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Neck masses in children, unlike those in adults, seldom represent ominous disease. Most (95%) masses are acute, enlarged, or inflamed lymph nodes and are transient in nature. In one series, 44% of children younger than 5 years had palpable lymph nodes. Other masses are congenital cysts and sinuses, vascular malformations, salivary and thyroid anomalies, benign and malignant neoplasms, traumatic injuries, and nonlymphatic infections. Malignancy accounts for 10%–15% of children hospitalized for a persistent neck mass. Although malignancy is uncommon, prompt diagnosis is crucial. US trends show that the incidence of head and neck malignancies among children younger than 15 years increased at a greater rate than childhood cancer in general.
Cervical lymphadenopathy (enlargement) and lymphadenitis (inflammation) are very common. There are few, current primary studies of etiology, and these represent a minority of cases. Attempts to delineate the causes and causative agents of acute lymphadenitis have been based on analysis of specimens obtained through needle aspiration and surgical biopsy, serologic testing, or current molecular methods. Staphylococcus aureus or Streptococcus pyogenes is isolated from 40% to 80% of culture-positive cases after aspiration of acutely inflamed, unilaterally inflamed nodes. , Community-acquired methicillin-resistant S. aureus (CA-MRSA) has emerged in the last two decades. , Anaerobic organisms outnumber aerobic and facultative organisms in normal oropharyngeal flora by more than 10:1. Anaerobic bacteria are isolated from 2% to 38% of cultures with higher yields if careful attention was paid to specimen collection and culture. , Pretreatment with antibiotics also is a factor in pathogen retrieval, with no pathogen isolated in at least 25% of cases of acutely inflamed nodes. ,
For persistent cervical masses, biopsy may be needed for diagnosis. One large study from 1963 examined the biopsy results of persistent cervical masses in 267 children. Congenital cyst or cystic hygroma was found in 60%, malignant tumors in 16%, benign tumors in 7%, nonspecific lymphoid hyperplasia in 10%, and tuberculous granuloma in 7%. The 46 malignant tumors were as follows: 20 lymphosarcoma or Hodgkin disease, 11 neurogenic tumors (neuroblastoma, neurofibroma, neurofibrosarcoma), 11 thyroid tumors, and 4 parotid tumors.
In a review of peripheral lymph node excisional biopsies performed in 239 children, a specific cause was found in only 41% of cases. Of all excised nodes, 76% were located in the neck. Reactive hyperplasia accounted for 52% of cases, granulomatous diseases for 32%, neoplasia for 13% (cases suggestive of lymphoma before biopsy were excluded), and chronic lymphadenitis for 3%. Of the 31 cases of malignancy, Hodgkin lymphoma and non-Hodgkin lymphoma (NHL) accounted for 24, neuroblastoma for 4, and rhabdomyosarcoma for 3 cases. In this series, generalized lymphadenopathy was associated most often with reactive hyperplasia. Close follow-up of patients with a diagnosis of nonspecific reactive hyperplasia is important because studies indicate that up to 25% of such patients ultimately develop life-threatening lymphoreticular disease. , , ,
The use of polymerase chain reaction (PCR) and other molecular testing of surgical specimens has improved diagnostics in persistent, culture- or serology-negative cases. In a 2009 study of 60 children treated surgically for suspected infectious persistent cervical lymphadenitis, an etiology was identified in 82%. Methods combining conventional stain and culture with PCR to amplify the eubacterial 16S ribosomal RNA followed by more specific pathogen amplification were used. Organisms identified were both tuberculous and nontuberculous Mycobacterium (NTM) spp. (62%), followed by Bartonella spp. (10%) and Legionella spp. (10%). Interestingly, this is the first case series describing Legionella as a cause of persistent cervical lymphadenitis; diagnosis was made by PCR testing. As additional modes of molecular diagnostics become more available, spectrum of causative agents of adenitis likely will increase.
Surgical biopsy results from children in developing countries show a slightly different spectrum of disease. A review of 1332 patients younger than 15 years over a 23-year span in South Africa showed 48% reactive nodes, 25% Mycobacterium tuberculosis, 11.6% neoplasm, and 11.5% chronic granuloma. Miscellaneous etiologies included syphilis, yaws, toxoplasmosis, sinus histiocytosis, and Kaposi sarcoma.
Age is useful in predicting etiology of infection ( Table 17.1 ). Group B Streptococcus and S. aureus are common causes of lymphadenitis in neonates and young infants and S. pyogenes, S. aureus, and NTM spp. are more common in children 1–4 years old. Acute cervical lymphadenitis may be more common in the young child because of an inability to localize the organism to the initial nasal or pharyngeal site of attachment. In children aged 5–15 years and adults, anaerobic infection, toxoplasmosis, cat-scratch disease, and tuberculosis are important considerations and dental origination becomes more prominent.
