ORIGINAL ARTICLE Childhood Cervical P Lymphadenopathy H Alexander K.C. Leung, MBBS, FRCPC, FRCP (UK & Irel), FRCPCH, & C W. Lane M. Robson, MD, FRCPC, FAAP, FRCP (Glasg) ABSTRACT Enlarged cervical lymph nodes are common in children (Leung & Rob- son, 1991). About 38% to 45% of otherwise normal children have palpable Cervical lymphadenopathy is a common problem in children. The cervical lymph nodes (Larsson et al., 1994). Cervical lymphadenopathy is condition most commonly repre- usually defined as cervical lymph nodal tissue measuring more than 1 cm sents a transient response to a be- in diameter (Darville & Jacobs, 2002; Margileth, 1995; Schreiber & Berman, nign local or generalized infection, 1996). Cervical lymphadenopathy most commonly represents a transient but occasionally it might herald the response to a benign local or generalized infection, but occasionally it presence of a more serious disorder. might herald the presence of a more serious disorder such as malignancy. Acute bilateral cervical lym- This article reviews the pathophysiology, etiology, differential diagnosis, phadenopathy usually is caused by clinical evaluation, and management of children with cervical lym- a viral upper respiratory tract infec- phadenopathy. tion or streptococcal pharyngitis. PATHOPHYSIOLOGY Acute unilateral cervical lym- phadenitis is caused by streptococ- The superficial cervical lymph nodes lie on top of the sternomastoid muscle cal or staphylococcal infection in and include the anterior group, which lies along the anterior jugular vein, 40% to 80% of cases. The most and the posterior group, which lies along the external jugular vein (Darville common causes of subacute or & Jacobs, 2002). The deep cervical lymph nodes lie deep to the sternomas- chronic lymphadenitis are cat toid along the internal jugular vein and are divided into superior and infe- scratch disease, mycobacterial in- rior groups. The superior deep nodes lie below the angle of the mandible, fection, and toxoplasmosis. Supra- whereas the inferior deep nodes lie at the base of the neck (Malley, 2000). clavicular or posterior cervical lym- The superficial cervical lymph nodes receive afferents from the mastoid, phadenopathy carries a much tissues of the neck, and the parotid (preauricular) and submaxillary nodes higher risk for malignancies than (Darville & Jacobs, 2002). The efferent drainage terminates in the upper does anterior cervical lym- phadenopathy. Generalized lym- deep cervical lymph nodes (Darville & Jacobs). The superior deep cervical phadenopathy is often caused by a nodes drain the palatine tonsils and the submental nodes. The lower deep viral infection, and less frequently cervical nodes drain the larynx, trachea, thyroid, and esophagus. by malignancies, collagen vascular Lymphadenopathy might be caused by proliferation of cells intrinsic to diseases, and medications. Labora- the node, such as lymphocytes, plasma cells, monocytes, and histiocytes, or tory tests are not necessary in the by infiltration of cells extrinsic to the node, as with neutrophils and malig- majority of children with cervical nant cells (Chesney, 1994). lymphadenopathy. Most cases of lymphadenopathy are self-limited and require no treatment. The treat- Alexander K.C. Leung is Clinical Associate Professor of Pediatrics, University of Calgary; Pediatric Consultant, ment of acute bacterial cervical Alberta Children’s Hospital; and Medical Director, Asian Medical Centre, an affiliate of the University of Cal- lymphadenitis without a known pri- gary Medical Clinic, Calgary, Alberta, Canada. mary source should provide ade- W. Lane M. Robson is Professor of Pediatrics, University of Oklahoma. quate coverage for both Staphylo- Reprint requests: Dr Alexander K.C. Leung, #200, 233-16th Ave NW, Calgary, Alberta, Canada T2M 0H5; coccus aureus and group A beta e-mail: [email protected]. hemolytic streptococci. 0891-5245/$30.00 J Pediatr Health Care. (2004). 18, Copyright © 2004 by the National Association of Pediatric Nurse Practitioners. 3-7. doi:10.1016/j.pedhc.2003.08.008 January/February 2004 3 PH ORIGINAL ARTICLE Leung & Robson C cervical lymphadenopathy, followed by BOX 1 Causes of cervical BOX 2 Differential diagnosis rhabdomyosarcoma and non-Hodgkin’s lymphadenopathy of cervical lymphadenopathy lymphoma (Leung & Robson, 1991). After 6 years, Hodgkin’s lymphoma is A. Infection • Mumps the most common tumor associated with 1. Viral • Thyroglossal cyst cervical lymphadenopathy, followed by a. Viral upper respiratory infection • Branchial cleft cyst non-Hodgkin’s lymphoma and rhab- b. Epstein-Barr virus • Sternomastoid tumor domyosarcoma. c. Cytomegalovirus • Cervical rib The presence of cervical lym- d. Rubella • Cystic hygroma phadenopathy is one of five diagnostic e. Rubeola • Hemangioma criteria for Kawasaki disease; the other f. Varicella-zoster virus • Laryngocele four are bilateral bulbar conjunctival in- g. Herpes simplex • Dermoid cyst jection, changes in the mucosa of the h. Coxsackievirus Data from Leung & Robson (1991). oropharynx, erythema or edema of the I. Human immunodeficiency peripheral extremities, and polymorph- virus ous rash. Generalized lymphadenopa- 2. Bacterial thy might be a feature of systemic onset a. Staphylococcus aureus (HIV). Bacterial cervical lymphadenitis juvenile rheumatoid arthritis, systemic β b. Group A β-hemolytic is usually caused by group A -he- lupus erythematosus, or serum sick- streptococci molytic streptococci or Staphylococcus ness. Certain drugs, notably phenytoin c. Anaerobes aureus. Anaerobic bacteria can cause and isoniazid, might cause generalized d. Diphtheria cervical lymphadenitis, usually in asso- lymphadenopathy (Malley, 2000). Cervi- e. Cat-scratch disease ciation with dental caries and peri- cal lymphadenopathy has been reported f. Tuberculosis odontal disease. Group B streptococci following immunization with diphthe- 3. Protozoal and Haemophilus influenzae type b are ria-pertussis-tetanus, poliomyelitis, or a. Toxoplasmosis less frequent causal organisms. Diphthe- typhoid fever vaccine (Leung & Rob- B. Malignancies ria is a rare cause. Bartonella henselae son, 1991). Rosai-Dorfman disease is 1. Neuroblastoma (cat scratch disease), atypical mycobac- a benign form of histiocytosis charac- 2. Leukemia teria, and mycobacteria are important terized by generalized proliferation of 3. Lymphoma causes of subacute or chronic cervical sinusoidal histiocytes. The disease usu- 4. Rhabdomyosarcoma ally manifests in the first decade of life C. Miscellaneous with massive and painless cervical lym- 1. Kawasaki disease phadenopathy and is often accompa- 2. Collagen vascular diseases nied by fever, neutrophilic leukocytosis, 3. Serum sickness Cervical masses are and polyclonal hypergammaglobuline- 4. Drugs mia (Swartz, 2000). Kikuchi-Fujimoto 5. Postvaccination common in children and disease (necrotizing lymphadenitis) is a 6. Rosai-Dorfman disease benign cause of lymphadenopathy that 7. Kikuchi-Fujimoto disease might be mistaken for primarily affects young Japanese fe- Data from Leung & Robson (1991). males. Fever, nausea, weight loss, night sweats, arthralgia, or hepatosplenomeg- enlarged cervical lymph aly might be present. ETIOLOGY nodes. DIFFERENTIAL DIAGNOSIS Common or important causes of cervi- Cervical masses are common in children cal lymphadenopathy are listed in Box and might be mistaken for enlarged cer- 1. The most common cause is reactive vical lymph nodes. The differential diag- hyperplasia resulting from an infec- lymphadenopathy (Spyridis, Malte- nosis of cervical lymphadenopathy is tious process, most commonly a viral zou, & Hantzakos, 2001). Chronic pos- listed in Box 2. In general, congenital le- upper respiratory tract infection (Peters terior cervical lymphadenitis is the sions are painless and are present at & Edwards, 2000). Upper respiratory most common form of acquired toxo- birth or identified soon thereafter tract infection might be caused by rhi- plasmosis and is the sole presenting (Twist & Link, 2002). Clinical features novirus, parainfluenza virus, influenza symptom in 50% of cases (Leung & that may help distinguish the various virus, respiratory syncytial virus, coro- Robson, 1991). conditions from cervical lymphadeno- navirus, adenovirus, or reovirus. Other More than 25% of malignant tumors pathy are discussed next in this article. viruses associated with cervical lym- in children occur in the head and neck, phadenopathy include Epstein-Barr and the cervical lymph nodes are the Mumps. The swelling of mumps virus (EBV), cytomegalovirus, rubella, most common site (Leung & Robson, parotitis crosses the angle of the jaw. rubeola, varicella-zoster virus, herpes 1991). During the first 6 years of life, On the other hand, cervical lymph simplex virus (HSV), coxsackievirus, neuroblastoma and leukemia are the nodes are usually below the mandible and human immunodeficiency virus most common tumors associated with (Leung & Robson, 1991). 4 Volume 18 Number 1 JOURNAL OF PEDIATRIC HEALTH CARE PH ORIGINAL ARTICLE Leung & Robson C Thyroglossal cyst. A thyroglossal group (Box 3). The prevalence of vari- cyst is a mass that can be distinguished ous childhood neoplasms changes BOX 3 Organisms with by the midline location between the with age. predilection for specific age thyoid bone and suprasternal notch groups and the upward movement of the cyst Laterality and chronicity. Acute bi- when the child swallows or sticks out lateral cervical lymphadenitis is usually A. Neonates his or her tongue. caused by a viral upper respiratory