CHAPTER 196 Pharyngitis and Adenotonsillar Disease
Infectious and inflammatory diseases involving the pharynx, tonsils, and adenoids account for a significant proportion of childhood illnesses and pediatric health care expenditures. They often result in two of the most common surgical procedures of childhood, tonsillectomy and adenoidectomy (Fig. 196-1). Clinical research has now helped illuminate this vast area of pediatric otolaryngology, including the effects of adenotonsillar hypertrophy on obstructive sleep apnea and the many possible sequelae of obstructive sleep apnea, the microbiologic flora of the tonsils and adenoids and their role in chronic adenotonsillar hypertrophy, the relationship between adenotonsillar hypertrophy and craniofacial growth, and new techniques for adenotonsillectomy with improved management of perioperative morbidity. This chapter reviews the current understanding of pharyngitis and adenotonsillar disease processes.
History
Celsus was the first to report removal of the tonsils.1 Describing his surgical technique, Celsus indicated that “the tonsils are loosened by scraping around them and then torn out.”1 Hemostasis was obtained using a vinegar mouthwash and painting the tonsillar fossa with a medication to reduce bleeding.2 Aëtius of Amida on the Tigris described a technique for tonsillectomy in the first half of the sixth century, in which a hook was used to snare the tonsil and a knife was used for amputation. He warned of the severe dangers of hemorrhage when excision was too deep.3 Subsequent surgical techniques were described by Paul of Aegina in 625, and Physick described a forceps to facilitate extirpation of the tonsil, which became the forerunner of the modern tonsil guillotine.1,4 Mackenzie improved on the Physick tonsillotome and popularized its use for surgery of the tonsils in the late nineteenth century.5
The adenoids were first described by the Danish physician Meyer. In his 1868 paper “Adenoid vegetations in the nasopharyngeal cavity,” Meyer described in detail his technique of posterior rhinoscopy to diagnose adenoid hypertrophy and recommended removal of adenoid tissue with the aid of a ring knife.6 In 1885, Gottstein described the first adenoid curette.6
Crowe and colleagues7 reviewed 1000 consecutive tonsillectomies performed between 1911 and 1917. In the study “Relation of tonsillar and nasopharyngeal infection to general systemic disorders,” they provided a detailed description of a meticulous surgical technique by sharp dissection and described using the Crowe-Davis mouth gag. The low complication rate they described compares favorably with rates in modern reports of tonsillectomy.
Anatomy
Palatine Tonsil
The palatine tonsil represents the largest accumulation of lymphoid tissue in Waldeyer’s ring and, in contrast to the lingual and pharyngeal tonsils, constitutes a compact body with a definite thin capsule on its deep surface.8 Tonsillar crypts, blind tubules from the epithelium on the surface of the tonsil that are lined with stratified squamous epithelium, extend deep into this tissue.
The tonsillar capsule is a specialized portion of the pharyngobasilar fascia that covers the surface of the tonsil and extends into it to form septa that conduct the nerves and vessels.8 The tonsil is not, therefore, easily separated from its capsule, but the capsule is united largely by loose connective tissue to the pharyngeal muscles. One can easily dissect the tonsil from its normal position by separating the capsule from the muscle through this loose connective tissue.
The tonsillar fossa is composed of three muscles: the palatoglossus muscle, which forms the anterior pillar; the palatopharyngeal muscle, which is the posterior pillar; and the superior constrictor muscle of the pharynx, which forms the larger part of the tonsillar bed.8 The muscular wall is thin, and immediately against it on the outer wall of the pharynx is the glossopharyngeal nerve. This nerve can be easily injured if the tonsillar bed is violated, and not uncommonly the nerve is temporarily affected by edema after tonsillectomy, which produces both a transitory loss of taste over the posterior third of the tongue and referred otalgia.
The arterial blood supply of the tonsil enters primarily at the lower pole, with branches also at the upper pole. There are typically three arteries at the lower pole: the tonsillar branch of the dorsal lingual artery anteriorly, the ascending palatine artery (a branch of the facial artery) posteriorly, and the tonsillar branch of the facial artery between them that enters the lower aspect of the tonsillar bed.8 At the upper pole of the tonsil, the ascending pharyngeal artery enters posteriorly, and the lesser palatine artery enters on the anterior surface. The tonsillar branch of the facial artery is the largest. Venous blood drains through a peritonsillar plexus about the capsule.8 The plexus drains into the lingual and pharyngeal veins, which in turn drain into the internal jugular vein.
