Chapter 18: Ear, Nose, & Throat

INFECTIONS OF THE EAR

1. Acute Otitis Externa

Differential Diagnosis

Acute or chronic otitis media with eardrum rupture, furunculosis of the ear canal, herpes zoster oticus, mastoiditis, referred temporomandibular joint pain, and chronic otitis externa.

Pathogenesis

Otitis externa (OE) is a cellulitis of the soft tissues of the external auditory canal (EAC), which can extend to surrounding structures such as the pinna, tragus, and lymph nodes. Humidity, heat, and moisture in the ear are known to contribute to the development of OE, thus it is more common in the summer months and in humid climates. Cerumen serves as a hydrophobic protective barrier to the underlying skin and its acidic pH inhibits bacterial and fungal growth. Trauma to the ear canal skin can break this skin-cerumen barrier, which is the first step in developing OE. Sources of trauma can include cotton swab use, earbuds, digital manipulation (scratching), and ear plugs. Dermatologic conditions such as atopic dermatitis can also predispose one to OE. The most common organisms causing OE are Staphylococcus aureus, Staphylococcus epidermidis, and Pseudomonas aeruginosa. However, anaerobic bacteria are also seen. Fungal infection occurs in 2%–10% of patients, usually following treatment for a bacterial OE.

Clinical Findings

Symptoms include acute onset of pain, aural fullness, decreased hearing, and itching in the ear. Symptoms tend to peak within 3 days. Manipulation of the pinna or tragus causes considerable pain. Discharge may be clear or purulent and may also cause secondary eczema of the auricle. The EAC is typically swollen and narrowed, and the patient may resist any attempt to insert an otoscope. Debris is often present in the canal, and it may be difficult to visualize the TM. However, it is important to determine the status of the eardrum to rule out secondary OE caused by middle ear drainage that may need to be managed differently.

Complications

If untreated, cellulitis of neck and face may result. Immunocompromised individuals can develop malignant OE, which is a spread of the infection to the skull base with resultant osteomyelitis. This is a life-threatening condition and should be evaluated emergently if suspected.

Treatment

Management of OE includes pain control, removal of debris from the canal, topical antimicrobial therapy, and avoidance of causative factors. Cultures are not routinely sent on initial presentation as most cases will resolve with these primary interventions. Fluoroquinolone eardrops alone are first-line therapy for OE in the absence of systemic symptoms. The topical therapy chosen must be nonototoxic because a perforation or patent tube may be present; if the TM cannot be visualized, a perforation should be presumed to exist. If the ear canal is too edematous to allow entry of the eardrops, a Pope ear wick (expandable sponge) should be placed to ensure antibiotic delivery. Oral antibiotics are indicated for any signs of invasive infection, such as fever, cellulitis of the face or auricle, or tender periauricular or cervical lymphadenopathy. In such cases, in addition to ototopical therapy, cultures of the ear canal discharge should be sent, and an antistaphylococcal antibiotic prescribed while awaiting culture results. The ear should be kept dry until the infection has cleared. If drainage persists despite therapy, cultures should be sent. Patients can have difficulty applying drops to the affected ear canal, which may cause prolonged symptoms and treatment failures. If this is suspected, thoroughly explain and demonstrate the application of the topical therapy as outlined in the Clinical Practice Guideline on Acute Otitis Externa. A new formulation of ciprofloxacin suspension has recently become available; it is liquid at cooler temperatures and thickens into a gel at body temperature. This allows a single application to work for multiple days and may be useful when there are compliance problems.

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2. Acute Otitis Media

Acute otitis media (AOM) is the most common reason antibiotics are prescribed for children in the United States. It is an acute infection of the middle ear space associated with inflammation, effusion, or, if a patent tympanostomy tube or perforation is present, otorrhea (ear drainage).

Differential Diagnosis

Otitis media with effusion (OME), bullous myringitis, acute mastoiditis, and middle ear mass.

Clinical Findings

Two findings are critical in establishing a diagnosis of AOM: a bulging TM and a MEE. The presence of MEE is best determined by visual examination and either pneumatic otoscopy or tympanometry (Figure 18–1). To distinguish AOM from OME, signs and symptoms of middle ear inflammation and acute infection must be present. Otoscopic findings specific for AOM include a bulging TM, impaired visibility of ossicular landmarks, yellow or white effusion (pus), an opacified and inflamed eardrum, and occasionally squamous exudate or bullae on the eardrum.

A. Pathophysiology and Predisposing Factors

1. Eustachian tube dysfunction (ETD)

The Eustachian tube regulates middle ear pressure and allows for drainage of the middle ear. The ciliated respiratory epithelium of the Eustachian tube also defends against pathogens by producing lysozyme and mucus, which helps rid the ear of microorganisms. It must periodically open to prevent the development of negative pressure and effusion in the middle ear space. If the Eustachian tube does not work properly, negative pressure leads to transudation of cellular fluid into the middle ear, as well as influx of fluids and pathogens from the nasopharynx and adenoids. Middle ear fluid may then become infected, resulting in AOM. The Eustachian tube of infants and young children is more prone to dysfunction because it is shorter, more compliant, and more horizontal than in adults. The Eustachian tube reaches its adult configuration by the age of 7 years. Children with craniofacial differences, such as those with Trisomy 21 or cleft palate, may be particularly susceptible to ETD because of abnormal anatomy of the Eustachian tube.

2. Bacterial colonization

Nasopharyngeal colonization with S pneumoniae, Haemophilus influenzae, or Moraxella catarrhalis increases the risk of AOM, whereas colonization with normal flora such as viridans streptococci may prevent AOM by inhibiting growth of these pathogens.

3. Viral upper respiratory infections

Upper respiratory infections (URIs) impair Eustachian tube function by causing adenoid hypertrophy and edema of the Eustachian tube itself. Viral infections also inhibit the antibacterial properties of mucus and mucociliary clearance.

4. Smoke exposure

Passive smoke increases the risk of persistent MEE by enhancing colonization, prolonging the inflammatory response, and impeding drainage of the middle ear through the Eustachian tube. For infants aged 12–18 months, cigarette exposure is associated with an 11% per pack increase in the duration of MEE.

5. Impaired host immune defenses

Immunocompromised children such as those with selective IgA deficiency usually experience recurrent AOM, rhinosinusitis, and pneumonia. However, most children who experience recurrent or persistent otitis only have selective impairments of immune defenses against specific otitis pathogens.

6. Bottle feeding

Bottle feeding especially with bottle propping in the crib or carseat increases the risk of AOM because of aspiration of contaminated secretions into the middle ear space. Breastfeeding reduces the incidence of acute respiratory infections and provides immunoglobulin A (IgA) antibodies that reduce colonization with otitis pathogens.

7. Season

The incidence of AOM correlates with the activity of respiratory viruses, accounting for the annual surge in otitis media cases during the winter months in temperate climates.

8. Daycare attendance

Children exposed to large groups of children have more respiratory infections and OM. The increased number of children in day care over the past three decades has undoubtedly played a major role in the increase in AOM.

9. Genetic susceptibility

Genetics is thought to play a role in 40%–70% of ear infections. Most of the genes responsible regulate immunity. However, environmental and pathogen-related causes also play a role. The role of genetics in AOM is an area of active research.

10. Age

Children ages 1–3 years are at greatest risk for AOM.

B. Microbiology of Acute Otitis Media

Bacterial or viral pathogens can be detected in up to 96% of middle ear fluid samples from patients with AOM. Peak activity of respiratory syncytial virus, metapneumovirus, and influenza A corresponds with increased visits for AOM and 71% of middle ear aspirates from children undergoing ear tube surgery for recurrent OM contain viruses. Polybacterial infections are seen in up to 55% of cases, with bacterial and viral coinfections occurring in up to 70%. S pneumoniae and H influenzae account for 35%–40% and 30%–35% of isolates, respectively. With widespread use of the pneumococcal conjugate vaccine starting in 2000, the incidence of AOM caused by H influenzae rose while that of the S pneumoniae vaccine serotypes declined. However, there has been an increase in disease caused by S pneumoniae serotypes not covered by the vaccine, as well as S aureus. The third most common pathogen cited is M catarrhalis, which causes 15%–25% of AOM cases in the United States (Table 18–1). The fourth most common organism in AOM is Streptococcus pyogenes, which is found more frequently in school-aged children than in infants. S pyogenes and S pneumoniae are the predominant causes of mastoiditis.

Drug-resistant S pneumoniae is a common pathogen in AOM and strains may be resistant to only one drug class (eg, penicillins or macrolides) or to multiple classes. Children with resistant strains tend to be younger and to have had more unresponsive infections. History of antibiotic treatment in the preceding 3 months increases the risk of harboring resistant pathogens.

C. Examination Techniques and Procedures

1. Pneumatic otoscopy

AOM is overdiagnosed, leading to inappropriate antibiotic therapy, unnecessary surgical referrals, and significant associated costs. Contributing to errors in diagnosis is the temptation to accept the diagnosis without removing enough cerumen to adequately visualize the TM, and the mistaken belief that a red TM establishes the diagnosis. Redness of the TM is often a vascular flush caused by fever or crying.

A pneumatic otoscope with a rubber suction bulb and tube is used to assess TM mobility. When used correctly, pneumatic otoscopy can improve diagnostic ability by 15%–25%. The largest possible speculum should be used to provide an airtight seal and maximize the field of view. When the rubber bulb is gently squeezed, the TM should move freely with a snapping motion; if fluid is present in the middle ear space, the mobility of the TM will be absent or resemble a fluid wave. The ability to assess mobility is compromised by failure to achieve an adequate seal with the otoscope and poor visualization of the TM.

2. Cerumen removal

In order to adequately visualize the TM, cerumen (ear wax) removal is an essential skill for anyone who cares for children. Please refer to section on cerumen impaction below.

3. Tympanometry

Tympanometry can be helpful in assessing middle ear status, particularly when pneumatic otoscopy is inconclusive or difficult to perform. Tympanometry can reveal the presence or absence of a MEE but cannot differentiate between acutely infected fluid (AOM) and a chronic effusion (also referred to as OME).

Tympanometry measures TM compliance and displays it in graphic form. It also measures the volume of the ear canal, which can help differentiate between an intact and perforated TM.

Standard 226-Hz tympanometry is not reliable in infants younger than 6 months. A high-frequency (1000 Hz) probe is used in this age group.

Tympanograms can be classified into four major patterns, as shown in Figure 18–2. The pattern shown in Figure 18–2A, characterized by maximum compliance at normal atmospheric pressure, indicates a normal TM, good Eustachian tube function, and absence of effusion. Figure 18–2B identifies a nonmobile TM with normal volume, which indicates MEE. Figure 18–2C indicates an intact, mobile TM with excessively negative middle ear pressure (> –150 daPa), indicative of poor Eustachian tube function. Figure 18–2D shows a flat tracing with a large middle ear volume, indicative of a patent tube or TM perforation.

Treatment

A. Pain Management

Pain is the primary symptom of AOM, and the 2013 clinical practice guidelines emphasize the importance of addressing this symptom. As it may take 1–3 days before antibiotic therapy leads to a reduction in pain, ibuprofen or acetaminophen should be administered as needed to relieve discomfort. Topical analgesics have a very short duration and studies do not support efficacy in children younger than 5 years.

