Chapter 378. Oral Pathology

While dental caries and its consequences represent the most common oral pathology encountered in children, both localized and systemic conditions of noncariogenic origin may manifest themselves in the mouth and surrounding tissues. This chapter will review some of the common and uncommon pathologies observed in or around the oral cavity of children.

Angioneurotic Edema

Angioneurotic edema is an allergic reaction often involving the oral soft tissues (see Chapter 189). Antigens precipitating this reaction include food and latex allergies, vaccines, insect bites, and medications. Typically, facial edema follows exposure to the antigen and may include swelling of intraoral soft tissues, potentially resulting in airway embarrassment. Initial management is provided with antihistamines. More aggressive therapy may require the administration of corticosteroids.1–10

Ankyloglossia

Ankyloglossia (tongue-tie; Fig. 378-1) is a congenital anomaly of the tongue characterized by a short and sometimes anteriorly inserted frenulum. Ankyloglossia reportedly occurs in approximately 2% to 5% of newborn infants with a male-to-female ratio of 2.6:1. Most do not require treatment. The clinical significance of ankyloglossia is a matter of controversy, particularly as it relates to breast-feeding difficulties such as sore nipples, poor infant weight gain, neonatal dehydration, and shortened breast-feeding duration. Infants with ankyloglossia and associated problems with latching may benefit from frenectomy. Rarely in the older child will it interfere with speech or contribute periodontal problems. Recent studies suggest some advantage of laser frenectomy over traditional surgical frenectomy relative to pain and recovery times.11–13

Bacterial Infections

Dental caries is the most common bacterial infection in the mouth that may progress to abscess and cellulitis if left untreated. (The caries process is described in Chapter 374). Nontooth-related intraoral bacterial infections are relatively uncommon. Extraoral bacterial infections tend to be far more common (see Chapter 367). Several other systemic conditions that have bacterial etiologies can present with oral lesions. These include streptococcal infections (see Chapter 285), diphtheria, tuberculosis, cat-scratch disease, actinomycosis, gonorrhea, and syphilis. Impetigo (see Chapter 367) is typically a superficial infection of the perioral tissues caused by Streptococcus pyogenes and Staphylococcus aureus. The classic clinical presentation is multiple delicate vesicles that spread and rupture to produce thickened amber crusts.14,15 Some cases may resemble exfoliative cheilitis or herpes labialis. Localized infections respond to topical antibiotics, while more widespread infections may require a 1-week course of clindamycin, cephalexin, or dicloxacillin. It commonly presents in younger children and is associated with poor oral hygiene; crowded living conditions; and hot, humid climates. Streptococcus pyogenes (Chapter 285) is also implicated in streptococcal tonsillitis, pharyngitis, and erysipelas, which are infections of the dermis. These infections can rapidly spread through the lymphatic channels.

Beta-hemolytic streptococci bacteria are responsible for scarlet fever (Chapter 285), which presents with unique intraoral lesions. It is most common in children between the ages of 3 and 12. The tonsils, soft palate, and pharynx become erythematous, and petechiae may be present on the soft palate. During the first 48 hours, the tongue’s dorsal surface has a white coating referred to as white strawberry tongue. A few days later, the white coating desquamates to reveal an erythematous dorsal surface with hyperplastic fungiform papillae referred to as red strawberry tongue. The condition has an excellent prognosis and is treated with penicillin.

Several other systemic conditions that have bacterial etiologies can present with oral lesions. These include diphtheria, tuberculosis, cat-scratch disease, actinomycosis, and gonorrhea. Syphilis (Chapter 288), produced by Treponema pallidum, can also present with oral lesions. Congenital syphilis often presents with an alteration in the formation of both the permanent incisors (Hutchinson’s incisors) and the molars (mulberry molars). The incisal edges of the anterior teeth present with a characteristic central hypoplastic notch. The classic pathognomonic diagnostic features of congenital syphilis are described as Hutchinson’s triad, which includes Hutchinsonian teeth, interstitial keratitis, and sensory neural deafness. Syphilis and all the bacterial infections are treated with an appropriate course of antibiotics.14–16

Bite Injuries

Accidental self-inflicted bite injuries are quite common in children, especially after having received intraoral local anesthesia. Not infrequently, children who have received a mandibular nerve block for dental treatment will chew their lip, buccal mucosa, or inner lip unknowingly, resulting in a significant area of epithelial necrosis accompanied by edema. Sites most frequently involved are the lateral border and tip of the tongue, buccal mucosa, and lower lip. The injury usually presents as a macerated, irregular lesion with painless white necrotic areas of epithelium surrounding the ulcerated surface. These children may complain of discomfort, particularly when exposed to foods high in salt or citric acid. Treatment, if necessary, is supportive and palliative since these areas of trauma rarely, if ever, become secondarily infected.17

