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contenttextbook· 125 Neck Masses in Infants and Children· item 126· p.827–833

782 SECTION 12: Pediatrics maxilla usually heal well without intervention. By contrast, the vascu larity around the mandibular frenulum often requires primary closure. Tongue lacerations can usually be treated conservatively, especially if the wound is <1 cm in length, is in the central portion of the tongue, and does not gape, and bleeding is controlled. Tongue lacerations greater than one third of the total diameter and those at the tip causing forking that may affect speech require suturing. In general, lacerations limited to the inner mucosal surface heal well on their own and do not require primary repair. By contrast, full-thickness lacerations and those that disrupt the vermillion border require suturing.  CARIES Children are particularly susceptible to developing caries soon after the initial eruption of teeth if care is not taken to properly examine and clean the new teeth. “Baby bottle” caries occur in 24% to 28% of all children age 2 to 5 years old. 44 Risk factors include prolonged breast or bottle feeding (beyond 12 months), prolonged pacifier use, frequent consumption of beverages high in sugar, and use of a bottle at bedtime. Encourage parents to minimize beverage choices high in sugar content. Teeth should be cleaned daily from time of eruption to 24 months of age with a soft toothbrush and increased to twice a day thereafter. FIGURE 124-10. Ellis type 2 fracture: Bilateral maxillary central incisor injuries with exposed enamel and dentin consistent with an Ellis class 2 fracture. [Reproduced with permission from Knoop K, Stack L, Storrow A: Atlas of Emergency Medicine, 2nd ed. © 2002, McGraw- Hill, Inc., New York, Figure 6-6.] FIGURE 124-11. Ellis type 3 fracture: A fracture demonstrating blood at the exposed dental pulp. This sign is pathognomonic for an Ellis class 3 fracture. [Reproduced with permission from Knoop K, Stack L, Storrow A: Atlas of Emergency Medicine, 2nd ed. © 2002, McGraw-Hill, Inc., New York, Figure 6-7.] Transition to a training cup by 1 year of age and removal of the bottle can also significantly reduce the occurrence of caries. In addition, an initial screening dental examination between 12 and 18 months of age is recommended to look for signs of decay or a need for fluoride supple mentation. In communities without fluoridated water, supplemental fluoride should be prescribed by the primary care provider. Dental neglect is a form of child abuse probably underreported or unrecognized by medical providers. It is defined as the “willful failure of parent or guardian to seek and follow through with treatment neces sary to ensure a level of oral health essential for adequate function and freedom from pain and infection. ” Poor oral hygiene may be secondary to family isolation, lack of finances, parental ignorance, unfluoridated water, bottle-propping, or lack of perceived value of oral health, and clinicians should determine whether one or more of these situations are contributing factors.  GINGIVITIS Gingivitis is inflammation of the gums that presents as tender, erythematous, often ulcerated or vesiculated areas of tissue. It is seen mainly in the setting of poor dental hygiene but can occur with viral and bacterial infections, certain medications (e.g., phenytoin [Dilantin ® ]), or even as a presentation of leukemia. Although there are many causes of gingivostomatitis, viral infections are particularly common in children.

