STEPHEN LUDWIG
BRANDON C. KU

DEFINITION

Neck swelling in children is a finding that elicits immediate parental concern and often prompts a visit to the physician. The finding of a neck mass invokes a response because it can be associated with malignancy. Malignancy, though part of the differential diagnosis, is a relatively uncommon cause of neck swelling by far. More common causes include inflammatory conditions, such as reactive lymphadenopathy from viral upper respiratory tract infections, bacterial adenitis, and congenital anomalies with or without bacterial superinfection. Because children often have palpable normal lymph nodes, a significant neck mass is typically defined as swelling that exceeds 2 cm in diameter. In rare cases, smaller nodes may have characteristics that prompt evaluation. Congenital anomalies, although present at birth, may not become clinically apparent until the child is school age or older.

Hospitalization is required if neck masses are present in conjunction with systemic symptoms such as fever, fatigue, or pallor; if the neck masses are large enough to comprise the airway; or if the neck masses have not responded to outpatient therapy.

CAUSE AND FREQUENCY

A differential diagnosis list for neck masses is presented in Table 13-1. Neck masses that require immediate evaluation include those that follow trauma and those that cause airway compromise (Table 13-2). The most common causes of neck swelling include benign reactive lymphadenopathy, bacterial lymphadenitis (including that caused by Bartonella henselae), hematoma, congenital causes (e.g., thyroglossal duct cyst, branchial cleft cyst, cystic hygroma), and benign tumors (e.g., lipoma, keloid). Table 13-3 indicates the type of mass by location.

QUESTIONS TO ASK AND WHY

• Is the airway compromised?

—The first and most important question to ask relates to the presence of airway compromise, since it demands immediate attention if present. Airway compromise may result from intrinsic occlusion or extrinsic compression of the airway. Airway edema may result from swelling caused by trauma or allergic reaction. Neck masses may also be associated with intra-thoracic masses that can cause respiratory distress.

• Are there systemic signs of illness?

—Other questions that help with the differential diagnosis process are the presence or absence of systemic signs, such as fever, weight loss, anorexia, night sweats, lethargy, or fatigue. Some elements of the differential diagnosis are associated with these systemic findings, such as malignancy, and others are clearly more localized.

• Is there history of or clinical concern for trauma?

—This question will help identify acute causes of neck swelling that may require surgical intervention, such as rapidly expanding hematoma. Acute bleeding prompts immediate identification of the source of bleeding and subsequent hemostasis. In addition, any trauma to the cervical spine requires stabilization of the cervical spine and evaluation.

• Is the swelling due to lymphadenitis or lymphadenopathy?

—This question will help elucidate whether there are signs of active infection (i.e., lymphadenitis) as opposed to enlargement without infection (i.e., lymphadenopathy). Signs of lymphadenitis include swelling, redness, warmth, and tenderness. Signs of lymphadenopathy may include swelling and no or mild tenderness, but the absence of significant overlying erythema, warmth, or tenderness.

• Is the swelling acute, subacute, or chronic?

—This question provides insight into possible causes of the neck swelling. Bacterial infections are usually acute and progressive. Other infections are more subacute, including Epstein-Barr virus (EBV) infection, cat-scratch disease, or tuberculosis. Congenital defects may be more chronic, with perhaps an acute superinfection that brings them to medical attention. A tumor may progressively increase in size over a variable time course depending on its histologic characteristic.

SUGGESTED READINGS

1. Pruden CM, McAneney CM. Neck mass. In: Fleisher GR, Ludwig S, Bachur RG, Gorelick MH, Ruddy RM, Shaw KN, eds. Textbook of Pediatric Emergency Medicine. 6th ed. Philadelphia: Lippincott Williams & Wilkins; 2010:385-391.

2. Friedman AM. Evaluation and management of lymphadenopathy in children. Pediatr Rev. 2008;29:53-59.

3. Leung AK, Davies HD. Cervical lymphadenitis: etiology, diagnosis and management. Curr Inf Dis Rep. 2009;11:183-189.

4. Nield LS, Kamat D. Lymphadenopathy in children: when and how to evaluate. Clin Pediatr. 2004;43:25-33.

5. Kandom N, Lee EY: Neck masses in children: current imaging guidelines and imaging findings. Semin Roentgenol. 2012;47:7-20.