Cheilitis

Cheilitis (Fig. 378-2) is a common inflammatory disorder in children. It typically arises from the chronic habit of licking the lips and perioral skin. Less frequently, cheilitis or angular cheilosis (perlèche) may occur from mechanical irritation, malocclusion, atopic dermatitis, fungal infection (typically candidiasis), bacterial infection, or nutritional deficiencies. Allergic cheilitis occurs far more frequently in females, typically from a sensitivity to lipstick or lip balm. Toothpaste flavoring agents such as spearmint oil are also a commonly identified allergen. The clinical features of cheilitis are erythema, mild edema, and thin scaling formation of the lips and perioral areas. When due to chronic lip-licking, treatment should be directed at breaking the habit. Empirically, this may be accomplished with lip balms with undesirable tastes. In severe cases, topical steroids, with or without fungicidal and bactericidal activity, may be beneficial. Obviously, in cases where a nutritional deficiency is thought to be the underlying etiology, nutritional supplements are indicated.18-20

Congenital Epulis

Congenital epulis (Fig. 378-3) or gingival granular cell tumor of the newborn is a relatively rare lesion typically arising from the maxillary anterior alveolus. Far more common in females (8:1), it presents as a smooth, firm, pedunculated soft-tissue lesion attached to the alveolus by a broad base. It can range in size from several millimeters to several centimeters, with larger lesions frequently being detected in utero.

Larger lesions require immediate surgical removal due to risks of obstruction, whereas surgery may be deferred with smaller lesions until the infant is older to minimize any anesthesia risk.21

Eruption Hematoma

An eruption hematoma (Fig. 378-4) is a soft tissue variant of the dentigerous cyst that develops around the crown of an erupting primary or permanent tooth. It is quite common and can occur in association with any erupting tooth. The lesion typically resolves on its own with the eruption of the underlying tooth. Rarely is it necessary to excise the roof of the cyst to allow for eruption of the involved root.

Fibroma

The irritation fibroma is considered the most common childhood reactive lesion of the oral cavity. In the vast majority of cases, the lesion arises as a reaction to a local irritant. While the lesion may involve any oral anatomic site, the buccal mucosa and interdental gingiva are the most common sites in children. The lesion usually presents as an asymptomatic pedunculated or sessile growth secondary to irritation. Treatment is surgical excision with the elimination of any associated source of local irritation.22,23 Failure to remove the source of irritation will likely result in recurrence.

Candidiasis

Candidiasis or candidosis is an infection with the yeastlike fungal organism Candida, the most common being Candida albicans. It often infects children and adults who are immunocompromised or who are on a prolonged course of antibiotic therapy. It is the most common oral fungal infection in humans and can be manifest in a variety of clinical presentations, which may make appropriate diagnosis difficult.24

Erythematous, or chronic atrophic candidiasis, may also be caused by immunosuppression, xerostomia, or chronic exposure to broad-spectrum antibiotic therapy. Patients often complain of a burning sensation on their tongue associated with a loss of filiform papilla and a denuded appearance of the tongue’s dorsal surface. Unlike the acute form characterized by sloughing white lesions, the erythematous form results in patchy lesions. Many forms of chronic candidiasis are asymptomatic, including central papillary atrophy and median rhomboid glossitis. In addition, angular cheilitis is a particular form of erythematous candidiasis that affects the commissures and is characterized by fissuring, erythema, and scalelike lesions. The treatment regimen is similar to that of acute candidiasis. The lesions may be caused by C albicans alone or by Candida in association with Staphylococcus bacterial infection.24–27

Pseudomembranous candidiasis (acute) is the most common form of oral candidiasis, which is usually seen on the buccal mucosa, dorsum of the tongue, and soft-palate (eFig. 378.1). It is commonly known as thrush and appears as white cottage cheese–like lesions that can be removed by scraping. It is the most common manifestation of HIV infection in children. Topical antifungal medications are often the drug of choice for localized candidiasis. While compliance for the “swish and swallow” routine with these medications is readily accomplished by older patients, in the infected infant, appropriate delivery of the topical medication is often problematic. A foam-headed device (Toothette) is a useful aid in applying the medication to the infected oral mucosa. When this condition is seen, it is imperative to rule out a depressed immune system, which may be contributing to the candidiasis. Appropriate pharmacotherapy includes Nystatin oral suspension, Clotrimazole troche, or systemic antifungal mediations such as ketoconazole or fluconazole.24–27

Geographic Tongue (Erythema Migrans)

Geographic tongue (Fig. 378-5) is a harmless common disorder of unknown etiology that primarily affects the dorsal surface of the tongue, although it may occasionally involve other mucosal surfaces, including the buccal and sublingual mucosa. Several studies have suggested an increased prevalence among patients with fissured tongue, allergies, seborrheic dermatitis, and psoriasis. Clinically, geographic tongue is characterized by an erythematous, well-circumscribed area of atrophy of the filiform papillae on the dorsum of the tongue, ranging in size form several millimeters to several centimeters. The erythematous area is most frequently surrounded by a slightly raised whitish margin. The involved area typically changes or “migrates” over several weeks or months, thus giving rise to the disorder’s name. Onset tends to be fairly rapid, with lesions typically persisting for several weeks, although occasionally they may last for several years. Patients are generally asymptomatic but may rarely complain of a “burning” sensation. No treatment is necessary; however, patients experiencing burning may benefit from palliative treatment with topical anesthetics.28