Patient Age | ||||
---|---|---|---|---|
Organism | Neonate | 2 Months–1 Year | 1–4 Years | 5–18 Years |
Group A Streptococcus | – | – | + | ++ |
Group B Streptococcus | ++ | + | – | – |
Staphylococcus aureus | + | ++ | ++ | ++ |
Nontuberculous Mycobacterium | – | – | ++ | + |
Bartonella henselae | – | + | ++ | ++ |
Toxoplasma gondii | – | – | + | + |
Anaerobic bacteria | – | – | + | ++ |
A complex and efficient lymphatic system has evolved to defend against microbial invasion of the head, neck, nasopharynx, and oropharynx, as shown in Table 17.2 and Fig. 17.1 . There are three interrelated lines of defense. The ring of Waldeyer is composed of a circle of adenoidal, tonsillar, and lingual lymphoid tissue (see Chapter 16 ). A collar of superficial satellite lymph nodes runs along the lower margins of the jaw and encircles this ring. This outlying collar of nodes of the head consists of the occipital, postauricular, preauricular, parotid, and facial groups. Nodes of the neck are the salivary gland−associated submaxillary and submental nodes and the vertically oriented superficial and deep cervical chains.
Lymph Node Group | Areas Drained |
---|---|
Head | |
Postauricular (mastoid) | Temporal and parietal scalp; posterior wall of ear canal; upper half of pinna |
Occipital | Posterior scalp; skin of upper, posterior side of neck |
Preauricular | Anterior and temporal regions of scalp; anterior ear canal and pinna; lateral conjunctivae |
Parotid | Root of nose; eyelids; temporal scalp; exterior auditory meatus; parotid glands; middle ear; floor nasal activity; posterior palate |
Facial | Eyelids, conjunctivae; skin and mucous membranes of nose and cheek; nasopharynx |
Neck | |
Submental | Central lower lip; floor of mouth; skin of chin; tongue tip |
Submandibular (submaxillary) | Buccal mucosa; side of nose; medial palpebral commissure; upper lip; lateral part of lower lip; gums, anterior part of tongue margin, teeth |
Superficial cervical | Anterior: superficial anterior neck tissues including skin, lower larynx, thyroid, cranial trachea |
Posterior: lower ear canal; parotid region | |
Superior deep cervical | Tonsil, adenoid; posterior scalp and neck; tongue, larynx, palate; thyroid; nose, nasopharynx; esophagus; paranasal sinuses; all nodes of head and neck except inferior deep cervical |
Inferior deep cervical (scalene, supraclavicular) | Dorsal scalp and neck; superficial pectoral region of arm; superior deep cervical nodes; larynx, trachea; thyroid |
Right: left lower lobe, lingula, right lung and pleura | |
Left: left upper lobe; entire abdomen |
Occipital nodes often are enlarged when generalized lymphadenopathy is present; regional enlargement is almost always infectious and is due to tinea capitis, pediculosis capitis, seborrheic dermatitis, or scalp cellulitis or abscess. Enlargement of preauricular nodes reflects local skin or conjunctival infection. Asymptomatically enlarged parotid glands raise the possibility of malignancy.
The superficial cervical nodes in the neck are a disparate group composed roughly of three vertical chains. The deep lateral or spinal accessory chain runs behind the posterior border of the sternocleidomastoid (SCM) muscle and along the spinal accessory nerve. The superficial cervical chain follows the external jugular vein, which runs obliquely across the surface of the SCM muscle to empty into the subclavian vein in the supraclavicular triangle. The superficial anterior chain runs in the midline from the chin to the suprasternal notch and comprises the infrahyoid, prelaryngeal, pretracheal, and anterior cervical nodes. The term posterior cervical nodes is nonspecific, referring to nodes of the spinal accessory chain and those of the superficial cervical chain that lie over and behind the SCM muscle; lymph nodes anterior to the SCM muscle are in the anterior triangle and those behind in the posterior triangle.
The deep cervical chain of lymph nodes runs from the base of the skull to the root of the neck next to the internal jugular vein on the carotid sheath, and under the SCM muscle. This chain contains numerous large nodes and is divided into superior and inferior deep cervical groups. The superior group is above and the inferior group is below the point low in the neck where the omohyoid muscle crosses the internal jugular vein. The lymphatic channels and nodes in the head and neck connect and empty into the thoracic duct on the left or the lymphatic duct on the right, each of which in turn immediately empties into the respective subclavian vein.