B. The Observation Option

The choice to observe an episode of AOM and not treat with antibiotics is an option in otherwise healthy children with mild to moderate otitis media without other underlying conditions such as cleft palate, craniofacial abnormalities, immune deficiencies, cochlear implants, or tympanostomy tubes. The decision should be made in conjunction with the parents, and a mechanism must be in place to provide antibiotic therapy if there is worsening of symptoms or lack of improvement within 48–72 hours. A Safety-Net Antibiotic Prescription (SNAP) given to parents with instructions to be filled only if symptoms do not resolve lowered overall antibiotic use in a large pediatric practice-based research network. The American Academy of Pediatrics clinical practice guidelines include age, presence of otorrhea, severity of symptoms, and laterality as criteria for antibiotic treatment versus observation (Table 18–2).

C. Antibiotic Therapy

Antibiotics have been shown to shorten the duration of AOM. Therefore, high-dose amoxicillin remains the first-line antibiotic for treating AOM, even with a high prevalence of drug-resistant S pneumoniae, because data show that isolates of the bacteria remain susceptible to the drug 83%–87% of the time.

Amoxicillin-clavulanate enhanced strength (ES), with 90 mg/kg/day of amoxicillin dosing (14:1 ratio of amoxicillin:clavulanate), is an appropriate choice when a child has had amoxicillin in the last 30 days, or is clinically failing after 48–72 hours on amoxicillin (Table 18–3) or has concomitant conjunctivitis. Purulent conjunctivitis is often caused by nontypeable H influenzae. The regular strength formulations of amoxicillin-clavulanate (7:1 ratio) should not be doubled in dosage to achieve 90 mg/kg/day of amoxicillin, because the increased amount of clavulanate will cause diarrhea.

Three oral cephalosporins (cefuroxime, cefpodoxime, and cefdinir) are more β-lactamase–stable and are alternative choices in children who develop a papular rash with amoxicillin (see Table 18–3). Of these, cefdinir suspension is most palatable; the other two have a bitter aftertaste which is difficult to conceal.

A second-line antibiotic is indicated when a child experiences symptomatic infection within 1 month of finishing amoxicillin; however, repeated use of high-dose amoxicillin is indicated if more than 4 weeks have passed without symptoms. Macrolides are not recommended as second-line agents because S pneumoniae is resistant in approximately 30% of respiratory isolates, and because virtually all strains of H influenzae have an intrinsic macrolide efflux pump, which pumps the antibiotic out of the bacterial cell. However, if there is a history of type 1 hypersensitivity reaction, macrolides may be used.

Reasons for failure to eradicate a sensitive pathogen include drug noncompliance, poor drug absorption, or vomiting of the drug. If a child remains symptomatic for longer than 3 days while taking a second-line agent, a tympanocentesis is useful to identify the causative pathogen. If a highly resistant pneumococcus is found or if tympanocentesis is not feasible, intramuscular ceftriaxone at 50 mg/kg/dose for 3 consecutive days is recommended. If a child has experienced a severe reaction, such as anaphylaxis, to amoxicillin, cephalosporins should not be substituted. Otherwise, the risk of cross-sensitivity is less than 0.1%. Multidrug-resistant S pneumoniae poses a treatment dilemma and newer antibiotics, such as fluoroquinolones or linezolid, may need to be employed. However, these drugs are not approved by the U.S. Food and Drug Administration (FDA) for the treatment of AOM in children.

In patients with tympanostomy tubes with uncomplicated acute otorrhea, ototopical antibiotics (fluoroquinolone eardrops) are first-line therapy. The eardrops serve two purposes: (1) They treat the infection and (2) they physically “rinse” drainage from the tube which helps prevent plugging of the tube. Oral antibiotics are not indicated in the absence of systemic symptoms.

If a TM perforation has occurred with otorrhea then topical antibiotics are recommended as first-line agents due to the ability to provide high concentrations of antibiotic directly to the middle ear. Topical fluoroquinolone otic drops (ofloxacin and ciprofloxacin) with or without steroids are considered safe for administration into the middle ear. If there is a large amount of debris or drainage in the ear canal, the canal may need to be suctioned first to allow drops to gain access to the middle ear.

D. Tympanocentesis

Tympanocentesis is performed by placing a needle through the TM and aspirating the middle ear fluid. The fluid is sent for culture and sensitivity. Indications for tympanocentesis are (1) AOM in an immunocompromised patient, (2) research studies, (3) evaluation for presumed sepsis or meningitis, such as in a neonate, (4) unresponsive otitis media despite courses of two appropriate antibiotics, and (5) acute mastoiditis or other suppurative complications.

E. Prevention of Acute Otitis Media

1. Antibiotic prophylaxis

Strongly discouraged due to poor efficacy and concern for antibiotic resistance.

2. Possible lifestyle modifications

Parental education plays a major role in decreasing AOM.

• Smoking is a risk factor both for URI and AOM. Primary care physicians should provide information on smoking cessation programs and measures.

• Breastfeeding protects children from AOM. Clinicians should encourage exclusive breastfeeding for 6 months.

• Bottle-propping in the crib should be avoided. It increases AOM risk due to the reflux of milk into the Eustachian tubes.

• Pacifiers are controversial. There may be a protective effect of pacifiers against SIDS but they may increase risk of AOM. Currently, the recommendation from the American Academy of Family Physicians is to wean pacifiers after 6 months of age to reduce the risk of AOM.

• Day care is a risk factor for AOM, but working parents may have few alternatives. Possible alternatives include care by relatives or child care in a setting with fewer children.

3. Surgery

Tympanostomy tubes are effective in the treatment of recurrent AOM as well as OME.

4. Immunologic evaluation and allergy testing

While immunoglobulin subclass deficiencies may be more common in children with recurrent AOM, there is no practical immune therapy available. More serious immunodeficiencies, such as selective IgA deficiency, should be considered in children who suffer from a combination of recurrent AOM, rhinosinusitis, and pneumonia. In the school-aged child or preschooler with an atopic background, skin testing may be beneficial in identifying allergens that can predispose to AOM.

5. Vaccines

The pneumococcal conjugate and influenza vaccines are recommended. The seven-valent pneumococcal conjugate vaccine (PCV7) was introduced in the United States in 2000, and the 13-valent pneumococcal conjugate vaccine (PCV13) in 2010. The transition from PCV7 to PCV13 has resulted in a decline of otitis media among children younger than 2 years due to decreased risk of both the first and subsequent episodes of otitis media.

3. Otitis Media With Effusion

Clinical Findings

OME is the presence of fluid in the middle ear space without signs or symptoms of acute inflammation. This is common, with 90% of children having one episode of OME by age 5. On examination, the TM may be opacified and thickened and the middle ear fluid can be clear, amber-colored, or opaque. Pneumatic otoscopy can confirm the presence of a MEE. If the pneumatic otoscopy examination is uncertain, then tympanometry should be performed to confirm presence of middle ear fluid.

Children with OME can develop AOM if the middle ear fluid should become infected. After AOM, fluid can remain in the ear for several weeks, with 60%–70% of children still having MEE 2 weeks after successful treatment. This drops to 40% at 1 month and 10%–25% at 3 months after treatment. It is important to distinguish OME from AOM because the former does not benefit from treatment with antibiotics.

Management

An audiology evaluation should be performed after approximately 3 months of continuous bilateral effusion in most children. However, children who are at risk of language delay due to socioeconomic circumstances, craniofacial anomalies, or other risk factors should undergo a hearing evaluation at the time that OME is diagnosed. Children with hearing loss or speech delay should be referred to an otolaryngologist for possible tympanostomy tube placement. Antibiotics, antihistamines, and steroids have not been shown to be useful in the treatment of OME.

In uncomplicated cases, OME is observed for 3 months prior to consideration for tympanostomy tube placement. Longer periods of observation may be acceptable in children with normal or very mild hearing loss on audiogram, no risk factors for speech and language issues, and no structural changes to the TM. These children should be followed every 3–6 months until the effusions clear or problems develop. Indications for tympanostomy tubes include hearing loss greater than 40 dB, TM retraction pockets, ossicular erosion, adhesive atelectasis, and cholesteatoma. In children older than age 4, adenoidectomy may be recommended with ear tubes as studies show this can reduce failure rate and need for additional surgeries.

Prognosis & Sequelae

Prognosis is variable based on age of presentation. Infants who are very young at the time of first otitis media are more likely to need surgical intervention. Other factors that decrease the likelihood of resolution are onset of OME in the summer or fall, history of prior tympanostomy tubes, presence of adenoids, and hearing loss greater than 30 dB.

4. Complications of Otitis Media

A. Changes of the Tympanic Membrane

Tympanosclerosis is an acquired disorder of calcification and scarring of the TM and middle ear structures secondary to inflammation. If tympanosclerosis involves the ossicles, a conductive hearing loss may result. The term myringosclerosis applies to calcification of the TM only and is a fairly common sequela of OME and AOM. Myringosclerosis may also develop at the site of a previous tympanostomy tube; tympanosclerosis is not a common sequela of tube placement. Myringosclerosis rarely causes hearing loss, unless the entire TM is involved (“porcelain eardrum”).

The appearance of a small defect or invagination of the pars tensa or pars flaccida of the TM suggests a retraction pocket. Retraction pockets occur when chronic inflammation and negative pressure in the middle ear space produce atrophy and atelectasis of the TM.

Continued inflammation can cause adhesions to form between the retracted TM and the ossicles. This condition, referred to as adhesive otitis, predisposes one to formation of a cholesteatoma or fixation and erosion of the ossicles.

B. Cholesteatoma

A greasy-looking or pearly white mass seen in a retraction pocket or perforation behind the eardrum suggests a cholesteatoma (Figure 18–3). If infection is superimposed, serous or purulent drainage will be seen, and the middle ear cavity may contain granulation tissue or even polyps. Persistent, recurrent, or foul-smelling otorrhea following appropriate medical management should make one suspect a cholesteatoma and prompt an ENT (Ear-Nose-Throat/Otolaryngology) referral.

C. Tympanic Membrane Perforation

Occasionally, an episode of AOM may result in rupture of the TM. Discharge from the ear is seen, and often there is rapid relief of pain. Perforations due to AOM usually heal spontaneously within a couple of weeks. Ototopical antibiotics are recommended for a 10- to 14-day course and patients should be referred to an otolaryngologist 2–3 weeks after the rupture for examination and hearing evaluation.

When perforations fail to heal, surgical repair may be needed. TM repair is generally delayed until the child is older and Eustachian tube function has improved. Repair of the eardrum (tympanoplasty) is generally deferred until around 7 years of age, which is approximately when the Eustachian tube reaches adult orientation.

In the presence of a perforation, water activities should be limited to surface swimming, preferably with the use of an ear plug.

D. Facial Nerve Paralysis

The facial nerve traverses the middle ear as it courses through the temporal bone to its exit at the stylomastoid foramen. Normally, the facial nerve is completely encased in bone, but occasionally bony dehiscence in the middle ear is present, exposing the nerve to infection and making it susceptible to inflammation during an episode of AOM. The acute onset of a facial nerve paralysis should not be deemed idiopathic Bell palsy until all other causes have been excluded. If middle ear fluid is present, prompt myringotomy and tube placement are indicated. CT is indicated if a cholesteatoma or mastoiditis is suspected.

E. Chronic Suppurative Otitis Media

Chronic suppurative otitis media (CSOM) is present when persistent otorrhea occurs in a child with tympanostomy tubes or TM perforation for greater than 6–12 weeks. It starts with an acute infection that becomes chronic with mucosal edema, ulceration, granulation tissue, and eventual polyp formation. Risk factors include a history of, multiple episodes of otitis media, living in crowded conditions, day care attendance, and being a member of a large family. The most common associated bacteria include P aeruginosa, S aureus, Proteus species, Klebsiella pneumoniae, and diphtheroids.