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eas of tissue. It is seen mainly in the setting of poor dental hygiene but can occur with viral and bacterial infections, certain medications (e.g., phenytoin [Dilantin ® ]), or even as a presentation of leukemia. Although there are many causes of gingivostomatitis, viral infections are particularly common in children. Acute necrotizing ulcerative gingivitis is a progressive infection of the gingiva, leading to pain, significant edema, and ulceration. The incidence typically peaks in the teens to early 20s, but may be seen in younger children in developing countries due to poor access to adequate dental care or malnutrition. Other factors that may predispose patients to acute necrotizing ulcerative gingivitis include smoking, immunosuppression, viral infections, stress, and sleep deprivation. Patients present with fever, halitosis, decreased appetite, and generalized malaise. Acute necrotizing ulcerative gingivitis is a mixed infection that includes spirochetes, specifically, Prevotella intermedia. Untreated, acute necrotizing ulcerative gingivitis can spread beyond the gingiva to involve deeper tissues or the tissues of the mouth floor (Ludwig’s angina) or face. Treatment consists of analgesia to facilitate better oral hygiene and antimicrobial oral rinses. Patients with more extensive disease or systemic symptoms may require admission for local debridement and parenteral antibiotic therapy with penicillin or metronidazole. The patient should be referred to a dentist for close follow-up care. REFERENCES The complete reference list is available online at www.TintinalliEM.com. Neck Masses in Infants and Children Charles E.A. Stringer Vikram Sabhaney INTRODUCTION AND EPIDEMIOLOGY Neck masses are common in childhood, and although most are benign, malignancy must remain a primary consideration. Among patients referred to tertiary centers for surgical excision of a cervical lesion, 90% to 96% of lesions are benign and are predominantly congenital. 1,2 While diagnosis is challenging, differentiating neck masses into inflammatory, congenital, or a neoplastic category is the first step toward diagnosis. CHAPTER Tintinalli_Sec12_p0669-0996.indd 782 8/2/19 7:51 PM

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rgical excision of a cervical lesion, 90% to 96% of lesions are benign and are predominantly congenital. 1,2 While diagnosis is challenging, differentiating neck masses into inflammatory, congenital, or a neoplastic category is the first step toward diagnosis. CHAPTER Tintinalli_Sec12_p0669-0996.indd 782 8/2/19 7:51 PM CHAPTER 125: Neck Masses in Infants and Children 783 TABLE 125-1 Key Features From History Historical Feature Associated Etiology Duration >4–6 weeks Granulomatous lymphadenitis, congenital lesions, malignancies Rate of growth Fluctuating → inflammatory Progressive → malignancy Pain Infectious, or infected congenital lesion Recent illness Viral URTI → Reactive lymphadenopathy Pharyngitis → EBV, GAS Neonatal period Group B Streptococcus Pain with meals Sialoadenitis Tuberculosis exposure (travel or sick contacts) Tuberculosis mycobacteria Undercooked meat or unpasteurized milk exposure Toxoplasmosis Cat exposure Cat-scratch disease or toxoplasmosis Animal exposure (including rabbits) Tularemia, cat-scratch disease, toxoplasmosis Mass since birth Congenital Birth trauma, forceps delivery Fibromatosis colli Constitutional symptoms (fever, weight loss, night sweats) Tuberculosis mycobacteria, malignancy Recent blood transfusions EBV, CMV, HIV Recent immunizations (DPT, polio, typhoid) Reactive lymphadenopathy Medications Phenytoin, carbamazepine, isoniazid, hydralazine, and others Abbreviations: CMV = cytomegalovirus; DPT = diphtheria, pertussis, and tetanus; EBV = Epstein-Barr virus; GAS = group A Streptococcus; HIV = human immunodeficiency virus; URTI = upper respiratory tract infection. TABLE 125-2 Key Features on Examination Physical Examination Finding Associated Etiology Laterality Bilateral → viral Unilateral → bacterial Size >3 cm more likely congenital or malignant; fluctuations in size more likely infectious Location Depends on lymph drainage pattern (see Figure 125-2) Hard, rubbery, fixed, matted nodes Possibly malignant Signs of inflammation (tenderness, erythema, warmth) Bacterial lymphadenitis or infected congenital lesion Fluctuance Abscess, often due to Staphylococcus aureus, or GAS Difficulty swallowing or drooling Deep space infection—retropharyngeal abscess Viral URTI (cough, rhinorrhea, conjunctivitis) Reactive lymphadenopathy Pharyngitis EBV mononucleosis, GAS Periodontal disease Anaerobic bacteria Purulent salivary duct drainage Sialoadenitis Crossing jaw—suggests parotid involvement Sialoadenitis, mumps, salivary gland malignancy Violaceous skin change Granulomatous lymphadenitis (TbM, NTM, CSD, tularemia) Cold node Granulomatous lymphadenitis or malignancy Midline, moves with swallowing or tongue protrusion Thyroglossal duct cyst Transillumination Lymphangioma (cystic hygroma) Torticollis, laterally mobile, vertically immobile Fibromatosis colli Supraclavicular mass Lymphoma, or less likely metastatic node Midline mass Thyroid cancer, thyroglossal duct cyst, dermoid cyst Posterior mass Rubella, toxoplasmosis, nasopharyngeal cancer Hepatosplenomegaly Malignancy, mononucleosis, systemic disease Wasting or cachexia Malignancy Abbreviations: CSD = cat-scratch disease; EBV = Epstein-Barr virus; GAS = group A Streptococcus; NTM = nontuberculous mycobacteria; TbM = tuberculous mycobacteria; URTI = upper respiratory tract infection. or antibiotics; however, all cases require 6-week follow-up to ensure resolution.  INFLAMMATORY NECK MASSES The most common pediatric neck mass is an enlarged lymph node caused by infection. Be careful to consider masquerading lesions such as salivary gland infections, acutely infected congenital lesions, and malignancies. Cervical lymph nodes drain the skin of the head and neck as well as the entire nasal, oral, and pharyngeal mucosa (Figure 125-2).

