Hereditary Gingival Fibromatosis: a Case Report with Seven-Year Follow-up.

INTRODUCTION: Hereditary gingival fibromatosis (HGF) is a rare disease characterized by gingival enlargement, normal color with benign and firm consistency. This growth may be exacerbated by use of drugs and plaque build-up. The treatment for this clinical condition is surgical excision of the enlarged gingival tissue or the extraction of all teeth. CASE REPORT: A 20-year-old Brazilian female handicapped patient with a chief complaint of exaggerated gingival enlargement who had been prescribed Carbamazepine and Gardenal was referred to our center. According to the clinical presentation and family history, the final diagnosis of gingival enlargement was HGF. Full dental treatment was performed, including basic periodontal treatment, restorations, sealants, and gingivoplasty with internal bevel. Special care was taken to ensure that there was no change in patient's anticonvulsant medication. The patient has been monitored for seven years without signs of recurrence of gingival hyperplasia due to constant professional and home control of plaque.

Introduction

Hereditary gingival fibromatosis (HGF) is a rare disease in both autosomal dominant and recessive types and has a frequency of 1:175,000 (). Clinically, it is characterized by benign gingival enlargement with normal color and firm consistency and non-hemorrhagic symptomatic illness (). Although the gingival enlargement does not directly affect the alveolar bone, the gingival swelling may add to the bacterial plaque accumulation, inducing gingivitis, periodontitis, bone resorption and halitosis (). Histologically, the connective tissue has increased collagen and few fibroblasts and the epithelial tissue presents hyperplasia areas and prominent buds. Despite these characteristics, the histologic features of HGF are not exactly specific, and the definitive diagnosis should be based on family history and clinical findings (). Some of the clinical complications of HGF can be excessive gingival growth, which results in pseudo pocketing and periodontal disease due to poor oral hygiene, diastemas, delayed tooth eruption, and facial disfigurement due to lip protrusion. In addition, in the most severe cases, gingival hyperplasia can result in limited tongue movement, speech impediments and difficulty chewing (-). There is no consensus among authors about the efficacy of HGF treatment () and the recurrence risk is real and needs to be avoided (). Depending on the severity of the of the growth, treatment involves the excision of the enlarged gingival tissues, using conventional surgery, electrosurgery, an apically positioned flap, or lasers through to the extraction of all teeth and reduction of the alveolar bone to prevent recurrence (, ). The success of dental treatment depends on scheduling a return appointment as a preventative for recurring disease (, ).

This paper aims to review the diagnosis, treatment, and follow-up of hereditary gingival fibromatosis by the presentation of a case report characterized by the exacerbated gingival enlargement through the use of anticonvulsant medication, associated with the inflammatory factor, i.e., high levels of plaque. In addition, this case report presents the local treatment plaque control as a differential without any interference with systemic medication.

Case report

A bedridden, 20-year-old female patient, who suffered from microcephaly, epilepsy, and mental retardation, was referred to our center in January 2009 with a chief complaint of ‘pain in the jaws’, according to her mother. In the course of taking medical history she reported another case of gum growth in her brother, who had died. The patient was on carbamazepine 200 mg (3 times a day), phenobarbital 100 mg (1 time a day), cyclobenzaprine hydrochloride 5 mg (1 time a day), ranitidine 50 mg (1 time a day), iron (40 drops a day), calcium (10 ml 1 time a day) and minerals (10 ml 1 time day). At that time, she had scored 50 points on the Karnofsky Performance Status Scale (), ate only a soft diet and weighed 14 kilograms. It is important to note that the patient had grand mal seizures about eight to ten times a day and sometimes there was need for hospitalization due to the severity of her seizures. At the time she went to our service, the seizures were in the initial control phase, since the doctor was adjusting the medication dose. After several alterations of dosage, the seizures stabilized with appropriate and regular use of anticonvulsant medication.

The intraoral examination revealed severe gingival hyperplasia associated with a high plaque index (3.16, according to IHOS index) - gingival calculus, false periodontal pockets, three decayed teeth and four teeth with active white spots (Figures 1, 2 and 3). Dental treatment was performed under general anesthesia because there were several dental procedures to be carried out and the patient’s general health status was classified as ASA II.