Visualization of the TM, meticulous cleaning with culture of the drainage, and appropriate antimicrobial therapy, usually topical, are the keys to management.

Occasionally, CSOM may be a sign of cholesteatoma or other disease process such as foreign body, neoplasm, Langerhans cell histiocytosis, tuberculosis, granulomatosis, fungal infection, or petrositis. If CSOM is not responsive to culture-directed treatment, imaging and biopsy may be needed to rule out other possibilities. Patients with facial palsy, vertigo, or other CNS signs should be referred immediately to an otolaryngologist.

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F. Labyrinthitis

Suppurative infections of the middle ear can spread into the membranous labyrinth of the inner ear. Symptoms include vertigo, hearing loss, and fevers. The child often appears extremely toxic. Intravenous antibiotic therapy is used, and intravenous steroids may also be used to help decrease inflammation. Sequelae can be serious, including a condition known as labyrinthitis ossificans, or bony obliteration of the inner ear, including the cochlea, leading to profound hearing loss.

G. Mastoiditis

Pathogenesis

Mastoiditis occurs when infection spreads from the middle ear space to the mastoid portion of the temporal bone, which lies just behind the ear and contains air-filled spaces. Mastoiditis can range in severity from inflammation of the mastoid periosteum to bony destruction of the mastoid air cells (coalescent mastoiditis) with abscess development. Mastoiditis can occur in any age group, but more than 60% of the patients are younger than 2 years. Many children do not have a prior history of recurrent AOM.

Clinical Findings

A. Symptoms and Signs

Patients with mastoiditis usually have postauricular pain, fever, and an outwardly displaced pinna. On examination, the mastoid area often appears indurated and red and with disease progression, it may become swollen and fluctuant. The earliest finding is severe tenderness on mastoid palpation. AOM is almost always present. Late findings include a pinna that is pushed forward by postauricular swelling and an ear canal that is narrowed due to pressure on the posterosuperior wall from the mastoid abscess. In infants younger than 1 year, the swelling occurs superior to the ear and pushes the pinna downward rather than outward.

B. Imaging Studies

The best way to determine the extent of disease is by CT scan. Early mastoiditis is radiographically indistinguishable from AOM, with both showing opacification but no destruction of the mastoid air cells. With progression of mastoiditis, coalescence of the mastoid air cells is seen with bone destruction. Mastoiditis is a clinical diagnosis, based on pain as well as physical exam findings.

C. Microbiology

The most common pathogens are S pneumoniae followed by H influenzae and S pyogenes. Rarely, gram-negative bacilli and anaerobes are isolated. In the preantibiotic era, up to 20% of patients with AOM developed mastoiditis requiring mastoidectomy. Antibiotics decrease the incidence and morbidity of acute mastoiditis. However, acute mastoiditis still occurs in children who are treated with antibiotics for an acute ear infection. In the Netherlands, where only 31% of AOM patients receive antibiotics, the incidence of acute mastoiditis is 4.2 per 100,000 person-years. In the United States, where more than 96% of patients with AOM receive antibiotics, the incidence of acute mastoiditis is 2 per 100,000 person-years. Despite the routine use of antibiotics, the incidence of acute mastoiditis has been rising in some cities. The pattern change may be secondary to the emergence of resistant S pneumoniae.

Differential Diagnosis

Lymphadenitis, parotitis, trauma, tumor, histiocytosis, OE, and furuncle.

Complications

Meningitis can be a complication of acute mastoiditis and should be suspected when a child has associated high fever, stiff neck, severe headache, or other meningeal signs. Lumbar puncture should be performed for diagnosis after imaging. Brain abscess occurs in 2% of mastoiditis patients and may be associated with persistent headaches, recurring fever, or changes in sensorium. Facial palsy, sigmoid sinus thrombosis, epidural abscess, cavernous sinus thrombosis, and thrombophlebitis may also be encountered.

Treatment

Intravenous antibiotic treatment alone may be successful if there is no evidence of coalescence or abscess on CT. However, if there is no improvement within 24–48 hours, surgical intervention should be undertaken. Minimal surgical management starts with tympanostomy tube insertion, during which cultures are taken. If a subperiosteal abscess is present, incision and drainage is also performed, with or without a cortical mastoidectomy. Intracranial extension requires complete mastoidectomy with decompression of the involved area.

Antibiotic therapy (intravenous and topical ear drops) is instituted along with surgical management and relies on culture-directed antibiotic therapy for 2–3 weeks. An antibiotic regimen should be chosen which is able to cross the blood-brain barrier. After significant clinical improvement is achieved with parenteral therapy, oral antibiotics are begun and should be continued for 2–3 weeks. A patent tympanostomy tube must also be maintained with continued use of otic drops until drainage abates.

Prognosis

Prognosis for full recovery is good. Children that develop acute mastoiditis with abscess as their first ear infection are not necessarily prone to recurrent otitis media.

ACUTE TRAUMA TO THE MIDDLE EAR

Head injuries, a blow to the ear canal, sudden impact with water, blast injuries, or the insertion of pointed objects into the ear canal can lead to perforation of the TM, ossicular chain disruption, facial nerve injury, hearing loss, vertigo, and hematoma of the middle ear. One study reports that 50% of serious penetrating wounds of the TM are due to parental use of a cotton swab.

If there is facial paralysis, severe vertigo or subjective hearing loss after ear trauma, urgent otolaryngology consultation is warranted. Middle ear trauma can lead to a perilymphatic fistula which is a breach of the inner ear that causes sensorineural (nerve) hearing loss and vertigo. This hearing loss can be prevented or reversed with emergent surgery. Facial nerve injury in the setting of middle ear trauma also often needs to be addressed emergently with possible facial nerve decompression. Other sequelae of middle ear trauma may be treated with observation. Blood collecting in the middle ear space may cause a conductive hearing loss that will resolve with time. Antibiotics are not necessary unless signs of infection appear. The patient needs to be followed with audiometry or by an otolaryngologist until hearing has returned to normal, which is expected within 6–8 weeks. If the conductive hearing loss does not resolve, there may be injuries to the ossicular chain. A CT scan may be needed to evaluate the middle ear structures in this case.

Traumatic TM perforations should be referred to an otolaryngologist for examination and hearing evaluation. Spontaneous healing may occur within 6 months of the perforation. In the acute setting, antibiotic eardrops are often recommended to provide a moist environment which is thought to speed healing.

EAR CANAL FOREIGN BODY & CERUMEN IMPACTION

Foreign bodies of the ear canal, both intentional (placement by patient or other person) or accidental (eg, insect, playground mulch), are common in childhood. Cerumen can also be obstructive, acting like a foreign body. These objects can be removed if they are easy to visualize and there is appropriate instrumentation. Factors that may make them difficult to remove include size of foreign body, particularly when large enough to obscure the TM, rounded or globular objects, and objects deep in the ear canal adjacent to the TM. If these conditions exist or you are unable to remove the object on the first attempt, an ENT referral is often necessary for resolution. Vegetable matter should never be irrigated as it can swell and become more difficult to remove. An emergency condition exists if the foreign body is a disk-type battery. An electric current is generated in the moist canal, and a severe burn can occur in less than 4 hours. If the TM cannot be visualized, assume a perforation and avoid irrigation or ototoxic medications.

Cerumen impaction is common in children and using cotton swabs or other devices in the ear canal predisposes to the problem. Not only do these objects block the natural outflow of cerumen, they can also increase cerumen production from irritation. Cerumen impaction should be removed if it is symptomatic or obstructs visualization of the TM. Education on the proper techniques of ear cleaning (such as only cleaning the external meatus) is important to avoid impactions. Explaining to parents that cerumen production is normal and cerumen protects the ear canal skin can help reinforce proper ear care.

AURICULAR HEMATOMA

Trauma to the outer ear can result in formation of a hematoma between the perichondrium and cartilage of the pinna. This is different from a bruise, which does not change the ear shape and where the blood is in the soft tissue outside of the perichondral layer. A hematoma appears as a boggy purple swelling of the cartilaginous auricle, and the normal folds of the ear are obscured. If untreated, neocartilage is deposited after 7–10 days resulting in “cauliflower ear.” To prevent this cosmetic deformity, physicians should urgently refer patients to an otolaryngologist for drainage and application of a pressure dressing.

CONGENITAL EAR MALFORMATIONS

The external ear and EAC start to develop at 3 weeks gestation. Variable anomalies can present depending on timing of abnormal development. Atresia is failure of the ear canal to form. This results in conductive hearing loss and should be evaluated within the first 3 months of life by an audiologist and otolaryngologist. Microtia is the term used for an external ear that is small, collapsed, or only has an earlobe present. Usually there is a deficiency of cartilage and tissue. Anotia refers to an absent external ear. Often, there is an associated atresia with microtia and anotia. Reconstruction of the auricle usually occurs around ages 6–8 years and requires introduction of additional tissue or implants.

Malformations of the auricle can occur that are not due to a deficiency of tissue. This can range from ears that lack the proper folds and therefore protrude from the skull (prominotia) to ears that are folded over due to lack of cartilage stiffness or in utero positioning. Taping of the ears into correct anatomic position is very effective if performed in the first 72–96 hours of life. Tape is applied over a molding of wax or plastic and continued for at least 2 weeks. If this window of opportunity is missed or taping is unsuccessful, surgical correction, called an “otoplasty,” can be performed at school age.

An ear is considered “low-set” if the upper pole is below eyebrow level. This condition is often associated with other congenital anomalies, and in these patients a genetics evaluation should be considered.

Preauricular tags, ectopic cartilage, fistulas, and cysts require surgical correction primarily for cosmetic reasons. Since the inner ear forms in conjunction with the outer ear children with ear malformations should have their hearing tested. Renal ultrasound should be considered, as external ear anomalies can also be associated with renal anomalies, as both structures form during the same period of embryogenesis. Most preauricular pits are asymptomatic but may become infected and require antibiotic treatment and possible surgical management.

IDENTIFICATION & MANAGEMENT OF HEARING LOSS

Hearing loss is classified as being conductive, sensorineural, or mixed in nature. Conductive hearing loss occurs when sound transmission is blocked somewhere between the opening of the external ear and the cochlear receptor cells. The most common cause of conductive hearing loss in children is fluid in the middle ear. Sensorineural hearing loss (SNHL) is due to a defect in the neural transmission of sound, arising from a defect in the cochlear hair cells or the auditory nerve. Mixed hearing loss is characterized by elements of both conductive and sensorineural loss.

Hearing is measured in decibels (dB). The threshold, or 0 dB, refers to the level at which a sound is perceived in normal subjects 50% of the time. Hearing is considered normal if an individual’s thresholds are within 20 dB of normal. In children, severity of hearing loss is commonly graded as follows: 20–40 dB mild, 41–55 dB moderate, 56–70 dB moderately severe, 71–90 dB severe, and 91+ dB profound.

Hearing loss can significantly impair a child’s ability to communicate and hinder academic, social, and emotional development. Studies suggest that periods of auditory deprivation may have enduring effects on auditory processing, even after normal hearing is restored. Even a unilateral loss may be associated with difficulties in school and behavioral issues. Early identification and management of any hearing loss is therefore critical.

Conductive Hearing Loss

The most common cause of childhood conductive hearing loss is otitis media and related conditions such as MEE and ETD. Other causes may include EAC atresia or stenosis, TM perforation, cerumen impaction, cholesteatoma, and middle ear abnormalities, such as ossicular fixation or discontinuity. Often, a conductive loss may be corrected with surgery.