The tonsillar or jugulodigastric node, which drains the palatine tonsil, belongs to the superior deep cervical group and lies at the angle of the mandible just below the posterior belly of the digastric muscle. Other deep cervical nodes lying under the SCM muscle along the internal jugular vein drain the adenoid, larynx, trachea, thyroid, palate, esophagus, paranasal sinuses, nasopharynx, occipital scalp, back of the neck, pinna, and much of the tongue. The large jugulo-omohyoid node that drains the tongue lies just above the omohyoid muscle where it crosses the internal jugular vein, which separates the superior and inferior deep cervical nodes.
Because most lymphatics of the head and neck drain to the submandibular and deep cervical nodes, these nodes are involved in more than 80% of cases of cervical lymphadenitis in young children.
The inferior deep cervical nodes lie low in the neck, below the omohyoid muscle and under and posterior to the anterior clavicular insertion of the SCM muscle. They lie in close approximation to the brachial plexus and to the entrance sites of the thoracic duct and right lymphatic duct into the left and right subclavian veins. All lymph from the head and neck, arms, superficial thorax, lungs, mediastinum, and abdomen passes through these nodes. The left supraclavicular nodes (Virchow-Troisier nodes) drain the left upper lobe of the lung, left mediastinum, stomach, small intestine, kidney, and pancreas. Enlargement of these nodes in the absence of cervical adenopathy suggests intra-abdominal tumor or inflammation (Troisier sign) or intrathoracic disease. The right supraclavicular nodes drain the left lingula and lower lobes as well as the entire right lung, pleura, and right mediastinum. Enlargement of these nodes most often indicates thoracic lesions, especially Hodgkin lymphoma and NHL. Prompt biopsy is indicated in the absence of easily documented pulmonary or cervical infection.
The fascial system of the neck is complex, but a general anatomic description is useful to understand the deep neck infections. The neck fascia is divided into two layers: the superficial layer and the deep layer. The deep fascia is further divided into the superficial, middle, and deep layers of the deep fascia ( Fig. 17.2 ). The deep neck spaces interconnect and include the lateral pharyngeal (parapharyngeal), retropharyngeal, prevertebral, “danger,” masticator, submandibular, carotid, pretracheal, peritonsillar, parotid, and temporal spaces.
The most clinically relevant deep neck spaces include the submandibular , peritonsillar , retropharyngeal , and lateral pharyngeal spaces . Infection in the submandibular space is known as Ludwig angina . Ludwig angina is a bilateral, brawny cellulitis that originates in the floor of the mouth. The peritonsillar space surrounds the palatine tonsil. Peritonsillar abscess is the most common deep neck infection, accounting for 50% of cases, and is more common in adolescents and young adults. Abscess usually is unilateral but can be bilateral. Cervical and submandibular lymphadenopathy also can occur through drainage of the superior deep cervical nodes. , Infection of the retropharyngeal space is most common in children aged 2–4 years because the several small lymph nodes and space atrophy by adolescence. Infection in this space in older children and adults usually is secondary to direct trauma rather than to lymphatic spread. Cervical adenitis is not a common manifestation because these nodes drain into the prevertebral space; however, mediastinitis and empyema can occur.
The lateral pharyngeal space is a central connection for all other neck spaces, and thus infections can follow infection at various sites; presentation with unilateral cervical adenitis is common. The lateral pharyngeal space is divided into anterior and posterior compartments. Suppurative jugular thrombophlebitis (Lemierre syndrome) is a well-described complication predominantly of Fusobacterium infection of the posterior compartment, most commonly spread from primary tonsillitis. Along with sepsis because of proximity to the carotid sheath, swelling around the SCM muscle or “bull neck,” neck pain and stiffness, and torticollis are common. , More detailed descriptions regarding neck space infections are found in Chapters 25 and 28 Chapter 25Chapter 28.
The oculoglandular syndrome of Parinaud consists of unilateral, chronic granulomas or ulcers of the conjunctivae associated with preauricular and submaxillary lymphadenitis. Causes are listed in Box 17.1 . Bartonella henselae infection is most common. Primary conjunctival inoculation causes grey or yellow granulomatous nodules or areas of focal necrosis, often surrounded by significant conjunctival chemosis and palpebral inflammation. Recovery without sequelae (except for occasional mild conjunctival scarring) occurs within 2–3 months. Antimicrobial therapy usually is given but has not been proved to hasten resolution.
Herpes simplex
Treponema pallidum
Mycobacterium tuberculosis
Nontuberculous Mycobacterium spp.
Bartonella spp.
