Keri L. Carstairs
Lymph nodes are commonly palpated in children’s necks. Cervical nodes >10 mm are considered abnormal and referred to as lymphadenopathy (1). Enlarged lymph nodes can arise in association with a wide variety of infectious, inflammatory, or neoplastic disease processes (2). The most common cause of a benign neck mass in children is cervical lymphadenopathy secondary to an upper respiratory tract infection, usually of viral origin.
Lymphadenitis is one type of lymphadenopathy and can be either acute or chronic. Although some define lymphadenitis broadly as an “inflammation of a lymph node,” others restrict the definition to include only bacterial infections (1,3).
After invading regional tissues, microorganisms enter the lymphatic system, where they are trapped and destroyed by phagocytes. This activity, coupled with the lymphocytic proliferation and transformation results in swelling of the node. Infectious organisms able to survive intracellularly can represent a persistent stimulus leading to a chronic lymphatic cell hyperplasia. Lymph nodes may become infiltrated with neutrophils in response to bacteria, with histiocytes in histiocytosis (and certain storage diseases), and with malignant cells in leukemia, lymphoma, or metastatic solid tumors (2).
In the case of bacterial infection, abscess formation may ensue when neutrophils accumulate within lymph nodes. (1,4). Group A β-hemolytic Streptococci sp and Staphylococcus aureus are the most frequent pathogens of bacterial lymphadenitis, accounting for nearly 95% of acute bacterial lymphadenitis. Children with staphylococcal infections generally have a longer history of symptoms and an increased incidence of fluctuance compared with those with streptococcal infections (1,4,5).
CLINICAL PRESENTATION
Cervical lymphadenitis presents most commonly in children between 1 and 4 years of age, and there is no significant difference in incidence between genders. It is helpful to determine whether the cervical lymphadenitis developed in an acute, subacute, or chronic manner. The acute bacterial nodal enlargement usually develops rather quickly and unilaterally. These nodes are typically very tender and sometimes feel fluctuant. Erythema of the overlying skin is occasionally noted. Affected children often experience malaise and irritability and may be febrile. Frequently, a concurrent pharyngitis, otitis media, upper respiratory tract infection, dental abscess, or adjacent skin infection can be detected, or a recent history of infection can be elicited. Subacute or chronic lymphadenitis develops in 2 to 3 weeks and tends to be relatively painless. Lymphadenitis may be caused by catscratch disease, atypical mycobacteria, or tuberculosis.
Acute bilateral lymphadenitis most often occurs in response to an acute upper respiratory infection such as pharyngitis caused by viral pathogens or Streptococcus pyogenes and Mycoplasma pneumoniae.These nodes are usually small, soft, can be either tender or nontender, and do not have associated erythema or warmth.
There are rarely significant sequelae when adenitis is managed appropriately. Of particular concern is the possibility of misdiagnosing and consequently mismanaging another more serious problem (6). It is sometimes difficult to distinguish lymphadenopathy or adenitis from a true neck mass. Neck masses may be congenital or acquired, infectious or neoplastic, benign or malignant. In children, the incidence of neoplastic lesions is low compared to adults. In neither group does the neck mass commonly present as an acutely emergent or life-threatening problem.
DIFFERENTIAL DIAGNOSIS
Obtaining a careful history and performing a complete examination can often differentiate the disorders most commonly confused with adenitis (Table 258.1) (6).
TABLE 258.1
Differential Diagnosis of Lymphadenitis
Viral Lymphadenopathy
Viral infections, especially those producing viremia, are the most common cause of lymphadenopathy in children and usually subside within a few days to 2 weeks. Often bilateral, causative agents include respiratory viruses such as herpes simplex gingivostomatitis, adenoviral syndromes, roseola, Enterovirus, human herpesvirus 6, Coxsackievirus, rubella, rubeola, varicella and infectious mononucleosis caused by Epstein–Barr virus (EBV), or cytomegalovirus (CMV) (1,2,4).
