CURRENT Diagnosis and Treatment Pediatrics, (Current Pediatric Diagnosis & Treatment) 22nd Edition
18. Ear, Nose, & Throat
Norman R. Friedman, MD
Melissa A. Scholes, MD
Patricia J. Yoon, MD
INFECTIONS OF THE EAR
ESSENTIALS OF DIAGNOSIS
Edema and erythema of the external auditory canal (EAC) with debris or thick, purulent discharge.
Severe ear pain, worsened by manipulation of the pinna.
Periauricular and cervical lymphadenopathy may be present.
Acute otitis media (AOM) with tympanic membrane (TM) rupture, furunculosis of the ear canal, and mastoiditis.
Otitis externa (OE) is a cellulitis of the soft tissues of the EAC, which can extend to surrounding structures such as the pinna, tragus, and lymph nodes. Also known as “swimmer’s ear,” humidity, heat, and moisture in the ear are known to contribute to the development of OE, along with localized trauma to the ear canal skin. Sources of trauma may include digital trauma, earplugs, ear irrigations, and the use of cotton-tipped swabs to clean or scratch the ear canal. Keeping the ear “too clean” can also contribute to the development of OE, since cerumen actually serves as a protective barrier to the underlying skin and its acidic pH inhibits bacterial and fungal growth. The most common organisms in OE are Staphylococcus aureus and Pseudomonas aeruginosa.
Symptoms include pain, aural fullness, decreased hearing, and sometimes itching in the ear. Manipulation of the pinna or tragus causes considerable pain. Discharge may start out as clear then become purulent. It may also cause secondary eczema of the auricle. The ear canal is typically swollen and narrowed, and the patient may resist any attempt to insert an otoscope. Debris is present in the canal and it is usually very difficult to visualize the TM due to canal edema.
If untreated, facial cellulitis 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.
Management includes pain control, removal of debris from the canal, topical antimicrobial therapy, and avoidance of causative factors. Fluoroquinolone eardrops are first-line therapy for OE. In the absence of systemic symptoms, children with OE should be treated with antibiotic eardrops only. The topical therapy chosen must be nonototoxic because a perforation or patent tube may be present; if the TM cannot be visualized, then 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 the 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.
Children with intact TMs who are predisposed to external otitis may be prophylaxed with two to three drops of a 1:1 solution of white vinegar and 70% ethyl alcohol in the ears before and after swimming.
Kaushik V, Malik T, Saeed SR: Interventions for acute otitis externa. Cochrane Database Syst Rev (1):CD004740, 2010 [PMID: 20091565].
Rosenfeld RM et al: Clinical practice guideline: acute otitis externa. Otolaryngol Head Neck Surg. 2006 Apr;134(4 Suppl):S4–S23 [PMID: 1668473].
Waitzman AA, et al: Otitis externa, http://emedicine.medscape.com/article/994550-overview. Accessed January 11, 2014.
Bullous myringitis (BM) is inflammation of the TM with hemorrhagic or serous bullae, and has been associated with viral upper respiratory infections and Streptococcus pneumoniae. It is often very painful and may be associated with otorrhea and decreased hearing. BM is treated with antibiotics, analgesics, anti-inflammatories, and occasionally steroids.
Acute Otitis Media
Acute otitis media (AOM) is the most common reason why 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).
ESSENTIALS OF DIAGNOSIS
Moderate to severe bulging of the TM or new otorrhea not associated with OE.
Mild bulging of the TM and less than 48 hours of otalgia (ear holding, tugging, or rubbing in a nonverbal child) or intense erythema of the TM.
Middle ear effusion (MEE), proven by pneumatic otoscopy or tympanometry, must be present.
Otitis media with effusion (OME), BM, acute mastoiditis, and middle ear mass.
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). In order 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 sometimes squamous exudate or bullae on the eardrum.
Figure 18–1. Tympanic membrane.
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. It must periodically open to prevent the development of negative pressure and effusion in the middle ear space. If this does not occur, 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, floppier, wider, and more horizontal than in adults. Infants with craniofacial disorders, such as Down syndrome or cleft palate, may be particularly susceptible to ETD.
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 (URI)—URIs increase colonization of the nasopharynx with otitis pathogens. They impair eustachian tube function by causing adenoid hypertrophy and edema of the eustachian tube itself.
4. Smoke exposure—Passive smoking 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—Breast-feeding reduces the incidence of acute respiratory infections, provides immunoglobulin A (IgA) antibodies that reduce colonization with otitis pathogens, and decreases the aspiration of contaminated secretions into the middle ear space which can occur when a bottle is propped in the crib.
7. Time of year—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 daycare over the past three decades has undoubtedly played a major role in the increase in AOM in the United States.
9. Genetic susceptibility—Although AOM is known to be multifactorial, and no gene for susceptibility has yet been identified, recent studies of twins and triplets suggest that as much as 70% of the risk is genetically determined.
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 if sensitive and comprehensive microbiologic methods are used. 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. The third most common pathogen cited is M catarrhalis, which causes up to 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.
Table 18–1. Microbiology of acute otitis media (AOM).
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. Antibiotic treatment in the preceding 3 months also increases the risk of harboring resistant pathogens. The prevalence of resistant strains no longer varies significantly among geographic areas within the United States, but it does vary worldwide. Lower incidences are found in countries with less antibiotic use. β-Lactamase resistance is seen in 34%–45% of H influenzae, while M catarrhalis approaches 100%.
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 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, poor visualization due to low light intensity, and mistaking the ear canal wall for the membrane. The bulb should be slightly compressed when placing the speculum to allow gentle retraction of the TM and avoid discomfort. The tip of the speculum should not be advanced past the cartilaginous EAC to avoid pressure on the bony canal, which is painful. Light, rapid squeezes of the bulb should be undertaken to maximize visualization and minimize pain.
2. Cerumen removal—Cerumen (ear wax) removal is an essential skill for anyone who cares for children. Impacted cerumen pushed against the TM can cause itching, pain, or hearing loss. Parents should be advised that cerumen actually protects the ear and usually comes out by itself through the natural sloughing and migration of the skin of the ear canal. Parents should never put anything into the ear canal to remove wax.
Cerumen may be safely removed under direct visualization through the operating head of an otoscope, provided two adults are present to hold the child. A size 0 plastic disposable ear curette may be used. Irrigation can also be used to remove hard or flaky cerumen. Wax can be softened with 1% sodium docusate solution, carbamyl peroxide solutions, or mineral oil before irrigation is attempted. Irrigation with a soft bulb syringe is performed with water warmed to 35–38°C to prevent vertigo. A commercial jet tooth cleanser (eg, Water Pik) can be used, but it is important to set it at low power to prevent damage to the TM. A perforated TM or patent tympanostomy tube is a contraindication to any form of irrigation.
