This article explores pediatric lingual and other intraoral lesions. First the embryology and anatomy of the oral anatomy is outlined. Then the article discusses infections and inflammatory diseases, congenital malformations, benign neoplasms, and malignant tumors.
Key points
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Sore throats and dysphagia are one of the more common presenting symptoms in patients seen by pediatricians.
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Many systemic diseases have manifestations in the oral cavity, and may be diagnosed by recognition of these characteristics.
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Most embryonic development of the oral cavity occurs between the fourth and eighth week of gestation.
Embryology and anatomy of the oral cavity
The oral cavity is bounded anteriorly by the lips, laterally by the cheeks, and superiorly by the hard and soft palate. The posterior border is defined by the circumvallate papillae and the junction of the hard and soft palate. The oral cavity is further divided into two parts. The vestibule is defined as the area between the teeth and lips and the oral cavity proper is defined as the area posterior to the teeth and alveolar ridge.
The Lips
In the fourth week of development the lower lip is formed as the mandibular prominences merge. In weeks 6 to 8 of development the medial frontonasal and lateral maxillary prominences merge to form the upper lip.
The lips are innervated by branches of the facial nerve. The buccal branch provides motor innervation to the upper lip and the marginal mandibular branch provides motor innervations to the lower lip. Sensory innervation to the upper and lower lips is provided by the infraorbital nerve and the mental nerve, respectively.
The Tongue
In the fourth week of gestation development of the tongue begins when two paired lateral lingual swellings and one central tuberculum impar appear on the floor of the oral cavity and pharynx. These two structures fuse and form the anterior two-thirds of the tongue. The posterior third of the tongue develops from a second median swelling known as the hypobranchial eminence or the copula. The glossopharyngeal and vagus nerves provide sensation and motor innervations to the posterior third of the tongue, whereas the trigeminal and chorda tympani nerves supply the anterior two-thirds of the tongue.
A V-shaped groove separates the anterior two-thirds and posterior one-third of the tongue. This groove is called the sulcus terminalis. The foramen cecum is a blind depression in the midline of the apex of the sulcus terminalis. The thyroid gland originates from the foramen cecum and migrates inferiorly.
The Palate
In the twelfth week of development the palate is formed from fusion of the primary and secondary palates. The primary palate is formed from the intermaxillary segment by the merger of two medial nasal swellings.
Beginning at the seventh week gestation the secondary palate is formed by the fusion of two palatal processes. The hard palate initially occurs in the midline anteriorly and proceeds posteriorly with the fusion of the horizontal palatal shelves.
The anterior two-thirds of the palate functions to separate the nasal cavity from the oral cavity, whereas the soft palate provides a barrier between the nasopharynx and the oropharynx. Proper functioning of the soft palate plays a key role in deglutition and normal articulation in speech.
Embryology and anatomy of the oral cavity
The oral cavity is bounded anteriorly by the lips, laterally by the cheeks, and superiorly by the hard and soft palate. The posterior border is defined by the circumvallate papillae and the junction of the hard and soft palate. The oral cavity is further divided into two parts. The vestibule is defined as the area between the teeth and lips and the oral cavity proper is defined as the area posterior to the teeth and alveolar ridge.
The Lips
In the fourth week of development the lower lip is formed as the mandibular prominences merge. In weeks 6 to 8 of development the medial frontonasal and lateral maxillary prominences merge to form the upper lip.
The lips are innervated by branches of the facial nerve. The buccal branch provides motor innervation to the upper lip and the marginal mandibular branch provides motor innervations to the lower lip. Sensory innervation to the upper and lower lips is provided by the infraorbital nerve and the mental nerve, respectively.
The Tongue
In the fourth week of gestation development of the tongue begins when two paired lateral lingual swellings and one central tuberculum impar appear on the floor of the oral cavity and pharynx. These two structures fuse and form the anterior two-thirds of the tongue. The posterior third of the tongue develops from a second median swelling known as the hypobranchial eminence or the copula. The glossopharyngeal and vagus nerves provide sensation and motor innervations to the posterior third of the tongue, whereas the trigeminal and chorda tympani nerves supply the anterior two-thirds of the tongue.