Bacterial Lymphadenitis
The presence of erythema, warmth, and tenderness overlying a node typically indicates the acute inflammatory response of pyogenic bacterial lymphadenitis. Most acutely infected nodes in children are rubbery or firm in consistency and are freely mobile. Left untreated, these nodes, often unilateral, tend to suppurate and may rupture onto the skin or, less commonly, dissect into the underlying soft tissues (1,4,5).
Infectious Mononucleosis
EBV causes infectious mononucleosis. It often presents as acute or subacute cervical lymphadenopathy. These nodes tend to be posterior cervical, bilateral, and moderately painful (4). They are present in 93% of infected children (2). Associated findings include malaise, fever, tonsillopharyngitis, hepatosplenomegaly, and generalized lymph node enlargement. A positive heterophil test (Monospot) confirms the diagnosis. In children younger than 4 years of age, the heterophil test is unreliable, and specific EBV titers should be done. Treatment is supportive. The patient should be instructed to avoid contact sports for several months after the illness because of the risk of splenic rupture. Rarely, cervical lymphadenopathy in infectious mononucleosis is associated with respiratory distress because of extremely enlarged (“kissing”) tonsils, and this condition may require hospitalization and steroid use.
Catscratch Disease
Most cases of catscratch disease are caused by Bartonella henselae. In approximately 50% of cases, there is a primary lesion at the site of a 7- to 14-day-old scratch or bite by a cat. This lesion begins as an indurated erythematous papule and later may become vesicular or pustular. The lesion resolves within 10 to 20 days. Catscratch disease usually presents subacutely. Several days to weeks later, the lymph nodes draining this area become progressively enlarged. The most common sites of lymphadenopathy are the axilla (52%) followed by the neck (28%) (2). These nodes are typically only moderately tender, but occasionally will suppurate. Sometimes, patients are febrile and may experience headache, myalgia, or malaise. Other complications, such as encephalitis, hepatosplenic abscesses, and osteolytic lesions, are uncommon. Although the diagnosis is usually made on clinical grounds, it can be confirmed serologically. The adenopathy generally resolves in 6 to 8 weeks with supportive treatment only. However, azithromycin is recommended for lymphadenopathy related to catscratch disease (5). Fluctuant nodes often need surgical intervention (1,8–10).
Methicillin-Resistant Staphylococcus aureus
With the changing epidemiology of methicillin-resistant Staphylococcus aureus (MRSA) infection in healthy individuals, this bacterium should be considered when patients present with soft tissue infections. MRSA should be considered in patients presenting with cervical lymph node swelling (11). MRSA infections can also occur in healthy newborns (11,12).
Mycobacterial Lymphadenitis
Atypical mycobacterial cervical lymphadenitis usually presents as subacute or chronic unilaterally enlarged lymph nodes that do not respond to antibiotics (1,13). Nontuberculous mycobacterial lymphadenitis generally occurs in early childhood. Infected nodes tend to be relatively painless but are sometimes tender and may even suppurate and drain. Systemic symptomatology is uncommon. Tuberculin skin testing is unreliable. Antituberculous drugs are not recommended, and excisional biopsy is both diagnostic and therapeutic (14).
Tuberculous lymphadenitis is rarely seen in the United States (10,15,16). Diseased nodes are similar to those of nontuberculous mycobacterial adenitis. To exclude the diagnosis, the family should be questioned about their exposure to tuberculosis, a tuberculin skin test should be done, and a chest radiograph ordered. Tuberculous lymph nodes should never be aspirated, because aspiration may lead to sinus development. Antituberculous drugs are effective in treating this condition. Lastly, infection should be reported to public health authorities.