CASE 13-1

Six-Year-Old Girl

STEPHEN LUDWIG

BRANDON C. KU

HISTORY OF PRESENT ILLNESS

The patient is a 6-year-old girl who was well until 2 weeks ago when she began to complain of tenderness on the right side of her neck. This continued for 1 week, after which the parents noticed swelling on the right side of her neck. The swelling and tenderness increased and the patient developed decreased range of neck motion. There was no dysphagia and no upper respiratory infection symptoms. The patient did not have antecedent trauma. She frequently played with the neighbor’s cat but had no known exposure to other animals such as rabbits. A tuberculin skin test, performed 1 week ago, was negative; an anergy panel had not been placed.

MEDICAL HISTORY

The patient had chicken pox 1 month ago. There are no known allergies. She did not receive any prescribed medications. There had been no recent travel.

PHYSICAL EXAMINATION

T 37.8°C; HR 96 bpm; RR 20; BP 106/69 mmHg; Weight 23 kg (75%-90%); Height 44.5 cm (25%-50%)

On general examination, the patient was alert and in no acute distress. She was normocephalic; optic discs were sharp; pupils were equal, round and reactive to light; extraocular muscles were intact; tympanic membranes were gray bilaterally, mobile; nose was without discharge; mouth was without lesions, but mucous membranes were notable for pallor, few petechiae of posterior pharynx. Neck on left side was supple with no prominent adenopathy. There was erythema and swelling that was midline and more prominent just to the left of the midline (Figure 13-1) that was tender to palpation and moved with swallowing. There was no movement with tongue thrust and the mass had well-defined borders. The following were also noted. Lungs: clear lung fields with decreased breath sounds at bases but no rales. Cardiac: regular rate and rhythm; II/IV systolic murmur, no gallop, no rub. Abdomen: soft, non-tender, nondistended; positive bowel sounds × 4; no hepatosplenomegaly. Nodes: no prominent adenopathy. Skin: no rash. Neurologic examination: CN II-XII intact; motor 5/5 throughout; DTR 2+ bilateral on upper extremities and lower extremities; proprioception intact.

DIAGNOSTIC STUDIES

WBC count, 9100/mm³ (56% segmented neutrophils, 34% lymphocytes, 5% eosinophils, 5% monocytes); hemoglobin, 11.2 mg/dL; platelets, 565 000/mm³; ESR, 45 mm/h; PT/PTT, 11.6/24.6 seconds; Barton-ella henselae antibody titers, 1:32; T3, 1.6; TSH, 4.3.

COURSE OF ILLNESS

The patient had evidence of an acute infection. There was tenderness, swelling, and limitation of range of motion. Because of the acute nature of this illness, the size of the mass (>3 cm), and the progression of neck swelling, hospitalization was indicated.

DISCUSSION CASE 13-1

DIFFERENTIAL DIAGNOSIS

The differential diagnosis in this case included an infected thyroglossal duct cyst. Lymphadenitis was possible, although the location was unusual. Bacteria that typically cause cervical lymphadenitis include Staphylococcus aureus, group A beta-hemolytic Streptococcus, Bartonella henselae, actinomycosis, and oral anaerobes. Cat-scratch lymphadenitis was less likely. The indirect fluorescent antibody (IFA) test has high sensitivity (>95%) and specificity (>98%) for the diagnosis of cat-scratch disease for patients who meet the classic case definition of CSD. Enzyme immunoassay (EIA) testing for IgM and IgG antibodies to B. henselae is also commercially available. A high antibody titer (>1:64) is suggestive of recent infection. However, 2%-6% of asymptomatic cat owners have a low-level positive antibody titer (~1:16 or 1:32). In such cases, paired serologic studies (antibody studies repeated 2 weeks later and performed at the same time as a sample of the serum from the initial test) to detect an increase in the antibody titer may be helpful to confirm the disease; those without any change in the antibody titer when repeated 2 weeks later are unlikely to have an acute infection with B. henselae. The low-level positive in this case was thought to be unrelated to the child’s symptoms.