A home remedy for recurrent cerumen impaction is a few drops of oil such as mineral or olive oil a couple of times a week, warmed to body temperature to prevent dizziness. Droppers are available at pharmacies.
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 (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 (greater than –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.
Figure 18–2. Four types of tympanograms obtained with Welch-Allyn MicroTymp 2. A: Normal middle ear. B: Otitis media with effusion or acute otitis media. C: Negative middle ear pressure due to eustachian tube dysfunction. D: Patent tympanostomy tube or perforation in the tympanic membrane. Same as B except for a very large middle ear volume.
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, mild to moderate pain should be treated with ibuprofen or acetaminophen. Severe pain should be treated with narcotics, but careful and close observation is required to address possible respiratory depression, altered mental status, gastrointestinal upset and constipation. Topical analgesics have a very short duration and studies do not support efficacy in children younger than 5 years.
B. The Observation Option
Since the release of clinical practice guidelines in 2004, watchful waiting with close observation has been recommended in certain groups of children with AOM. The updated 2013 guidelines modify the initial recommendations, including the laterality of infection and otorrhea as criteria (Table 18–2). The choice to observe is an option in otherwise healthy children 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. For infants younger than 6 months, antibiotics are always recommended on the first visit, regardless of diagnostic certainty.
Table 18–2. Recommendations for initial management of uncomplicated AOM.a
C. Antibiotic Therapy
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 is generally considered safe, low-cost, palatable, and has a narrow microbiologic spectrum.
Amoxicillin-clavulanate enhanced strength (ES), with 90 mg/kg/d 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). The regular strength formulations of amoxicillin-clavulanate (7:1 ratio) should not be doubled in dosage to achieve 90 mg/kg/d of amoxicillin, because the increased amount of clavulanate will cause diarrhea.
Table 18–3. Antibiotic therapies for acute otitis media.
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). Unfortunately, coverage of highly penicillin-resistant pneumococci with these agents is poor and only the intermediate-resistance classes are covered. Of these drugs, cefdinir suspension is most palatable; the other two have a bitter after-taste which is difficult to conceal. Newer flavoring agents may be helpful here.
A second-line antibiotic is indicated when a child experiences symptomatic infection within 1 month of finishing amoxicillin; however, repeat use of high-dose amoxicillin is indicated if more than 4 weeks have passed without symptoms. Macrolides such as azithromycin and clarithromycin are not recommended as second-line agents because S pneumoniae is resistant to macrolides in approximately 30% in respiratory isolates, and because virtually all strains of H influenzae have an intrinsic macrolide efflux pump, which pumps antibiotic out of the bacterial cell.
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%. Multi-drug resistant S pneumoniae poses a treatment dilemma and newer antibiotics may need to be employed such as fluoroquinolones or linezolid. 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.
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. Tympanocentesis can be performed with an open-headed operating otoscope or a binocular microscope with either a spinal needle and 3-mL syringe, or if available, an Alden-Senturia trap (Storz Instrument Co., St. Louis, MO) or the Tymp-Tap aspirator (Xomed Surgical Products, Jackson, FL) (Figure 18–3).
Figure 18–3. Operating head and Alden-Senturia trap for tympanocentesis. Eighteen-gauge spinal needle is attached and bent.
E. Prevention of Acute Otitis Media
1. Antibiotic prophylaxis—Strongly discouraged. Antibiotic resistance is a concern and studies have shown poor efficacy.
2. Lifestyle modifications—Parental education plays a major role in decreasing AOM. These are some items to consider in children with RAOM:
• Smoking is a risk factor both for upper respiratory infection and AOM. Primary care physicians can provide information on smoking cessation programs and measures.
• Breast-feeding protects children from AOM. Clinicians should encourage exclusive breast-feeding 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. In Finland, removing pacifiers from infants was shown to reduce AOM episodes by about one-third compared to a control group. The mechanism is uncertain but likely to be related to effects on the eustachian tube. However, the benefit of pacifier use in reducing sudden infant death syndrome (SIDS)–related deaths is also important to consider. Currently, the recommendation from the American Academy of Family Physicians is to wean a pacifier if used 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) has been used in the United States since 2000, and the 13-valent pneumococcal conjugate vaccine (PCV13) was introduced in 2010. PCV7 was found to produce a 29% reduction in AOM resulting from the seven serotypes found in the vaccine, and overall, PCV7 reduced AOM by 6%–7%, according to a 2009 Cochrane database review. Data are not yet available regarding the effect of PCV13 on AOM at this time. In recent studies, intranasal influenza vaccine reduced the number of influenza-associated cases of AOM by 30%–55%.
Jones WH, Kaleida PH: How helpful is pneumatic otoscopy in improving diagnostic accuracy? Pediatrics 2003;112;510 [PMID: 12949275].
Lieberthal AS, et al: The diagnosis and management of acute otitis media. Pediatrics 2013; 131:e964-e999 [PMID: 23439909].
Wald E: Acute otitis media and acute bacterial sinusitis. Clin Infect Dis 2011;52(S4):S277–S283 [PMID: 21460285].
Sexton S, Natale R: Risks and benefits of pacifiers. Am Fam Physician 2009 Apr 15;79(8):681–685 [PMID: 19405412].
Stockmann C et al: Seasonality of acute otitis media and the role of respiratory viral activity in children. Pediatr Infect Dis J 2012 Dec 17; 314-319 [PMID: 23249910].
http://www.entnet.org/HealthInformation/. Accessed January 11, 2014.
http://www.entnet.org/EducationAndResearch/upload/AAO-PGS-9-4-2.pdf. Accessed January 11, 2014.
http://www.cdc.gov/drugresistance/. Accessed January 11, 2014.
Otitis Media With Effusion
ESSENTIALS OF DIAGNOSIS
MEE with decreased TM mobility.
No signs or symptoms of acute inflammation.
May precede or follow an episode of AOM.
Otitis media with effusion (OME) is the presence of fluid in the middle ear space without signs or symptoms of acute inflammation. There may be some discomfort, but acute pain is not characteristic. A retracted or neutral TM with decreased mobility is seen on pneumatic otoscopy. The TM may be opacified or may have a whitish or amber discoloration.
Children with OME can develop AOM if the middle ear fluid should become infected. OME often follows an episode of AOM. 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.
An audiology evaluation should be performed after approximately 3 months of continuous bilateral effusion in children younger than 3 years and those at risk of language delay due to socioeconomic circumstances, craniofacial anomalies, or other risk factors. 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
Traditionally, OME was observed for 3 months in uncomplicated cases prior to consideration for tympanostomy tube placement. More recent studies have found that longer periods of observation do not necessarily have significant negative effects on literacy, attention, academic achievement, and social skills. 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. Absolute indications for tympanostomy tubes include hearing loss greater than 40 dB, TM retraction pockets, ossicular erosion, adhesive atelectasis, and cholesteatoma. If a patient clears a persistent MEE, the physician should follow the patient monthly.