Francisella tularensis
Corynebacterium diphtheria
Spirillum minor
Chlamydia trachomatis
Bacillus anthracis
Nocardia brasiliensis
Yersinia spp.
Listeria monocytogenes
Histoplasma capsulatum
Coccidioides immitis
Paracoccidioides spp.
Sporothrix schenckii
Trypanosoma spp.
Rickettsialpox
Rickettsia spp.
For purposes of predicting etiology, most cases of infectious cervical lymphadenitis can be divided into the following three categories (with some overlap): (1) acute bilateral lymphadenitis; (2) acute unilateral lymphadenitis; and (3) subacute (chronic) lymphadenitis.
Acute bilateral lymphadenopathy (less commonly lymphadenitis) most often is a localized response to acute pharyngitis or part of a generalized lymphoreticular response to systemic inflammation in older children. Usually, lymph nodes are small and soft, may or may not be tender, and are not associated with erythema or warmth of the overlying skin. Viral upper respiratory tract infection is the most common cause, followed by pharyngitis due to S. pyogenes and Mycoplasma pneumoniae.
Although generalized lymphadenopathy is common in Epstein-Barr virus (EBV) infection, cervical adenopathy is most prominent and is present in 93% of infected children. Enlargement is bilateral and most prominent in the posterior cervical chain, followed by the anterior cervical chain. Single or groups of lymph nodes vary from 5 to 25 mm in diameter and are firm, discrete, and minimally tender. Moderate splenomegaly occurs in 75% of patients. Significant enlargement of the lymphoid tissue in Waldeyer ring can result in nasopharyngeal and oropharyngeal airway obstruction.
Cytomegalovirus infection (CMV) presents similar EBV in children with normal immune function, although it is more frequent in young children. Cervical adenopathy is less common in CMV infection (75%) than in EBV (95%) infection. Tonsillopharyngitis and sore throat are more common in EBV infection, whereas hepatosplenomegaly, upper airway obstruction, and rashes are more common in CMV infection.
Cervical, submaxillary, and submental nodes frequently are enlarged and tender during the course of primary gingivostomatitis due to herpes simplex virus (HSV). Primary infections of the conjunctivae and lids are accompanied by preauricular adenopathy. Localized or regional necrotizing lymphadenitis from primary HSV infection also has been reported as a rare occurrence.
Pharyngoconjunctival fever due to adenovirus is characterized by abrupt onset of fever, pharyngitis, and granular conjunctivitis (unilateral or bilateral). Hyperplasia of the tonsillar, adenoidal, and pharyngeal lymphoid tissue is present and can be accompanied by cervical node or more generalized lymphadenopathy. Preauricular adenopathy is surprisingly infrequent and hepatosplenomegaly is common.
Preauricular lymphadenitis occurs in 90% of patients with epidemic keratoconjunctivitis. One half of patients with the usually unilateral, acute, follicular conjunctivitis also have pharyngitis and rhinitis.
Acute respiratory disease due to adenoviruses generally has significant constitutional as well as localized respiratory symptoms. Bilateral cervical adenopathy is expected.
Nonspecific febrile illness due to coxsackieviruses and echoviruses generally has an abrupt onset. Manifestations include fever, malaise, sore throat, nausea, vomiting, and abdominal pain. Minimal conjunctival and pharyngeal erythema with bilateral cervical adenopathy is present.
In one reported series of human herpesvirus 6 (HHV-6) infections in 688 children, mild bilateral cervical adenopathy was present in 31% of cases. Typically, the occipital, posterior auricular, and posterior cervical nodes are involved, but enlargement is modest. HHV-7 also can cause roseola; however, the complete clinical picture of disease has not been defined.
HHV-8 generally is the etiologic agent of Kaposi sarcoma in severely immunosuppressed patients with human immunodeficiency virus infection. HHV-8 also has been associated with primary effusion lymphoma and multicentric Castleman disease. Primary infection in immunocompetent children can cause fever, sore throat, morbilliform rash, and cervical lymphadenopathy. ,
In patients with experimentally induced rubella, lymph node enlargement begins as early as 7 days before the onset of the rash. Although generalized lymphadenopathy occurs, the nodes most commonly involved are the posterior auricular, suboccipital, and cervical nodes. Although lymph node tenderness and swelling are severe on day 1–2 of the rash, this quickly subsides. Enlargement can persist for weeks.
A mononucleosis-like syndrome due to parvovirus B19 infection can be associated with bilateral cervical and intraparotid lymphadenopathy in which facial palsy and parotitis can also be seen. Generalized lymphadenopathy and hepatosplenomegaly also can be present.
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