In the location of this neck swelling, one must also consider thyroid-related masses such as tumors arising from the thyroid or aberrant thyroid gland. Thyroid function studies were normal. The CT scan showed a mass that was larger than clinically appreciated. There was also minimal tracheal deviation that was suggestive of a thyroid nodule. Thus, it was determined that a tissue diagnosis was required without delay.

DIAGNOSIS

A neck CT scan revealed a 4 × 5 cm solid neck mass with minimal tracheal deviation. Biopsy of the mass did not reveal lymph node or thyroid tissues; fibrotic changes along with numerous neutrophils, lymphocytes, and monocytes were consistent with an inflammatory process. Bacterial cultures from the mass obtained during a needle biopsy were positive for Eikenella corrodens; smears for acid-fast bacilli (AFB) were negative. The diagnosis was that of a thyroglossal duct cyst that was infected with Eikenella corrodens.

INCIDENCE AND EPIDEMIOLOGY OF THYROGLOSSAL DUCT CYST

Thyroglossal duct cysts are congenital remnants that occur when one thyroid migrates from the base of the tongue to its position in the neck. It occurs anywhere from the base of the tongue through the hyoid bone to just above the thyroid cartilage. Although asymptomatic while dormant, they may become symptomatic when infected.

CLINICAL PRESENTATION

Thyroglossal duct cysts may be evident at birth or may be dormant for many years until they become infected producing redness, swelling, and pain. The mass of a thyroglossal duct cyst may enlarge to the point of respiratory compromise. Eikenella corrodens is a slow growing organism that has been reported to cause a variety of clinical infections from infected neck masses to brain abscesses, lung infection, bite wound infection, and bone and joint space infections.

DIAGNOSTIC APPROACH

The diagnosis is suspected clinically by position and movement with swallowing or protrusion of the tongue. Ultrasound is the preferred imaging modality though CT scan may further delineate the size and location of the mass. The possibility of an aberrant thyroid must be ruled out. A diagnostic clue to the thyroglossal duct cyst is that it moves when the patient is asked to swallow. The cyst may contain elements of thyroid tissue; thus, if the cyst shows solid elements they should be identified with a nuclear scan prior to surgery, lest they be inadvertently removed. Thyroglossal duct cysts are the most common midline lesions.

Eikenella corrodens was isolated in this case. It is an unusual organism but one that should be considered if the patient has active periodontal disease or deteriorated oral health. It is also more common in individuals who are immunocompromised although that was not the circumstance in our patient. Excisional biopsy is the usual method to confirm the diagnosis; the excisional biopsy is typically performed after resolution of the acute superinfection.

TREATMENT

The ultimate treatment is surgical. Removal of the cyst and the other remnants of the migration tract are important. This is difficult and detailed surgery that involves surgery in the field of many other important vital structures. Prior to surgery, antibiotic therapy may be helpful in lessening the inflammation and aiding in the postoperative healing process. Antibiotics to cover Gram-positive organism are customary. Eikenella corrodens is a usual Gram-negative organism that probably derives from active periodontal disease. Treatment may be effective with a wide range of antibiotics, however, usually the combination of antimicrobial therapy and surgical draining is required to effect complete recovery.

SUGGESTED READINGS

1. Paul K, Patel SS. Eikenella corrodens infections in children and adolescents: case reports and review of the literature. Clin Infec Dis. 2001;33:545-61.

2. Marra S, Hotaling AJ. Deep neck infections. Am J Otolaryngol. 1996;17:287-298.

3. Sheng WS, Hsueh PR, Hung CC, et al. Clinical features of patients with invasive Eikenella corrodens infections and microbiological characteristics of the causative isolates. Euro J Clin Microbiol Infect Dis. 2001;20:231-236.

4. Cheng AF, Man DW, French GL. Thyroid abscess caused by Eikenella corrodens. J Infect. 1988;16:181-185.

5. LaRiviere CA, Waldhausen JH. Congenital cervical cysts, sinuses and fistulae in pediatric surgery. Surg Clin North Am. 2012;92:583-597.

