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Bilateral cervical lymphadenopathy is generally associated with viral infections, whereas node infection of bacterial origin tends to be unilateral.6,16 Congenital neck masses are usually noted shortly after birth and are painless. Some congenital lesions may not be apparent until years later when there is an obstruction and the subsequent infection causing it to grow in size. Hemangiomas are red or purplish in color, flat or raised, and blanch with pressure. They grow rapidly in the first few months of life, slowly regress over months to years, and may even disappear with time.
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In the absence of trauma, a child presenting with dysphagia, stiff neck, trismus, stridor, or muffled speech should be suspected of having a deep neck infection. Deep neck space infections involving the retropharyngeal and parapharyngeal spaces are potentially life-threatening conditions that continue to occur despite the widespread use of antibiotics. These infections originate from a tonsillitis, pharyngitis, or sinusitis that has spread through the regional lymph nodes of the neck17,18 and are generally polymicrobial often with both aerobic and anaerobic bacteria. Over two-thirds will have beta-lactamase producing organisms.19 Deep neck infections with Fusobacterium necrophorum have been associated with Lemierre's syndrome (spiking fevers, tenderness of the sternocleidomastoids with internal jugular vein thrombosis, and metastatic pulmonary abscesses). Complications of deep neck infections include spontaneous rupture into the pharynx, extension to the lateral side of the neck, or dissection into the mediastinum and prevertebral space leading to aspiration, airway obstruction, compression of major blood vessels, and death.19 Therefore, a suspicion of a possible deep neck infection calls for early consultation with surgical subspecialists.
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Occasionally, the emergency physician may encounter a patient with a neck mass that is minimally tender, nonfluctuant, slowly enlarging over a few days or weeks with no obvious source or systemic symptoms. The differential diagnosis is wide and consideration should be given to atypical mycobacterium as well as Mycobacterium tuberculosis infection, infectious mononucleosis, cat scratch disease, HIV infection, sarcoidosis, actinomycosis, and toxoplasmosis. If a patient has not been treated, a course of an appropriate oral antibiotic is indicated with close follow-up to assess response to treatment. Ancillary testing may be initiated depending on the history and physical examination. This may include a complete blood count and a chest radiograph to detect pulmonary infiltrates or mediastinal adenopathy. Persistence of the mass despite antibiotic therapy, suspicion of malignancy, or other esoteric etiology necessitates a referral to an infectious disease specialist or to an otolaryngologist for a biopsy.
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Nontuberculous mycobacterial infection may present as a chronic cervical adenitis. This is usually seen in children between 1 and 5 years of age. The bacteria gain entry from a breakdown in the mucous membranes of the oropharynx and tonsils and then invade the regional lymph nodes. The usual presentation is an enlarged lymph node in the submandibular region that has a rubbery consistency, minimal tenderness, and a dull reddish color (Fig. 10-4). Occasionally, a draining sinus is present. Although the treatment of choice has been surgical resection, recent studies have shown some respond with medical management alone.5
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Bartonella henselae, the organism responsible for cat scratch disease, is a common cause of regional lymphadenopathy. The enlarged node usually involves the axillary area but occasionally can affect the cervical, epitrochlear, or inguinal nodes as well. Recent contact with a cat or kitten can be obtained in the majority of patients. A papule at the site of the inoculation is frequently noted, followed in 1 to 2 weeks by the development of tender, indurated, erythematous skin overlying the enlarged node in the lymphatic chain that drains the site of infection. Systemic symptoms such as fever and malaise are seen in about a third of the patients. Management consists of symptomatic relief, as the disease is usually self-limited, resolving spontaneously in 2 to 4 months. However, a 5-day course of azithromycin may help shorten course of illness.
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In patients who are acutely or severely ill, particularly in those with hepatic or splenic involvement, painful adenitis, or who are immunocompromised, a longer antibiotic course is indicated. The choice of antibiotic and duration of therapy20 is best chosen in consultation with an infectious disease specialist.
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Other inflammatory conditions to consider are infections of the salivary glands or sialadenitis. It presents as a tender and swollen mass in the area of the submandibular or parotid glands. Occasionally, the condition can be bilateral as in mumps parotitis. The majority can be treated with conservative measures such as hydration, pain relief, application of moist heat, and sialogogues. In those with bacterial superinfection, broad-spectrum antibiotics effective against Staphylococcus, Streptococcus, and anaerobic flora should be started.