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neck mass is an enlarged lymph node caused by infection. Be careful to consider masquerading lesions such as salivary gland infections, acutely infected congenital lesions, and malignancies. Cervical lymph nodes drain the skin of the head and neck as well as the entire nasal, oral, and pharyngeal mucosa (Figure 125-2). Enlarged submandibular and cervical nodes are most common because they drain much of the oropharynx, including the adenoids and tonsils. 3,4 Supraclavicular lymphadenopathy is suspicious for metastasis as these nodes drain the abdomen and thorax. Palpable cervical lymph nodes are found in about 28% to 44% of healthy infants and children, with the incidence peaking in early childhood. 3,5,6 Lymph nodes ≤1 cm in children <12 years old are considered normal. 7,8 Most lymphadenopathy represents nonspecific reactive hyperplasia, often due to a viral upper respiratory tract infection. 9 Lymphadenitis is lymph node inflammation (swelling, tenderness, warmth, erythema) and is most commonly due to viral or bacterial causes. Infection with a GENERAL APPROACH  CLINICAL FEATURES A thorough history and physical examination help narrow the broad differential for cervical lymphadenopathy. Both acuity and laterality of node swelling are helpful for diagnosis. Acute bilateral lymph nodes are typically due to a viral infection, acute unilateral nodes are due to a bacterial cause, and subacute/chronic nodes (>4 to 6 weeks) are due to granulomatous bacteria or noninfectious causes. This framework is most effective for diagnosis when combined with the general and specific features of different etiologies (Tables 125-1 and 125-2). Carefully document the features (size, location) of all head and neck masses for future comparison. Lymph node location and characteristics give clues based on lymphatic drainage patterns. Look for systemic disease by assessing for generalized lymphadenopathy, hepatosplenomegaly, testicular masses and/or enlargement in males, and the child’s overall condition.  GENERAL MANAGEMENT Ideally, the treatment of a childhood neck mass is directed at the specific cause; however, due to the many etiologies, this is not always possible at first presentation. The majority of presentations are benign; however, the priority of childhood neck masses is to correctly identify those that are neoplastic. Figure 125-1 provides an approach to management based on suspected etiology and should be adapted to the individual patient. Follow-up of patients who are treated with antibiotics is necessary at 48 to 72 hours: if improvement of symptoms is not seen, then expand antibiotic coverage to include periodontal flora or methicillin-resistant Staphylococcus aureus and consider aspiration of fluctuant nodes for culture. Most lymphadenopathy will improve after 2 weeks of observation Tintinalli_Sec12_p0669-0996.indd 783 8/2/19 7:51 PM

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: if improvement of symptoms is not seen, then expand antibiotic coverage to include periodontal flora or methicillin-resistant Staphylococcus aureus and consider aspiration of fluctuant nodes for culture. Most lymphadenopathy will improve after 2 weeks of observation Tintinalli_Sec12_p0669-0996.indd 783 8/2/19 7:51 PM 784 SECTION 12: Pediatrics FIGURE 125-1. Approach to the pediatric neck mass in the ED. ABC = airway, breathing, circulation. Pediatric neck mass ABCs with imaging/consultation if concern for deep tissue infection Yes Distress Very likely malignant or congenital Acute (<4 wk) Subacute/chronic (>4 wk) Antibiotics with follow-up in 48-72 hr Signs of inflammation (consistent with bacterial lymphadenitis) 2-wk follow-up for evidence of improvement 6-wk follow-up for evidence of resolution No improvement Improvement Yes Yes Referral for definitive diagnostic & therapeutic management Initiate workup for chronic infectious etiologies and refer for diagnosis and management Resolution No resolution Preauricular Mastoid Jugulodigastric Posterior auricular Occipital Posterior cervical Supraclavicular Deep cervical Superficial cervical Submental Submaxillary Tonsillar FIGURE 125-2. Cervical lymph node drainage. Tintinalli_Sec12_p0669-0996.indd 784 8/2/19 7:51 PM