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.
The presence of biofilms has been found to explain the “sterile” effusion sometimes present when culturing middle ear fluid in OME. On electron microscopy, biofilms have been found to cover the middle ear mucosa and adenoids in up to 80% of patients with OME. Currently, work is being done to find ways to eradicate biofilms, including physical disruption, reduction, and eradication.
Paradise JL et al; Tympanostomy tubes and developmental outcomes at 9 to 11 years of age. New Eng J Med 2007;356:3 [PMID: 17229952].
Rosenfeld RM et al: Clinical practice guideline: otitis media with effusion. Otolaryngol Head Neck Surg 2004;130:S95 [PMID: 15138413].
Smith A, Buchinsky FJ, Post JC: Eradicating chronic ear, nose, and throat infections: a systematically conducted literature review of advances in biofilm treatment. Otolaryngol Head Neck Surg 2011 Mar;144(3):338–347 [Review] [PMID: 21493193].
Complications of Otitis Media
A. Tympanosclerosis, Retraction Pockets, Adhesive Otitis
Tympanosclerosis is an acquired disorder of calcification and scarring of the TM and middle ear structures from inflammation. 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. If tympanosclerosis involves the ossicles, a conductive hearing loss may result. Myringosclerosis rarely causes a hearing loss, unless the entire TM is involved (“porcelain eardrum”). Myringosclerosis may be confused with a middle ear mass. However, use of pneumatic otoscopy can often help in differentiation, as the plaque will move with the TM during insufflation.
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.
A greasy-looking mass or pearly white mass seen in a retraction pocket or perforation suggests a cholesteatoma (Figure 18–4). 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.
Figure 18–4. Attic cholesteatoma, formed from an in-drawing of an attic retraction pocket.
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 after 3–6 months, surgical repair may be needed. TM repair is generally delayed until the child is older and eustachian tube function has improved. Procedures include paper patch myringoplasty, fat myringoplasty, and tympanoplasty. Tympanoplasty is generally deferred until around 7 years of age, which is approximately when the eustachian tube reaches adult orientation. In otherwise healthy children, some surgeons perform a repair earlier if the contralateral, nonperforated ear remains free of infection and effusion for 1 year.
In the presence of a perforation, water activities should be limited to surface swimming, preferably with the use of an ear mold.
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
ESSENTIALS OF DIAGNOSIS
Ongoing purulent ear drainage.
Nonintact TM: perforation or tympanostomy tubes.
May be associated with cholesteatoma.
Chronic suppurative otitis media (CSOM) is present when persistent otorrhea occurs in a child with tympanostomy tubes or TM perforations. It starts with an acute infection that becomes chronic with mucosal edema, ulceration, and granulation tissue. 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.
Roland PS, et al: Chronic suppurative otitis media. http://emedicine.medscape.com/article/859501-overview. Accessed January 11, 2014.
Suppurative infections of the middle ear can spread into the membranous labyrinth of the inner ear through the round window membrane or abnormal congenital communications. Symptoms include vertigo, hearing loss, fevers, and 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.
ESSENTIALS OF DIAGNOSIS
AOM otitis media is almost always present.
Postauricular pain and erythema.
Ear protrusion (late finding).
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.
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. 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 computed tomography (CT) scan. Early mastoiditis may be 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.
The most common pathogens are S pneumoniae followed by H influenzae and S pyogenes . Rarely, gram-negative bacilli and anaerobes are isolated. Antibiotics may 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. Moreover, 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.
Lymphadenitis, parotitis, trauma, tumor, histiocytosis, OE, and furuncle.
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. 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.
In the preantibiotic era, up to 20% of patients with AOM underwent mastoidectomy for mastoiditis. Now, the occurrence is 5 cases per 100,000 persons with AOM. 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 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.
Bakhos D et al: Conservative management of acute mastoiditis in children. Arch Otolaryngol Head Neck Surg 2011 Apr;137(4):346–350 [PMID: 21502472].
Brook I: Pediatric mastoiditis. http://emedicine.medscape.com/article/966099-overview. Accessed January 11, 2014.
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 disruption, and hematoma of the middle ear. One study reported that 50% of serious penetrating wounds of the TM were due to parental use of a cotton swab.
Treatment of middle ear trauma consists mainly of watchful waiting. An audiogram may show a conductive hearing loss. Antibiotics are not necessary unless signs of infection appear. The prognosis for normal hearing depends on whether the ossicles have been dislocated or fractured. 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. A CT scan may be needed to evaluate the middle ear structures. Occasionally, middle ear trauma can lead to a perilymphatic fistula (PLF) which is disruption of the barrier between the middle ear and inner ear, usually in the area of the oval window. PLF can occur from an acute blow to the ear, foreign body, or occasionally from more innocuous mechanisms such as Valsalva or sneezing. Symptoms include sudden hearing loss and vertigo. This requires emergent otolaryngology evaluation.
Traumatic TM perforations should be referred to an otolaryngologist for examination and hearing evaluation. Spontaneous healing may occur within 6 months of the perforation. If the perforation is clean and dry and there is no hearing change, there is no urgency for specialty evaluation. In the acute setting, antibiotic eardrops are often recommended to provide a moist environment which is thought to speed healing.
CERUMEN IMPACTION AND EAR CANAL FOREIGN BODY
Foreign bodies in the ear are common, whether intentional (self-placement, placement by another child) or accidental (eg, insect, broken-off cotton swab after cleaning attempt). Cerumen can also be obstructive, acting like a foreign body. If the cerumen or object is large or difficult to remove with available instruments, otolaryngology referral is recommended, as the child may be traumatized by further removal attempts, and trauma to the ear canal may cause edema, necessitating removal under general anesthesia. Vegetable matter should never be irrigated as it can swell causing increased pain and removal difficulty. An emergency condition exists if the foreign body is a disk-type battery, such as those used in clocks, watches, and hearing aids. 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.
Sharpe SJ, Rochette LM, Smith GA: Pediatric battery-related emergency department visits in the United States, 1990–2009. Pediatrics 2012 Jun;129(6):1111–1117 [PMID: 22585763].
Trauma 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 perichondrial layer. A hematoma appears as a boggy purple swelling of the cartilaginous auricle, and the normal folds of the ear are obscured. If untreated, pressure necrosis of the underlying cartilage may occur, resulting in “cauliflower ear.” To prevent this cosmetic deformity, physicians should urgently refer patients to an otolaryngologist for drainage and application of a carefully molded pressure dressing.