The nerve supply of the tonsillar region is through the tonsillar branches of the glossopharyngeal nerve about the lower pole of the tonsil and through the descending branches of the lesser palatine nerves, which course through the pterygopalatine ganglion.8 The cause of referred otalgia with tonsillitis is through the tympanic branch of the glossopharyngeal nerve. Efferent lymphatic drainage courses through the upper deep cervical lymph nodes, especially the jugulodigastric or tonsillar node located behind the angle of the mandible.8
Adenoids
The adenoid or pharyngeal tonsils form the central part of the ring of lymphoid tissue surrounding the oropharyngeal isthmus. The adenoid is composed of lymphoid tissue, with its apex pointed toward the nasal septum and its base toward the roof and posterior wall of the nasopharynx. The adenoid is covered by a pseudostratified ciliated columnar epithelium that is plicated to form numerous surface folds. The adenoid develops as a midline structure by the fusion of two lateral primordia that become visible during early fetal life, are fully developed during the seventh month of gestation, and continue to grow until the fifth year of life, often causing some airway obstruction.6,9 Thereafter, the adenoid gradually atrophies, the nasopharynx grows, and the airway improves.10
The blood supply and drainage are from the ascending pharyngeal artery, the ascending palatine artery, the pharyngeal branch of the maxillary artery, the artery of the pterygoid canal, and contributing branches from the tonsillar branch of the facial artery.9 Venous drainage is to the pharyngeal plexus, which communicates with the pterygoid plexus and then drains into the internal jugular and facial veins. The nerve supply is from the pharyngeal plexus. The efferent lymphatic drainage of the adenoids is to the retropharyngeal and pharyngomaxillary space lymph nodes.9
Immunology of the Adenoids and Tonsils
The adenoids and tonsils are predominantly B-cell organs; B cells account for 50% to 65% of all adenoid and tonsillar lymphocytes.11 Approximately 40% of adenoid and tonsillar lymphocytes are T cells, and 3% are mature plasma cells. Conversely, 70% of the lymphocytes in peripheral blood are T cells.12 The immunoreactive lymphoid cells of the adenoids and tonsils are found in four distinct areas: the reticular cell epithelium, the extrafollicular area, the mantle zone of the lymphoid follicle, and the germinal center of the lymphoid follicle.11
Ample evidence shows that the adenoids and tonsils are involved in inducing secretory immunity and regulating secretory immunoglobulin production. They contain a system of channels covered by specialized endothelium that can mediate antigen uptake much like Peyer’s patches of epithelium in the bowel.13 Both the adenoids and tonsils are favorably located to mediate immunologic protection of the upper aerodigestive tract as they are exposed to airborne antigens. Both organs, specifically the tonsils, are particularly designed for direct transport of foreign material from the exterior to the lymphoid cells.11 This is in contrast to lymph nodes, which depend on antigenic delivery through afferent lymphatics. The tonsillar crypts are covered by stratified squamous epithelium. There are 10 to 30 of these crypts in the tonsils, and they are ideally suited to trapping foreign material and transporting it to the lymphoid follicles.11
The tonsils and adenoids rank among the secondary lymphatic organs. Intratonsillar defense mechanisms eliminate weak antigenic signals. Only when additional higher antigenic concentrations are presented does proliferation of antigen-sensitive B cells occur in the germinal centers.11 Low antigen doses effect the differentiation of lymphocytes to plasma cells, whereas high antigen doses produce B-cell proliferation. The generation of B cells in the germinal centers of the tonsils is considered by Siegel to be one of the most essential tonsillar functions.14
Immunoglobulins (Igs) produced by the adenoid include IgG, IgA, IgM, and IgD.11 IgG appears to pass into the nasopharyngeal lumen by passive diffusion.11 The tonsil produces antibodies locally as well as B cells, which migrate to other sites around the pharynx and periglandular lymphoid tissues to produce antibodies.
T-cell functions, such as interferon-γ production and, presumably, production of other important lymphokines, have been shown to be present in tonsils and adenoids.11 The role played by tonsillar and adenoid T cells in tumor response is still unknown.
The human tonsils are immunologically most active between ages 4 and 10 years. Involution of the tonsils begins after puberty, resulting in a decrease of the B-cell population and a relative increase in the ratio of T to B cells.11,15 Although the overall Ig-producing function is affected, considerable B-cell activity is still seen in clinically healthy tonsils even at age 80 years.16 The situation is different in disease-associated changes, such as when recurrent tonsillitis and adenoid hyperplasia are observed. Inflammation of the reticular crypt epithelium results in shedding of immunologically active cells and decreasing antigen transport function with subsequent replacement by stratified squamous epithelium.17 These changes lead to reduced activation of the local B-cell system, decreased antibody production, and an overall reduction in density of the B-cell and germinal centers in extrafollicular areas.17 In contrast to recurrent tonsillitis, the changes are less pronounced in adenoid hyperplasia, in which the immunoregulatory conditions necessary for maintenance of the B-cell population are well preserved. The reason is most likely that the reticular epithelium is less affected in inflammation of adenoids than of tonsils.
