Gingival enlargement among the paediatric population: an overview and case report

Presentations of gingival enlargement in children

Children's gingivae typically appear reddish in colour with rounded and rolled gingival margins (Figure 1).¹ In a healthy state, the gingival margin is several millimetres coronal to the cemento-enamel junction. The gingival sulcus may be 0.5–3 mm deep on a fully erupted tooth.² Contact points between primary teeth are not as tight as those between the permanent dentition, which can allow for an increased area of potential bacterial growth.¹ Diseases and disorders affecting the gingivae can be divided into those that are related to local inflammatory factors and those that are related to systemic factors (Table 1).

Chronic inflammatory gingival conditions

Plaque-induced gingivitis

Gingivitis is a plaque-induced gingival inflammation that is reversible following improvements in oral hygiene and removal of plaque.⁴ Its clinical presentation in children is frequently gingival colour change and swelling. Figure 2 is an example of significant gingival hyperplasia secondary to poor oral hygiene. Gingivitis among children is common, with a number of modifying factors, including difficulty negotiating around the erupting dentition, developing manual dexterity, lacking parental guidance and puberty. In addition to this, for some children, the presence of orthodontic appliances can become a plaque retentive factor and promote plaque-induced gingivitis.¹

Fibrous epuli

Fibrous epuli are the most common benign soft tissue tumour seen in the oral cavity. They are, however, more frequently seen in adults. Fibrous epuli present as a firm, pink mass emerging from the interdental papilla, most typically in the anterior gingival region. They occur as a result of a chronic trauma resulting in the formation of granulation tissue and a subsequent submucosal fibrous mass.⁵

Pyogenic granuloma

Pyogenic granulomas present as a mass growing from beneath the gingival margin and are often ulcerated. They are highly vascular, compressible and have the potential to bleed readily. Oral pyogenic granuloma can occur in persons of all age groups. However, they are more frequently reported in pubertal females due to an association with elevated levels of oestrogen and progesterone.⁷

Traumatic lesions

Traumatic lesions can arise following chemical, thermal, electrical or physical injuries. Chronic irritation caused by fixed orthodontic treatment and removable appliances can result in gingival hyperplasia or an acute inflammatory reaction on the gingivae.^1,7

Systemic factors

Viral infection

A number of viral infections can cause oral lesions (Table 2). Primary herpetic gingivostomatitis (PHGS) is commonly observed among children and is the most common clinical manifestation of primary herpes simplex virus (HSV) infection. The first peak of PHGS onset is between the ages of 6 months and 5 years. Signs and symptoms include fever, chills, irritability, malaise and headache. Pain in the mouth, refusal to drink and submandibular lymphadenitis is also seen. The gingivae have an erythematous appearance with oedema and gingival bleeding. Ulcerated lesions are also seen, which usually heal within 1–3 weeks.^1,8

Table 2. Summary of common viral diseases seen in the oral cavity of children.9,10,11

Virus	Clinical manifestations
Herpes simplex virus	Herpetic gingivostomatitis
	Herpes labialis
Coxsackie virus	Herpangina
	Hand, foot and mouth disease
Varicella-zoster virus	Chicken pox
Paramyxovirus	Measles
Epstein–Barr virus	Oral hairy leukoplakia
Rubella virus	German measles

Nutritional deficiency

Scorbutic gingivitis (scurvy) is a result of vitamin C deficiency, which causes haemorrhage, collagen degeneration and oedema of the gingival connective tissues.¹ Scurvy, however, is now generally rare and more common among populations in the developing world. The clinical presentation of these cases included erythematous, haemorrhagic and oedematous gingivae.¹²

Granulomatous inflammation

Orofacial granulomatosis (OFG) is a chronic granulomatous condition that presents with recurrent lip swelling and intra-oral involvement that may include diffuse, erythematous enlargement of the gingivae and cobblestone mucosa.^7,12 This condition can occur independently, but can also be indicative of systemic diseases including tuberculosis, sarcoidosis and Crohn's disease. Patients who present with OFG should be referred for blood screening so that any co-ocurring systemic disease can be identified.^3,12

