferred as the dense fibrous consistency of the tissue makes it difficult to cut with a blade, and lasers have more superficial effect. Furthermore, a bloodless field and quick ablation of the tissues are beneficial. Some authors recommend surgical excision only after complete eruption of the permanent teeth.[4]

These cases usually show a high rate of recurrence. It is mostly seen in children and teenagers. Genetic and psychological counseling of the patient and family members and stringent maintenance of oral hygiene should be followed. It is also important for multidisciplinary collaboration to rehabilitate the patient as these patients have multiple un-erupted or malpositioned teeth and early loss of teeth associated with the enlargement. In our case, it was decided to follow-up the patient for additional time before undertaking orthodontic or prosthetic treatment.

CONCLUSION

Early diagnosis, emphasis on conservative management, and a multi-disciplinary treatment protocol are important factors in the management of patients with idiopathic GF. More genetic research is needed to identify their molecular basis for a more definitive line of treatment.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.