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for diagnosis and management Resolution No resolution Preauricular Mastoid Jugulodigastric Posterior auricular Occipital Posterior cervical Supraclavicular Deep cervical Superficial cervical Submental Submaxillary Tonsillar FIGURE 125-2. Cervical lymph node drainage. Tintinalli_Sec12_p0669-0996.indd 784 8/2/19 7:51 PM CHAPTER 125: Neck Masses in Infants and Children 785 FIGURE 125-3. Suppurative lymphadenitis. [Reproduced with permission from Shah BR, Lucchesi M, Amodio J (eds): Atlas of Pediatric Emergency Medicine , 2nd ed. © 2013, McGraw-Hill Education, New York, NY, Figure 3-2.] pyogenic organism may lead to liquefactive necrosis (suppuration) and abscess formation ( Figure 125-3). If the immune system is unable to eradicate a particular organism, macrophages will attempt to contain it, forming a chronic granulomatous lymphadenitis. ACUTE BILATERAL LYMPHADENOPATHY Acute bilateral lymphadenopathy is usually due to viral infection and is self-limited. Common viruses include rhinovirus, parainfluenza, influenza, respiratory syncytial virus, coronavirus, reovirus, and adenovirus. 10 Treatment is symptomatic and expectant. Alternative viral causes include infectious mononucleosis, characterized by fever, exudative pharyngitis, and significant lymphadenopathy (Figure 125-4). Heterophile antibodies or Epstein-Barr virus–specific immunoglobulin M confirms the diagnosis, and in immunocompetent patients, treatment is symptomatic. Some viral causes of lymphade nopathy are associated with classic exanthems such as measles (Koplik spots, conjunctivitis, and a descending rash) and rubella (Forchheimer spots, rash, and polyarthritis). Acute bilateral lymphadenopathy and oral lesions may also be due to herpes simplex virus (gingivostomatitis) or coxsackie virus (herpangina). Pharyngitis caused by group A Streptococcus is accompanied by cervical lymphadenopathy. 11 Bilateral swelling that extends over the jaw suggests parotid gland involvement due to mumps and may be associated with orchitis and a rash. ACUTE UNILATERAL LYMPHADENOPATHY Acute unilateral lymphadenopathy is most often due to bacterial lymphadenitis (Figure 125-5) caused by S. aureus and group A Streptococcus.12 Lymph nodes typically have signs of inflammation (erythema, warmth, tenderness), and if an abscess has developed, fluctuance may be appreciated. Often the source of group A Streptococcus is the pharynx, whereas S. aureus originates from a break in the skin. Careful examination of the head, neck, throat, skin, and ears may identify a source that can be cultured. Generally, lymph nodes <1 cm do not need treatment. If lymphad enitis measures between 1 and 3 cm, initiate antibiotics to treat group A Streptococcus and Staphylococcus for up to 10 to 14 days. 13 Nodes >3 cm raise suspicion for malignancy, but if the nodes are acute and inflammatory, a course of antibiotics and observation are reasonable. Due to increasing β-lactamase–resistant Staphylococcus, first-generation cephalosporins or amoxicillin-clavulanic acid are first-line choices,12,14,15 although clindamycin as a first-line agent is reasonable if local methicillinresistant S. aureus prevalence is high. 16 If the child is unwell or immunocompromised, use IV cefazolin, nafcillin, or clindamycin. All patients require reassessment in 48 hours, and if no improvement is appreciated, expand antibiotic coverage to treat methicillin-resistant S. aureus. Clindamycin and trimethoprim-sulfamethoxazole are both effective choices if local resistance FIGURE 125-4. Infectious mononucleosis typically presents with bilateral lymphade nopathy. A 6-year-old girl demonstrating bilateral lymphadenopathy. [Reproduced with per mission from Shah BR, Lucchesi M: Atlas of Pediatric Emergency Medicine.