Hairy Tongue

An accumulation of keratin and elongation and hypertrophy of the filiform papillae of the tongue results in the clinical appearance of hairy tongue. While the etiology is unknown, it is correlated with several predisposing factors, including poor oral hygiene, mouthrinses containing sodium perborate, long-term antibiotics, steroid therapy, head and neck irradiation, and C albicans. Clinically, the elongated filiform papillae can range in color from white to brown to black. Although patients are typically asymptomatic, they may occasionally complain of a bad taste or halitosis. Treatment involves removing the precipitating factors and improved oral hygiene, including brushing the tongue. A unique form of hairy tongue is associated with HIV and is referred to as hairy leukoplakia. While this condition is usually observed in adults with HIV, there have been several reports of its occurrence in HIV-infected children. The lateral border of the tongue is most commonly affected bilaterally and is thought to be associated with Epstein-Barr virus infection.29-31

Melanotic Neuroectodermal Tumor of Infancy

Melanotic neuroectodermal tumor of infancy is a rare condition that could potentially be confused with the congenital epulis. Similarities to the congenital epulis include its appearance in infancy (most common age being 1 to 3 months), it being more common in females (3:2), and it most commonly involving the anterior maxilla. However, unlike congenital epulis, the melanotic tumor of infancy is a locally aggressive lesion capable of causing significant tissue damage. It is believed the tumor arises from neural crest. The most common manifestation of the tumor is a soft tissue mass with an intact mucosal surface. The lesion may contain blue or black areas of pigmentation and may grow large enough to obliterate the labial buccal vestibule with resultant displacement and elevation of the lip. Pain is rarely associated with this tumor, whose growth rate is variable. Surgical excision or enucleation is the recommended treatment. Recurrence is relatively common, with rates as high as 20%.32

Mucocele

Mucoceles (Fig. 378-6) are the most common salivary gland problem of childhood. They typically arise in the mucosa of the lower lip as a result of trauma to a minor salivary gland, although they may occur less frequently on the buccal mucosa, soft palate, or upper lip. The extravasation of saliva into the surrounding soft tissue causes the formation of a pseudocyst. This lesion clinically appears as a painless, raised, dome-shaped lesion and may be clear, pink to red, or bluish in color, or it may be pale and fibrotic. Size is typically less than a centimeter in diameter. A common sign is a periodic reduction in swelling due to rupture and partial release of fluid, followed by recurrence due to new accumulations of fluid. While mucoceles will occasionally resolve spontaneously, most require surgical excision.33

Neonatal Cysts

Neonatal cysts (Fig. 378-7) are benign developmental lesions. Previously, they were differentiated based upon locations. Epstein’s pearls are remnants of epithelial tissue trapped during the palatal fusion that occurs during the 8th to 10th gestational week. Bohn’s nodules are remnants of mucous gland tissue found on the buccal or lingual aspects of the dental ridges. Dental lamina cysts are found along the crest of the ridges.1,4,8 These terms, however, are frequently confused and used synonymously. Currently, the terms palatal cysts (designating epithelial cells that persist at the site of fusion of the palatal shelves) and alveolar cysts (remnants of degenerating dental lamina on the buccal, lingual, or crest portion of the alveolar ridge), are used in descriptions to avoid confusion.4 Alveolar cysts have also been referred to as “gingival cysts” or “inclusion cysts” in some texts. The clinical description of palatal and alveolar cysts varies in color from white, to gray, to yellow nodules, in size from a pinhead to 3 mm, and in numbers from 1 to 6. The frequency of inclusion cysts is high in newborns, but they are rarely seen after 3 months of age. The prevalence in term infants varies from 65% to 85% for palatal cysts and depending on location, 9% for mandibular alveolar cysts to 36% for maxillary alveolar cysts. Prevalence is lower in premature infants.34 White infants are more likely to have palatal and alveolar cysts than African-American infants. No gender difference has been reported.

Since these lesions resolve spontaneously over the course of a few months, No treatment is necessary.35

Obstructive Sialadenitis

Obstructive sialadenitis occurs as the result of a sialolith or foreign body blocking the normal flow of saliva, most commonly from the submandibular gland. Patients often complain of a painful swelling related to meals. The swelling generally dissipates over several hours. Symptoms may include pain, erythema of the overlying skin, fever, and an elevated white blood cell count. Treatment includes antibiotics, salivary flow stimulation, and heat. Surgery may be indicated to remove the sialolith and to establish patency of the duct. In recurrent cases, excision of the submandibular gland may be necessary.36