Reports conflict regarding the immunologic consequences of tonsillectomy and adenoidectomy, yet it is clear that no major immunologic deficiencies result from these procedures.16,18 Ogra19 showed a three- to fourfold drop in titers in children previously immunized with live poliovirus vaccine. Attempts to vaccinate seronegative children subjected to tonsillectomy and adenoidectomy have resulted in delayed and lowered nasopharyngeal secretory immune responses as measured by IgA antibodies to the poliovirus.19 Fortunately, poliovirus epidemics are no longer an annual threat. Serum IgA levels in patients who had undergone tonsillectomy were lower than in age-matched controls, but this immunologic change did not appear to be clinically significant. Some studies actually point to improved immunologic activity after tonsillectomy. One study showed better neutrophil chemotaxis after tonsillectomy, and another demonstrated increased IgG and IgM production, possibly as a result of unblocking of the suppression that the immune system was subject to before tonsillectomy.20,21 One large study, with a cohort of 1328 children, showed no higher incidence of atopic disease (asthma, allergic rhinitis, and eczema) in children who had adenotonsillectomy prior to age 8 than in those who had not undergone surgery.22
Bacteriology
Establishment of normal flora in the upper respiratory tract begins at birth. Actinomyces, Fusobacterium, and Nocardia are acquired by age 6 to 8 months.23 Subsequently, Bacteroides, Leptotrichia, Propionibacterium, and Candida are also established as part of the oral flora.24 Fusobacterium populations reach high numbers after dentition and reach maximal numbers at 1 year of age.23 The ratio of anaerobic to aerobic bacteria in saliva is approximately 10 : 1,24 because of variations in oxygen concentration throughout the oral cavity.
Healthy children up to 5 years of age can harbor known aerobic pathogens. Ingvarsson and colleagues24 reported that Streptococcus pneumoniae was recovered in 19% of healthy children, Haemophilus influenzae in 13%, group A Streptococcus in 5%, and Moraxella (Branhamella) catarrhalis in 36%. The frequency of pathogens decreases with age, possibly because of greater immunity. Changes in the pharyngeal bacteria flora noted during viral illnesses are thought to be a result of the increased adherence of Staphylococcus aureus as well as gram-negative enteric organisms.25 Oral pharyngeal colonization during illness-free periods was found to vary from 12% to 18% for gram-negative enteric organisms and from 5% to 14% for S. aureus.26 During an episode of viral upper respiratory tract infection, the colonization rates for gram-negative organisms and S. aureus increased to 60% and 43%, respectively.
Brodsky and Koch27 found substantive differences between the types and numbers of aerobic bacteria found in nondiseased and diseased adenoids. The core samples of normal adenoids showed that 75% of children who were relatively free of upper respiratory disease, otitis media, and symptoms of adenoid obstruction had either no bacterial growth on culture or bacteria that are considered part of the normal flora and not pathogenic. Core samples in adenoids of only 45% of children who had chronic adenoid infection and 39% who had obstructive adenoid hypertrophy had no bacteria growth or only normal flora; the bacteria found in these children were more likely to be β-lactamase producers.
Infections of Waldeyer’s Ring
Many organisms can induce inflammation of Waldeyer’s ring. These include aerobic as well as anaerobic bacteria, viruses, yeasts, and parasites. Some of the infectious organisms are part of the normal oral pharyngeal flora, and others are external pathogens. Because the oropharynx is colonized by many organisms, most infections of Waldeyer’s ring are polymicrobial. These organisms work synergistically and can be demonstrated in mixed aerobic and anaerobic infections.28
Another feature of mixed infections is the ability of organisms resistant to an antimicrobial agent to protect an organism susceptible to that agent by the production of an antibiotic-degrading enzyme that is secreted into the tissues.29 Because of the polymicrobial nature of most infections around Waldeyer’s ring, it is often difficult to interpret data derived from clinical samples obtained from mucosal surfaces and to differentiate between organisms that are colonized and those that are invaders.
Viruses
Herpangina caused by coxsackievirus is characterized by small vesicles with erythematous bases that become ulcers and are spread over the anterior pillar tonsils, palate, and posterior pharynx (Fig. 196-2). Herpes simplex virus commonly causes the well-known “cold sore.” This virus can also cause exudative or nonexudative pharyngitis, mainly in older children and young adults. In younger children, the herpesvirus may induce gingivostomatitis.
Management of viral infections is nonspecific and symptomatic. Antibiotics are helpful in cases of secondary bacterial infection.30
Epstein-Barr Virus
One particular type of viral infection that deserves special attention is the Epstein-Barr virus (EBV). EBV induces the mononucleosis syndrome, which consists of high fever; general malaise; large, swollen, dirty-gray tonsils (Fig. 196-3); sore throat; dysphagia; and odynophagia. Petechiae located at the junction of the hard and soft palate are highly suggestive of EBV infection, although not pathognomonic. Often patients with EBV infection have hepatosplenomegaly with resultant liver damage. The most common method of transmission is oral contact.