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trimethoprim-sulfamethoxazole are both effective choices if local resistance FIGURE 125-4. Infectious mononucleosis typically presents with bilateral lymphade nopathy. A 6-year-old girl demonstrating bilateral lymphadenopathy. [Reproduced with per mission from Shah BR, Lucchesi M: Atlas of Pediatric Emergency Medicine. © 2006, McGraw-Hill Education, New York, NY, Figure 11-14.] FIGURE 125-5. An infant with unilateral lymphadenitis due to Staphylococcus aureus infection. [Photo contributed by Dr. J. P. Ludemann, BC Children’s Hospital, Vancouver, British Columbia, Canada.] Tintinalli_Sec12_p0669-0996.indd 785 8/2/19 7:51 PM

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trimethoprim-sulfamethoxazole are both effective choices if local resistance FIGURE 125-4. Infectious mononucleosis typically presents with bilateral lymphade nopathy. A 6-year-old girl demonstrating bilateral lymphadenopathy. [Reproduced with per mission from Shah BR, Lucchesi M: Atlas of Pediatric Emergency Medicine. © 2006, McGraw-Hill Education, New York, NY, Figure 11-14.] FIGURE 125-5. An infant with unilateral lymphadenitis due to Staphylococcus aureus infection. [Photo contributed by Dr. J. P. Ludemann, BC Children’s Hospital, Vancouver, British Columbia, Canada.] Tintinalli_Sec12_p0669-0996.indd 785 8/2/19 7:51 PM 786 SECTION 12: Pediatrics is low.16-18 Failure to improve at 48 hours is not an indication for surgical drainage as outcomes after switching to second-line antibiotics without surgery are similar.19 Fluctuance of the node suggests abscess formation due to pyogenic bacteria, typically from a tonsillar source.15 While fluctuance is associated with a higher rate of surgical drainage,20 neck abscesses often respond to antibiotics alone.15,21 Needle aspiration may be helpful to avoid incision in cosmetically important areas.15 If a superficial abscess is pointing or not resolving within 2 weeks of antibiotics, then evaluation by US and incision and drainage may be necessary. 22 Early US for inflammatory neck masses results in unnecessary cost and drainage, particularly in patients with less than 3 days of symptoms and who are older than 1 year of age. When associated with torticollis or trismus, consider a deep space infection and obtain imaging and/or surgical consultation (see Chapter 126, “Stridor and Drooling in Infants and Children”). Infants have a higher incidence of infection with group B Streptococcus than S. aureus or group A Streptococcus . Group B streptococcal infec tion can be associated with bacteremia, pneumonia, and meningitis. 24,25 Infections originating from the oral mucosa, seen most commonly in children age 5 to 15 years with periodontal disease, are more likely to contain anaerobic bacteria requiring coverage with penicillin V , amoxicillin-clavulanic acid, or clindamycin. 14 Sialadenitis is characterized by unilateral swelling crossing the jaw and tenderness after meals and can be associated with purulent discharge from Wharton’s and Stensen’s ducts (Figure 125-6). Sialolithiasis can cause recurrent infections due to outflow obstruction of the gland. SUBACUTE/CHRONIC LYMPHADENOPATHY Chronic lymphadenopathy is defined as persistence for 6 weeks without resolution. Persistent lymphadenopathy, generalized lymphadenopathy, or failure to respond to 2 to 4 weeks of antibiotics requires evaluation for uncommon infectious, congenital, and neoplastic causes. Persistent infectious agents evade eradication by granuloma formation. Gener ally, the course of evolution is slower than with typical inflammatory lymphadenitis and may be characterized by a violaceous color of the overlying skin. Refer to otolaryngology for definitive diagnosis and management. 26-28 Refer immunocompromised patients to an infectious disease specialist. Some of the more common causes of granulomatous lymphadenitis are covered below.  MYCOBACTERIAL LYMPHADENITIS Chronic cervical lymphadenitis may be due to tuberculous ( Mycobacterium tuberculosis) or nontuberculous mycobacterial strains. Clinically, both varieties of lymphadenitis are characterized by a chronic, minimally FIGURE 125-6. Sialadenitis. A. Unilateral swelling crossing the angle of the mandible. B and C. Purulent drainage from Stensen’s and Wharton’s salivary ducts, respectively. [Reproduced with permission from Knoop KJ, Stack LB, Storrow AB: Atlas of Emergency Medicine, 2nd ed. © 2002, McGraw-Hill, Inc., New York, Figures 5-32 and 5-33.] Tintinalli_Sec12_p0669-0996.indd 786 8/2/19 7:51 PM