Pyogenic Granuloma

The pyogenic granuloma is a relatively common benign vascular lesion of the skin and mucosa that arises from an exaggerated tissue response to local irritation. Typically found on the gingiva in the the oral cavity, it can be found on the tongue, lips, and buccal mucosa. The granuloma appears as a painless, nodular, erythematous mass that is typically either pedunculated or sessile. The color ranges from bluish to purple and may resemble a hemangioma. The surface may be smooth or lobulated and is frequently ulcerated and covered with a whitish-yellow pseudomembrane. Probing or palpating this soft lesion will frequently result in bleeding. It grows rapidly and ranges in size from 5 to 10 mm. Since these lesions arise from an excessive tissue response to a local irritant, treatment involves both surgical excision and removal of the source of irritation. When on the gingiva, the irritant is typically calcified plaque (calculus), which must be removed from the tooth surface to prevent recurrence.37

Ranula

A ranula is a form of mucous-retention cyst that occurs on the floor of the mouth in association with the sublingual gland or, less frequently, the submandibular gland. While they may be congenital, most arise from trauma. The ranula presents as a smooth, dome-shaped, painless, fluctuant swelling. The color is typically bluish, but deep lesions may appear as normal mucosa. Smaller ranulas are typically excised, while larger lesions may require marsupialization.38-40

Recurrent Aphthous Stomatitis

Recurrent aphthae, or canker sores, are the most common forms of oral ulcerations in both children and adults. Epidemiological studies indicate that the prevalence of recurrent aphthous stomatitis (ARS) in the general population is between 2% and 50%, with onset typically occurring in childhood. These episodic ulcerations generally continue past childhood and adolescence and occur throughout the patient’s life span, with no age, sex, race, or geographic-related predilection.

There are three forms of the disorder: minor, major (Sutton’s disease), and herpetiform. Minor aphthae are typically round, small (less than 10 mm in diameter), well-circumscribed ulcerations surrounded by an erythematous halo. These usually occur on movable or unbound (nonkeratinized) mucosa. Several may be present simultaneously. Healing is spontaneous within 7 to 10 days, usually without scarring.

Major aphthae are similar but are larger in size (greater than 1 cm in diameter), are generally much deeper, and may be debilitating. Healing may take 10 to 30 days and may result in scarring.

Herpetiform ulcers are very small (1 mm in diameter) and often occur in clusters. There is a tendency for adjacent ulcers to coalesce and form a larger affected area. Healing takes place usually within 7 to 10 days. The name results from the clinical manifestation resembling a recurrent intraoral herpetic infection. All three clinical types are associated with varying degrees of pain and discomfort, the extent of which depends on the location, size, and depth of the ulcers. The etiology of recurrent aphthous stomatitis is unknown, although an altered local immune response is the suspect. Precipitating factors include stress, endocrine alterations, allergies, trauma, and food hypersensitivity. Patients with frequent recurrences should be screened for potential underlying diseases such as anemia, nutritional deficiencies (eg, iron, folate, vitamin B12), diabetes mellitus, immunosuppression, and inflammatory bowel disease (Table 378-1). Treatment is primarily aimed at pain relief and accelerating the healing process. All therapies are palliative, and none have resulted in permanent remission. Although the ulcer’s etiology is not clearly understood, some treatment regimens focus on either preventing or at least reducing the recurrence rate of an episode. A variety of topical and systemic therapies have been tried, but so far, no studies have shown clear evidence of effectiveness, either as preventive or a pain-relieving agents. Empirically, effective treatment includes topical anesthetics, corticosteroids (topical and systemic), antimicrobial mouthrinses, topical anti-inflammatory agents, and topical barriers. Severe, persistent, recurrent aphthous ulcers may be treated with medications such as systemic corticosteroids, pentoxyphylline, colchicines, and thalidomide, but these should not be routinely used, as they have potential adverse side effects. All therapies are palliative, and none have resulted in permanent remission. Since several systemic disorders are associated with chronic aphthous ulcer formation, potential underlying conditions (ie, periodic fever apthous stomatitis–pharyngitis and adenopathy (PFAPA), inflammatory bowel disease, Behçet’s syndrome, Reiter’s syndrome, and cyclic neutropenia) must be ruled out (see Table 378-1). RAS represents a highly common mucosal disorder, the etiology remains poorly understood, and the efficacy of therapies remains undefined.41-47

Condyloma Accuminatum

This is a benign papillary lesion most commonly associated with human papillomavirus (HPV) 16 and 18 and is usually sexually transmitted. Oral condyloma accuminatum reportedly results from genital-oral sexual transmission or hand-to-mouth autoinoculation. The presence of condylomata in children may be secondary to child abuse. The common location of children’s oral condyloma accuminatum is the palate, which is different from adults, who usually get the lesions on the lower lip and tongue. The course of treatment and recurrence rate is similar to that for other types of HPV.48-50

Epstein-Barr Virus

The Epstein-Barr virus (EBV) is a gamma-herpes virus of which humans are its only natural reservoir. EBV causes several diseases that may have oral or maxillofacial manifestations, such as infectious mononucleosis, hairy leukoplakia, Burkitt’s lymphoma, and nasopharyngeal carcinoma (see also Chapter 311). EBV infects different human cell types, including epithelial cells and lymphocytes. Symptomatic primary infection is more likely to occur in immunocompromised individuals. Infectious mononucleosis commonly has oral manifestations in children and young adults. Clinical signs include pharyngitis and tonsillitis that extends into the soft palate. The involved mucosa is erythematous with erosions and small characteristic petechiae. The area is often coated with a grayish exudate and is associated with tender cervical lymphadenopathy. Detection of specific anti-EBV IgM antibodies confirms acute infection. Symptoms will usually resolve in a month but may persist for longer in some cases. EBV can be reactivated from the latent state.