A V-shaped groove separates the anterior two-thirds and posterior one-third of the tongue. This groove is called the sulcus terminalis. The foramen cecum is a blind depression in the midline of the apex of the sulcus terminalis. The thyroid gland originates from the foramen cecum and migrates inferiorly.
The Palate
In the twelfth week of development the palate is formed from fusion of the primary and secondary palates. The primary palate is formed from the intermaxillary segment by the merger of two medial nasal swellings.
Beginning at the seventh week gestation the secondary palate is formed by the fusion of two palatal processes. The hard palate initially occurs in the midline anteriorly and proceeds posteriorly with the fusion of the horizontal palatal shelves.
The anterior two-thirds of the palate functions to separate the nasal cavity from the oral cavity, whereas the soft palate provides a barrier between the nasopharynx and the oropharynx. Proper functioning of the soft palate plays a key role in deglutition and normal articulation in speech.
Infections and inflammatory diseases of the oral cavity
Sore throats and dysphagia are one of the more common presenting symptoms in patients seen by pediatricians. There are many causative organisms from localized viral, bacterial, and fungal infections to more systemic diseases that manifest in the oral cavity.
Viral Infections
Many viruses can cause dysphagia and orpharyngitis. In the oral cavity, herpetic infection is the most common type of viral illness.
Herpes simplex virus
There are two forms of herpes simplex virus infections. Herpes simplex virus 1 is a primary severe infection affecting the oral mucosa. Herpes simplex virus 2 is a secondary infection that is more common and affects the genitalia.
Primary herpetic infection classically presents in children younger than 5 years old. After a brief prodrome of constitutional symptoms, stomatitis develops with a tingling sensation of the oral mucosa followed by erythema and edema. Vesicles then develop and rupture turning into ulcers that heal spontaneously within 10 to 14 days. A gray pseudomembrane then covers the ulcers, which may coalesce or become secondarily infected with oral bacteria. There may be associated fever and flulike symptoms with rare progression to herpes or meningoencephalitis. Treatment involves antiviral drugs, such as acyclovir either topically or parentally.
Once primary infection has occurred with herpes simplex virus, the virus may lie dormant in the regional neuroganglia until activated. Fever, excessive sun exposure, stress, or immunodeficiency can result in activation of the virus. A burning or tingling sensation signals the formation of mucosal erythema and vesicle formation shortly after. The vesicles usually rupture within the first day leaving a superficial ulceration that spontaneously heals within 10 to 14 days. These ulcers usually reoccur in the same sites on the vermillion border of the lips or intraorally on the mucosa of the gingival.
Herpes zoster
Herpes zoster, or shingles, presents as skin or mucosal vesicles along the distribution of the trigeminal nerve and can involve the mucosa of the lips and oral mucosa. This is classically seen in patients who are immunocompromised. This is thought to be caused by the reactivation of the varicella virus that is dormant in the sensory ganglia. Vesicles usually resolve within 10 days and patients are treated symptomatically.
Herpangia
This usually occurs in young children caused commonly by coxsackievirus A. It is characterized by the sudden onset of fever, malaise, with an intense sore throat. Multiple small vesicles are usually located in the posterior oral cavity, which ruptures to form small ulcers. This condition spontaneously resolves in 7 to 10 days.
Hand-foot-and-mouth disease
This viral illness mimics herpangia in every respect. It is also caused by the coxsackievirus A. Vesiculaopapular lesions on the palms of the hand and soles of the feet differentiate this illness from herpangia. Spontaneous resolution usually occurs within a week.
Papilloma
Human papilloma virus types 6, 14, and 22 usually infect the mucosa of the upper aerodigestive system. Although papilloma of the larynx are more common and problematic, papilloma may also occur on the soft palate, tonsillar pillars, and uvula ( Fig. 1 ). Excision is recommended to avoid risk of distal seeding into the supraglottis and glottis.
Infectious mononucleosis
This infection is caused by the Epstein-Barr virus and usually effects adolescents and young adults. It is characterized by malaise, fatigue, and generalized lymphadenopathy. The tonsils become hypertrophic and erythematous with a gray membrane ( Fig. 2 ). Airway compromise is a possible morbidity, especially in small children, and the airway must be managed immediately via endotracheal intubation. Usually supportive treatment is all that is required, with some patients requiring intravenous steroids in cases of worsening respiratory obstruction.