6. Goff CJ, Allred C, Glade RS. Current management of congenital cysts sinuses and fistulae. Curr Opin Otolarygol Head Neck Surg. 2012;20:533-539.

CASE 13-2

Two-Year-Old Girl

STEPHEN LUDWIG
BRANDON C. KU

HISTORY OF PRESENT ILLNESS

The patient is a 2-year-old Vietnamese girl who has been well until her mother noted a bump under her jaw a few days ago. She has not had any fever or upper respiratory tract symptoms (cough, rhinorrhea), or sore throat. She has been acting well. The bump does not hurt, is not red, but during the last 2-3 days it has increased in size from pea-sized to 4-5 cm, and it now looks purple. She had normal oral intake and urine output. There was no vomiting, diarrhea, or rash. The patient sweats at night, but this is not new. There has been no weight loss or change in appetite. Sleep pattern is normal. There was no history of animal bites. There was no dyspnea or wheezing.

MEDICAL HISTORY

The girl was born at term by spontaneous vaginal delivery without complications. She had pyelonephritis twice, once at 14 months of age and again at 16 months of age. A voiding cystourethrogram performed after the second episode of pyelonephritis revealed grade 3 vesicoureteral reflux. She did not have frequent upper respiratory infections, otitis media, or pneumonia. She has not had any skin or soft tissues infections. Her only medication was trimethoprim-sulfamethoxazole which she received as prophylaxis against urinary tract infections. She has received her routinely recommended immunizations, including measles, mumps, rubella, and varicella vaccination at 15 months of age. She has no known allergies. There is no family history of recurrent infections, immune dysfunction or autoimmune disease. The patient lives with parents in a semi-rural area of New York state. Her maternal grandmother assists with childcare. No family members or recent visitors have been incarcerated or live or work in nursing homes or other chronic care facilities. The family does not have any pets. The only animal with whom the child interacts is an older cat owned by a maternal aunt who lives nearby.

PHYSICAL EXAMINATION

T 36.0°C axillary; HR 108; RR 22; BP 121/60; Weight 12.8 kg (30th percentile); Height 91 cm (30th percentile); Head Circumference 48.1 cm (25th percentile)

On general examination, the patient was alert, well nourished, and well developed. She cooperated with the examination in a manner appropriate for her age. She had a 3 × 2 cm firm nodule with purplish hue of overlying skin at the angle of the right mandible (Figure 13-2). It was not tender. There was no overlying warmth or discharge. There were no cardiac murmurs or rubs. The lungs were clear to auscultation bilaterally. With the exception of the neck, there were no masses or enlarged lymph nodes elsewhere.

DIAGNOSTIC STUDIES

Hemoglobin, 13 g/dL; platelets, 210000/mm³;

WBC count, 6400/mm³ (45 segmented neutrophils, 46 lymphocytes, 8 monocytes); sodium, 139 mEq/L; chloride, 100 mEq/L; blood urea nitrogen, 17 mg/dL; glucose, 84 mg/dL; PT, 12.8; PTT, 31.9 seconds; phosphorous, 4.5 mg/dL; calcium, 8.2 mg/dL; magnesium, 1.6 mg/dL; cat-scratch titers, <1:32.

COURSE OF ILLNESS

The primary pediatrician initiated treatment with oral amoxicillin-clavulanate with an increase in the size of the mass over 2 weeks. Therefore, the antibiotic regimen was changed to clindamycin without any substantive change in the lymph node after 1 week. Tuberculin skin testing by purified protein derivative resulted in a 10-mm area of induration. A chest X-ray (Figure 13-3) revealed additional findings that prompted an excisional biopsy of the lymph node and bronchoscopy.