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le of the mandible. B and C. Purulent drainage from Stensen’s and Wharton’s salivary ducts, respectively. [Reproduced with permission from Knoop KJ, Stack LB, Storrow AB: Atlas of Emergency Medicine, 2nd ed. © 2002, McGraw-Hill, Inc., New York, Figures 5-32 and 5-33.] Tintinalli_Sec12_p0669-0996.indd 786 8/2/19 7:51 PM CHAPTER 125: Neck Masses in Infants and Children 787 TABLE 125-3 Common Congenital Neck Cysts by Location, Signs, and Typical Age of Onset Type Location Signs Age Thyroglossal duct Midline (infrahyoid mostly) Cyst moves with swallowing or tongue protrusion. Birth–elderly Dermoid/ epidermoid Midline (suprahyoid) Cyst may move with swallowing. Birth–adult Branchial Anterior triangle anterior to sternocleidomastoid muscle near angle of the mandible May be associated with draining sinus. Childhood– adult Lymphangioma (cystic hygroma) Posterior triangle Soft; enlarges in first few weeks of life. Transilluminates. Birth–infancy FIGURE 125-7. Large thyroglossal duct cyst. tender, “cold abscess, ” with overlying violaceous skin. Spontaneous drainage can transform into a chronic draining sinus. 27 It is important to differentiate between tuberculous and nontuberculous strains because treatment is different. Differential Mantoux testing with a combination of antigens can identify strains in about 93% of patients. 29 Consider M. tuberculosis lymphadenitis in children with tuberculosis exposure, 30 in those exhibiting constitutional signs, and in those with an abnormal chest radiograph and with a strongly reactive purified protein derivative skin test, 31 and treat for 8 to 12 months. Treatment for nontuberculous lymphadenitis is surgical excision.32  CAT-SCRATCH DISEASE Cat-scratch disease is caused by Bartonella henselae, with inoculation by a scratch or a bite from a kitten. The inoculation site develops into a painless, erythematous vesicle or pustule after 3 to 10 days, which may disappear before other symptoms develop. After 1 to 3 weeks, regional lymphadenopathy typically develops; suppuration is uncommon. Diag nosis involves serologic testing, but because antibody development may be delayed and the primary lesion may have resolved, rely on a strong history of cat exposure and an inoculating lesion. No treatment is needed due to high rates of spontaneous resolution, although azithromycin may hasten resolution. 33 Serious complications (encephalitis) can develop in immunodeficient patients, with treatment options based primarily on case reports.  TOXOPLASMOSIS Toxoplasma gondii is a protozoan parasite with a complex life cycle that can infect humans through ingestion of undercooked meat, exposure to oocysts in cat feces, or through maternal-fetal transmission (congenital toxoplasmosis). Infection is widespread: an estimated 22.5% of children in the United States are infected by the age of 12 years old, and in some countries, up to 95% of the population has been infected. The lymphatic system is the most common organ system involved. Diagnosis is usu ally made serologically with antibody titers. Treatment is not typically needed in healthy children.  TULAREMIA Tularemia is caused by the bacteria Francisella tularensis, which is transmitted by arthropod bites, handling infected animals (classically rab bits), and contaminated food and water. The most common forms are ulceroglandular and glandular tularemia. In the ulceroglandular form, the inoculation site develops from an erythematous tender papule into an exudative ulcer within days. Regionally draining lymphadenopathy subsequently develops that may spontaneously suppurate and drain if not treated. Tularemia is diagnosed serologically with microagglutina tion testing. Gentamicin is the treatment of choice.