Haematological conditions

Cyclic neutropenia is a rare haematological disorder characterized by cyclic depressions in neutrophil numbers with onset in early childhood. The neutropenic phase is associated with clinical symptoms, such as recurrent fever, malaise, headaches, pharyngitis, ulcers of the oral mucous membrane and gingival inflammation (Figure 3).¹³

Another rare haematological disorder, familial benign neutropenia can also present with intra-oral signs and symptoms including ulcerative gingivitis and gingival oedema.⁷

Gingival enlargement and/or spontaneous or excessive bleeding has been reported as the initial clinical presentation of leukaemia. The bleeding is thought to be due to thrombocytopenia associated with acute and chronic forms of leukaemia.^1,14

Drug-induced gingival lesions

Gingival enlargement is a known unwanted side-effect of some drugs, most commonly calcium channel blockers, anti-convulsant medications and cyclosporin. Children are less commonly prescribed these medications, hence there is a lower incidence of drug-induced gingival enlargement in this group. However, evidence shows that children who do take these medications are more likely have this adverse reaction.^1,3,14

Genetic conditions

Hereditary gingival fibromatosis is a rare inherited condition which manifests itself clinically as enlarged gingival tissue that can partially cover teeth. It usually affects the maxillary tuberosities and mandibular retromolar pads; however, labial gingivae can also be involved. In severe cases eruption may be affected.^3,7,14

Papillon–Lefèvre syndrome (PLS) is an autosomal recessive disorder that manifests orally as severe gingivostomatitis and periodontitis (Figure 4). Eruption of the dentition occurs at the expected time and usually with normal structure and form. However, due to the onset of aggressive periodontitis and subsequent destruction of periodontium and resorption of bone, patients experience premature loss of primary and permanent dentition at a very young age. The incidence of the syndrome is rare, with the syndrome affecting one to four per million with no sex and racial predominance.¹⁵

Chédiak–Higashi syndrome (CHS) is another rare autosomal recessive disorder that can present orally as unexplained gingivitis and periodontitis among young children. This condition is otherwise characterized clinically by oculocutaneous albinism, recurrent bacterial infections, and giant granules in peripheral blood leukocytes or bone marrow cells.¹⁶

Case report

An 18-month-old boy attended Birmingham Children's Hospital (BCH) emergency department with a 4-day history of unexplained bleeding from the gingivae. The patient's mother had first noticed a patch of blood in the child's cot, and bleeding was visible from the child's gums.

The patient's mother had previously seen a local general dental practitioner (GDP) who diagnosed dental infection or pain related to teething. The child was prescribed broad spectrum oral antibiotics and discharged. In the following days, the bleeding from the gingivae continued to increase with the antibiotics having no effect. Following advice from a second GDP accessed via an emergency medical helpline, the patient was brought to the emergency department.

The patient was medically fit and well, taking no regular medications. There was no previous history of prolonged bleeding or easy bruising. The child, although visibly upset and tired, displayed no systemic symptoms of being unwell, and all vital observations were within normal ranges.

Intra-oral examination revealed a large blood clot present overlying the upper primary incisors. On removal of the clot, there was generalized enlargement of the maxillary gingivae covering over two-thirds of the crown of the upper dentition. The gingivae were pink with loss of stippling and a spongy appearance (Figure 5). There was no evidence of a traumatic injury or laceration to the gingivae. This seemed to be predominantly affecting the upper dentition with no bleeding or enlargement seen from the lower arch.

Discussion

Leukaemia is the most commonly diagnosed childhood cancer. Leukaemia is a group of haematological disorders resulting from the uncontrolled proliferation and abnormal development of neoplastic cells. Normal haematopoiesis is supressed, leading to a deficiency of mature leukocytes, erythrocytes and platelets, and hence patients may present with infections, anaemia or bleeding.¹⁷ Leukaemia can be classified into acute or chronic, and lymphoblastic or myeloid.¹⁸

Acute lymphoblastic leukaemia (ALL) is the most common form of leukaemia, with approximately 78% of all leukaemia diagnosed according to UK charity organization, Children with Cancer.¹⁹ ALL is caused by an overproduction of immature lymphoid cells, called lymphoblasts. The peak incidence of ALL is among children aged 2–3 years with boys at greater risk of developing ALL with a ratio of 4:3.