DISCUSSION CASE 13-2

DIFFERENTIAL DIAGNOSIS

The patient was characterized by having an increased neck mass. The mass in this case did not hurt. This differential is from an acute adenoiditis with a usual bacterial organism. The location of the mass was over the mandible in a position typical for a lymph node. The patient was treated with antibiotics without response. The chest radiograph revealed bilateral mediastinal lymphadenopathy (Figure 13-3). A chest CT confirmed hilar lymphadenopathy but did not identify any focal lung abnormalities. She was admitted for excisional biopsy of the lymph nodes of the neck and bronchoscopy.

Lymph node pathology revealed necrotizing granulomas consistent with atypical mycobacterial infection. In addition, there were two other findings of importance, a positive PPD and positive chest radiograph. These findings required that a tissue diagnosis be made and so the patient was sent for biopsy and bronchoscopy after less invasive diagnostic studies failed to reveal an etiology. The pathology showed a pattern consistent with atypical mycobacterial infection. This was also consistent with the child’s course of an acute enlargement of a lymph node but not the signs of an acute bacterial infection. There was no history of trauma. The location of the mass was not usual for a congenital lesion.

DIAGNOSIS

Nontuberculous mycobacterial infection causing adenitis in neck and mediastinum. There was no underlying immunodeficiency found in this patient.

INCIDENCE AND EPIDEMIOLOGY OF ADENITIS

Most cases of mycobacterial adenitis in children are usually caused by nontuberculosis mycobacteria rather than Mycobacterium tuberculosis. Nontuberculous mycobacteria typically include M. abscessus, M. chelonae, M. fortuitum, M. avium complex, and M. kansasii. They are typically found in soil and water, including natural and treated water sources.

CLINICAL MANIFESTATIONS

Cervical or submandibular adenitis most commonly occurs in otherwise healthy children 1-5 years of age. The likely mechanism is ingestion of contaminated sources such as soil. Mycobacterium avium complex is identified most often. The presentation is protracted lymphadenopathy; the affected lymph nodes are usually unilateral and nontender. In the absence of effective therapy, the affected lymph nodes may resolve or progression to liquefaction with violaceous discoloration of the overlying skin, followed by spontaneous and prolonged drainage from a cutaneous fistula. Nontuberculous mycobacteria may also involve other lymph node groups, including the mediastinal lymph nodes as in this case. In the chest, the findings are relatively silent and relate primarily to enlargement of paratracheal lymph nodes.

DIAGNOSTIC APPROACH

In this case, the diagnosis was strengthened by findings of enlarged mediastinal nodes and the notation of a positive PPD.

Tuberculin skin testing. Tuberculin skin tests (i.e., purified protein derivative) may be negative or positive. Many children have intermediate induration (5-10 mm) though up to one-third of affected children may have induration more than 10 mm. In this case, although there was strong suspicion for nontuberculous mycobacteria, there was the need to confirm the diagnosis with biopsy or bronchoscopy findings.

Biopsy. As much material as possible is required; swabs typically have poor yield. The specimens should be placed in both broth and solid media. Cultures in broth media have a higher yield and tend to produce more rapid results than specimens plated on solid media; solid media, however, permit observation of colony morphology and recognition of multiple mycobacterial species. Excisional biopsy is often performed for simultaneous diagnosis and treatment.

TREATMENT

The treatment in cases of atypical mycobacteria is lymph node excision. Many nodes may regress on their own if untreated. However, the need to establish a diagnosis usually prompts excision. If excision is not possible because of location (e.g., facial nerve injury may occur with excision of preauricular lymph nodes), antimicrobial therapy may be beneficial. Treatment depends on the nontuberculous mycobacteria isolated. Treatment typically involves at least two effective drugs. Empiric therapy, while awaiting culture and susceptibility results, often includes clarithromycin in combination with rifabutin, rifampin, ciprofloxacin, or ethambutol. A randomized trial compared a “wait-and-see” approach with 12-weeks of clarithromycin plus rifabutin; eligible patients all had red, fluctuant lymphadenitis. The median time to resolution of disease (defined as a 75% or greater reduction in lymph node size with cured fistula and total skin closure without recurrence) was 36 weeks for the treated group and 40 weeks for the “wait-and-see” group.

SUGGESTED READINGS

1. Altman RP, Margileth AM. Cervical lymphadenopathy from atypical mycobacteria: diagnosis and surgical treatment. J Pediatr Surg. 1975;10:419-422.