Other conditions associated with EBV, but less common in the United States, are nasopharyngeal carcinoma (Asia) and Burkitt’s lymphoma (Africa). Patients who have compromised cellular immunity—for example, those with HIV infection—are particularly prone to developing EBV-associated lymphomas. Lastly, hairy leukoplakia is an EBV-induced epithelial hyperplasia that causes hypertrophic corrugated white lesions on the lateral border of the tongue. Hairy leukoplakia can occur in the absence of HIV infection in organ transplant patients who are immunosuppressed. Unlike acute candidiasis, this lesion cannot be wiped off and is entirely asymptomatic.51-54

Hand, Foot, and Mouth Disease

Hand, foot, mouth disease (HFMD) is a common enteroviral infection caused by Coxsackie type A16 and is another common cause of intraoral vesicular lesions (see also Chapter 306). It has also been linked to Coxsackie A 4-7, 9, 10; B 1-3; and 5 serotypes.55 It has a distinctly different presentation than the lesions seen in herpangina, which is also caused by the Coxsackie virus. These are both common viral illnesses of infants and children. Infection usually occurs by the fecal-oral route, leading to viremia and invasion of the mucosa and skin. The infection begins with a mild fever, poor appetite, malaise, and frequently a sore throat. One or 2 days after the fever begins, painful sores develop in the mouth. They begin as small red spots that blister and then often become ulcers between 1 and 2 cm in diameter. The associated lesions of the hands and feet are usually painless and fade in 1 to 2 weeks without any apparent scarring. It is important to note that outbreaks occur more commonly in summer and autumn and can be transmitted from child to child or from child to adult.

No specific treatment is available for this or other enterovirus infections. Symptomatic treatment is given to provide relief from fever, aches, or pain from the mouth ulcers. The lesions regress and heal within 7 to 10 days. If an outbreak occurs in the childcare setting, it is imperative that meticulous personal hygiene measures be enforced. It may also be beneficial to thoroughly wash and disinfect contaminated items and surfaces using a diluted solution of chlorine-containing bleach.55-59

Herpangina

Herpangina has a similar presentation to that of hand, foot, mouth disease, but the vesicular lesions are solely seen in the region of the soft palate (see also Chapter 306 and Figure 306-3). Herpangina is also usually caused by the Coxsackie virus but may also be caused by an echovirus. It is relatively common in children between the ages of 1 and 4 and is seen more often in summer and autumn. Herpangina is associated with headache, quick-onset fever, dysphagia, sore throat, pain, drooling, and a sudden decrease in appetite. The incubation period is 2 to 9 days, and fever usually lasts from 1 to 4 days. As in any viral infection, antibiotics are ineffective and treatment is supportive and palliative, consisting of hydration and acetaminophen for any associated fever. The lesions usually resolve in 7 to 10 days.55-59

Human Immunodeficiency Virus (HIV)

HIV infection predisposes an infected child to a myriad of characteristic oral lesions and manifestations. Candidiasis has been documented in several studies as the most frequently occurring oral manifestation in HIV-infected children, with a prevalence ranging from 20% to 72%.60-63 There is a significant relationship between oral mycotic infections and declining CD4 T-cell and neutrophil counts in HIV-infected children.64Other lesions in these immunosuppressed children include parotid enlargement, angular cheilitis, herpetic stomatitis, oral hairy leukoplakia, petechiae, aphthous stomatitis, linear gingival erythema, and cervical lymphadenopathy. It is important to emphasize that parotitis has been recognized as a unique sequela of pediatric HIV infection and does not require specific treatment. It has been reported in 10% to 30% of children with symptomatic HIV infection.65,66 The presence of parotitis has been associated with a positive prognosis and long-term survival in HIV-infected children. Therefore, it has been suggested that both candidal infection and parotitis be included in prognostic indices and decisions regarding therapy for these patients. Undoubtedly, pediatric dentists and pediatricians can both play a role in the early detection of HIV-related oral lesions.60-67