Figure 1

Intraoral view of the maxillary right side, showing HGF before surgical dental treatment under general anesthesia.

Figure 2

Intraoral view of the maxillary left side, showing HGF before surgical dental treatment under general anesthesia.

Figure 3

A front view of mandibular incisors before surgical dental treatment under general anesthesia.

We performed the basic periodontal treatment and composite resin restorations, sealing the active white spots using modified, atraumatic, restorative techniques, and we surgically removed the third molars. Regarding the gum, specifically, after the marking the gingival pockets, we removed the excessive tissue by means of internal bevel gingivectomy on some sections of buccal and palatal-lingual surfaces. The incisions in the internal bezel were designed to preserve a proper amount of gum tissue after surgery as well as to allow the passive replacement of surgical flap, thus achieving healing all at once. We opted for this technique based on the patient’s special care needs and the large amount of tissue to be excised in one session.

The patient is still undergoing follow-up every three months in our center, seven years after the surgical procedures. Currently, her health status is under control, including her oral health, without any signs of gingival hyperplasia, false periodontal pockets, or caries (Figures 4, 5, 6, 7 and 8). Informed consent has been obtained from the patient’s mother.

Figure 4

A: Immediately after surgery. B: Fifteen days after surgery.

Figure 5

One-year follow-up.

Figure 6

An intraoral view of the maxillary right side seven years after dental treatment under general anesthesia.

Figure 7

An intraoral view of the maxillary left side seven years after dental treatment under general anesthesia.

Figure 8

The patient undergoing monitoring without evidence of caries or periodontal disease.

Discussion

Hereditary gingival fibromatosis is a rare condition of gingival tissues that can be associated with several other clinical manifestations and feature some syndromes (, ). This growth is slow and progressive and may be exacerbated by the use of drugs. It can occur in isolation or may be associated with syndromes, such as Zimmermann-Laband, Rutherford, and Ramon syndromes (, ). It is important to note that oral health of the patient, including the gums and teeth, was recovered without interfering with her medication, as the doctor took a long time to choose the drug and its dose to control convulsive seizures. For this reason, our team evaluated the three main causes of gingival hyperplasia, which were genetics, the use of anticonvulsant medication and the presence of plaque, i.e., the inflammatory factor. It is noteworthy that in this case, there was no intervention done concerning the genetic and medical factors. On the other hand, the control of the inflammatory factor, through periodic visits to the dentist and the family’s participation at home, has been responsible for the absence of the recurrence of gingival hyperplasia to date. This fact leads us to rule out the hypothesis of medical gingival hyperplasia. In this context, this situation underscores the importance of plaque control to prevent new and recurrent diseases, especially manifestations of rare and severe pathologies, such as HGF, in which the recurrence rate after surgery is relatively high (). Therefore, despite the fact that HGF is not caused by plaque increase; it can be exacerbated by it ().

It is worth noting that due to the patient's neurological impairment and disabled protective reflexes, such as coughing, the diet recommended by her medical team had a “doughy consistency”. In addition, her oral health care had been relatively neglected in the context of general health framework that she presented. Such a situation is in accordance with Escribano-Hernández et al (), who alleged that mentally disabled patients often have their oral health care neglected. They have further stated that the rate of dental caries and periodontal disease is substantially increased in this population because of a pureed, high carbohydrate diet and physiological limitations that prevent adequate teeth cleaning due to impaired muscle function.

Actually, the use of dental general anesthesia has been indicated for handicapped patients that do not have good comprehension as well as children infants and pre-school children who do not have good behavior and patients with excessive fear associated with several dental needs (, ). However, it is important to highlight that dental general anesthesia had good results only when associated with a postoperative prevention program (). We endorse these results and believe that clinical success over seven years of follow-up has been achieved due to the correct diagnosis, precise treatment and family agreement with the professional team’s treatment proposal.

Conclusion

HFG is a rare disease that must be treated very carefully, especially in systemically compromised patients such as mentally disabled patients. This case report has relevance since the correct diagnosis may lead to proper treatment. In this case, it was of fundamental importance not to change the anticonvulsant medication. Associated with this, the differential was exactly the control of inflammatory factors during the postoperative period. Thus, it is extremely important that these patients are regularly seen by the dentist and their plaque index is kept under control because the disease can recur.