MEE may be serous, mucoid, or purulent, as in AOM. Effusions are generally associated with a mild conductive hearing loss that normalizes once the effusion is gone. The American Academy of Pediatrics recommends that hearing and language skills be assessed in children who have recurrent AOM or MEE lasting longer than 3 months.

Sensorineural Hearing Loss

SNHL arises due to a defect in the cochlear receptor cells or the auditory nerve (cranial nerve VIII). The loss may be congenital (present at birth) or acquired. In both the congenital and acquired categories, the hearing loss may be either hereditary (due to a genetic mutation) or nonhereditary. It is estimated that SNHL affects 2–3 out of every 1000 live births, making this the most common congenital sensory impairment. The incidence is thought to be considerably higher among the neonatal intensive care unit population. Well-recognized risk factors for SNHL in neonates include positive family history of childhood SNHL, birthweight less than 1500 g, low Apgar scores (0–4 at 1 minute or 0–6 at 5 minutes), craniofacial anomalies, hypoxia, in-utero infections (eg, TORCH syndrome), hyperbilirubinemia requiring exchange transfusion, and mechanical ventilation for more than 5 days.

A. Congenital Hearing Loss

Approximately 50% of congenital hearing loss is nonhereditary. Examples include loss due to infection, teratogenic drugs, and perinatal injuries. The other 50% is attributed to genetic factors. Among children with hereditary hearing loss, approximately one-third of cases are thought to be due to a known syndrome, while the other two-thirds are considered nonsyndromic.

Syndromic hearing loss is associated with malformations of the external ear or other organs, or with medical problems involving other organ systems. Over 400 genetic syndromes that include hearing loss have been described. All patients being evaluated for hearing loss should be also evaluated for features commonly associated with these syndromes. These include branchial cleft cysts or sinuses, preauricular pits, ocular abnormalities, white forelock, café au lait spots, and craniofacial anomalies. Some of the more frequently mentioned syndromes associated with congenital hearing loss include the following: Waardenburg, branchio-oto-renal, Usher, Pendred, Jervell and Lange-Nielsen, and Alport.

Over 70% of hereditary hearing loss is not related to a syndrome (ie, there are no associated visible abnormalities or related medical problems). The most common known mutation associated with nonsyndromic hearing loss is in the GJB2 gene, which encodes the protein Connexin 26. The GJB2 mutation has a carrier rate of about 3% in the general population. Most nonsyndromic hearing loss, including that due to the GJB2 mutation, is autosomal recessive.

B. Acquired Hearing Loss

Hereditary hearing loss may be delayed in onset, as in Alport syndrome and most types of autosomal dominant nonsyndromic hearing loss. Vulnerability to aminoglycoside-induced hearing loss has also been linked to a mitochondrial gene defect.

Nongenetic etiologies for delayed-onset SNHL include exposure to ototoxic medications, meningitis, autoimmune or neoplastic conditions, noise exposure, and trauma. Infections such as syphilis or Lyme disease have been associated with hearing loss. Hearing loss associated with congenital cytomegalovirus (CMV) infection may be present at birth, or may have a delayed onset. The loss is progressive in approximately half of all patients with congenital CMV-associated hearing loss. Other risk factors for delayed-onset, progressive loss include a history of persistent pulmonary hypertension and extracorporeal membrane oxygenation therapy.

C. Congenital CMV Infection and Hearing Loss

Congenital CMV (cCMV) associated hearing loss can be congenital, but is more often acquired. It deserves special mention as it is believed to be the most common cause of nongenetic hearing loss in the US pediatric population, and it can present at birth or have a delayed onset as long as several years. Congenital CMV is the most common in utero infection in the United States today, and an estimated 0.5%–1% of all newborns are infected in the prenatal period. While some manifest severe symptoms at birth such as petechiae, hyperbilirubinemia, hepatosplenomegaly, seizures, neurologic deficits and retinitis, 90%–95% of newborns with cCMV infection are completely asymptomatic. Thirty to fifty percent of symptomatic and 8%–12% of asymptomatic infants will go on to develop SNHL in early childhood. Congenital CMV infection can only be confirmed in the newborn period, so given that most newborns are asymptomatic during this period, it has been very difficult to accurately determine the percentage of SNHL attributable to cCMV. There is currently no universal screening for cCMV, but with the increased awareness of this problem, some centers are now performing targeted screening on newborns who fail newborn hearing screening. There is no consensus on the management of cCMV but trials of valganciclovir have shown promise in the treatment of cCMV hearing loss.

Identification of Hearing Loss

A. Newborn Hearing Screening

Prior to the institution of universal newborn screening programs, the average age at identification of hearing loss was 30 months. Recognizing the importance of early detection, in 1993, a National Institutes of Health Consensus Panel recommended that all newborns be screened for hearing impairment prior to hospital discharge. Today, all 50 states and the District of Columbia have Early Hearing Detection and Intervention (EHDI) laws or screening programs, and as of 2014, 97% of newborns in the United States were screened for hearing loss. The EHDI goal is loss identification and confirmation of hearing loss by 3 months of age, and appropriate intervention by the age of 6 months. Subjective testing is not reliable in infants, and therefore objective, physiologic methods are used for screening. Auditory brainstem response and otoacoustic emission testing are the two commonly employed screening modalities.

B. Audiologic Evaluation of Infants and Children

A parent’s report of his or her infant’s behavior cannot be relied upon for identification of hearing loss. A deaf infant’s behavior can appear normal and mislead parents and professionals. Deaf infants are often visually alert and able to actively scan the environment which may be mistaken for an appropriate response to sound. In children, signs of hearing loss include inconsistent response to sounds, not following directions, speech and language delays, and turning the volume up on televisions or radios. Any child who fails a hearing screening or is suspected to have hearing loss should be referred for formal audiometric testing by an audiologist who is knowledgeable about testing the pediatric population.

Audiometry subjectively evaluates hearing. There are several different methods used, based on patient age:

• Behavioral observational audiometry (birth to 6 months): Sounds are presented at various intensity levels, and the audiologist watches closely for a reaction, such as change in respiratory rate, starting or stopping of activity, startle, head turn, or muscle tensing. This method is highly tester-dependent and error-prone.

• Visual reinforcement audiometry (6 months to 2.5 years): Auditory stimulus is paired with positive reinforcement. For example, when a child reacts appropriately by turning toward a sound source, the behavior is rewarded by activation of a toy that lights up. After a brief conditioning period, the child localizes toward the tone, if audible, in anticipation of the lighted toy.

• Conditioned play audiometry (2.5–5 years): The child responds to sound stimulus by performing an activity, such as putting a peg into a board.

• Conventional audiometry (5 years and up): The child indicates when he or she hears a sound.

Objective methods such as auditory brainstem response and otoacoustic emission testing may be used if a child cannot be reliably tested using the above methods.

In addition to infants who fall into the high-risk categories for SNHL as outlined earlier, hearing should be tested in children with a history of developmental delay, bacterial meningitis, ototoxic medication exposure, neurodegenerative disorders, or a history of infection such as mumps or measles. Children with bacterial meningitis should be referred immediately to an otolaryngologist, as cochlear ossification can necessitate urgent cochlear implantation. Even if a newborn screening was passed, all infants who fall into a high-risk category for progressive or delayed-onset hearing loss, such as in utero CMV exposure, should be referred for regular audiologic monitoring for the first 3 years and at appropriate intervals thereafter to avoid a missed diagnosis.

Management of Hearing Loss

While identification of hearing loss is the critical first step, it is largely meaningless without appropriate follow-up and management. Prompt and appropriate intervention can minimize the potential lifelong detrimental effects that hearing loss can have on language, academic, emotional and social development, and hearing-related quality of life. Optimal management requires a multidisciplinary approach. The team may include audiologists, otolaryngologists, speech-language pathologists, early intervention specialists, deaf-hard of hearing educational specialists and family counselors.

Any child with confirmed hearing loss should be referred to an otolaryngologist for further evaluation and possible medical and/or surgical management. Etiologic workup can include radiographic imaging and/or laboratory tests and should be tailored to each individual patient’s history, examination, and audiometric results. Within the past decade, major advances have made comprehensive genetic testing for syndromic and nonsyndromic hearing loss using next-generation sequencing technology available to the public.

The medical management of hearing loss depends upon the type and severity. Conductive hearing loss is sometimes fixable if the point at which sound transmission is compromised can be corrected. For example, hearing loss due to chronic effusions usually normalizes once the fluid has cleared, whether by natural means or by the placement of tympanostomy tubes. As of yet, SNHL is not reversible. Most sensorineural loss is managed with amplification. Cochlear implantation is an option for some children when benefit is no longer achieved with amplification. It is FDA approved down to the age of 12 months. Unlike hearing aids, cochlear implants do not amplify sound, but directly stimulate the cochlea with electrical impulses.

When a hearing loss is diagnosed, it is not known whether it will remain stable, or if it will fluctuate or worsen. Therefore, children with hearing loss should receive ongoing audiologic monitoring, as well as periodic assessments of global development and functional performance.

The Individuals with Disabilities Education Act 2004 (IDEA 2004) is a United States law mandating free access to individualized educational opportunities with qualified instructors for children with disabilities, including hearing loss. Part C provides early intervention for children up to age 3 years. Audiologists play an important role in the transition from diagnosis to intervention, as they are required to initiate a referral to their state’s Part C program within 7 days of a new confirmed permanent hearing loss diagnosis. An Individualized Family Service Plan (IFSP) is developed and may enlist the services of audiologists, speech-language pathologists, and other related professionals. The IFSP is family-centered, and family support services are often available. Between the ages of 3 and 21 years, children are covered under Part B of IDEA 2004. Part B is managed through the local school systems’ special education programs. Under Part B, an Individualized Education Plan (IEP) is developed with a goal of maximizing a child’s academic success.

Prevention

Appropriate care may treat or prevent certain conditions causing hearing deficits. Aminoglycosides and diuretics, particularly in combination, are potentially ototoxic and should be used judiciously and monitored carefully. Given the association of a mitochondrial gene defect with aminoglycoside ototoxicity, use should be avoided when possible, in patients with a family history of aminoglycoside-related hearing loss. Reduction of repeated exposure to loud noises may prevent high-frequency hearing loss associated with acoustic trauma. Any patient with sudden-onset SNHL should be seen by an otolaryngologist immediately, as in some cases, steroid therapy may reverse the loss if initiated right away.

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ACUTE VIRAL RHINITIS

The common cold (viral URI) is the most common pediatric infectious disease, and the incidence is higher in early childhood than in any other period of life (Common Cold; See also Chapter 40). Children younger than 5 years typically have 6–12 colds per year. Approximately 30%–40% are caused by rhinoviruses, of which there are over 100 subtypes. Other culprits include adenoviruses, coronaviruses, enteroviruses, influenza and parainfluenza viruses, and respiratory syncytial virus. Since so many viruses can cause a cold, development of a vaccine has not been possible.

Differential Diagnosis

Rhinosinusitis (acute or chronic), allergic rhinitis, nonallergic rhinitis, influenza, pneumonia, gastroesophageal reflux disease, asthma, and bronchitis.