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ulation site develops from an erythematous tender papule into an exudative ulcer within days. Regionally draining lymphadenopathy subsequently develops that may spontaneously suppurate and drain if not treated. Tularemia is diagnosed serologically with microagglutina tion testing. Gentamicin is the treatment of choice.  OTHER CAUSES Other causes of cervical lymphadenopathy include recent immunizations (diphtheria, pertussis, and tetanus; polio; typhoid), blood transfusions (through Epstein-Barr virus, cytomegalovirus, or human immunode ficiency virus exposure), medications (including phenytoin, carbam azepine, isoniazid, and hydralazine), and systemic disease (Kawasaki’s disease; sarcoid; periodic fever, aphthous stomatitis, pharyngitis, and adenitis; and autoimmune) (Tables 125-1 and 125-2).  CONGENITAL NECK MASSES Congenital lesions are the most common cause of noninflammatory pediatric neck masses, 1 but may not be recognized until infection develops. The sternocleidomastoid muscle divides the neck into anterior and posterior triangles, and congenital neck masses can be organized into midline, anterior, and posterior groups ( Table 125-3). The most common congenital neck masses and their characteristic features are discussed below. THYROGLOSSAL DUCT CYSTS Thyroglossal duct cysts ( Figure 125-7) account for approximately 70% of congenital neck masses and are the second most common benign neck mass after lymphadenopathy.34 Thyroglossal duct cysts result from the persistence of any segment of the thyroglossal duct along its course from the foramen cecum of the tongue to the pyramidal lobe of the thyroid and are located in the midline of the neck within the anterior triangle. 35 Most of the lesions are infrahyoid (65%), whereas suprahyoid cysts account for 20%, and another 15% of lesions are at the level of Tintinalli_Sec12_p0669-0996.indd 787 8/2/19 7:51 PM

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amen cecum of the tongue to the pyramidal lobe of the thyroid and are located in the midline of the neck within the anterior triangle. 35 Most of the lesions are infrahyoid (65%), whereas suprahyoid cysts account for 20%, and another 15% of lesions are at the level of Tintinalli_Sec12_p0669-0996.indd 787 8/2/19 7:51 PM 788 SECTION 12: Pediatrics FIGURE 125-8. Second arch branchial cleft cyst. [Reproduced with permission from Knoop K, Stack L, Storrow A, Thurman RJ: Atlas of Emergency Medicine , 4th ed. © 2016. McGraw-Hill, Inc., New York, Figure 14-69, p. 469. (Photo contributor: Scott Manning, MD.)] FIGURE 125-9. Lymphangioma. [Reproduced with permission from Knoop KJ, Stack LB, Storrow AB, Thurman RJ: Atlas of Emergency Medicine , 2nd ed. © 2002, McGraw-Hill, Inc., New York, Figure 14-35.] the hyoid bone. 36,37 The pathognomonic feature is a painless fluctuant mass that moves with swallowing or protrusion of the tongue. Acutely infected cysts require antibiotics. Further investigations and surgical excision are undertaken after infection subsides because they carry a very small risk of malignant transformation. DERMOID CYSTS Dermoid cysts are developmental anomalies involving pluripotent embryonal stem cells and occur most often in children <3 years old. Dermoid cysts are usually suprahyoid 39 and midline and are often misdiagnosed as thyroglossal duct cysts. 40,41 Dermoid cysts are mobile, but they do not move with tongue protrusion. Management is surgical excision. BRANCHIAL CLEFT CYSTS Incomplete obliteration of the branchial apparatus, predominantly the cleft, leads to branchial cleft anomalies: cysts, sinuses, or fistulae. 