Primary Herpetic Gingivostomatitis

Primary oral herpetic gingivostomatitis is caused by herpes simplex virus type 1 (HSV-1) (see also Chapter 315). Most individuals experience a subclinical infection with HSV-1, while a small percentage of the population develops the full manifestations of the infection. It is frequently referred to as herpetic gingivostomatitis and is characterized by vesicular lesions on the gingival tissue, lips, tongue, buccal mucosa, and soft palate. These lesions will often appear in clusters and may frequently coalesce, creating large areas of ulcerations that resemble aphthous ulcerations. The tissue is fiery red in appearance and is painful. Herpes simplex is easily transmitted when the shedding herpes virus comes in direct contact with mucous membranes or broken skin. Most primary infections are contracted from others who are shedding HSV-1 but who are free of obvious lesions. Childcare facilities such as daycare centers or primary school classrooms are ideal grounds for transmission. Primary herpes simplex infection peaks between the ages of 2 and 3 but may present in children between 6 months and 5 years of age or older. There is no sex predilection.68-71

Symptoms manifest themselves after a brief prodromal period. The child may become febrile, have associated lymphadenopathy, and may not be able to eat properly. There is an associated risk of dehydration, so care must be taken to assure hydration. Intravenous hydration therapy might be necessary in severe cases. The viral infection typically lasts between 10 to 14 days, with the most severe ulcerations appearing between day 3 and day 7.

Prior to the development of acyclovir, treatment was limited to palliative care consisting of administration of fluids to prevent dehydration, topical and systemic analgesics to relieve pain, and antipyretics to combat elevated temperature. The use of oral acyclovir is thought to be most effective if used during the prodromal period. The suggested oral dosage for HSV-1 infections is 15 mg/kg, 5 times daily, for a period of 7 days. Palliation may be provided with topical agents, including viscous lidocaine (2%), Kaopectate and Benadryl elixir, or dyclonine. Over-the-counter topical anesthetics in gel form contain 20% benzocaine and may be helpful for those with a minimal number of lesions. Parents may have concerns about their child having a “herpes infection,” since herpes is so often linked to sexual transmission. It should be understood that HSV-1 (rather than HSV-2) is considered the infecting organism for their child’s condition.69-75

Recurrent Herpes Labialis

After the initial infection ameliorates, herpes simplex virus persists in the dorsal root ganglion and causes recurrent disease in approximately 15% of the population. The recurrent herpes labialis or “cold sore” lesions occur on the lips and perioral tissues and are most often seen unilaterally. The infection is thought to be reactivated by exposure to stress, sun, illness, fever, and depressed immune systems. Since sunlight seems to be the most common precipitating agent, daily application of a lip balm containing sunblock maybe a beneficial preventative in the susceptible individual. The viral infection has a course of 7 to 10 days, progressing from a vesicular stage to crusting. Therapy is generally palliative, as topical and systemic antiviral agents have been shown to be of limited efficacy. It must be stressed that patients keep their hands and fingers clean and away from the nidus of infection, as autoinoculation of the contralateral side of the mouth, eye, or other mucous membrane can occur readily.72-75

Rubeola

The measles virus is a single-stranded RNA virus of the family Paramyxoviridae. It has a prodromal period of 7 to 14 days. Intraoral lesions called Koplik spots are present prior to the rash on the skin and appear on day 2 or 3. These blue-white spots are about the size of salt grains and are surrounded by a reddish halo. They are located on the buccal mucosa opposite the premolar teeth and are pathognomonic for measles. The spots usually regress at or around day 6.76

Squamous Papilloma

The human papillomavirus (HPV) is a benign group of DNA-based viruses that can infect the skin and mucous membranes. Infection with HPV can result in numerous exophytic white or pink fingerlike projections that resemble cauliflower or warts. These lesions are pedunculated and represent a proliferation of stratified squamous epithelium. There are over 100 strains of HPV. The average age of occurrence for a squamous papilloma is in the fourth decade of life, but nearly 20% of cases occur before 20 years of age.77-85 Over 50% of oral lesions are linked to HPV-6 and HPV-11 subtypes. The oral lesions are most commonly seen on the lips and on the palate, uvula, and dorsum of the tongue. They are transmitted through direct contact, and the only definitive treatment is excision, cryotherapy, or electrosurgery. Recurrence is rare.49,54

Intraoral hard tissue pathologies refer to conditions afflicting the skeletal components of the masticatory system, namely the maxilla, mandible, and alveolar bone, or the dentition. Both the skeletal conditions and anomalies of tooth number, size, shape, and color may manifest as isolated occurrences or may reflect an underlying systemic disturbance. Intraoral tumors and cysts involving the facial skeleton are relatively uncommon in children. An analysis of oral and maxillofacial pathology over a 30-year period revealed that only 8% of the specimens came from children. The vast majority of oral pathology in children is related to the dentition and oral soft tissues, as demonstrated by several large biopsy surveys. Dental or tooth-related pathology, salivary gland disease, and mucosal pathology accounted for the vast majority of biopsies in these studies. And while most intraosseous pathology is discovered as a result of a routine dental radiograph, enlargement of these tissues occasionally will be clinically detectable.77-79

Flaitz and Coleman suggest that bony enlargements of the maxilla and mandible be divided into three categories: (1) inflammatory lesions, (2) benign cystic and neoplastic lesions, (3) aggressive and malignant lesions. The inflammatory lesions are the most frequently encountered, followed closely by the benign cystic and neoplastic lesions. Aggressive and malignant lesions are rare in children, with a reported prevalence of less than 1% of biopsied lesions.80

Aggressive and Malignant Neoplasms of the Jaws

Aggressive and malignant jaw neoplasms and tumors are rare in children. These include the melanotic neuroectodermal tumor of infancy (described elsewhere), Langerhans cell histiocytosis, Ewing’s sarcoma, primitive neuroectodermal tumor, osteosarcoma, and desmoplastic fibroma of bone.