Acute myeloid leukaemia (AML) is an overproduction of immature myeloid cells, called myeloblasts. Children with Cancer, reports that approximately 70 cases of AML are diagnosed every year in the UK, which accounts for 15% of UK childhood leukaemia cases.¹⁹ The highest incidence of the disease is in children aged under 2 years and, similarly to ALL, boys are a higher risk of developing AML at a ratio of 5:4.¹⁹

Treatment of AML involves multidrug chemotherapy and, in many patients, bone marrow transplantation. The mortality rate of the disease is high, hence the need for early and accurate diagnosis. AML generally presents in children as fatigue, pallor, abnormal bleeding and lymphadenopathy. However, oral lesions can present as the initial clinical feature of acute leukaemia and, therefore, can be an important diagnostic indicator.^17,20

The most common oral manifestations seen in patients diagnosed with leukaemia include petechiae or spontaneous bleeding (56% of patients), mucosal ulceration (53%) and gingival enlargement (36%).)^17,18

In 5% of AML cases, enlarged gingivae of leukemic cells presents as the initial manifestation of the disease. Gingival enlargement and ulcerations may be due to direct infiltration of leukemic cells or neutropenia. This infiltration results in an increased gingival thickness, formation of gingival pocketing and secondary inflammatory infiltration. Poor oral hygiene increases the risk of infection of the gingival lesions.¹⁷ Gingival enlargement has been shown to regress within 3–4 weeks of commencement of chemotherapy regimens.¹⁸

Patients who report to dental practitioners with gingival enlargement as the primary presentation of AML have been reported previously and included in a number of reviews and overviews of gingival enlargement and gingival disease.^{1,3,7,14,17,18,20}

The importance of asking about general symptoms is highlighted on review of previous case reports. Fatigue is a common symptom. Fever and malaise could also be indicative of viral infection and all differential diagnoses should be considered.

In this case, the first dentist who reviewed this patient had considered teething or dental infection as potential differential diagnoses and prescribed antibiotics. Teething can be painful for young children with symptoms of teething, including drooling and soreness of the gingivae, associated with irritability and sleep disturbance. However, the significant gingival enlargement and bleeding seen in this case made teething a highly unlikely cause. Similarly, although dental infection or abscess can cause systemic symptoms, localized swelling is more common.

Antibiotic prescription resulted in no resolution of the symptoms experienced in this case. Antibiotics could be justified because the dentist had considered dental infection as the potential diagnosis. However, the patient was discharged and no review made. FGDP guidelines recommend a review for patients prescribed antibiotics for dento-alveolar infections in 2–3 days, with onward referral recommended for those with no resolution of symptoms and if unable to establish drainage.²¹ Given the unusual presentation and age of this patient, a consideration of these guidelines would have been pertinent in this case.

In this case, it is important to consider whether the ongoing COVID-19 pandemic may have played a part in the reluctance for this child to be seen for a second face-to-face review. Similarly, the disruption in regular check-ups and recalls for patients in primary care could have prevented this from potentially being noticed at a routine examination.

This case also highlights that telephone review can act as a useful screening tool, with the second dentist correctly referring to the emergency department for urgent review and investigations. Early diagnosis and treatment of leukaemia can increase the patient's chances for remission, therefore maintaining a high degree of suspicion for unusual oral presentations can allow for prompt referral and treatment of these patients.

Conclusion

There is a wide range of gingival conditions that present among children that can be associated with local and systemic factors and, in some cases, be indicative of malignancy. Gingival overgrowth and bleeding may be the first clinical presentation of blood dyscrasias. Patients may initially present to dentists before seeing any other healthcare professional. Early intervention and treatment can be critical in such cases, hence the importance for dental professionals to be aware of and to consider this potential diagnosis. In cases of gingival bleeding or enlargement, where other differential diagnoses have been ruled out, a referral to secondary care should be made where necessary.