35 Up to 95% of branchial cleft cysts arise from remnants of the second branchial cleft, 42 which are most often anterior to the sternocleidomastoid muscle near the angle of the mandible ( Figure 125-8). The cysts are round, smooth, and mobile, but are not tender unless they become infected. There may be a history of recurrent swelling or infection in the same area. Cysts may spontaneously rupture, forming an external sinus or fistula. Oral antibiotics are indicated for infected cysts prior to defini tive surgery. LYMPHANGIOMAS Lymphangiomas (also known as cystic hygromas) result from sequestration of lymphatic channels that fail to communicate with the internal jugular vein leading to a blockage of the lymphatic system. 43 They can occur anywhere in the body, but nearly 75% arise within the neck, most commonly along the jugular chain of lymphatics. Cervical lymphangiomas are soft, painless, and compressible, and can be very large (Figure 125-9). Morbidity is usually secondary to compression of surrounding structures, and large neck lesions may cause serious airway and feeding problems. Sixty-five percent present at birth, and 90% are clinically detected by the end of the second year. 36,43 Sudden enlargement can occur from infection or hemorrhage into the lesion. Careful inspection of the oral cavity and palpation of the trachea for deviation are important in assessing the airway of patients with large cystic hygromas. Injection of sclerosing agents can be used in some lesions, although surgical excision is the definitive treatment.  NEOPLASTIC NECK MASSES Malignancy must always be considered as a potential etiology in the evaluation of a child with a neck mass (see Chapter 145, “Oncologic Emergencies in Infants and Children”). Of childhood malignancies, 5% to 12% present as a head or neck mass, of which 99% are primary tumors. 1,44-47 Diagnosing malignancies is made challenging by their rar ity; only 1% of pediatric superficial lumps are malignant.

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f a child with a neck mass (see Chapter 145, “Oncologic Emergencies in Infants and Children”). Of childhood malignancies, 5% to 12% present as a head or neck mass, of which 99% are primary tumors. 1,44-47 Diagnosing malignancies is made challenging by their rar ity; only 1% of pediatric superficial lumps are malignant. 48 Accordingly, a high index of suspicion and attention to worrisome features from history and physical examination are required ( Table 125-4).12,31,46-50 One review demonstrated that in 80% of superficial masses, malignancy can be ruled out with 99.7% accuracy based on the absence of five clinical features (Table 125-4). 48 The presence of any worrying features should prompt urgent referral for further diagnostic workup that may include fine-needle aspiration cytology, imaging, or excisional biopsy. BENIGN NEOPLASMS Benign noninflammatory pediatric neck masses compose 5% of presentations. 51 An exhaustive review is not included in this chapter; instead, benign masses with specific identifying features are highlighted.  HEMANGIOMAS Hemangiomas are congenital vascular tumors that present at 2 to 4 weeks, usually grow rapidly until 9 to 10 months of age, and typically regress thereafter. When palpable, they are soft, mobile, and frequently have a bluish hue. 52 Almost 90% of hemangiomas resolve spontaneously without the need for therapy. Lesions showing unusually rapid growth, hemor rhage, recurrent infection, or compression of adjacent structures may require treatment by a specialist, including beta-blockers, steroids, and TABLE 125-4 Worrisome Features of Head and Neck Masses •  Constitutional  symptoms (fever, weight loss, night sweats, fatigue) •  History  of malignancy •   Mass features (onset in the neonatal period*; size >3 cm; duration >4–6 wk; supraclavicular, posterior, or midline location; rapid and progressive enlargement *; hard or rubbery consistency; fixation to skin or deep fascia*; associated skin ulceration*) •  Generalized  lymphadenopathy •  Inflammatory  mass >3 cm persistent for >6 wk, despite treatment •  Mass  >3 cm with firm or hard consistency* *If these five features are absent, then there is 99.7% accuracy that a superficial lump is not malignant in 80% of cases.41 Tintinalli_Sec12_p0669-0996.indd 788 8/2/19 7:52 PM