Approximately 10% of Langerhans cell histiocytosis cases have oral involvement. Localized destruction of the tooth-supporting bone frequently results in mobile teeth, usually in the mandible.81,82 Typically, these hard tissue lesions are accompanied by soft tissue involvement characterized by localized ulceration and necrosis.

Ewing’s sarcoma and primitive neuroectodermal tumors involve the jaws in fewer than 3% of the cases. Typical symptoms are jaw enlargement frequently accompanied by pain.

Approximately 6% to 7% of all osteogenic sarcomas occur in the maxillofacial region. The most common clinical manifestations of osteosarcoma of the jaws is rapid enlargement of the affected bone that may or may not be accompanied with pain. Ulceration and paresthesia or anesthesias are commonly reported symptoms. One study found these lesions frequently misdiagnosed as odontogenic infections.83-88

Benign Cystic and Neoplastic Lesions of the Jaws

Intraosseous benign cysts and tumors are typically classified as either odontogenic, meaning arising from tooth-forming tissues, or nonodontogenic. Most odontogenic cysts in children are associated with impacted teeth and are most frequently discovered from dental radiographs. Occasionally, these cysts will cause localized expansion that may be evident clinically. One odontogenic cyst of particular concern is the odontogenic keratocyst, which typically is multiple when associated with basal cell nevus syndrome. These cysts have a relatively high recurrence following excision. The nonodontogenic cysts and tumors that may present with facial enlargement include the aneurysmal bone cyst, fibro-osseous lesions, fibrous dysplasia, cherubism, and central giant cell granuloma. Biopsy is necessary to establish a definitive diagnosis.99

Attrition and Erosion

After the initial mineralization process, tooth structure may be lost from a myriad of factors besides caries and traumatic fracture. These mechanisms include attrition, abrasion, and dental erosion (eFig. 378.2).

Attrition is loss of tooth structure caused by tooth-to-tooth contact during mastication and occlusion. When the amount of tooth loss is extensive, it may begin affecting the appearance and function of the primary or permanent dentition. This wear may present in the form of habitual bruxism or involuntary grinding of the teeth. Bruxism is defined as an interruption of the normal rest position of the mandible during rest or sleep resulting from the forceful, rhythmic contractions of the masticatory musculature. It can be potentially harmful to the health of the oral tissues and restorative work. The prevalence of bruxism in children noted in the literature varies from 7% to 88%.86-89 This has been reported to occur in association with parasomnial activity and various dental, mental, neurological, and psychological risk factors. It has also been noted as a factor of other dental conditions such as malocclusion, eruption, or localized conditions, including mobile teeth, cuspal interferences, high restorations, and occlusal disharmony.89-91

Dental erosion is an irreversible dental hard-tissue loss due to acid exposure in the absence of bacterial involvement. Erosion may be caused by intrinsic factors, including recurrent vomiting (as in bulemia nervosa), regurgitation, gastroesophageal reflux (GER), or rumination. Extrinsic sources of acid are also possible causes of dental erosion such as diets composed of acidic foods and drinks, carbonated beverages, and alcohol. Continuous ingestion of acidic medications like chewable vitamin C and aspirin are also known causes of dental erosion.92,93

Erosion is a relatively common finding in the dentition of children. Kazoullis and colleagues (2007) reported that 68% of 714 healthy children examined (ages 5 to 14 years old) presented with dental erosion.94-96 They indicated a threefold greater prevalence of dental erosion in primary teeth than permanent teeth and stated that erosion was associated with low socioeconomic status, enamel hypoplasia, and dental caries. They speculate that the increase in dental caries may be from the acidic process that is common in both entities. They point out that erosion in primary dentition may be more severe than in the permanent dentition and may be due to (1) the pattern of mineralization being different in primary teeth, (2) thinner primary enamel, and (3) primary teeth being present in the mouth a relatively longer period of time than permanent teeth at the time of the data-gathering in the study. Dental erosion can result in pain, malocclusion, and even temporomandibular joint dysfunction (as the hard tissues wears, the bite deepens, causing changes in the temporomandibular joint).95

Gastroesophageal reflux has been associated with varying degrees of dental erosion of both the primary and permanent dentition.92-96 In four of these studies, pediatric-age patients with GERD underwent dental examinations to determine the prevalence of dental erosion.91,94-96 The reported prevalence of erosive lesions in these studies ranged from 14% to 87%. Only one study of children with significant erosion has confirmed the presence of GERD using the standard pH probe.97 This study group consisted of six children, four of whom were confirmed to have GERD. Interestingly, one of the four children diagnosed with GERD had no symptoms of the disease. Kocatas and colleagues also studied dental erosion in children (mean age of 6.5 years) who were diagnosed with GERD. They reported that streptococcus mutans (MS) and salivary yeast colonization were higher in patients with dental erosion.98