Kawasaki Disease
Also known as mucocutaneous lymph node syndrome, Kawasaki disease is diagnosed in the presence of fever, plus four of the five diagnostic criteria (cervical lymphadenopathy, rash, oral exanthem, conjunctival injection, and swelling and redness of palms and soles) and exclusion of other illnesses. Cervical lymphadenopathy is found in only 50% of cases. Nodes are usually unilateral and confined to the anterior triangle, moderately tender, nonfluctuant, and firm, with or without overlying erythema. The early diagnosis of Kawasaki disease is critical, because treatment with salicylates and intravenous immunoglobulin can prevent serious complications, namely, coronary aneurysm (10).
Group B Streptococcal Cellulitis–Adenitis Syndrome
The group B streptococcal cellulitis adenitis syndrome occurs in neonates (5). The neonate presents with the abrupt onset of fever, facial or submandibular erythema and swelling, irritability, poor feeding, and toxicity. About 80% have an ipsilateral otitis media. Meningitis may accompany lymphadenitis. The organism can be isolated from blood and from aspirates of the lymph node or the cellulitis. A complete sepsis workup should be performed, and the infant must be admitted to the hospital for intravenous antibiotic therapy (5,10,17).
Toxoplasmosis
Acquired toxoplasmosis sometimes presents as a single, enlarged, posterior cervical node that may or may not be tender (1,18,19). These nodes tend to vary in size but do not suppurate. The diagnosis can be made by performing serial toxoplasma titers. Pharmacologic therapy should be withheld unless fever or a complication such as pneumonitis, myocarditis, meningitis, or encephalitis ensues.
Other Infectious Etiologies
Other uncommon causes of cervical lymphadenopathy include Pasteurella multocida, Yersinia pestis, histoplasmosis, and CMV.
Rheumatologic Lymphadenopathy
A number of rheumatologic illnesses can cause cervical lymphadenopathy; chief among them is juvenile rheumatoid arthritis.
Neoplasms
Neoplasms are rare in children, occurring in 1.4% of patients younger than 17 years of age with a superficial lump on any part of the body. Careful follow-up of all lymphadenopathy is mandatory, especially if it is associated with fever or weight loss or if it fails to resolve (1,9,20).
Benign neoplasms in children that present as neck masses include hemangiomas, lymphangiomas, fibromas, neurofibromas, and lipomas. Malignant neck neoplasms in children tend to be single, nontender, immobile, and fast growing and are located in the posterior triangle of the neck. Age is a key element in the occurrence of the various malignant neck neoplasms. From ages 1 to 5, neuroblastoma, rhabdomyosarcoma, histiocytic lymphoma, and Hodgkin disease are the most common. In the older child, histiocytic lymphoma and Hodgkin disease occur equally often. The adolescent most commonly acquires Hodgkin disease. Rhabdomyosarcoma is the most common pediatric solid tumor of the head and neck and one of the most common tumors in children younger than age 6; frequently presenting with rapid growth in the orbit, nasopharynx, ear, mastoid, face, or tongue. Neuroblastoma is the most common solid tumor in children overall, but presents as a neck mass only about 5% of the time.
ED EVALUATION AND MANAGEMENT
Many of the aforementioned diagnoses can be made simply by taking a complete history and performing a thorough examination. The following points should be addressed when questioning the family: duration of adenopathy or mass; history of an adjacent skin lesion, earache, upper respiratory tract infection, sore throat, or dental problem; presence of fever or weight loss; exposure to tuberculosis; contact with cats; and travel (1,4). When attempting to determine whether a neck mass may be a lymph node, it is important to recognize the location of the normal chain of cervical lymph nodes. The size, consistency, mobility, and tenderness of each affected node must be noted (1,10). Marking the dimensions with ink and recording them in the emergency department (ED) record can be helpful to the next physician who evaluates the patient. The head, ears, eyes, nose, oropharynx, and neck are examined, and the child is evaluated for hepatomegaly, splenomegaly, and other adenopathy (1).
