Cervical Lymphadenopathy

LYMPH NODE SWELLINGS

Aetiology

Lymph nodes increase in size and become easily palpable or even visible (Figure 1) in response to a range of disease processes, especially those:

• with an inflammatory component;
•  affecting leukocytes and other cells of the lymphoreticular system(lymph nodes, liver, spleen);
• involving lymphatics.

It is not uncommon to find swollen lymph nodes in the neck (cervical lymphadenopathy). The most commoncause of cervical lymphadenopathy is infection in the area of drainage, which

includes virtually any site in the head and neck (Table 2). Lymphadenopathy in the anterior triangle of the neck alone is often due to local disease, usually infection, and a local cause is especially likely if the nodes are enlarged on only one side. Most common of all is an enlarged jugulodigastric (tonsillar) lymph node, inflamed secondary to a viral upper respiratory tract infection.

This sentinel node should always be carefully palpated.

Enlarged cervical lymph nodes may also be a manifestation of systemic infection (e.g. HIV/AIDS), or related to malignant disease in the drainage area (e.g. carcinoma) or elsewhere (e.g.

leukaemia or lymphoma). Bilateral lymphadenopathy may indicate generalized disease. Generalized lymphadenopathy with or without enlargement of other lymphoid tissue such as liver and spleen(hepatosplenomegaly), suggests a systemic cause.

Clinical Features

Lymph nodes that are increasing in size and are tender may be inflammatory. In infective disorders the nodes are usually firm, discrete, tender and mobile.Lymph nodes that are hard or rubbery

may be malignant from metastases from oral or other neoplasms in the head and neck. In the lymphomas, particularly, the nodes may be rubbery, matted together and fixed to deeper structures. Such enlarged nodes are also found as part of generalized lymphadenopathy, in neoplasms such as lymphomas and leukaemias, and in these lymphoproliferative states there is also usually hepatosplenomegaly. It may well be important therefore to obtain a specialist opinion as to whether lymph nodes elsewhere in the body are enlarged.

Diagnosis and Management

Every effort should be made to establish the cause of the lymphadenopathy because lymphadenopathy can usually be adequately treated only by eliminating the underlying cause. The diagnosis is

usually made primarily on the findings from the history and clinical examination but various special investigations may be indicated (these are discussed under the relevant diseases).

However, the cause may not always be found despite a careful search. For example, children occasionally develop a Staphylococcus aureus lymphadenitis (usually in a submandibular node) in the

absence of any obvious portal of infection. More serious is the finding of a malignant enlarged cervical lymph node in the apparent absence of a primary neoplasm. A hidden nasopharyngeal or tonsillar carcinoma is a classic cause of this, and specialist opinion should therefore be sought in such cases.

INFECTIVE INFLAMMATORY CONDITIONS

Viral Infections with Predominantly Upper Respiratory and Oral Manifestations
Cervical lymphadenopathy may be seen in any viral upper respiratory infection (such as the common cold or viral onsillitis), and oral viral infections that also produce mouth ulcers (e.g. herpes
simplex stomatitis, herpangina, and occasionally herpes zoster of the trigeminal nerve: These infections affect
children and young adults predominantly.
Main features:

• the mouth or oropharynx is often sore;
• the cervical lymph nodes may be tender;
• usually several nodes in the anterior triangle of the neck especially the jugulodigastric nodes are enlarged, often bilaterally;
• the posterior triangle nodes are not usually enlarged;
• there is no generalized lymphadenopathy unless there are systemic complications or lesions elsewhere;
• there is no hepatosplenomegaly unless there are systemic complications or lesions elsewhere;
•  there is sometimes fever and/or malaise

Non-bacterial Infections with Multiple Systemic Manifestations
These include mainly systemic viral infections: hand, foot and mouth disease, viral exanthemata (chickenpox, measles, rubella), glandular fever syndromes
(mainly Epstein-Barr virus, HIV, cytomegalovirus and HHV-6 infections and the parasitic infestation toxoplasmosis). In these disorders (summarized in Table 3) it is usual to find:

• sore mouth or oropharynx;
• tender enlarged cervical lymph nodes;
• several anterior (and often posterior) triangle lymph nodes enlarged;
• generalized lymph node enlargement; and, in some instances:
• rash;
• fever;
• malaise and anorexia;
• hepatosplenomegaly.

Many of these infections predominantly affect children or young adults.

Toxoplasmosis
This is a rare parasitic infection, contracted mainly from cats.

Aetiology

Toxoplasmosis is caused by a parasite, Toxoplasma gondii, which infests members of the cat family who then excrete it in faeces. T. gondii survives in soil for up to one year, and infection is generally by ingestion of its cysts or oocysts directly from infected soil or present in infected lamb or pork.