CONGENITAL EAR MALFORMATIONS
Atresia is agenesis of the EAC. 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. Often, there is an associated EAC atresia. Reconstruction can be performed in girls around age 6 and in boys around age 8–10 years.
“Lop ears,” folded down, or protruding ears (“Dumbo ears”) can be a source of much teasing and ridicule. 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 and continued for 2 weeks. If this window of opportunity is missed or taping is unsuccessful, another option for this sort of deformity is an otoplasty procedure.
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. Children exhibiting any of these findings should have their hearing tested. Renal ultrasound should be considered, as external ear anomalies can be associated with renal anomalies, as both structures form during the same period of embryogenesis. Most preauricular pits cause no symptoms but can become infected. If one should become infected, the patient should receive antibiotic therapy and be referred to an otolaryngologist for eventual excision.
Brent B: Microtia repair with rib cartilage grafts: a review of personal experience with 1000 cases. Clin Plast Surg 2002;29:257 [Classic] [PMID: 12120682].
Fritsch MH: Incisionless otoplasty. Otolaryngol Clin North Am 42(6):1199–1208 [PMID: 19962016].
Yotsuyanagi T et al: Nonsurgical treatment of various auricular deformities. Clin Plast Surg 2002;29:327 [Classic] [PMID: 12120687].
IDENTIFICATION & MANAGEMENT OF HEARING LOSS
Hearing loss is classified as being conductive, sensorineural, or mixed in nature. Conductive hearing loss occurs when there is a blockage of sound transmission 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 15 dB of normal. In children, severity of hearing loss is commonly graded as follows: 16–25 dB slight, 26–40 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 impairment 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 eustachian tube dysfunction. Other causes of conductive hearing loss 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
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
Nonhereditary causes account for approximately 50% of congenital hearing loss. These include prenatal infections, 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 impairment 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 also be 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 hearing loss include the following: Waardenburg, branchio-oto-renal, Usher, Pendred, Jervell and Lange-Nielsen, and Alport.
Over 70% of hereditary hearing loss is nonsyndromic (ie, there are no associated visible abnormalities or related medical problems). The most common known mutation associated with nonsyndromic hearing loss is in the GJB2gene, 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 impairment. 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.
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, every state and territory in the United States has an Early Hearing Detection and Intervention (EDHI) program, with a goal of hearing loss identification 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 as well as professionals. Deaf infants are often visually alert and able to scan the environment so actively that this can 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, unclear speech, and turning the volume up on equipment such as televisions or radios. If there is any suspicion of hearing loss, it is important to refer the patient for formal audiometric testing by an audiologist who is experienced in 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 these listed methods.
A child who fails newborn hearing screening or has a suspected hearing loss should be referred for further audiologic evaluation, and any child with hearing loss should be referred to an otolaryngologist for further workup and treatment. In addition to the 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 occur, necessitating 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 should receive ongoing audiologic monitoring for 3 years and at appropriate intervals thereafter to avoid a missed diagnosis.
Appropriate care may treat or prevent 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 certain mitochondrial gene defect and aminoglycoside ototoxicity, use should be avoided, if 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.
Management of Hearing Loss
Children with hearing loss should be referred to an otolaryngologist for etiologic workup which may include radiographic imaging and/or laboratory tests. Workup is directed by each individual patient’s history, examination, and audiologic results. As discussed previously, hearing loss may be an isolated abnormality or may be part of a larger syndrome. Within the past couple of years, comprehensive genetic testing for syndromic and nonsyndromic hearing loss using next-generation sequencing technology has become widely available to the public.
The importance of early intervention for receptive and expressive development is well-established. The management of hearing loss depends on the type and severity of impairment. Conductive hearing loss is typically correctable by addressing the point in sound transmission at which efficiency is compromised. 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, although cochlear hair cell regeneration is an area of active research. Most sensorineural loss is managed with amplification. Cochlear implantation is an option for some children with severe-profound loss, and at the time of this writing, 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. Children with hearing loss should receive ongoing audiologic monitoring.
Smith RJH, Shearer AE, Hildebrand MS, Van Camp G: Deafness and hereditary hearing loss overview. Last update January 3, 2013. http://www.ncbi.nlm.nih.gov/books/NBK1434/. Accessed January 11, 2014.
http://www.asha.org/public/hearing/Prevalence-and-Incidence-of-Hearing-Loss-in-Children/. Accessed January 11, 2014.
http://www.infanthearing.org/screening/. Accessed January 11, 2014.
THE NOSE & PARANASAL SINUSES
ACUTE VIRAL RHINITIS (COMMON COLD; SEE ALSO CHAPTER 38)
The common cold (viral upper respiratory infection) is the most common pediatric infectious disease, and the incidence is higher in early childhood than in any other period of life. Children younger than 5 years typically have 6–12 colds per year. Approximately 30%–40% of these are caused by rhinoviruses. Other culprits include adenoviruses, coronaviruses, enteroviruses, influenza and parainfluenza viruses, and respiratory syncytial virus.
ESSENTIALS OF DIAGNOSIS
Clear or mucoid rhinorrhea, nasal congestion, sore throat.
Possible fever, particularly in younger children (under 5–6 years).
Symptoms resolve by 7–10 days.
Rhinosinusitis (acute or chronic), allergic rhinitis, nonallergic rhinitis, influenza, pneumonia, gastroesophageal reflux disease, asthma, and bronchitis.
The patient usually experiences a sudden onset of clear or mucoid rhinorrhea, nasal congestion, sneezing, and sore throat. Cough or fever may develop. Although fever is usually 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 2–3 weeks following resolution of other symptoms.
Treatment for the common cold is symptomatic (Figure 18–5). Because colds are vial infections, antibiotics will not cure or shorten their length. Acetaminophen or ibuprofen can be helpful for 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.
Figure 18–5. Algorithm for acute nasal congestion and rhinosinusitis.
Available scientific data suggest that over-the-counter cold and cough medications are generally not effective in children. These medications may be associated with serious adverse effects, and it is recommended that they not be used in children under the age of 4 years. Antihistamines have not proven effective in relieving cold symptoms; in rhinoviral colds, increased levels of histamine are not observed. Oral decongestants have been found to provide some symptomatic relief in adults but have not been well-studied in children. Cough suppression at night is the number one goal of many parents; however, studies have shown that antitussives, antihistamines, antihistamine-decongestant combinations and antitussive-bronchodilator combinations are no more effective than placebo. Use of narcotic antitussives is discouraged, as these have been associated with severe respiratory depression.
Education and reassurance may be the most important “therapy” for the common cold. Parents 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.
Arroll B, Kenealy T: Are antibiotics effective for acute purulent rhinitis? Systematic review and meta-analysis of placebo controlled randomized trials. BMJ 2006;333(7562):279. Epub 2006 July 21. [PMID: 16861253].