Diagnosis of EBV is confirmed by laboratory studies. A differential blood count showing 50% lymphocytosis with 10% atypical lymphocytes is characteristic of EBV infection. Serologic studies include monospot and other serum heterophil antibody titer measurements. Results of these tests may be negative initially, and repeat testing in 1 to 2 weeks is warranted if clinical suspicion of EBV infection is strong. The heterophil antibody titers are detected by the Paul-Bunnell-Davidsohn or ox-cell hemolysis test. If the heterophil antibody agglutination test result is negative, the disease may still be present. Only 60% of patients with infectious mononucleosis have a positive result within the first 2 weeks after onset of the illness; 90% have a positive result 1 month after onset.31 EBV-specific serologic assays have become the method of choice for confirmation of acute or convalescent EBV infection. Figure 196-4 shows the serologic response time. Management of this condition is symptomatic. Recovery may take weeks, and antibiotics are used to treat secondary bacterial infections. Ampicillin should be avoided because a rash may occur despite previous intake without similar reactions. Upper airway obstruction from severely enlarged tonsils can be life-threatening and should be managed promptly with the insertion of a nasopharyngeal airway and short-term high-dose steroid therapy. If the obstruction is severe and unrelieved by these measures, a tonsillectomy or tracheotomy may be necessary.
Neisseria
Pharyngitis as a result of Neisseria gonorrhoeae is common in homosexual men. It has been detected in 6% of adolescents with acute pharyngitis.32 Although the infection is often asymptomatic, it can result and persist after treatment. Acute exudative tonsillitis is a manifestation of gonococcal pharyngitis. The clinical syndrome may range from an asymptomatic to an exudative pharyngitis, but most cases tend to fall toward the exudative pharyngitis end of the spectrum. Nonetheless, disseminated gonococcemia can result even from mild or asymptomatic infection. Penicillin and tetracycline are the most effective therapeutic agents.
Vincent’s Angina
Vincent’s angina is secondary to Spirochaeta denticulata and Vincent’s fusiform bacillus Borrelia vincentii (Treponema vincentii). This condition arises slowly, manifesting both mild local and systemic symptoms. The infection arises most commonly in overcrowded conditions. Patients present with complaints of high fever, headache, sore throat, and physical findings of cervical lymphadenopathy and a membrane on the tonsil that, when removed, reveals an ulcer that remains confined to the surrounding tissue and usually heals in 7 to 10 days.33 Management consists of penicillin therapy. Local treatment in the form of oral hygiene is helpful. This condition may be confused with trench mouth, which is caused by the same organisms but in which oral cavity ulcers include the gums and oral mucous membranes.34
Corynebacterium diphtheriae
The incidence of Corynebacterium diphtheriae infection has declined markedly since the introduction of diphtheria vaccination. This organism causes an early exudative pharyngotonsillitis with a thick pharyngeal membrane. Infection can then spread to the throat, tonsils, palate, and larynx.35 C. diphtheriae organisms also produce a lethal exotoxin that can damage cells in distant organs. Today only 200 to 300 cases of diphtheria occur in the United States each year, usually—but not exclusively—in people who have not been immunized.36 The organism is a gram-positive pleomorphic aerobic bacillus that can be identified in a routine throat culture, particularly when the microbiologist is made aware of a clinical suspicion of this diagnosis. Only toxigenic strains infected with a bacteriophage can cause diphtheria.30 Laryngeal inflammation combined with an exudative, necrotic, gray pharyngeal membrane may result in airway obstruction. Removal of this membrane causes bleeding. Early diagnosis is critical, and the goal of therapy is neutralization of unbound toxin with antitoxin. Myocarditis and neurologic sequelae resembling features of Guillain-Barré syndrome or poliomyelitis may result.35,36 The organism is identified by fluorescent antibody studies. The presence of Klebs-Löffler bacillus in the membrane can be diagnosed with Gram staining.34 Because diphtheria is an emergency condition, antitoxin must be given in the first 48 hours of onset to be effective. Airway obstruction should be managed with tracheotomy. Penicillin in high doses should be administered.
Streptococcal Tonsillitis-Pharyngitis
Group A Streptococcus is the most common bacterial cause of acute pharyngitis.37 The public health importance of this infection lies not only in its frequency but also on the fact that it is a precursor of two serious sequelae, acute rheumatic fever and poststreptococcal glomerulonephritis. Although the incidence of rheumatic fever has decreased, all groups of β-hemolytic streptococci have been associated with rheumatic fever.23
Acute streptococcal tonsillitis is a disease of childhood, with a peak incidence at about 5 to 6 years of age, but can occur in children younger than 3 years and in adults older than 50 years.34 Outbreaks may arise in epidemic forms in institutional settings such as recruit camps and daycare facilities. Acute tonsillitis manifests as a dry throat, malaise, fever, fullness of the throat, odynophagia, dysphagia, otalgia, headache, limb and back pain, cervical adenopathy, and shivering. Examination reveals a dry tongue, erythematous, enlarged tonsils, and yellowish white spots on the tonsils. In severe cases, a tonsillar or pharyngeal membrane or purulent exudate may exist along with jugulodigastric lymph node enlargement.34
The diagnosis of acute tonsillitis is made mainly on clinical grounds. The clinical manifestations of streptococcal and nonstreptococcal pharyngitis overlap so broadly that it is often impossible to make the diagnosis with certainty. For this reason, most authorities recommend that the diagnosis of group A β-hemolytic streptococcal (GABHS) pharyngitis be verified or ruled out by microbiologic tests in patients who appear likely, on the basis of clinical and epidemiologic considerations, to have this illness.37,38 The time-honored method of diagnostic confirmation is the throat culture. This is a simple and extremely useful test but sampling must be skillfully performed by swabbing the posterior pharynx and tonsillar areas.37 The specimen must also be appropriately processed and read. Such selective use of throat cultures represents good medical practice.