Clinical Findings

The patient usually experiences a sudden onset of sore throat followed by clear or mucoid rhinorrhea, nasal congestion, and sneezing. Cough or fever may develop. Although fever is not a prominent feature in older children and adults, in the first 5 or 6 years of life it can be as high as 40.6°C without superinfection. The nose, throat, and TMs may appear red and inflamed. The average duration of symptoms is about 1 week. Nasal secretions tend to become thicker and more purulent after day 2 of infection due to shedding of epithelial cells and influx of neutrophils. This discoloration should not be assumed to be a sign of bacterial rhinosinusitis, unless it persists beyond 10–14 days, by which time the patient should be experiencing significant symptomatic improvement. A mild cough may persist for several weeks following resolution of other symptoms.

Treatment

Treatment for the common cold is symptomatic (Figure 18–4). Because colds are viral infections, antibiotics are not helpful. Acetaminophen or ibuprofen can relieve fever and pain. Humidification may provide relief for congestion and cough. Nasal saline drops and bulb suctioning may be used for an infant or child unable to blow his or her nose.

Available scientific data suggest that over-the-counter cold and cough medications are not effective in children and may be associated with serious adverse effects. Use in children younger than 4 years is not recommended. Antihistamines have not proven effective in relieving cold symptoms in children; in rhinoviral colds, increased levels of histamine are not observed. Oral decongestants may provide symptomatic relief in adults but have not been well studied in children. Studies have shown that most cough medicines are no better than placebo and use of narcotic antitussives is discouraged, as these may be associated with respiratory depression. There is no convincing evidence that naturopathic or alternative therapies are effective in children.

Only time cures the common cold. Education and reassurance may be the most important “therapy” for parents. They should be informed about the expected nature and duration of symptoms, efficacy and potential side effects of medications, and the signs and symptoms of complications of the common cold, such as bacterial rhinosinusitis, bronchiolitis, or pneumonia.

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RHINOSINUSITIS

The use of the term rhinosinusitis has replaced sinusitis. Rhinosinusitis acknowledges that the nasal and sinus mucosa are involved in similar and concurrent inflammatory processes.

1. Acute Bacterial Rhinosinusitis

Acute bacterial rhinosinusitis (ABRS) is a bacterial infection of the paranasal sinuses which lasts less than 30 days and the symptoms resolve completely. It is almost always preceded by a cold. Other predisposing conditions include allergies and trauma. The diagnosis of ABRS is made when a cold does not improve by 10–14 days or worsens after 5–7 days. The maxillary and ethmoid sinuses are most commonly involved and these sinuses are present at birth. Other sinuses are involved in older children as the sphenoid sinuses typically form by the age of 5 years, and the frontal sinuses by age 7–8 years. Frontal sinusitis is unusual before age 10 years.

Pathogenesis

Situations which lead to inflammation of sinonasal mucosa and obstruction of sinus drainage underlie the development of rhinosinusitis. A combination of anatomic, mucosal, microbial, and immune factors are involved. Both viral and bacterial infections play integral roles in the pathogenesis. Viral URIs may cause sinus mucosal injury and swelling, resulting in sinus outflow obstruction, loss of ciliary activity, and mucous hypersecretion. The bacterial pathogens that commonly cause acute rhinosinusitis are S pneumoniae, H influenzae (nontypeable), M catarrhalis, and β-hemolytic streptococci.

Clinical Findings

The onset of symptoms in ABRS may be gradual or sudden and commonly include nasal drainage, nasal congestion, facial pressure or pain, postnasal drainage, hyposmia or anosmia, fever, cough, fatigue, maxillary dental pain, and ear pressure or fullness. The physical examination is rarely helpful in making the diagnosis, as the findings are essentially the same as those in a child with an uncomplicated cold.

In complicated or immunocompromised patients, sinus aspiration and culture by an otolaryngologist should be considered for diagnostic purposes and to facilitate culture-directed antibiotic therapy. Gram stain or culture of nasal discharge does not necessarily correlate with cultures of sinus aspirates. If the patient is hospitalized because of rhinosinusitis-related complications, blood cultures should also be obtained.

Imaging of the sinuses during acute illness is not recommended unless evaluating for possible complications, or for patients with persistent symptoms which do not respond to medical therapy. As with the physical examination, the radiographic findings of ABRS, such as sinus opacification, fluid, and mucosal thickening, are indistinguishable from those seen in the common cold.

Complications

Complications of ABRS occur when infection spreads to adjacent structures like the eye and the brain. S aureus (including methicillin-resistant S aureus) is frequently implicated in complicated ABRS, as well as Streptococcus anginosus (milleri), which has been found to be a particularly virulent organism.

Orbital complications are the most common, arising from the ethmoid sinuses. These complications usually begin as a preseptal cellulitis, but can progress to postseptal cellulitis, subperiosteal abscess, orbital abscess, and cavernous sinus thrombosis. Associated signs and symptoms include eyelid edema, restricted extraocular movements, proptosis, chemosis, and altered visual acuity (see Chapter 16).

The most common complication of frontal sinusitis is osteitis of the frontal bone, also known as Pott’s puffy tumor. Intracranial extension of infection can lead to meningitis and to epidural, subdural, and brain abscesses. Frequently, children with complicated rhinosinusitis have no prior history of sinus infection.

Treatment

For children with cold symptoms that are not improving by 10 days, observation for up to 3 more days or antibiotic therapy may be chosen, depending upon individual circumstances, such as ability to follow-up and treat with antibiotics if needed. For children with uncomplicated ABRS who have worsening or severe symptoms (fever of at least 39°C and purulent nasal drainage for at least 3 consecutive days), antibiotic therapy is recommended. Antibiotics are generally thought to decrease duration and severity of symptoms.

First-line antibiotic therapy should be amoxicillin or amoxicillin-clavulanate. Cefuroxime, cefpodoxime, and cefdinir are recommended for patients with a non–type I hypersensitivity to penicillin, and in most cases may be safely used in patients with an anaphylactoid reaction, as recent studies seem to indicate almost no risk of a serious reaction to second- and third-generation cephalosporins among these patients. Other agents which may be used, particularly in more severe cases where resistant S pneumoniae and H influenzae are suspected, include clindamycin, linezolid, and quinolone antibiotics. Due to high resistance of S pneumoniae and H influenzae, the use of trimethoprim-sulfamethoxazole and azithromycin is not advised.

Duration of therapy should be for 7 days after symptoms have resolved.

Failure to improve after 48–72 hours of antibiotic therapy suggests a resistant organism or potential complication. Second-line therapies should be initiated at this point, or, if the patient is already on amoxicillin-clavulanate or a cephalosporin, intravenous antibiotic therapy should be considered. Imaging and referral for sinus aspiration should be strongly considered as well.

Patients who are toxic, or who have evidence of invasive infection or CNS complications, should be hospitalized immediately. Intravenous therapy with nafcillin or clindamycin plus a third-generation cephalosporin such as ceftriaxone should be initiated until culture results become available.

Decongestants, antihistamines, and nasal saline irrigations are frequently used in acute rhinosinusitis to promote drainage. To date, there are no methodologically sound studies supporting their efficacy in children. If used, topical nasal decongestants, such as oxymetazoline or phenylephrine sprays, should not be used for more than 3 days due to risk of rebound edema. Patients with underlying allergic rhinitis may benefit from intranasal cromolyn or corticosteroid nasal spray.

2. Recurrent or Chronic Rhinosinusitis

Recurrent rhinosinusitis occurs when episodes of ABRS clear for at least 10 days but recur at least four times per year. Chronic rhinosinusitis (CRS) is diagnosed when the child has not cleared the infection in 90 days, but has not developed acute complications. Both symptoms and physical findings are required to support the diagnosis, and CT scan may be a useful in making the diagnosis. Although recent meta-analysis evaluations have resulted in recommendations for ABRS, there is a paucity of data for the treatment of recurrent or CRS. Important factors to consider include allergies, anatomic variations, and disorders in host immunity. Mucosal inflammation leading to obstruction is most commonly caused by allergic rhinitis and occasionally by nonallergic rhinitis. There is a great deal of evidence that allergic rhinitis, rhinosinusitis, and asthma are all manifestations of a systemic inflammatory response. Gastroesophageal reflux has also been implicated in CRS. Less commonly, CRS is caused by anatomic variations, such as septal deviation, polyp, or foreign body.

Allergic nasal polyps are unusual in children younger than 10 years and should prompt a workup for cystic fibrosis. In cases of chronic or recurrent pyogenic pansinusitis, poor host resistance (eg, an immune defect, primary ciliary dyskinesia, or cystic fibrosis)—though rare—must be ruled out by immunoglobulin studies, electron microscopy studies of respiratory cilia, nasal nitric oxide measurements if available, a sweat chloride test, and genetic testing (see Chapter 19). Anaerobic and staphylococcal organisms are often responsible for CRS. Evaluation by an allergist and an otolaryngologist may be useful in determining the underlying causes.

Treatment

A. Medical Therapy

Antibiotic therapy is similar to that used for ABRS, but the duration is longer, typically 3–4 weeks. Antimicrobial choice should include drugs effective against staphylococcal organisms. Nasal saline irrigations and intranasal steroid sprays have been shown to be helpful in the reduction of symptoms of CRS.

B. Surgical Therapy

The mainstay of treatment for pediatric CRS is medical management, with appropriate antibiotic therapy and treatment of comorbid conditions such as allergic rhinitis and asthma. Only a small percentage of children will warrant surgical management.

1. Antral lavage

Antral lavage, generally regarded as a diagnostic procedure, may have some therapeutic value. An aspirate or a sample from the maxillary sinus is retrieved under anesthesia. The maxillary sinus is then irrigated. In the very young child, this may be the only procedure performed.

2. Adenoidectomy

Adenoidectomy is thought to be effective in 50%–75% of children with CRS, up to the age of 12 years. The adenoids serve as a reservoir of pathogenic bacteria and may also interfere with mucociliary clearance and drainage. Biofilms have been reported in the adenoids of children with CRS and may explain the resistance of these infections to standard antibiotic therapy.

3. Endoscopic sinus surgery

Endoscopic sinus surgery in children was controversial because of concerns regarding facial growth. However, recent studies have not supported this concern. Endoscopic sinus surgery is reported to be effective in over 80% of cases, and may be indicated if adenoidectomy or balloon dilation is not effective.

4. External drainage

External drainage procedures are reserved for complications arising from ethmoid and frontal sinusitis.

CHOANAL ATRESIA

Choanal atresia occurs in approximately 1 in 7000 live births. The female-male ratio is 2:1, as is the unilateral-bilateral ratio. Bilateral atresia results in severe respiratory distress at birth and requires immediate placement of an oral airway or intubation, and otolaryngology consultation for surgical treatment. Unilateral atresia usually appears later as a unilateral chronic nasal discharge that may be mistaken for CRS. Diagnosis may be suspected if a 6F catheter cannot be passed through the nose and is confirmed by axial CT scan. Approximately 50% of patients with bilateral choanal atresia have CHARGE association (Coloboma, Heart disease, Atresia of the choanae, Retarded growth and retarded development or CNS anomalies, Genital hypoplasia, and Ear anomalies or deafness) (see Chapter 37) or other congenital anomalies.

RECURRENT RHINITIS

Recurrent rhinitis is frequently seen in pediatrics. The child is brought in with the chief complaint of having “one cold after another,” “constant colds,” or “always being sick.” Approximately, two-thirds of these children have recurrent colds; the rest have either allergic rhinitis or recurrent rhinosinusitis.