Smith SM, Schroeder K, Fahey T: Over-the-counter medications for acute cough in children and adults in ambulatory settings. Cochrane Database Syst Rev 2008;(1):CD001831 [PMID: 18253996].
Taverner D, Latte J: Nasal decongestants for the common cold. Cochrane Database Syst Rev 2007;(1):CD001953 [PMID: 17253470].
U.S. Food and Drug Administration: Using over-the-counter cough and cold products in children. Posted Oct 22, 2008. http://www.fda.gov/ForConsumers/ConsumerUpdates/ucm048515.htm. Accessed January 11, 2014.
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 in which the symptoms resolve completely. It is almost always preceded by a viral upper respiratory infection (cold). Other predisposing conditions include allergies and trauma. The diagnosis of ABRS is made when a child with a cold does not improve by 10–14 days or worsens after 5–7 days. The maxillary and ethmoid sinuses are most commonly involved. These sinuses are present at birth. 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.
ESSENTIALS OF DIAGNOSIS
Upper respiratory infection symptoms are present 10 or more days beyond onset, or symptoms worsen within 10 days after an initial period of improvement.
Symptoms may include nasal congestion, nasal drainage, postnasal drainage, facial pain, headache, and fever.
Symptoms resolve completely within 30 days.
Situations which lead to inflammation of sinonasal mucosa and obstruction of sinus drainage pathways underlie the development of rhinosinusitis. A combination of anatomic, mucosal, microbial, and immune pathogenic factors are believed to be involved. Both viral and bacterial infections play integral roles in the pathogenesis. Viral upper respiratory infections may cause sinus mucosal injury and swelling, resulting in osteomeatal obstruction, loss of ciliary activity, and mucus hypersecretion. The bacterial pathogens that commonly cause acute rhinosinusitis are S pneumoniae, H influenzae (nontypeable), M catarrhalis, and β-hemolytic streptococci.
The onset of symptoms in ABRS may be gradual or sudden, and may 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. Occasionally, sinuses may be tender to percussion, but this is typically seen only in older children and is of questionable reliability.
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 indicated except when 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 of ABRS occur when infection spreads to adjacent structures—the overlying tissues, the eye, or the brain. S aureus (including methicillin-resistant S aureus [MRSA]) 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 15).
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.
For children who are not improving by 10 days, or who have more severe symptoms, with fever of at least 39°C and purulent nasal drainage for at least 3–4 consecutive days, antibiotic therapy is recommended. Although some discrepancy exists, antibiotics are generally thought to decrease duration and severity of symptoms.
To minimize the number of children who receive antimicrobial therapy for uncomplicated viral upper respiratory infections, and to help combat antibiotic resistance, the American Academy of Pediatrics in 2001 issued guidelines for treatment. This algorithm is presented in Figure 18-6. Key decision points include severity of disease and risk factors for resistant organisms
Figure 18–6. Management of children with uncomplicated acute bacterial rhinosinusitis (ABRS). (Reproduced, with permission, from the American Academy of Pediatrics: Clinical practice guidelines: management of sinusitis. Pediatrics 2001;108:798.)
For patients with mild-moderate symptoms, who are not in day care, and have not been on recent antibiotic therapy, high-dose amoxicillin is considered first-line therapy. For those with severe symptoms, in day care, or who were on antibiotics within the past 1–3 months, high-dose amoxicillin–clavulanate is recommended as first-line therapy. Cefuroxime, cefpodoxime, and cefdinir are recommended for patients with a non–type I hypersensitivity to penicillin. Macrolides should be reserved for patients with an anaphylactic reaction to penicillin. Other options for these patients include clindamycin or trimethoprim–sulfamethoxazole. However, it should be remembered that clindamycin is not effective against gram-negative organisms such as H influenzae.
Failure to improve after 48–72 hours 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 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 cefotaxime 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 is no evidence-based data supporting their efficacy, and concern has been raised about potential adverse effects related to impaired ciliary function, decreased blood flow to the mucosa, and reduced diffusion of antibiotic into the sinuses with the use of decongestants. 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.
Principi N, Esposito S: New insights into pediatric rhinosinusitis. Pediatr Allergy Immunol 2007;18 (Suppl 18):7–9 [PMID: 17767598].
Shaikh N, Wald ER, Pi M: Decongestants, antihistamines and nasal irrigation for acute sinusitis in children. Cochrane Database Syst Rev 2012;9:CD007909. doi: 10.1002/14651858.CD007909.pub3 [PMID: 22972113].
2. Recurrent or Chronic Rhinosinusitis
Recurrent rhinosinusitis occurs when episodes of ABRS clear with antibiotic therapy but recur with each or most upper respiratory infections. Chronic rhinosinusitis (CRS) is diagnosed when the child has not cleared the infection in the expected time but has not developed acute complications. Both symptoms and physical findings are required to support the diagnosis, and CT scan may be a useful adjuvant 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 chronic rhinosinusitis. 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 Chapters 18 and 31). Anaerobic and staphylococcal organisms are often responsible for CRS. Evaluation by an allergist and an otolaryngologist may be useful in determining the underlying causes.
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. 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. Balloon catheter dilation—This procedure opens up the maxillary sinuses without removal of tissue to promote drainage. Preliminary studies indicate that this may be effective in children who have failed adenoidectomy.
4. 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.
5. External drainage—External drainage procedures are reserved for complications arising from ethmoid and frontal sinusitis.
Harvey R, Hannan SA, Badia L, Scadding G: Nasal saline irrigations for the symptoms of chronic rhinosinusitis. Cochrane Database Syst Rev 2007;3:CD006394. doi: 10.1002/14651858.CD006394.pub2 [PMID: 17636843].
Makary CA, Ramadan HH: The role of sinus surgery in children. Laryngoscope 2013 Jan 29. [Epub ahead of print] [PMID: 23361382].
Snidvongs K, Kalish L, Sacks R, Craig JC, Harvey RJ: Topical steroid for chronic rhinosinusitis without polyps. Cochrane Database Syst Rev 2011:CD009274. doi: 10.1002/14651858.CD009274 [PMID: 21833974].
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 6-Fr 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 35) or other congenital anomalies.
Recurrent rhinitis is frequently seen in the office practice of 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 symptoms, 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.
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 which can be very difficult to treat. Oral decongestants, nasal corticosteroids, antihistamines, and ipratropium spray have all been shown to offer symptomatic relief.
Rachelefsky G, Farrar JR: A control model to evaluate pharmacotherapy for allergic rhinitis in children. JAMA Pediatr 2013;167(4):380–386 [PMID: 23440263].