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Thyroglossal duct cysts arise from the vestiges of the thyroglossal duct that runs in the middle of the neck from the base of the tongue to the thyroid gland (Fig. 10-5). These cysts enlarge after bouts of upper respiratory infections. Support for this diagnosis in addition to its midline location is that protrusion of the tongue will produce retraction of the lesion. Most of these cysts manifest between the ages of 2 and 10 years, although a third do not become apparent until after the second decade of life.4,10 Infection is common because of the persistent communication with the base of the tongue provides access for the oral flora. Once the infection has been treated, surgical excision to remove the cyst and its entire tract is indicated.
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Cystic hygromas are formed when primordial lymphatic ducts fail to establish drainage into the venous system. They are often multiloculated and very large. They are found in areas where lymphatic ducts drain into large veins such as the neck, axilla, and mediastinum. The left side of the neck is frequently involved because this is where the thoracic duct enters the subclavian vein.6 Most cystic hygromas are identified at birth, although some may not be diagnosed until the second decade of life usually because they become infected. There is a strong association between congenital cystic hygromas of the neck and Turner syndrome.21,22 Cystic hygromas in infants can extend from the tongue to the mediastinum and in rare cases, rapidly enlarge, causing extrinsic compression of the adjacent vital structures and leading to respiratory distress. Such rapid enlargement can occur secondary to trauma, infection, or hemorrhage into the cyst.6
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Anomalous development of the branchial arches and clefts produces brachial cysts, sinuses, fistulae, or skin tags in the neck. Most branchial cleft anomalies originate from the second cleft. These painless cysts or sinuses are usually seen at the anterior border of the middle to lower third of the sternocleidomastoid muscle. They can become secondarily infected. Brachial cysts are more commonly diagnosed after the first decade of life, whereas fistulas are usually diagnosed shortly after birth.6,7
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Minor trauma to the neck and a variety of other conditions can cause spasm of the cervical muscles, primarily the sternocleidomastoid. Underlying etiologies of torticollis include upper respiratory infection, cervical adenitis, retropharyngeal abscess, atlantoaxial rotatory subluxation and rarely, dystonic reactions or intracranial and spinal cord tumors.23,24
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Congenital torticollis is suspected when an infant, usually at 2 to 8 weeks of age, presents with an ipsilateral neck mass with the head tilted toward it and the chin in the opposite direction. The cause of congenital torticollis is still not clear, although it may be related to bleeding into the sternocleidomastoid muscle from a difficult delivery.25 The onset of marked facial hypoplasia or asymmetry is an indication for surgical transection of the middle third of the affected sternocleidomastoid muscle.26
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Supraclavicular lymphadenopathy in any age group is a serious concern and should be promptly investigated for an underlying malignancy.6,27 Other characteristics of a neck mass that should increase suspicion of a malignancy include the presence of irregular margins, hard consistency, size of more than 3 cm, adherence to surrounding areas, and association with other systemic symptoms.7,27,28
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Malignant neoplasms of the head and neck account for approximately 5% of all malignancies in childhood.29 Eighty percent of children with Hodgkin's disease and 40% with non-Hodgkin's lymphoma will present with a neck mass.7 The neck is second to the orbit as the site for rhabdomyosarcoma. The tumor presents as a rapidly enlarging, painless neck mass that is hard and immobile. Other tumors include neuroblastoma and lymph node metastasis from malignancies of the skin and thyroid. These masses tend to be hard and fixed to the underlying structures.
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Laboratory testing is often not necessary. A neck mass that is rapidly enlarging and does not respond to the standard antibiotic regimen or has been present for a few weeks requires further laboratory and radiographic evaluation. A complete blood count with differential and either erythrocyte sedimentation rate or C-reactive protein may be obtained.
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Lateral neck films are useful in the rapid assessment of the patient with potential airway obstruction. Chest radiographs can detect pulmonary infiltrates or mediastinal involvement in patients suspected of having tuberculosis, sarcoidosis, or primary lung tumors. Ultrasound has replaced computerized axial tomography (CT) as the initial imaging modality for the assessment of neck masses. It avoids ionizing radiation of sensitive tissues in a child's neck and has good sensitivity and specificity in detecting abscesses.11,12 CT will obtain better images of deep structures and anatomical plains than ultrasound but is limited in differentiating abscess from cellulitis.12,13 Magnetic resonance imaging also has the advantage of not using ionizing radiation and can be useful in evaluating deep neck masses, especially those that may extent into the head or spine.14,15 There is, however, the potential drawback as young children need to be sedated for this study.