1. Allergic Rhinitis

Allergic rhinitis is a chronic disorder of the upper airway which is induced by IgE-mediated inflammation secondary to allergen exposure. It is more common in children than in adults and affects up to 40% of children in the United States. It significantly affects quality of life, interfering with physical and social activities, concentration, school performance, and sleep. Allergic rhinitis can contribute to the development of rhinosinusitis, otitis media, and asthma. Symptoms may include nasal congestion, sneezing, rhinorrhea, and itchy nose, palate, throat, and eyes. On physical examination, the nasal turbinates are swollen and may be red or pale pink-purple. Several classes of medications have proven effective in treating allergic rhinitis, including intranasal corticosteroids, oral and intranasal antihistamines, leukotriene antagonists, and decongestants. Ipratropium nasal spray may also be used as an adjunctive therapy. Nasal saline rinses are helpful to wash away allergens. Recent studies have indicated that use of intranasal steroid sprays may not only decrease the impairment caused by allergic rhinitis symptoms, but also help prevent progression to more severe disease and decrease the risk of related comorbidities such as asthma and sleep-disordered breathing. These intranasal steroids can be used in children as young as 2 years of age.

2. Nonallergic Rhinitis

Nonallergic rhinitis also causes rhinorrhea and nasal congestion, but does not seem to involve an immunologic reaction. Its mechanism is not well understood. Triggers can include sudden changes in environmental temperature, air pollution, and other irritants such as tobacco smoke. Medications can also be associated with nonallergic rhinitis. Nasal decongestant sprays, when used for long periods of time, can cause rhinitis medicamentosa, which is a rebound nasal congestion and can be very difficult to treat. Oral decongestants, nasal corticosteroids, antihistamines, and ipratropium spray have all been shown to offer symptomatic relief.

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EPISTAXIS

The nose is a highly vascular structure. In most cases, epistaxis (nosebleed) arises from the anterior portion of the nasal septum (Kiesselbach area). It is often due to dryness, nose rubbing, nose blowing, or nose picking. Examination of the anterior septum usually reveals a red, raw surface with fresh clots or old crusts. Presence of telangiectasias, hemangiomas, or varicosities should also be noted. If a patient has been using a nasal corticosteroid spray, check the patient’s technique to make sure he or she is not directing the spray toward the septum. If proper technique does not reduce the nosebleeds, the spray should be discontinued.

In fewer than 5% of cases, epistaxis is caused by a bleeding disorder such as von Willebrand disease. A hematologic workup is warranted if any of the following is present: family history of a bleeding disorder; medical history of easy bleeding, particularly with circumcision or dental work; spontaneous bleeding at any site; bleeding that lasts for more than 30 minutes or blood that will not clot with direct pressure by the physician; onset before age 2 years; or a drop in hematocrit due to epistaxis. High blood pressure may rarely predispose to prolonged nosebleeds in children.

Juvenile nasopharyngeal angiofibroma (JNA) is a benign, but often aggressive, tumor that tends to bleed and may present as recurrent epistaxis (45%–60%). JNA is only seen in males; females with JNA should undergo karyotyping. CT and MRI are diagnostic.

Treatment

The patient should sit up and lean forward so as not to swallow the blood. Swallowed blood may cause nausea and hematemesis. The nasal cavity should be cleared of clots by gentle blowing. The soft part of the nose below the nasal bones is pinched and held firmly enough to prevent arterial blood flow, with pressure over the bleeding site (anterior septum) being maintained for 5 minutes. For persistent bleeding, a one-time only application of oxymetazoline into the nasal cavity may be helpful. If bleeding continues, the bleeding site needs to be visualized. A small piece of gelatin sponge (Gelfoam) or collagen sponge (Surgicel) can be inserted over the bleeding site and held in place.

Friability of the nasal vessels is often due to dryness and can be decreased by increasing nasal moisture. This can be accomplished by daily application of a water-based ointment to the nose. A pea-sized amount of ointment is placed just inside the nose and spread by gently squeezing the nostrils. Twice-daily nasal saline irrigation and humidifier use may also be helpful. Aspirin and ibuprofen should be avoided, as should nose picking and vigorous nose blowing. Otolaryngology referral is indicated for refractory cases. Cautery of the nasal vessels is reserved for treatment failures.

NASAL INFECTION

A nasal furuncle is an infection of a hair follicle in the anterior nares. Hair plucking or nose picking can provide a route of entry. The most common organism is S aureus. A furuncle presents as an exquisitely tender, firm, red lump in the anterior nares. Treatment includes dicloxacillin or cephalexin orally for 5 days to prevent spread. The lesion should be gently incised and drained as soon as it points with a sterile needle. Topical antibiotic ointment may be of additional value. Because this lesion is in the drainage area of the cavernous sinus, the patient should be followed closely until healing is complete. Parents should be advised never to pick or squeeze a furuncle in this location—and neither should the physician. Associated cellulitis or spread requires hospitalization for administration of intravenous antibiotics.

A nasal septal abscess is usually the result of nasal trauma or a nasal furuncle. Examination reveals fluctuant gray septal swelling, which is usually bilateral. The possible complications are the same as for nasal septal hematoma (see following discussion). In addition, spread of the infection to the CNS is possible. Treatment consists of immediate hospitalization, incision and drainage by an otolaryngologist, and antibiotic therapy.

NASAL TRAUMA

Newborn infants rarely present with subluxation of the quadrangular cartilage of the septum. In this disorder, the top of the nose deviates to one side, the inferior septal border deviates to the other side, the columella leans, and the nasal tip is unstable. This disorder must be distinguished from the more common transient flattening of the nose caused by the birth process. In the past, physicians were encouraged to reduce all subluxations in the nursery. Otolaryngologists are more likely to perform the reduction under anesthesia for more difficult cases.

After nasal trauma, it is essential to examine the inside of the nose in order to rule out hematoma of the nasal septum, as this can quickly lead to septal necrosis, causing permanent nasal deformity. This diagnosis is confirmed by the abrupt onset of nasal obstruction following trauma and the presence of a boggy, widened nasal septum. The normal nasal septum is only 2–4 mm thick. A cotton swab can be used to palpate the septum. Treatment consists of immediate referral to an otolaryngologist for drainage and packing of the nose.

Most blows to the nose result in epistaxis without fracture. A persistent nosebleed after trauma, crepitus, instability of the nasal bones, and external deformity of the nose indicate fracture. Septal injury cannot be ruled out by radiography, and can only be ruled out by careful intranasal examination. Patients with suspected nasal fractures should be referred to an otolaryngologist for definitive therapy. Since the nasal bones begin healing immediately, the child must be seen by an otolaryngologist within 48–72 hours of the injury to allow time to arrange for fracture reduction before the bones become immobile.

FOREIGN BODIES IN THE NOSE

If this diagnosis is delayed, unilateral foul-smelling rhinorrhea, halitosis, bleeding, or nasal obstruction often result.

There are many ways to remove nasal foreign bodies. The obvious first maneuver is vigorous nose blowing if the child is old enough. The next step in removal requires nasal decongestion, good lighting, correct instrumentation, and physical restraint. Topical tetracaine or lidocaine may be used for anesthesia in young children. Nasal decongestion can be achieved by topical phenylephrine or oxymetazoline. When the child is properly restrained, most nasal foreign bodies can be removed using a pair of alligator forceps or right-angle instrument through an operating head otoscope. If the object seems unlikely to be removed on the first attempt, is wedged in, or is quite large, the patient should be referred to an otolaryngologist rather than worsening the situation through futile attempts.

Because the nose is a moist cavity, the electrical current generated by disk-type batteries—such as those used in clocks, watches, and hearing aids—can cause necrosis of mucosa and cartilage destruction in less than 4 hours. Batteries constitute a true foreign body emergency.

ACUTE STOMATITIS

Stomatitis is inflammation in the oral cavity, and can arise from infection, autoimmune disorders, drug reactions, and chemoradiation. Acute episodes are often painful, and limit oral intake.

1. Recurrent Aphthous Stomatitis

Aphthous ulcers, or canker sores, are small painful ulcers (3–10 mm) usually found on the inner aspect of the lips, gingiva, or tongue. Typically lasting 1–2 weeks, there is usually no associated fever or cervical adenopathy. These may reoccur throughout life, with infectious or autoimmune cause suspected. Treatment is symptomatic with acetaminophen or ibuprofen and temporary choice of a bland diet. A topical corticosteroid, such as triamcinolone dental paste, may also reduce symptomatic duration and intensity. A Cochrane review was unable to promote a single systemic treatment for those unresponsive to local therapy.

Less common causes of recurrent oral ulcers include Behçet disease, familial Mediterranean fever, and PFAPA syndrome (Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenopathy). Behçet disease requires two of the following: genital ulcers, uveitis, and erythema nodosum–like lesions. Patients with Mediterranean fever usually have a positive family history, serosal involvement, and recurrent fever. PFAPA typically begins before the age of 5 years, continues through adolescence, and then spontaneously resolves. Fever and other symptoms recur at regular intervals. Episodes last approximately 5 days and are not associated with other URI symptoms or illnesses. Steroids may shorten episodes but do not prevent recurrence. PFAPA has been shown to resolve with prolonged cimetidine use and adenotonsillectomy.

2. Herpes Simplex Gingivostomatitis

HSV-1 is more often associated with oral ulcerations than HSV-2 (See Chapter 40 Infections: Viral & Rickettsial). HSV transmission is almost always through direct contact, and the virus migrates to reside in the trigeminal ganglion. Reactivation from a dormant state can arise from a variety of stimuli, leading to symptomatic ulcerated lesions of the lips and oral cavity.

3. Thrush

Candida is normally found in the oral flora of 60% of the population (See Chapter 43 Infections: Parasitic & Mycotic). In infants it presents as white plaques with an erythematous base that typically involves the buccal mucosa and dorsal tongue. Spread to the pharynx and larynx causes odynophagia and can be seen in older children using inhaled steroids and those with compromised immune systems. The breast-feeding mother-infant dyad require simultaneous treatment—the mother with oral diflucan and the infant with a one-time application of Gentian Violet or a course of Nystatin suspension.

4. Traumatic Oral Ulcers

Mechanical trauma most commonly occurs on the buccal mucosa secondary to biting by the molars. Thermal trauma, from very hot foods, can also cause ulcerative lesions. Chemical ulcers can be produced by mucosal contact with aspirin or other caustic agents. Oral ulcers can occur with leukemia or on a recurrent basis with cyclic neutropenia.

PHARYNGITIS

Figure 18–5 is an algorithm for the management of a sore throat.

1. Acute Viral Pharyngitis

Over 90% of sore throats and fever in children are due to viral infections. The findings seldom point toward any particular viral agent, but four types of viral pharyngitis are sufficiently distinctive to warrant discussion below.

Clinical Findings

A. Infectious Mononucleosis

Findings include exudative tonsillitis, generalized cervical adenitis and fever, usually in patients older than 5 years. A palpable spleen or axillary adenopathy increases the likelihood of the diagnosis. The presence of more than 10% atypical lymphocytes on a peripheral blood smear or a positive mononucleosis spot test supports the diagnosis, although these tests are often falsely negative in children younger than 5 years. Epstein-Barr virus serology showing an elevated IgM-capsid antibody is definitive. Amoxicillin is contraindicated in patients suspected of having mononucleosis because the drug often precipitates a rash.

B. Herpangina

Herpangina ulcers are classically 3 mm in size, surrounded by a halo and are found on the anterior tonsillar pillars, soft palate, and uvula; the anterior mouth and tonsils are spared. Herpangina is caused by the Coxsackie A group of viruses. Polymerase chain reaction testing is available, but not typically indicated, as this is a self-limited illness.