One of the major problems in the use of throat cultures in everyday medical practice has been the delay in obtaining results. The delay can range from 18 to 48 hours, which holds up the start of appropriate management but does not increase the likelihood of development of rheumatic fever. Nevertheless, it can be difficult for physicians to persuade parents of the wisdom of withholding antibiotics until results are known, especially if their children are cranky and febrile. If group A streptococcal pharyngitis is treated early in the clinical course, the period of communicability is reduced.39 Management is thus initiated before culture results are available and unfortunately may not be terminated even when negative results are obtained. Development of rapid strep detection tests for the detection of the group A streptococcal antigen has therefore represented a useful advance.
Several rapid tests to detect group A streptococcal antigen in the pharynx have been developed. They employ either latex agglutination or enzyme-linked immunosorbent assay (ELISA) methods to extract the antigen from a swab. The streptococcal group A carbohydrate may be detected within a matter of minutes. The test kits are suitable for use in a physician’s office. Although the rapid detection tests are highly specific, they are unfortunately not as sensitive as routine throat culture.37,40,41 A negative rapid detection test result may prompt the practitioner to withhold antibiotic therapy while awaiting culture results. Most guidelines suggest that throat cultures should be performed when the body temperature is greater than 38.3° C or when the illness is characterized exclusively by a sore throat.42 The most accurate and cost-effective method to diagnose acute GABHS infection is the use of the rapid strep test. This is followed by standard throat culture in patients with a negative rapid strep test result and a strong suspicion of streptococcal tonsillitis.
Even optimally obtained and processed, throat cultures are not without flaws. Throat culture cannot reliably differentiate acute from chronic infection. The treatment of all patients who have positive culture results leads to over-management. There are occasional false-negative results (approximately 10% of cases), although one report has found that patients with false-negative results are most likely carriers who do not require treatment.37 Studies have reported that a single throat culture is 90% to 97% sensitive and 90% specific for GABHS growth.43 The carrier state can be elucidated by serologic testing. A true infection is demonstrated by a positive throat culture result and at least a two-dilutional rise in the antistreptolysin-O titer.44 A GABHS carrier without acute infection has a positive culture result with no change in dilution titer.45 Excluding the diagnosis of group A streptococcal pharyngitis is quite important because the majority of patients with acute pharyngitis do not have “strep” throat.
Therapy has usually been directed at the aerobic pathogens traditionally associated with tonsillitis (e.g., GABHS). Penicillin is still the agent of choice in most cases. However, anaerobic bacteria play a major role in the complications associated with tonsillitis, so they are probably also involved in recurrent tonsillitis. One study has documented the prevalence of Bacteroides cultured from chronically inflamed tonsils.46 Anaerobes also have been implicated in acute tonsillitis.23 Clinical failure of penicillin should lead to the suspicion of β-lactamase–producing organisms. The reason for the treatment failure could be that these organisms either act as pathogens themselves, so-called direct pathogens, or protect susceptible pathogens from the effects of β-lactamase antibiotics, making them so-called indirect pathogens. In such cases, the patient complains of sore throat that never resolves completely despite penicillin management. An alternative to the use of penicillin is to use a penicillin plus a β-lactamase inhibitor such as clavulanic acid (e.g., amoxicillin/clavulanic acid). Other alternatives are clindamycin and a combination of erythromycin and metronidazole. Institution of antibiotic therapy within 24 to 48 hours of symptom onset will result in decreased symptoms associated with sore throat, fever, and adenopathy 12 to 24 hours sooner than without antibiotic administration. The use of antibiotics also minimizes the chance of suppurative complications and diminishes the likelihood of acute rheumatic fever.47,48 Ten full days of therapy is necessary, as eloquently demonstrated by Schwartz and colleagues,49 who showed that children receiving 10 days of therapy have lower clinical and bacteriologic recurrence rates than children receiving only 7 days of therapy.