2. Dhooge I, Dhooge C, DeBaets F, et al. Diagnostic and therapeutic management of atypical mycobacterial infections in children. Euro Arch Oto-Rhin-Laryngol. 1993;250:387-391.

3. Benson-Mitchell R, Buchanan G. Cervical lymphadenopathy secondary to atypical mycobacterial in children. J Laryngol Otolo. 1996;110:48-51.

4. Danielides V, Patrikakos G, Moerman M, et al. Diagnosis, management and surgical treatment of non- tuberculous mycobacterial head and neck infection in children. J Oto-Rhino-Laryngol. 2002;64:284-289.

5. Scott CA, Atkinson SH, Sodha A, et al. Management of lymphadenitis due to non-tuberculous mycobacterial infection in children. Pediatr Surg Int. 2012;28: 461-466.

6. Tortoli E. Clinical manifestations of nontuberculous mycobacteria infections. Clin Microbiol Infect. 2009;15:906-910.

7. Clark JE. Nontuberculous lymphadenopathy in children: using evidence to plan optimal management. Adv Exp Med Biol. 2011;719:117-121.

8. Griffith DE, Aksamit T, Brown-Elliott BA, et al. An official ATS/IDSA statement: diagnosis, treatment, and prevention of nontuberculous mycobacterial disease. Am J Resp Crit Care Med. 2007;175:367-416.

9. Hazra R, Robson CD, Perez-Atayde AR, Husson RN. Lymphadenitis due to nontuberculous mycobacteria in children: presentation and response to therapy. Clin Infect Dis. 1999;28:123-129.

10. Lindeboom JA. Conservative wait-and-see therapy versus antibiotic treatment for nontuberculous mycobacterial cervicofacial lymphadenitis in children. Clin Infect Dis. 2011;52:180-184.

CASE 13-3

Two-Month-Old Boy

PAUL L. ARONSON

HISTORY OF PRESENT ILLNESS

A 2-month-old male presented with a 3-day history of neck swelling and 1 day of neck redness. The parents did not notice any swelling prior to the past 3 days. He has had a fever at home for 1 day. His oral intake has been adequate. The patient had no emesis, diarrhea, respiratory symptoms, or rash elsewhere on the body. There was no cat or TB exposure.

MEDICAL HISTORY

The patient was born at term during an uncomplicated delivery. There have been no hospitalizations. The patient had not yet received any immunizations. There were no known allergies and the patient had not received any medications. Family history is notable for no significant illnesses. The mother and father had arrived from West Africa 6 months ago.

PHYSICAL EXAMINATION

T 38.5°C; HR 176 bpm; RR 44/min; BP 112/76 mmHg; SpO₂ 97% in room air; Weight 6.2 kg (75th-90th percentile); Length 60 cm (90th percentile); Head Circumference 41 cm (90th percentile)

The infant was not in any distress. The head was normocephalic and the anterior fontanelle was open and flat. The oropharynx was clear. A tender, mildly indurated erythematous mass arose in the superior portion of the neck at the angle of the jaw. There was no fluctuance. The lung and heart sounds were normal. There was no hepatomegaly, and no axillary or inguinal lymphadenopathy.

DIAGNOSTIC STUDIES

The complete blood count revealed the following: 15 300 WBCs/mm³ (9% band forms, 65% segmented neutrophils, and 26% lymphocytes); hemoglobin, 11.7 g/dL; platelets, 296 000/mm³. Serum electrolytes revealed the following: sodium, 135 mEq/L; potassium, 5.3 mEq/L; chloride, 103 mEq/L; bicarbonate, 24 mEq/L; blood urea nitrogen, 4 mg/dL; creatinine, 0.2 mg/dL; and glucose, 113 mg/dL. Cerebrospinal fluid examination revealed 1 WBC, no RBCs, and a glucose of 68 mg/dL. There were no bacteria on gram stain. Blood, cerebrospinal fluid, and urine cultures were obtained. CT scan of neck with intravenous contrast was obtained (Figure 13-4).

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