American Academy of Allergy, Asthma, and Immunology: Rhinitis. http://www.aaaai.org/conditions-and-treatments/allergies/rhinitis.aspx. Accessed January 11, 2014.
The nose is an extremely vascular structure. In most cases, epistaxis (nosebleed) arises from the anterior portion of the nasal septum (Kiesselbach area). It is often due to dryness, vigorous 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 extraction; 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.
A nasopharyngeal angiofibroma may manifest as recurrent epistaxis. Adolescent boys are affected almost exclusively. CT scan with contrast of the nasal cavity and nasopharynx is diagnostic.
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 by the clock. 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.
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 usually follows 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.
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 these can cause septal necrosis, leading to 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 evacuation of the hematoma 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 at removal.
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.
THE THROAT & ORAL CAVITY
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 or on the tongue; rarely they may appear on the tonsils or palate. There is usually no associated fever or cervical adenopathy. They can last 1–2 weeks and may recur numerous times throughout life. The cause is unknown, although an allergic or autoimmune basis is suspected.
A topical corticosteroid, such as triamcinolone dental paste, may reduce duration of the lesion. Pain can also be reduced by eating a bland diet, avoiding salty or acidic foods and juices, and by taking acetaminophen or ibuprofen. A recent 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). PFAPA syndrome was first described in 1987, and its cause is unknown, although an immune etiology is suspected. It usually 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. Episodes may be dramatically improved with prednisone bursts, but recurrences typically continue. PFAPA may resolve with prolonged cimetidine treatment. Tonsillectomy has been shown to be effective in curing this condition. Otolaryngology referral is appropriate.
A diagnosis of 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.
Brocklehurst P et al: Systemic interventions for recurrent aphthous stomatitis (mouth ulcers). Cochrane Database Syst Rev 2012 Sep 12;9:CD005411. doi: 10.1002/14651858.CD005411.pub2 [Review] [PMID: 22972085].
Licameli G, Lawton M, Kenna M, Dedeoglu F: Long-term surgical outcomes of adenotonsillectomy for PFAPA syndrome. Arch Otolaryngol Head Neck Surg 2012 Oct;138(10):902–906. doi: 10.1001/2013.jamaoto.313 [PMID: 23069819].
2. Herpes Simplex Gingivostomatitis (See also Chapter 38)
In the initial infection with the herpes simplex virus, children usually develop 10 or more small ulcers (1–3 mm) on the buccal mucosa, anterior tonsillar pillars, inner lips, tongue, and gingiva; the posterior pharynx is typically spared. The lesions are associated with fever, tender cervical adenopathy, and generalized oral inflammation which precedes the development of the ulcers. This gingivostomatitis lasts 7–10 days. Exposure to the virus occurs 3–50 days prior to the onset of symptoms. Affected children are commonly younger than 3 years. Treatment is symptomatic, as described earlier for recurrent aphthous stomatitis, with the exception that corticosteroids are contraindicated. Acyclovir therapy may be initiated if the child is seen early in the course. Because of pain, dehydration occasionally develops, requiring hospitalization. Herpetic laryngotracheitis is a rare complication.
3. Thrush (See also Chapter 41)
Oral candidiasis mainly affects infants and occasionally older children in a debilitated state. Candida albicans is a saprophyte that normally is not invasive unless the mouth is abraded or the patient is immunocompromised. The use of broad-spectrum antibiotics and systemic or inhaled corticosteroids may be contributing factors. Symptoms include oral pain and refusal of feedings. Lesions consist of white curdlike plaques, predominantly on the buccal mucosa, which cannot be washed away after a feeding. Another less common variation of oral candidal infection is erythematous candidiasis, which produces erythematous patches on the palate and dorsum of the tongue. This condition is associated primarily with patients who are taking broad-spectrum antibiotics or corticosteroids, or are human immunodeficiency virus (HIV) positive.
Treatment consists of nystatin oral suspension. Large plaques may be removed with a moistened cotton swab, and the nystatin may be applied to the lesions with a swab. Patients who do not respond to oral therapy or are immunocompromised may require systemic antifungal agents. Parents should be advised to replace any items, such as a pacifier, that may have become contaminated with Candida.
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 also occur with leukemia or on a recurrent basis with cyclic neutropenia.
Figure 18–7 is an algorithm for the management of a sore throat.
Figure 18–7. Algorithm for pharyngitis. CBC, complete blood count; EBV, Epstein-Barr virus; ENT, ear, nose, and 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.
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 age 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.
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. Enteroviral polymerase chain reaction testing is widely available, but not typically indicated, as herpangina 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
ESSENTIALS OF DIAGNOSIS
At least one of the following:
Cervical lymphadenopathy (lymph nodes tender or > 2 cm)
Positive group A β-hemolytic streptococcus culture
Fever greater than 38.3°C
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 infection. It is most common in children between 5 and 15 years of age 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.
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 upper respiratory infection, 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.
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.
Table 18–4. Treatment of group A streptococcal pharyngitis.a
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 group A strep 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.
Baugh RF et al; American Academy of Otolaryngology-Head and Neck Surgery Foundation: Clinical practice guideline: tonsillectomy in children. Otolaryngol Head Neck Surg 2011 Jan;144 (1 Suppl):S1–S30 [PMID: 21493257].
Wessels MR: Clinical practice. Streptococcal pharyngitis. New Engl J Med 2011;364(7):648–655 [PMID: 21323542].
National Institute of Mental Health PANDAS information: http://intramural.nimh.nih.gov/pdn/web.htm. Accessed January 11, 2014.
PERITONSILLAR CELLULITIS OR ABSCESS (QUINSY)
ESSENTIALS OF DIAGNOSIS
Severe sore throat.
Unilateral tonsillar swelling.
Deviation of the uvula.
Trismus (limited mouth opening).
Tonsillar infection occasionally penetrates the tonsillar capsule, spreading to the surrounding tissues, causing peritonsillar cellulitis. If untreated, necrosis occurs and a peritonsillar abscess forms. The most common pathogen is β-hemolytic streptococcus, but others include group D streptococcus, S pneumoniae, and anaerobes.
The patient complains of a severe sore throat even before the physical findings become marked. A 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.
It is often difficult to differentiate peritonsillar cellulitis from abscess. In some children, it is possible to aspirate the peritonsillar space to diagnose and treat an abscess. However, it is reasonable to admit a child for 12–24 hours of intravenous antimicrobial therapy, because aggressive treatment of cellulitis can usually prevent suppuration. Therapy with a penicillin or clindamycin is appropriate. Failure to respond to therapy during the first 12–24 hours indicates a high probability of abscess formation. An otolaryngologist should be consulted for incision and drainage or for aspiration under local or general anesthesia.