There appears to be no need for further management for asymptomatic carriers because the carrier state does not lead to suppurative or nonsuppurative complications nor is the patient likely to spread the disease to others.50 Although asymptomatic patients need neither culture nor management if results of follow-up culture are positive, certain high-risk situations should be approached differently. For example, if a family member had rheumatic fever or if a family has been experiencing recurrent streptococcal illness, another course of antibiotics would be recommended for the carrier. Patients who become symptomatic after an appropriate course of therapy may indeed represent true management failures for which a second course of therapy would also be justified.50
Tonsillar Concretions/Tonsilloliths
Tonsillar concretions or tonsilloliths (Fig. 196-5) arise from retained material and bacterial growth in the tonsillar or adenoid crypts and may exist in patients with or without a history of inflammatory disorders in either the tonsils or adenoids.51 The clinical presentation of fetor oris (halitosis) and sore throat as well as the presence of whitish, expressible, foul-tasting, and foul-smelling cheesy lumps from the tonsils characterizes the tonsillar concretions in many patients. Local management involves simple expression of the concretions by the patient, the use of pulsating jets of water to clean the pockets of debris mechanically, or application of topical silver nitrate to the tonsillar crypts in an effort to chemically cauterize and obliterate them. Persistent problems with pain, halitosis, foreign body sensation, or otalgia may require surgical removal of the tonsils as definitive therapy.
Complications of Tonsillitis
Nonsuppurative Complications
Scarlet fever is secondary to acute streptococcal tonsillitis or pharyngitis with production of endotoxins by the bacteria.33 Manifestations include an erythematous rash; severe lymphadenopathy with a sore throat; vomiting; headache; fever; erythematous tonsils and pharynx; tachycardia; and a yellow exudate over the tonsils, pharynx, and nasopharynx. The membrane that is present over the tonsils is usually more friable than that seen with diphtheria. A strawberry tongue with a rash and large glossal papillae is a good diagnostic sign. Diagnosis of scarlet fever is made by culture and positive result of the Dick test, which is an intradermal injection of dilute streptococcal toxin.34 Management of this condition involves intravenous administration of penicillin G. Otologic complications may include necrotizing otitis media with complete loss of the tympanic membrane and ossicles.
Fortunately, acute rheumatic fever is an uncommon illness in the United States today. The incidence of rheumatic fever following sporadic streptococcal infection is approximately 0.3%.50 A patient with rheumatic fever who does not comply with penicillin prophylaxis should have a tonsillectomy and adenoidectomy because patients who have undergone surgery have a lower infection rate with β-hemolytic streptococcus.52
Poststreptococcal glomerulonephritis may be seen after both pharyngeal and skin infections. The incidence is approximately 24% after exposure to nephrogenic strains, but these strains account for less than 1% of the total pharyngeal strains.50 Typically, an acute nephritic syndrome develops 1 to 2 weeks after a streptococcal infection. The infection is secondary to the presence of a common antigen of the glomerulus with the streptococcus. Penicillin management may not decrease the attack rate, and there is no evidence that antibiotic therapy affects the natural history of glomerulonephritis. A tonsillectomy may be necessary to eliminate the source of infection.
Suppurative Complications
Peritonsillar Infections
Peritonsillar abscess most commonly occurs in patients with recurrent tonsillitis or in those with chronic tonsillitis that has been inadequately treated. The spread of infection is from the superior pole of the tonsil with pus formation between the tonsil bed and the tonsillar capsule.33 This infection usually occurs unilaterally and the pain is quite severe, with referred otalgia to the ipsilateral ear a few days after the onset of tonsillitis. Drooling is caused by odynophagia and dysphagia. Trismus is frequently present as a result of irritation of the pterygoid musculature by the pus and inflammation. There is gross unilateral swelling of the palate and anterior pillar with displacement of the tonsil downward and medially with reflection of the uvula toward the opposite side. Cultures of the peritonsillar abscess usually show a polymicrobial infection, both aerobic and anaerobic.53
Cellulitis should be differentiated from abscess in the management of peritonsillar infections. Some abscesses may be clinically obvious, whereas others are less obvious. When there is extension of infection of the peritonsillar abscess, computed tomography (CT) with contrast enhancement may be indicated (Fig. 196-6).
The choice between needle aspiration and incision and drainage in the management of peritonsillar abscesses is controversial. Traditional management has consisted of incision and drainage, with tonsillectomy 4 to 12 weeks later. Some surgeons advocate immediate tonsillectomy or Quinsy tonsillectomy as definitive management to ensure complete drainage of the abscess and to alleviate the need for a second hospitalization for an interval tonsillectomy.54 Each therapeutic modality has advantages in certain situations. If incision and drainage or needle aspiration fails to drain an abscess adequately, a Quinsy tonsillectomy is indicated. In patients with a prior history of recurrent peritonsillar abscess or recurrent tonsillitis severe enough to warrant tonsillectomy, a Quinsy tonsillectomy should be considered. Quinsy tonsillectomy is particularly favored in children because they are likely to experience further episodes of tonsillitis, and needle aspiration or incision and drainage with a child under local anesthesia is often difficult or impossible.