Discoloration and Staining

The color of normal teeth varies and can be affected by a variety of factors, such as hygiene practices, diet, habits, bacteria, and underlying medical conditions. This discoloration can be classified as either extrinsic or intrinsic in nature (eFig. 378.3). Extrinsic stains, or superficial pigmentation of the enamel, are caused by such things as bacterial stains, tobacco, foods, restorative materials, and medications like iron supplements. Generally they can be removed with a thorough dental prophylaxis. For example, chromogenic bacteria can produce stains that vary from orange to black-brown, and extensive use of tobacco, tea, and coffee often results in significant brown discolorations on the enamel’s surface. However, the most common cause of such extrinsic stains is poor oral hygiene.

Intrinsic stains are secondary to endogenous factors, resulting in discoloration of the dentin. These may be secondary to hereditary conditions such as amelogenesis or dentinogenesis imperfecta. They may also be attributed to dental fluorosis, erythropoietic porphyria, hyperbilirubinemia, and medications. Historically, tetracycline use in pregnant women and young children was one of the more common etiologies of intrinsic staining. Their ability to cross the placenta barrier and chelate calcium results in staining of both teeth and bone in the developing fetus and child. Consequently, package warnings regarding this complication became commonplace, with tooth staining a rarity. An exception to this phenomenon is minocycline, which can result in abnormal pigmentation of the skin, thyroid gland, nails, bone,sclerae, and conjunctivae in adults. Unlike tetracycline, minocyclinehas been reported to cause generalized intrinsic tooth stainingposteruption. The mechanism of this staining is unclear but is likely related to the dynamic nature of the tooth dentin.100-102

Fusion and Gemination

Fusion and gemination refer to two distinct conditions resulting in a similar clinical appearance of “twinned” or “double tooth.” They represent disturbances in the morphodifferentiation stage of tooth development.

Gemination is an attempt of a single tooth bud to divide, resulting in an incomplete formation of both the teeth and their clinical crowns. This may result in crowns that are partially or totally separate but have a single root canal. The total tooth count is normal. Fusion, however, is a complete joining of enamel or dentin of two distinct tooth buds. Usually there is one fewer tooth, unless this fusion occurs with an extra or supernumerary tooth. Fusion and germination are seen more commonly in the primary than in the permanent dentition. Fusion or gemination is found relatively frequently, occurring in 0.5% to 2.5% of the population studied.103,104

Hypodontia

Congenitally missing teeth (tooth agenesis) are one of the most common dental diagnoses in the pediatric population. The congenital absence of teeth results from disturbances during the initial stages of tooth formation, initiation, and proliferation. Missing teeth can occur in isolation or may be associated with an underlying syndrome or medical condition and can range from complete anodontia (all teeth) to hypodontia (one or more teeth). Additionally, these dental anomalies have been reported in children who have cleft lip, cleft palate, ectodermal dysplasia, Down syndrome, and Hallermann-Streiff (oculomandibulofacial) syndrome.

Isolated missing teeth in the permanent dentition are not rare. The most common missing permanent teeth are third molars, with prevalence rates reported at 9% to 37%. This is followed by mandibular second premolars and maxillary lateral incisors, depending on the population studied. It is important to note that it is possible for any tooth in the dental arch to be congenitally absent. Referral to a pediatric dentist may be necessary to determine whether the tooth is, indeed, congenitally absent or has failed to erupt.105-107

Supernumerary Teeth/Hyperdontia

Supernumerary teeth and conditions of hyperdontia occur when there is more than the normal complement of primary or permanent teeth. Overall, the prevalence of supernumerary teeth ranges from 1.5% to 3.9%. Single-tooth hyperdontia is most commonly found in the anterior maxillary region in the form of a mesiodens. A mesiodens is a supernumerary tooth (or teeth) that is present in the maxillary midline between the permanent central incisors and is often an incidental finding on dental radiographs. It is usually located palatally to the permanent incisors and may interfere with eruption or, if the incisors erupt, may result in a malposition. In the absence of timely eruption of the maxillary permanent incisors, referral to a pediatric dentist is appropriate, and removal may be necessary. Occasionally, supernumerary teeth may be found in the primary dentition and may signal the presence of extra permanent teeth as well.

Multiple supernumerary teeth may be a feature of several syndromes, including Gardner’s syndrome, cleidocranial dysplasia (CCD), cleft lip and palate, Hallermann-Streiff, Down syndrome, and chondroectodermal dysplasia. Overall, supernumerary teeth are five times more common in the maxilla, with a male-to-female ratio of 2:1. They are more commonly associated with permanent dentition. The etiology of supernumerary teeth is not completely understood.108,109