Recurrent peritonsillar abscesses are so uncommon (7%) that routine tonsillectomy for a single incident is not indicated unless other tonsillectomy indications exist. Hospitalized patients can be discharged on oral antibiotics when fever has resolved for 24 hours and dysphagia has improved.
Retropharyngeal lymph nodes, which drain the adenoids, nasopharynx, and paranasal sinuses, can become infected, commonly due to β-hemolytic streptococci and S aureus. If this pyogenic adenitis goes untreated, a retropharyngeal abscess forms. This occurs most commonly during the first 2 years of life. After this age, the most common cause of retropharyngeal abscess is superinfection of a penetrating injury of the posterior wall of the oropharynx.
The diagnosis of retropharyngeal abscess should be strongly suspected in a child with fever, respiratory symptoms, and neck hyperextension. Dysphagia, drooling, dyspnea, and gurgling respirations are also found. 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. On lateral neck x-ray, the retropharyngeal tissues are wider than the C4 vertebral body. But plain films are not specific; a CT scan with contrast is more helpful.
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. Immediate surgical drainage is required when a definite abscess is seen radiographically or when the airway is compromised. 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 increase. A child with retropharyngeal abscess will not improve and may continue to deteriorate. A surgeon should incise and drain the abscess under general anesthesia to prevent its extension.
Ludwig angina is a rapidly progressive cellulitis of the submandibular space that can cause airway obstruction and death. The submandibular space extends from the mucous membrane of the floor of the mouth to the muscular and fascial attachments of the hyoid bone. This infection is unusual in infants and children. The initiating factor in over 50% of cases is dental disease, including abscesses and extraction. Some patients have a history of injury to the floor of the mouth. Group A strep is the most common organism identified.
Symptoms include fever and tender swelling of the floor of the mouth. The tongue can become tender and inflamed. Upward displacement of the tongue may cause dysphagia, drooling, and airway obstruction.
Treatment consists of high-dose intravenous clindamycin or ampicillin plus nafcillin until the culture results and sensitivities are available. Because the most common cause of death in Ludwig angina is sudden airway obstruction, the patient must be monitored closely in the intensive care unit and intubation provided for progressive respiratory distress. 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, and the remainder to viruses, atypical mycobacteria, and Bartonella henselae. MRSA must also be considered.
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. Blood should be drawn 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.
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.
Hegde AN, Mohan S, Pandya A, Shah GV: Imaging in infections of the head and neck. Neuroimaging Clin N Am 2012 Nov;22(4):727–754. doi: 10.1016/j.nic.2012.05.007 [Epub 2012 Aug 11] [PMID: 23122264].
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 firm to hard in 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 in the midline of the neck, usually located 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% of lymphatic malformations are noted at birth; the remaining malformations are usually seen by the age of 2 years. If these become large enough, they can compromise the patient’s ability to swallow and breathe.
4. Parotitis—The most common pitfall is mistaking parotitis 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 cyst in the floor of the mouth caused by obstruction of the sublingual salivary gland. A “plunging” ranula extends 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.
SNORING, MOUTH BREATHING, & UPPER AIRWAY OBSTRUCTION
The American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) defines sleep-disordered breathing (SDB) as an abnormal respiratory pattern during sleep that includes snoring, mouth breathing, and pauses in breathing. SDB encompasses a spectrum of disorders that ranges in severity from snoring to obstructive sleep apnea. SDB is a presumptive diagnosis; OSA can be diagnosed only by polysomnogram (PSG).
In 2012, the American Academy of Pediatrics (AAP) updated their clinical practice guideline for the diagnosis and management of uncomplicated childhood obstructive sleep apnea syndrome. The guideline emphasizes that pediatricians should screen all children for snoring. If the child exhibits additional signs and symptoms of SDB, referral for a sleep study is recommended, but referral to an otolaryngologist or sleep specialist is also an option. The American Academy of Sleep Medicine (AASM) has similar recommendations.
The gold standard for diagnosis of OSA is a polysomnogram (PSG), commonly called a “sleep study”. A patient’s history and clinical examination cannot predict the presence or severity of OSA. Similarly, an overnight oximetry study is a poor screening test for OSA, as it may detect patients with severe disease but miss those with milder forms, since obstructive respiratory events can occur without oxygen desaturations. The criteria for diagnosing OSA differ between children and adults. An obstructive event occurs when airflow stops despite persistence of respiratory effort. A hypopnea is counted when airflow and respiratory effort decrease with an associated oxygen desaturation or arousal. Normative values for children are just being established. The AASM states that for children, the occurrence of more than one apneic or hypopneic event per hour with duration of at least two respiratory cycles is abnormal. However, the AASM has qualified its recommendation, stating that the criteria may be modified once more comprehensive data become available. One study of children aged 6–11 years found that a respiratory disturbance index of at least one event per hour, when associated with a 3% oxygen desaturation, was associated with daytime sleepiness and learning problems. If oxygen desaturations were absent, a respiratory disturbance index of five events per hour was associated with clinical symptoms.
Although an obstructive apnea index greater than one event per hour may be statistically significant, whether it is clinically relevant remains unclear. Children with an apnea-hypopnea index of greater than five events per hour appear to have clinically significant OSA. The dilemma is how to manage children with an apnea-plus-hypopnea index of more than one but fewer than five events per hour, as some of these children experience neurocognitive symptoms. Of note, many research studies have shown little correlation between the severity of OSA by PSG and neuropsychological morbidity.
Clinical Evaluation & Management
The AAO-HNS has developed clinical guidelines to help otolaryngologists determine when to proceed with surgical treatment (usually adenotonsillectomy) or request a polysomnogram. A PSG is generally recommended for children with suspected sleep apnea if they have any of the following comorbid conditions: obesity, Down syndrome, craniofacial abnormalities, neuromuscular disorders, sickle cell disease, or mucopolysaccharidoses. PSG is also recommended for children without the above comorbidities if the need for surgery is uncertain, or if there is discordance between tonsillar size on physical examination and the reported severity of symptoms.
Most pediatric otolaryngologists perform adenotonsillectomy in healthy patients with SDB without obtaining a PSG. An adenotonsillectomy without PSG is usually recommended in a healthy child if the following are present:
1. Nighttime symptoms: habitual snoring along with gasping, pauses, or labored breathing. Other symptoms which may be related to SDB include night terrors, sleep walking, and secondary enuresis.
2. Daytime symptoms: unrefreshed sleep, attention deficit, hyperactivity, emotional lability, temperamental behavior, poor weight gain, and daytime fatigue. Other signs include daytime mouth breathing or dysphagia.
3. Enlarged tonsils.
If the three findings cannot be confirmed, but the child has other indications for an adenotonsillectomy—that is, recurrent tonsillitis or markedly enlarged (4+) tonsils with dysphagia—surgery is also warranted.