Parapharyngeal Space Abscess
An abscess can develop in the parapharyngeal space if infection or pus drains from either the tonsils or from a peritonsillar abscess through the superior constrictor muscle.33 The abscess is located between the superior constrictor muscle and the deep cervical fascia and causes displacement of the tonsil on the lateral pharyngeal wall toward the midline. Involvement of the adjacent pterygoid and paraspinal muscle with the inflammatory process results in trismus and a stiff neck. The thickness of the overlying sternocleidomastoid muscle often prevents the detection of fluctuance by palpation.
Retropharyngeal Space Infections
The superior limit of the retropharyngeal space is the cranial base. Inferiorly, the retrovisceral space extends into the mediastinum to approximately the level of the tracheal bifurcation. The buccal pharyngeal fascia is adherent to the prevertebral fascia in the midline, so that infection in the retropharyngeal space is unilateral. Lateral neck radiography, CT, or ultrasonography may help ascertain whether there is cellulitis or a true abscess (Fig. 196-7). The source of the retropharyngeal abscess is a chain of lymph nodes present on either side of the midline in the retropharyngeal space. These lymph nodes receive drainage from the nose, paranasal sinuses, pharynx, and eustachian tube.
Figure 196-7. A, Lateral neck radiograph showing a retropharyngeal abscess. B, Computed tomography scan of the same patient.
A transoral approach is recommended for incision and drainage of retropharyngeal space abscesses. If the abscess extends inferiorly below the hyoid bone (shown on the CT scan), an external approach should be used. The patient must undergo oral intubation, which can be done safely by introduction of the tube on the side opposite the abscess to avoid aspiration of purulent material. The patient must be positioned in the head-down Trendelenburg position, and packing should be placed around the endotracheal tube inferiorly. Gram staining, culture, and antimicrobial sensitivity staining should be performed on the purulent material. A small vertical incision is made in the lateral aspect of the posterior pharyngeal wall at a point between the junction of the lateral one third and medial two thirds of the distance between the midline of the pharynx and the medial aspect of the retromolar trigone.55 The space is gently probed with a hemostat to break up the loculations and drain the abscess. A drain is not used because of the possibility of aspiration if swallowed postoperatively. If the abscess extends laterally to involve the parapharyngeal space, it should be drained through an external approach.
Chronic Adenotonsillar Hypertrophy
Etiology
Chronic adenotonsillar hypertrophy—manifesting as various degrees of airway obstruction in children—has become the most common indication for adenotonsillectomy in the United States.56 Typically, the tonsils and adenoids are very small at birth and progressively enlarge over the first four years of life as a result of increased immunologic activity (Figs. 196-8 and 196-9). Brodsky and colleagues27,57 reported that hypertrophied and chronically infected tonsils and adenoids had greater loads of pathogenic bacteria, especially β-lactamase producers, than nondiseased tonsils and adenoids.57,58 These studies were based on core samples that were believed to be more accurate than surface cultures of the tonsils and adenoids. It is possible that equilibrium exists between the normal flora of the adenotonsillar tissue and their local immunologic response and that this equilibrium can become disrupted with recurrent acute viral or bacterial infections or colonization with pathogenic bacteria, resulting in hypertrophied lymphoid tissue.27
Figure 196-9. A, Endoscopic view of mild adenoid hypertrophy. B, Severe adenoid hypertrophy with total choanal obstruction.
In addition to chronic bacterial infection, exposure to second-hand smoke has been implicated as a cause of adenotonsillar hypertrophy in children.8
Airway Obstruction
Obstructive sleep apnea is the most common indication for tonsillectomy in the pediatric population. Pediatric obstructive sleep apnea, confirmed by polysomnography, is reported to have an incidence of 1% to 3%.59–61 The obstructive apnea is almost always associated with hypertrophy of the tonsils and adenoids. The tonsil and adenoid tissue, when large, fills the area of the oropharynx and nasopharynx, obstructing airflow. This obstruction is worse when the patient is supine and asleep owing to the effects of gravity and the relaxation of surrounding nasopharyngeal and oropharyngeal soft tissue. The obstruction can result in a mildly compromised airway, which leads to snoring, and most children with airway obstruction related to adenotonsillar hypertrophy have a history of significant snoring at night.28,31,59,62,63 The obstruction may lead to intermittent complete airway obstruction with subsequent apnea and oxygen desaturation. The apnea typically is short and usually associated with a brief arousal wherein the patient repositions himself or herself and opens the airway. However, if the apnea is prolonged, oxygen desaturation can occur. Such desaturation episodes put stress on the cardiovascular system.