Bacterial Infections
Bacterial infections of the oral cavity are rare and usually occur as the result of normal oral flora that becomes pathologic when the host defense is impaired because of immunodeficiency. Approximately 80% of the oral flora is anaerobic streptococci and diphtherias.
Gingivitis
Gingivitis is caused by oral bacteria manifesting as tender swelling and bleeding of the gingivae. It is usually seen in patients with poor oral hygiene, wearing dental appliances, or that are immunosuppressed.
Acute necrotizing ulcerative gingivitis is caused by an anaerobic spirochete. Also known as Vincent infection, the gingival papillae necrotize to produce a psuedomembrane with exposure of the dental roots and loosening of the teeth. The gingivae are very painful and hemorrhagic and symptoms include high fever, halitosis, and malaise. Treatment includes local debridement of necrotic tissues, mouthwash, and penicillin.
Ludwig cellulitis
A bacterial infection precipitated by poor oral hygiene or dental extractions can result in a rapidly progressive cellulitis of the sublingual and submandibular space. This manifests rapidly with erythematous skin, pitting edema, trismus, and high fever. If inadequately treated, Ludwig cellulitis can result in airway compromise and the need for fiberoptic intubation or tracheostomy. Treatment includes surgical drainage in combination with broad-spectrum antibiotics.
Streptococcal infection
Pharyngotonsillitis is most commonly caused by respiratory viruses with symptoms including rhinosinusits, sneezing, and cough. Approximately 20% to 30% of patients with symptoms of sore throat, fever, and lymphadenopathy are attributed to bacterial infection ( Fig. 3 ). The most common bacterial cause is group A β-hemolytic Streptococcus pyogenes . Early detection and treatment is necessary given the systemic complications of streptococcal infections including scarlet fever, rheumatic fever, septic arthritis, and glomerulonephritis. The treatment of choice for streptococcal tonsillitis is penicillin-based antibiotics and rehydration.
Diphtheria
Pharyngitis caused by the pathogen Corynebacterium diptheriae is very rare in the United States as a result of widespread immunization. However, cases have been reported in immunosuppressed children and recent immigrants. Diphtheria is characterized by high fever and sore throat with hypertrophic erythematous tonsils. A thick yellow gray pseudomembrane on the pharyngeal mucosa is pathognomonic for diphtheria. Delayed treatment may result in airway obstruction and neurologic and cardiac complications. Immediate treatment with intravenous penicillin and anitioxin is necessary.
Fungal Infections
Oral candidiasis is common in milk-fed infants, especially those infants who have taken broad-spectrum antibiotics. It presents with erythematous macules on the oral mucosa with a white membranous exudates that does not bleed with debridement. Treatment includes oral nystatin or ketoconazole.
Systemic Disease
Many systemic diseases have manifestations in the oral cavity, and may be diagnosed by recognition of these characteristics. Children with significant immunodeficiency, such as AIDS and leukemia, are at an increased risk for recurrent viral and bacterial infections of the oral cavity. Angular chelitis, hairy leukoplakia, and Kaposi sarcoma are three oral manifestations or systemic diseases that affect these children.
Common childhood viral infections, such as measles and varicella (chicken pox), also have oral manifestations. Koplik spots, nontender red lesions with a pale center, on the buccal mucosa are pathognomonic for measles.
Behçet syndrome is a systemic disease characterized by ulcers of the genitalia and urethra, uveitis, iridocyclitits, and recurrent ulcers of the oral cavity. Treatment includes systemic steroids and varied mouthwashes.
Kawasaki disease is a disease of uncertain cause. It is characterized by 4 to 5 days of high fever; facial rash; and erythematous and painful lips, tongue, and oral mucosa. Other conditions resulting in tongue edema and erythema include iron deficiency anemia, vitamin B and C deficiency, and Sjögren syndrome.
Granulomatous diseases in the oral mucosa are exceedingly rare, but there can be oral manifestations in children with tuberculosis, Wegener granulomatosis, and leprosy.
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