Figure 18–8 is an algorithm for management of SDB complaints in an otherwise healthy child. The pathway relies on clinical symptoms and tonsil size. Of note, tonsil size alone does not predict the presence of significant SDB. Children without significant tonsil hypertrophy may still have SDB. Factors such as low muscle tone can contribute to an individual’s propensity to experience SDB. Although the pathway states that an asymptomatic child with markedly enlarged tonsils (4+) should undergo PSG, a period of observation is reasonable. One’s clinical suspicion of SDB should be heightened if a child has enlarged tonsils, especially if the parents cannot provide a reliable history. If the child has no clinical symptoms and the tonsils are only moderately enlarged (3+), observation is appropriate. Educating the parents about the risks of SDB and what to look for is paramount.
Figure 18–8. Algorithm for evaluation of snoring in a healthy child.
A polysomnographic study is recommended for a child who has no adenotonsillar hypertrophy or nasal obstruction, but has significant symptoms of SDB. Other conditions, especially a periodic limb movement disorder, may mimic the daytime symptoms of SDB. If a polysomnogram indicates OSA in a child without tonsillar hypertrophy, a complete evaluation of the upper airway by awake flexible laryngoscopy should be performed to look for other possible sites of obstruction: nose, nasopharynx (adenoid), hypopharynx (base of tongue or lingual tonsils), and larynx. The adenoid can also be assessed with a lateral neck x-ray. Alternative methods to evaluate a child for anatomical sites of obstruction include sedated ciné MRI of the upper airway or sleep endoscopy.
As for postoperative PSG, the AASM and AAP agree that a child with mild OSA (typically fewer than five obstructive events an hour) does not require a follow-up study. However, those with persistent symptoms, more severe OSA, obesity, or other comorbidities should routinely have a postoperative study.
Allergic rhinitis is a common cause of nasal obstruction. If allergy is the suspected cause of SDB, a trial of intranasal corticosteroid spray is indicated. If enlarged tonsils (Figure 18–9) or adenoids are present, a referral to an otolaryngologist or a pediatric sleep laboratory is appropriate.
Figure 18–9. A grading scale for tonsil size that ranges from 0 to 4. With grade 0 the tonsils are small and contained within the tonsillar fossa; in grade 4 the tonsils are so large they almost touch (“kissing”). (Reprinted, with permission, from Brodksy L: Modern assessment of tonsils and adenoids. Pediatr Clin North Am 1989;36:1551.)
Complications & Sequelae
The importance of diagnosing SDB in children cannot be underestimated. SDB has been associated with problems in a multitude of realms, including social, behavioral, and neurocognitive. It has been shown to significantly impact quality of life and has been associated with growth impairment and cardiovascular complications. Recent studies also suggest that SDB is associated with systemic inflammation.
Aurora RN et al; American Academy of Sleep Medicine: Practice parameters for the respiratory indications for polysomnography in children. Sleep 2011 Mar 1;34(3):379–388 [PMID: 21359087; PMCID: PMC3041715].
Marcus CL et al; American Academy of Pediatrics: Diagnosis and management of childhood obstructive sleep apnea syndrome. Pediatrics 2012 Sep;130(3):e714–e55. doi: 10.1542/peds.2012-1672 [Epub 2012 Aug 27] [Review] [PMID: 22926176].
Marcus CL et al; American Academy of Pediatrics: Diagnosis and management of childhood obstructive sleep apnea syndrome. Pediatrics 2012 Sep;130(3):576–584. doi: 10.1542/peds. 2012-1671 [Epub 2012 Aug 27] [PMID: 22926173].
Roland PS et al; American Academy of Otolaryngology—Head and Neck Surgery Foundation: Clinical practice guideline: polysomnography for sleep-disordered breathing prior to tonsillectomy in children. Otolaryngol Head Neck Surg 2011 Jul;145 (1 Suppl):S1–S15 [Epub 2011 Jun 15] [PMID: 21676944].
American Academy of Sleep Medicine educational site: http://www.sleepeducation.com. Accessed January 11, 2014.
Child-friendly website: http://www.sleepforkids.org/. Accessed January 11, 2014.
National Sleep Foundation: http://www.sleepfoundation.org/. Accessed January 11, 2014.
http://www.sleepfoundation.org/article/hot-topics/snoring-children. Accessed January 11, 2014.
TONSILLECTOMY & ADENOIDECTOMY
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 earlier discussion of OSA and SDB). Adenotonsillar hypertrophy may also cause dysphagia or dental malocclusion. Rarely, hypertrophied tonsils may lead to pulmonary hypertension or cor pulmonale.
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 section on Recurrent Aphthous Stomatitis, earlier), in which fever recurs predictably, typically every 4–8 weeks. Tonsillectomy has been shown to be an effective treatment.
Baugh RF et al; American Academy of Otolaryngology-Head and Neck Surgery Foundation: Clinical practice guideline: tonsillectomy in children. Otolaryngol Head Neck Surg 2011 Jan;144(1 Suppl):S1-S0. doi: 10.1177/0194599810389949 [PMID: 21493257].
American Academy of Otolaryngology/Head and Neck Surgery–sponsored site dedicated to children: http://www.entnet.org/kidsent/. Accessed January 11, 2014.
The adenoids, composed of lymphoid tissue in the nasopharynx, are 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 is pinched and the maxilla narrowed because the molding pressures of the orbicularis oris and buccinator muscles are unopposed by the tongue. The adenoids 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
Adenoids 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 often 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. It usually is asymptomatic and disappears after cup feeding is initiated.
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.
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.
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
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 hospitalization and 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. Recently, endoscopy of Stensen duct has been investigated not only to confirm the 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.
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. Refer to an otolaryngologist for surgical management.
CONGENITAL ORAL MALFORMATIONS
1. Tongue-Tie (Ankyloglossia)
A short lingual frenulum can hinder both protrusion and elevation of the tongue. Puckering 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. In our practice, neonatal frenulectomy is performed in clinic. Early treatment is favored, as when an infant is even several months old, general anesthesia is required for the procedure to be performed safely.
Buryk M et al: Efficacy of neonatal release of ankyloglossia: a randomized trial. Pediatrics 2011 Aug;128(2):280–288 [PMID: 21768318].
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 (See Chapter 35)
A. Submucous Cleft Palate
A bifid uvula is present in 3% of healthy children. 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. During feeding, some of these infants experience 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.
C. Pierre Robin Sequence
This group of congenital malformations is characterized by the triad of micrognathia, cleft palate, and glossoptosis. Affected children present as emergencies in the newborn period because of infringement on the airway by the tongue. 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. Recently, 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.
Mackay DR: Controversies in the diagnosis and management of the Robin sequence. J Craniofac Surg 2011 Mar;22(2):415–420 [PMID: 21403570].