Before the syndrome of obstructive sleep apnea was widely recognized, children sometimes presented with pulmonary hypertension and cor pulmonale, failure to thrive, and developmental delay.64 Such severe consequences rarely occur now, but the sleep disturbance manifests as multiple clinical symptoms that are commonly seen. Some of the more common symptoms that occur during the sleep of affected children are loud “heroic” breathing, diaphoresis, apnea, gasping, mouth-breathing, restless sleep, enuresis, drooling, night terrors, and sleepwalking. During the day affected children may suffer from daytime sleepiness, morning headache, dry mouth, halitosis, swallowing difficulty, behavioral difficulties and hyponasal speech, or, rarely, hypernasal speech (rhinolalia aperta) caused by enlarged tonsils impinging on normal palatal movement.63 Behavioral difficulties include hyperactivity, inattentiveness in the classroom, problems with academic performance, and rebellious or aggressive behavior. The aforementioned behavioral difficulties are also clinical manifestations of the most commonly diagnosed psychiatric diagnosis in children—attention deficit–hyperactivity disorder (ADHD).
Today obstructive sleep apnea and its consequences are widely recognized, and for that reason it is the primary indication for tonsillectomy in this country.65 There is no debate about the existence of the obstructive sleep apnea syndrome or that adenotonsillectomy is the treatment of choice. However, there is considerable debate about the methods of diagnosis and therefore considerable discussion about exactly how large a group of children is affected by the syndrome (see Chapter 183 for a full discussion).
Attention Deficit–Hyperactivity Disorder
Numerous articles in the literature have shown a significant relationship between sleep-disordered breathing and the symptoms of ADHD. One study investigated 996 children aged 4 to 5 years seen consecutively in a community health clinic. Of the 782 for whom the questionnaires were completed, 95 or 12% of the children were found to snore on most nights. A group of 66 children considered at high risk for sleep disturbance was selected from these 95 children and compared with a control group who had no symptoms of sleep disturbance. The study used a modified version of the Conner’s behavioral scale to assess symptoms of hyperactivity. These 66 children were studied with overnight oximetry and video monitoring. According to results of the overnight study, only 7 of the 66 children in the high-risk group had documented obstructive sleep apnea syndrome, but all the children in this high-risk group had significantly higher scores on parental and teacher reports of hyperactive behavior.60
Another study looked at the relationship between the symptoms of sleep-disordered breathing and problem behavior including hyperactivity and inattention. The study enrolled 3019 5-year-old children. The researchers used a questionnaire to evaluate for symptoms of sleep-disordered breathing as well as problems with behavior, and had a subset of 219 children’s families complete the Revised Conners’ Parent Rating Scale as a means of validating the results obtained with limited initial questioning. Symptoms of sleep-disordered breathing were present in 25% of the children. A strong association was found between parent-reported sleep problems and the parent-reported incidence of inattention, hyperactivity, and aggressiveness. Interestingly, a dose-response effect on problem behaviors was seen for both snoring frequency and snoring loudness. These results remained significant even when data were adjusted for sex, race, maternal education level, maternal marital status, household income, and respiratory history. Such adjustments had not been made in previous reports on the link between SDB and ADHD.66
A different study evaluated 2076 children and found 71 with behavioral or academic problems. These children had significantly more problems with snoring and difficulty breathing. This association was found to be strongest with children who had academic problems or in children whose specific behavioral problem was consistent with ADHD.67
Other studies have examined this issue from the opposite direction, evaluating a group of children diagnosed with neuropsychological problems and searching for signs and symptoms of SDB. These studies show that children with neuropsychological problems have a higher percentage of sleep problems than normal controls. Marcotte and associates looked at 200 children referred for psychiatric evaluation for possible ADHD; 79 children were diagnosed with ADHD, learning disability, or combined ADHD–learning disability. Through parent questionnaires, the investigators found a high incidence of reported sleep problems at night in comparison with the incidence in a control group of children. They did not find any difference in the reported length of sleep. Thus, the effect seems to depend on quality, not quantity, of sleep.68
Another study surveyed a group of parents at a child psychiatry unit and a group of patients at a general pediatric clinic. Children at the psychiatry clinic with the diagnosis of ADHD had a 33% incidence of habitual snoring, compared with 11% of the other children at the psychiatry clinic and 9% in those at the general pediatric clinic. Higher snoring scores derived from the validated pediatric sleep questionnaires were associated with higher levels of inattention and hyperactivity, again pointing to an apparent dose-response relationship.69
The pathophysiology of ADHD is unknown, but there are suggestions that an abnormal sleep pattern may be one causal factor. It has long been known that stimulants improve behavior in patients with ADHD. Stimulants are believed to work because ADHD is a disorder caused by hypovigilance rather than hypervigilance.70 If hypovigilance is indeed the mechanism, it makes sense that a child with decreased or poor sleep secondary to obstructive sleep apnea syndrome would be much more likely to suffer from symptoms of ADHD. Consequently, if the sleep obstruction was removed, it should follow that the quality of sleep would improve and therefore symptoms of ADHD would diminish.
One small article in the European literature did show that symptoms of ADHD diminished after adenotonsillectomy.71