Useful studies include a complete blood cell count, erythrocyte sedimentation rate, C reactive protein, a Monospot or EBV serology, a throat culture for group A Streptococcus sp, and a tuberculin skin test. It is unnecessary to order all of these tests on every patient. The tests prove most helpful when the cause of the adenopathy is unknown. A blood culture should be obtained if the patient appears toxic. Older children with cervical adenitis are rarely bacteremic, but neonates often are and must be evaluated for possible sepsis (17). Additional studies may be needed in the older child, depending on the history and physical examination (e.g., serology for catscratch disease or EBV). Sometimes, a definitive diagnosis is not made until a biopsy is performed.
Needle aspiration is useful both diagnostically and therapeutically, and any fluctuant node should be aspirated if tuberculous lymphadenitis cannot be excluded. An ultrasound can help determine whether a node or mass is cystic or solid, if necessary (22). The largest, most fluctuant node is selected, the overlying skin is cleansed, a 20-gauge needle attached to a 20-mL syringe is inserted into the fluctuant region, and aspiration is done. If pus is not obtained, 1 to 2 mL of sterile, nonbacteriostatic saline is injected into the node, which is reaspirated. The aspirate is sent for Gram stain and cultures for aerobic bacteria, anaerobic bacteria, mycobacteria, and fungi (1,2,4).
If bacterial cervical lymphadenitis is suspected, treatment with antibiotics effective against the principal bacterial pathogens, group A β-hemolytic Streptococcus sp and S. aureus, should be initiated. Analgesics and warm compresses may also be helpful (1). Most patients can be successfully managed as outpatients with follow-up arranged. Outpatient and inpatient therapy is outlined in Table 258.2(1,18,23). Failure to respond to oral antibiotics does not necessarily mean the cause of adenitis is not bacterial; parenteral antibiotics may be required. If the lymphadenitis is associated with dental disease, anaerobic infection needs to be considered (1,23).
TABLE 258.2
Management of Bacterial Lymphadenitis
KEY TESTING
• History and physical examination should drive testing and treatment decisions
• Neonates and children who are septic-appearing should have CBC, CRP, ESR, and blood cultures obtained
• Ultrasound can be used to differentiate solid from cystic masses
• Aspiration of fluid for Gram stain and culture when indicated by history and physical examination.
CRITICAL INTERVENTIONS
• Take a detailed history, including travel and animal contact, to differentiate lymphadenitis from other disorders.
• Arrange follow-up for all case of lymphadenitis, especially for patients with large nodes, fever, weight loss, or other concerning systemic signs.
DISPOSITION
Most children can be managed successfully as outpatients. They should be reevaluated, to monitor their progress, within 2 to 3 days of the ED visit. Follow-up is very important and is done best by the patient’s primary care physician, who should reexamine the child serially until the node regresses.
Admission for intravenous antibiotics is warranted if the child appears toxic, if the lymphadenitis is severe (e.g., node diameter >3 to 4 cm or high fever), if the lymphadenitis fails to respond to oral medications, or if the patient is a neonate or cannot tolerate oral antibiotics (18). Admission for further evaluation is recommended if there is mediastinal lymphadenopathy on the chest radiograph, weight loss, fever for more than 1 week, or a suspicion of Kawasaki disease or malignancy (9).
Biopsy is indicated if the node increases in size or does not decrease in 4 to 6 weeks or return to normal in 8 to 12 weeks (9). Particularly worrisome findings are weight loss, persistent fever, or fixation to adjacent tissues or overlying skin (1,4,7).
Common Pitfalls
• Treating patients with severe lymphadenitis orally and delaying an inevitable admission for intravenous antibiotics.
• Failure to consider atypical mycobacterial lymphadenitis, Kawasaki disease, and catscratch disease in the differential diagnosis.
ACKNOWLEDGMENTS
The author gratefully acknowledges the contributions of Mobeen H. Rathore and Sabiha Hussain to the content of this chapter.
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