Clinical Features

In otherwise healthy patients, symptomatic toxoplasmosis manifests with glandular fever syndrome with a negative Paul-Bunnell test, or chorioretinitis and loss of visual acuity. In immunocompromised patients, such as those with HIV, toxoplasmosis is a more serious problem and may be life threatening. Such patients are at particular risk of clinical infection. Many develop CNS involvement.
Transplacental transmission may result in learning disability and other foetal defects.

Diagnosis and Management

Toxoplasmosis is diagnosed from the history and examination findings, confirmed by:

•  detection of serum antibodies by the Sabin-Feldman dye test, indirect haemagglutination test or IgM fluorescent antibody test;
•  isolation of T gondii;
•  histological demonstration in tissues of T. gondii trophozoites. Toxoplasmosis is usually treated with pyrimethamine plus sulphadiazine.

Acute Non-specific Bacterial Infections
Any bacterial infection in the area of drainage can cause enlargement of anterior cervical lymph nodes. Most of these infections are non-specific and odontogenic, and there may or may not be fever and malaise. Usually:

• there are only one or two cervical lymph nodes enlarged;
• the nodes are tender;
• lymphadenopathy is unilateral; 
• nodes are enlarged only in the anterior triangle;
• there is no generalized lymphadenopathy;
• there is no hepatosplenomegaly;
• a focus of infection can be identified in the head and neck region.

However, lesions on the back of the scalp or neck may cause enlargement of posterior cervical nodes.

Acute Specific Bilateral Infections

Aetiology
Occasionally, specific bacterial infections (particularly Staphylococcus aureus) and atypical mycobacteria involve cervical lymph nodes.

Clinical Features

S. aureus usually infects a single submandibular lymph node in young children to cause acute lymphadenitis in the absence of any detectable entry point
for the organism. Atypical mycobacteria may cause a similar clinical picture and are increasingly seen in immunocompromised persons.

Diagnosis and Management

Diagnosis is often empirical, though it may be possible to aspirate the infected gland and identify the causal bacterium. Specialist referral is warranted. The condition should be treated with
antibioticsóusually flucloxacillin for S. aureus because the organism is often penicillinase-producing. Mycobacterial infections are treated with anti-tubercular therapy. If a lesion is fluctuant and pointing, surgical drainage is needed.

Cat Scratch Disease

This rare bacterial infection is contracted from cats, and usually seen in children.

Aetiology
Cat scratch disease is almost always, but not invariably, acquired from a scratch or close contact with a cat infected with Bartonella (Rochalimaea) quintana, a Gram-negative bacterium.

Clinical Features

Most patients develop a primary, tender non-pruritic papule, usually at the site of the scratch, then unilateral regional lymphadenitis, and sometimes mild fever and malaise. There is indolent,
sometimes suppurative, regional lymphadenitis.

Diagnosis and Management
The history, together with an intradermal skin test (and occasionally biopsy), facilitate the diagnosis specialist referral may thus be indicated. There is also often a mild leukocytosis and raised
erythrocyte sedimentation rate (or plasma viscosity).
As the condition is self-limiting, no treatment is usually actually needed, apart from supportive care. However, erythromycin is effective and is often indicated if the patient is immunocompromised.

Mucocutaneous Lymph Node Syndrome (Kawasaki Disease)

This is an uncommon childhood disease, which may result in cardiac damage and is lethal in up to 10% of patients. The aetiology is unknown but apparently infectious.

Clinical Features

The condition manifests with generalized lymphadenopathy, a rash with desquamation of hands and feet, oedematous lips and pharyngitis. It may be complicated by myocarditis.

Diagnosis and Management
There is no specific diagnostic test or treatment. Specialist care is mandatory.

NON-INFECTIVE INFLAMMATORY CONDITIONS

These include sarcoidosis, Crohn's disease and various connective tissue diseases. Cervical lymph nodes may be enlarged in rheumatoid arthritis or lupus erythematosus.

NEOPLASTIC CONDITIONS

Metastatic Malignancy (Other than Lymphoid) The usual neoplasms that metastasize to cervical lymph nodes are:

•  oral squamous cell carcinoma;
•  oral Kaposiís sarcoma;
•  other oral malignant neoplasms;
•  nasopharyngeal carcinoma; and
•  thyroid tumours.

Rare metastases to lower cervical nodes include tumours from the stomach and testes.
The usual findings are:

•  one or more anterior cervical nodes enlarged;
•  firm or hard node, fixed to deeper tissues in later stages;
•  unilateral lymphadenopathy in oral neoplasms anteriorly in the mouth;
•  nodes on both sides may occasionally be involved;
• no generalized lymphadenopathy;
• no hepatosplenomegaly.

In some patients with a malignant cervical lymph node, the primary tumour is never located. Lymph node and tonsillar biopsy may well be needed to help the diagnosis of malignancy and
sometimes to establish the origin of the tumour.