C. Hand, Foot, and Mouth Disease

This entity is caused by several enteroviruses, one of which (enterovirus 71) can rarely cause encephalitis. Ulcers occur anywhere in the mouth. Vesicles, pustules, or papules may be found on the palms, soles, interdigital areas, and buttocks. In younger children lesions may be seen on the distal extremities and even the face.

D. Pharyngoconjunctival Fever

This disorder is caused by an adenovirus and often is epidemic. Exudative tonsillitis, conjunctivitis, lymphadenopathy, and fever are the main findings. Treatment is symptomatic.

2. Acute Bacterial Pharyngitis

Differential Diagnosis

Viral pharyngitis, infectious mononucleosis, bacterial pharyngitis other than streptococcal, diphtheria, and peritonsillar abscess.

Approximately 20%–30% of children with pharyngitis have a group A streptococcal (GAS) infection. It is most common in children between 5 and 15 years in the winter or early spring. Less common causes of bacterial pharyngitis include Mycoplasma pneumoniae, Chlamydia pneumoniae, groups C and G streptococci, and Arcanobacterium hemolyticum. Of the five, M pneumoniae is by far the most common and may cause over one-third of all pharyngitis cases in adolescents and adults.

Clinical Findings

Sudden onset of sore throat, fever, tender cervical adenopathy, palatal petechiae, a beefy-red uvula, and a tonsillar exudate suggest streptococcal infection. Other symptoms may include headache, stomachache, nausea, and vomiting. The only way to make a definitive diagnosis is by throat culture or rapid antigen test. Rapid antigen tests are very specific, but have a sensitivity of only 85%–95%. Therefore, a positive test indicates S pyogenes infection, but a negative result requires confirmation by performing a culture. Diagnosis is important because untreated streptococcal pharyngitis can result in acute rheumatic fever, glomerulonephritis, and suppurative complications (eg, cervical adenitis, peritonsillar abscess, otitis media, cellulitis, and septicemia). The presence of conjunctivitis, cough, hoarseness, symptoms of URI, anterior stomatitis, ulcerative lesions, viral rash, and diarrhea should raise suspicion of a viral etiology.

Occasionally, a child with group A streptococcal infection develops scarlet fever within 24–48 hours after the onset of symptoms. Scarlet fever is a diffuse, finely papular, erythematous eruption producing a bright red discoloration of the skin, which blanches on pressure. The rash is more intense in the skin creases. The tongue has a strawberry appearance.

A controversial but possible complication of streptococcal infections is pediatric autoimmune neuropsychiatric disorders associated with Streptococcus (PANDAS). PANDAS is a relatively newly recognized condition. It describes a subset of pediatric patients who experience a sudden onset of obsessive-compulsive disorder and/or tics, or worsening of such symptoms in children who previously had these, following a strep infection.

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Treatment

Suspected or proven group A streptococcal infection should be treated with penicillin (oral or intramuscular) or amoxicillin as outlined in Table 18–4. For patients allergic to penicillin, alternative treatments include cephalexin, azithromycin, and clindamycin. Tetracyclines, sulfonamides (including trimethoprim-sulfamethoxazole) and quinolones should not be used for treating GAS infections.

Repeat culture after treatment is not recommended and is indicated only for those who remain symptomatic, have a recurrence of symptoms, or have had rheumatic fever. Of note, children who have had rheumatic fever are at a high risk of recurrence if future GAS infections are inadequately treated. In this group of patients, long-term antibiotic prophylaxis is recommended, sometimes life-long in patients with residual rheumatic heart disease (see Chapter 20).

In general, the carrier state is harmless, self-limited (2–6 months), and not contagious. An attempt to eradicate the carrier state is warranted only if the patient or another family member has frequent streptococcal infections, or if a family member or patient has a history of rheumatic fever or glomerulonephritis. If eradication is chosen, a course of clindamycin for 10 days or rifampin for 5 days should be used.

In the past, daily penicillin prophylaxis was occasionally recommended; however, because of concerns about the development of drug resistance, tonsillectomy is now preferred for patients with recurrent streptococcal tonsillitis.

PERITONSILLAR CELLULITIS OR ABSCESS (QUINSY)

Tonsillar infection extending to the surrounding tissues is called peritonsillar cellulitis. If untreated, necrosis occurs, with peritonsillar abscess formation. The most common pathogen is β-hemolytic Streptococcus, but others include group D Streptococcus, S pneumoniae, and anaerobes. A severe sore throat and high fever is usually present, and the process is almost always unilateral. The tonsil bulges medially, and the anterior tonsillar pillar is prominent. The soft palate and uvula on the involved side are edematous and displaced toward the uninvolved side. As the infection progresses, trismus, ear pain, dysphagia, and drooling may occur. The most serious complication of untreated peritonsillar abscess is a lateral pharyngeal abscess.

While peritonsillar cellulitis will often respond to parenteral antibiotics (penicillin, cephalosporin, or clindamycin), an abscess within the peritonsillar space will typically require drainage. Failure to respond to therapy during the first 12–24 hours indicates a high probability of abscess formation and need for otolaryngology consultation. A peritonsillar abscess may be needle aspirated or formally incised and drained. The risk of bilateral and/or recurrent peritonsillar abscess is low (7%–10%), so tonsillectomy is not usually indicated for a single incident.

RETROPHARYNGEAL ABSCESS

Retropharyngeal lymph nodes drain the oropharynx, nasopharynx, and paranasal sinuses. Infections of these nodes are most often due to β-hemolytic streptococci and S aureus. The diagnosis of retropharyngeal abscess should be suspected in a child with fever, respiratory symptoms, and neck hyperextension. Dysphagia, drooling, dyspnea, and gurgling respirations may also be present. This occurs most commonly during the first 2 years of life. After this age, the most common cause of retropharyngeal abscess is penetrating injury of the posterior pharyngeal wall.

Prominent swelling on one side of the posterior pharyngeal wall is characteristic. Swelling usually stops at the midline because a medial raphe divides the prevertebral space. Although nonspecific, lateral neck x-ray may demonstrate retropharyngeal tissues wider than the C4 vertebral body; CT scan with contrast can distinguish between soft tissue swelling versus abscess.

Although a retropharyngeal abscess is a surgical emergency, frequently it cannot be distinguished from retropharyngeal adenitis. Immediate hospitalization and intravenous antimicrobial therapy with a semisynthetic penicillin or clindamycin is the first step for most cases. In most instances, a period of 12–24 hours of antimicrobial therapy will help differentiate the two entities. In the child with adenitis, fever will decrease and oral intake will improve. A child with retropharyngeal abscess will typically not improve. A surgeon should incise and drain the abscess under general anesthesia to prevent its extension. Immediate surgical drainage is indicated if there is concern for airway compromise.

LUDWIG ANGINA

Ludwig angina is a rapidly progressive cellulitis of both submandibular spaces that pushes the tongue posteriorly against the pharyngeal wall, causing life threatening airway obstruction. Symptoms include fever and tender swelling of the tongue and floor of the mouth. Most often arising from an odontogenic source, this infection is unusual in infants and children. Group A strep is the most common culprit. Treatment consists of high-dose intravenous clindamycin or ampicillin plus nafcillin until culture and sensitivities are available. Treatment for airway obstruction in patients with Ludwig angina has transitioned through the years from tracheotomy to intensive care unit monitoring and intubation. An otolaryngologist should be consulted for airway evaluation and management, and to perform a drainage procedure if needed.

ACUTE CERVICAL ADENITIS

Local infections of the ear, nose, and throat can involve a regional lymph node and cause abscess formation. The typical case involves a unilateral, solitary, anterior cervical node. About 70% of these cases are due to β-hemolytic streptococcal infection, 20% to staphylococci (including MRSA), and the remainder to viruses, atypical mycobacteria, and Bartonella henselae.

The initial evaluation of cervical adenitis should generally include a rapid group A streptococcal test, and a complete blood count with differential looking for atypical lymphocytes. A purified protein derivative skin test, looking for nontuberculous mycobacteria, should also be considered. If multiple enlarged nodes are found, a rapid mononucleosis test is useful. Early treatment with antibiotics prevents many cases of adenitis from progressing to suppuration. However, once abscess formation occurs, antibiotic therapy alone is often insufficient and a drainage procedure may be necessary. Because of the increase in community-acquired MRSA, it is a prudent to send a specimen for culture and sensitivity.

Cat-scratch disease, caused by B henselae, causes indolent (“cold”) adenopathy. The diagnosis is supported if a primary papule is found at the scratch site on the face. In over 90% of patients, there is a history of contact with kittens. The node is usually only mildly tender but may, over a month or more, suppurate and drain. About one-third of children have fever and malaise; rarely neurologic sequelae and prolonged fever occur. Cat-scratch disease can be diagnosed by serologic testing, but testing is not always confirmatory. If blood is to be drawn, one should wait 2–8 weeks after onset of symptoms. Because most enlarged lymph nodes infected with B henselae spontaneously regress within 1–3 months, the benefit of antibiotics is controversial. In a placebo-controlled trial, azithromycin for 5 days caused a more rapid decrease in node size. Other drugs likely to be effective include rifampin, trimethoprim-sulfamethoxazole, erythromycin, clarithromycin, doxycycline∗, ciprofloxacin^∗, and gentamicin (^∗be aware of age restrictions).

Cervical lymphadenitis can also be caused by nontuberculous mycobacterial species or Mycobacterium avium complex. Mycobacterial disease is unilateral and may involve several matted nodes. A characteristic violaceous appearance may develop over a prolonged period of time without systemic signs or much local pain. Atypical mycobacterial infections are often associated with positive purified protein derivative skin test reactions less than 10 mm in diameter, and a second-strength (250-test-unit) purified protein derivative skin test is virtually always positive.

Differential Diagnosis

A. Neoplasms and Cervical Nodes

Malignant tumors usually are not suspected until adenopathy persists despite antibiotic treatment. Classically, malignant lymph nodes are painless, nontender, and of firm consistency. They may be fixed to underlying tissues. They may occur as a single node, as unilateral nodes in a chain, bilateral cervical nodes, or as generalized adenopathy. Common malignancies that may manifest in the neck include lymphoma, rhabdomyosarcoma, and thyroid carcinoma.

B. Imitators of Adenitis

Several structures in the neck can become infected and resemble a lymph node. The first three masses are of congenital origin.

1. Thyroglossal duct cyst

These are midline, usually near the level of the hyoid bone. Thyroglossal duct cysts move upward when the tongue is protruded or with swallowing. Occasionally, a thyroglossal duct cyst may have a sinus tract with an opening just lateral to the midline. When infected, these can become acutely swollen and inflamed.

2. Branchial cleft cyst

These masses are found along the anterior border of the sternocleidomastoid muscle and are smooth and fluctuant. Sometimes a branchial cleft cyst may be attached to the overlying skin by a small dimple or a draining sinus tract. When infected, they can become a tender mass 3–5 cm in diameter.

3. Lymphatic malformation

Most lymphatic cysts are located in the posterior triangle just above the clavicle. These are soft and compressible and can be transilluminated. Over 60% are noted at birth; the remaining malformations usually become apparent by the age of 2 years. If large enough, they can compromise the patient’s ability to swallow and breathe.

4. Parotitis

Parotitis is commonly mistaken for cervical adenitis. The parotid salivary gland crosses the angle of the jaw. Parotitis may be bacterial or viral and may occur unilaterally or bilaterally. Mumps was once the most common cause of viral parotitis, but because of routine vaccinations, parainfluenza is the primary viral cause in the United States. An amylase level will be elevated in parotitis.

5. Ranula

A ranula is a saliva filled cyst in the floor of mouth caused by obstruction of the sublingual salivary gland. A “plunging” ranula extends down through the mylohyoid muscle and can present as a neck mass.

6. Sternocleidomastoid muscle hematoma

Also known as fibromatosis colli, these are noted at age 2–4 weeks. On examination, the mass is found to be part of the muscle body and not movable. An associated torticollis usually confirms the diagnosis. A neck ultrasound can help confirm the diagnosis. Treatment involves physical therapy, with range of motion exercises.

TONSILLECTOMY & ADENOIDECTOMY

Tonsillectomy

A tonsillectomy, with or without adenoidectomy, is most often performed for either hypertrophy or recurrent infections. The most common indication for adenotonsillectomy is adenotonsillar hypertrophy associated with an obstructive breathing pattern during sleep (see Chapter 19). Adenotonsillar hypertrophy may also cause other problems such as dysphagia or dental malocclusion.

Recurrent tonsillitis is the second most common reason for tonsillectomy. Tonsillitis is considered “recurrent” when a child has seven or more documented infections in 1 year, five per year for 2 years, or three per year for 3 years. For an infection to be considered clinically significant, there must be a sore throat and at least one of the following clinical features: cervical lymphadenopathy (tender lymph nodes or > 2 cm), OR tonsillar exudate, OR positive culture for group A β-hemolytic Streptococcus, OR temperature greater than 38.3°C.

Tonsillectomy is reasonable with fewer infections if the child has missed multiple school days due to infection, has a complicated course, or under other circumstances such as recurrent peritonsillar abscess, persistent streptococcal carrier state, or multiple antibiotic allergies. Unless neoplasm is suspected, tonsil asymmetry is not an indication.

A new indication for tonsillectomy is PFAPA syndrome (see earlier section on Recurrent Aphthous Stomatitis), in which fever recurs predictably, typically every 4–8 weeks. Tonsillectomy has been shown to be an effective treatment.

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Adenoidectomy

The adenoid is composed of lymphoid tissue in the nasopharynx and is a part of the Waldeyer ring of lymphoid tissue, which also includes the palatine and lingual tonsils. Enlarged adenoids, with or without infection, can obstruct the nose, alter normal orofacial growth, and interfere with speech, swallowing, and Eustachian tube function. Children who are persistent mouth breathers can develop dental malocclusion and “adenoid facies,” where the face appears “pinched” and the maxilla narrowed because the molding pressures of the orbicularis oris and buccinator muscles are unopposed by the tongue. The adenoid can also harbor biofilms, which have been associated with CRS and otitis media.

Indications for adenoidectomy with or without tonsillectomy include upper airway obstruction, orofacial conditions such as mandibular growth abnormalities and dental malocclusion, speech abnormalities, persistent MEE, recurrent otitis media, and CRS.

Complications of Tonsillectomy & Adenoidectomy

The mortality rate associated with tonsillectomy and adenoidectomy is reported to approximate that of general anesthesia alone. The rate of hemorrhage varies between 0.1% and 8.1%, depending on the definition of hemorrhage; the rate of postoperative transfusion is 0.04%. Other potential complications include permanently hypernasal speech (< 0.01%) and, more rarely, nasopharyngeal stenosis, atlantoaxial subluxation, mandibular condyle fracture, and psychological trauma.

Contraindications to Tonsillectomy & Adenoidectomy

A. Palatal Abnormalities

Adenoid tissue should not be removed completely in a child with a cleft palate or submucous cleft palate because of the risk of velopharyngeal incompetence which may cause hypernasal speech and nasal regurgitation. If needed, a partial adenoidectomy can be performed in at-risk children. A bifid uvula can be a sign of a palatal abnormality.

B. Bleeding Disorder

When suspected, bleeding disorders must be diagnosed and treated prior to surgery.

C. Acute Tonsillitis

An elective tonsillectomy and adenoidectomy can usually be postponed until acute tonsillitis is resolved. Urgent tonsillectomy may occasionally be required for tonsillitis unresponsive to medical therapy.

DISORDERS OF THE LIPS

1. Labial Sucking Tubercle

A young infant may present with a small callus in the mid-upper lip. The cause is likely strong sucking in utero but can persist into early infancy. It usually is asymptomatic and disappears after cup feeding is initiated.

2. Cheilitis

Dry, cracked, scaling lips are usually caused by sun or wind exposure. Contact dermatitis from mouthpieces or various woodwind or brass instruments has also been reported. Licking the lips exacerbates cheilitis. Liberal use of lip balm gives excellent results.

3. Inclusion Cyst

Inclusion or mucous retention cysts are due to obstruction of mucous glands or other mucous membrane structures, such as minor salivary glands. In the newborn, they occur on the hard palate or gums and are called Epstein pearls. These resolve spontaneously in 1–2 months. In older children, inclusion cysts usually occur on the palate, uvula, or tonsillar pillars. They appear as taut yellow sacs varying in size from 2 to 10 mm. Inclusion cysts that do not resolve spontaneously may undergo incision and drainage. Occasionally, a mucous cyst on the lower lip (mucocele) will require excision for cosmetic reasons.

DISORDERS OF THE TONGUE

1. Geographic Tongue (Benign Migratory Glossitis)

This condition of unknown etiology occurs in 1%–2% of the population with no age, sex, or racial predilection. It is characterized by irregularly shaped patches on the tongue that are devoid of papillae and surrounded by parakeratotic reddish borders. The pattern changes as alternating regeneration and desquamation occurs. The lesions are generally asymptomatic and require no treatment.

2. Fissured Tongue (Scrotal Tongue)

This condition is marked by numerous irregular fissures on the dorsum of the tongue. It occurs in approximately 1% of the population and is usually a dominant trait. It is also frequently seen in children with trisomy 21.

3. Coated Tongue (Furry Tongue)

The tongue becomes coated if mastication is impaired and the patient is limited to a liquid or soft diet. Mouth breathing, fever, or dehydration can accentuate the process.

4. Macroglossia

Tongue hypertrophy and protrusion may be due to trisomy 21, Beckwith-Wiedemann syndrome, glycogen storage diseases, cretinism, mucopolysaccharidoses, lymphangioma, or hemangioma. Tongue reduction procedures should be considered in otherwise healthy subjects if macroglossia affects airway patency.

HALITOSIS

Bad breath is usually due to acute stomatitis, pharyngitis, rhinosinusitis, nasal foreign body, or dental hygiene problems. In older children and adolescents, halitosis can be a manifestation of CRS, gastric bezoar, bronchiectasis, or lung abscess. The presence of orthodontic devices or dentures can cause halitosis if good dental hygiene is not maintained. Halitosis can also be caused by decaying food particles embedded in cryptic tonsils. Mouthwashes and chewable breath fresheners give limited improvement. Treatment of the underlying cause is indicated, and a dental referral may be in order.

SALIVARY GLAND DISORDERS

1. Parotitis

A first episode of parotitis may safely be considered to be of viral origin, unless fluctuance is present. Mumps was the leading cause until adoption of vaccination; now the leading viruses are parainfluenza and Epstein-Barr virus. HIV infection should be considered if the child is known to be at risk.

2. Suppurative Parotitis

Suppurative parotitis occurs chiefly in newborns and debilitated elderly patients. The parotid gland is swollen, tender, and often erythematous, usually unilaterally. The diagnosis is made by expression of purulent material from Stensen duct. The material should be cultured. Fever and leukocytosis may be present. Treatment includes intravenous antibiotic therapy. S aureus is the most common causative organism.

3. Juvenile Recurrent Parotitis

Some children experience recurrent nonsuppurative parotid inflammation with swelling or pain and fever. Juvenile recurrent parotitis (JRP) is most prevalent between the ages of 3 and 6 years, and it generally decreases by adolescence. The cause is unknown, but possible etiologic factors include ductal anomaly, autoimmune, allergy, and genetic. It usually occurs unilaterally. Treatment includes analgesics and some recommend an antistaphylococcal antibiotic for prophylaxis of bacterial infection and quicker resolution. Endoscopy and irrigation of Stensen duct is being performed more frequently, not only to confirm diagnosis but also to provide treatment.

4. Tumors of the Parotid Gland

Mixed tumors, hemangiomas, sarcoidosis, and leukemia can manifest in the parotid gland as a hard or persistent mass. A cystic mass or multiple cystic masses may represent an HIV infection. Workup may require consultation with oncology, infectious disease, hematology, and otolaryngology.

5. Ranula

A ranula is a retention cyst of a sublingual salivary gland. It occurs on the floor of the mouth to one side of the lingual frenulum. It is thin walled and can appear bluish. Referral is indicated to an otolaryngologist for surgical management.

CONGENITAL ORAL MALFORMATIONS

1. Tongue-Tie (Ankyloglossia)

A short lingual frenulum can hinder protrusion and elevation of the tongue. Dimpling of the midline tongue tip is noted with tongue movement. Ankyloglossia can cause feeding difficulties in the neonate, speech problems, and dental problems. If the tongue cannot protrude past the teeth or alveolar ridge or move between the gums and cheek, referral to an otolaryngologist is indicated. A frenulectomy should be performed in the neonatal period if the infant is having difficulty breast-feeding, specifically inefficient milk transfer or persistent pain in the mother. Early treatment is favored because it can easily be performed in clinic. When an infant is even a few months old, general anesthesia is required for the procedure to be performed safely. Lip tie concerns have become more common as breast-feeding initiation is increasing but there is little evidence that this has any effect on nursing.

2. Torus Palatini

Torus palatini are hard, midline, palate masses which form at suture lines of the bone. They are usually asymptomatic and require no therapy, but they can be surgically reduced if necessary.

3. Cleft Lip & Cleft Palate

A. Submucous Cleft Palate

A bifid uvula is present in 3% of healthy children (see Chapter 37). However, a close association exists between bifid uvula and submucous cleft palate. A submucous cleft can be diagnosed by noting a translucent zone in the middle of the soft palate (zona pellucida). Palpation of the hard palate reveals absence of the posterior bony protrusion. Affected children have a 40% risk of developing persistent MEE. They are at risk for velopharyngeal incompetence, or an inability to close the palate against the posterior pharyngeal wall, resulting in hypernasal speech and nasal regurgitation of food. Children with submucous cleft palate causing abnormal speech or significant nasal regurgitation of food should be referred for possible surgical repair.

B. High-Arched Palate

A high-arched palate is usually a genetic trait of no consequence. It also occurs in children who are chronic mouth breathers and in premature infants who undergo prolonged oral intubation. Some rare causes of high-arched palate are congenital disorders such as Marfan syndrome, Treacher Collins syndrome, and Ehlers-Danlos syndrome. Orthodontic treatment of a high arched palate can be an effective treatment for childhood OSA.

C. Pierre Robin Sequence

This group of congenital malformations is characterized by the triad of micrognathia, cleft palate, and glossoptosis. Affected children often present as emergencies in the newborn period because of infringement on the airway by the tongue and also because of feeding difficulties. The main objective of treatment is to prevent asphyxia until the mandible becomes large enough to accommodate the tongue. In some cases, this can be achieved by leaving the child in a prone position when unattended. Other airway manipulations such as a nasal trumpet may be necessary. Distraction osteogenesis has been used to avoid tracheostomy. In severe cases, a tracheostomy is required. The child requires close observation and careful feeding until the problem is outgrown.