1.  Lymphadenopathy has been reported in up to 100% of children.

2.  On initial presentation, children with suspicious lymphadenopathy should undergo screening, laboratory tests and a complete history and physical examination.

3.  Biopsy is indicated for lymph nodes which are large (2-3 cm), in an unusual location or do not regress with conjunctive therapy.

4.  An antibiotic trial, consisting of an anti-staphylococcal drug should be given.  If an inadequate response takes place, the node should be biopsied.  A repeat trial should not be given. 

5.  Both open excisional biopsy and fine needle biopsy have a place in the management of lymphadenopathy.

6.  Congenital cysts in children are common.  When enlarging or infected, they should be excised.  Ideally, this is done when the child is three to four years of age.

Masses in the neck of children are caused by a wide variety of etiologies.  Table 1.  They may historically be characterized by the presence or absence of pain, their rate of growth, and their location.1   Physical examination confirms their location and provides information on how solid they are, how distinct their edges are, and whether or not they are fixed to the skin or underlying tissues.  The status of overlying skin or mucosa is also of importance.

Differentiation of a clinically suspicious versus a non-suspicious neck mass is very important.  Thirty-eight percent of children from zero to six months of age have palpable lymphadenopathy in the head and neck region beginning as early as six weeks of age.3   In general, the younger child usually has occipital and postauricular adenopathy as the older child usually has cervical lymphadenopathy.  Lymph nodes which are greater than 3cm should always be viewed with a great deal of suspicion.  Likewise, posterior and lower cervical nodes are more apt to harbor a malignancy.  Lymph nodes are not normally found in the supraclavicular region.  Nodes in this area have been reported to have up to a 60% malignancy rate.29   Tender neck masses in the jugulodigastric area (beneath the superior portion of the sternocleidomastoid muscle) presenting in a febrile child, are often infectious in etiology.  In those cases, lymphadenitis and infected congenital cysts are at the top of the differential diagnosis.  The latter must be seriously considered if an etiology for the infected nodes are not found.  Non-tender small lymph nodes (less than 1 cm) in this area are common in children and may represent a slowly resolving lymphadenitis or a reactive lymphadenitis from a low grade chronic tonsillitis.

Initial screening tests including a chest x-ray, complete blood count with differential, monospot, taxoplasmosis, and cytomegalovirus, and cat scratch fever titers should be considered.  A physician must interpret these tests carefully because infections from Toxoplasma gondii,  Epstein-Barr virus, and cytomegalovirus occur in a large percentage of the population.  These infections can be indolent, sub-clinical and last for a protracted period of time.  Thus, one must be very careful to form a diagnosis based on a positive laboratory test, because it may be an associated rather than an etiological finding.  The child should also be placed on an anti-staphylococcal antibiotic for at least two weeks and re-evaluated in four weeks.  If the node has not reduced in diameter by 50%, biopsy is indicated.  The choice between needle aspiration and open biopsy will depend upon the clinical situation and the available pathological facilities.  It should be noted that there is a small but definite false negative rate with needle aspiration.  Adult needle aspirations are often done to differentiate squamous cell carcinoma from lymphadenitis, as in children, the differential of lymphoma from lymphadenitis is often more important.  If an open biopsy is performed, the tissue should be sent for culture (fungal, viral, acid fast, and bacterial), touch preparations (to evaluate for lymphomas) should be done and special stains (acid fast and fungal) ordered.  If the tissue is immediately put in formalin and sent to pathology, cultures and touch preparations will not be obtainable.

Lymphadenitis:  Enlargement of lymph nodes in children are most often in response to infection either acute or chronic and maybe a local or systemic phenomenon.  Lymph nodes in the neck drain into fairly predictable areas of the head and face.  These should diligently be searched for a source of inflammation.  Table 2 describes the general pattern of drainage.  Pustules, furuncles, acne, dental infection and oral lesions may all cause localized lymphadenitis.  Children in particular, have a tendency to go on to suppurative lymphadenitis which may grow out staphylococcus, streptococcus, or may be sterile.  These present as a tender, red, enlarging mass which eventually point like any other abscess.  Usually they are treated with needle aspiration and penicillin, but occasionally they require formal incision and drainage.

Chronic, painless lymphadenitis may be caused by many bacterial, fungal, viral, and parasitic infiltrations.  Many of the more common etiologies are listed in Table 3.  Often, no etiologic agent is identified and on biopsy the node is found to have follicular hypertrophy and is described as “reactive”.

Systemic lymphadenitis may present initially in the head and neck.  Mononucleosis classically presents with bilateral posterior triangle lymphadenitis along with malaise, fever, anorexia, and often, adenotonsillitis.  Lymphoma may also present with a similar picture and appropriate laboratory tests should be done for any lymphadenitis or tonsillitis that does not properly respond to antibiotics.

Solitary masses of non-lymph node origin:  While relatively uncommon, solitary firm, enlarging masses do occur in children and are usually congenital (such as dermoids or teratomas) or neoplastic.  Rhabdomyosarcoma is the most common soft tissue sarcoma, but the variety of childhood sarcomas are exhausting and beyond the reach of this discussion.  Fine needle aspiration is playing an increasing role in diagnosing such neck masses.  The increased survival of such patients when treated by wide surgical excision and aggressive radiation and chemotherapy mandates their early recognition.  Dermoids are not uncommon in the submental area.

Cystic masses:  Cystic masses are fairly common in the necks of children.  They usually represent a congenital disorder and may or may not be inflamed at the time of presentation.   A cystic hygroma or lymphangioma presents as a spongy, large neck mass, usually present since birth.  They often have ill-defined borders and be trans-illuminated to an extraordinary extent.  Treatment is surgical excision, but the child is usually allowed to reach the age of three to four years.  Persistent enlargement with resulting airway obstruction or acute inflammation may force a definitive procedure at an earlier age.  These may now respond medically to alpha interferon (1995).

Branchial cleft cysts result from remnants of the embryologic gill clefts that fail to resorb.   They usually present in the mid-lateral neck just under the border of the sternocleidomastoid muscle.  They are often part of a track which may start as a dimple of skin at the anterior neck, run across or between the internal and external carotid arteries, over cranial nerves X through X12 (and sometimes IX) and end at either the tonsillar pillar or pyriform sinus.  Simple incision and drainage or incomplete excision will result in a recurrence and a difficult revision surgery.

Thyroglossal duct cysts are analogous to those of branchial cleft origin, but present in the midline anterior neck, usually below the hyoid bone.  They elevate with swallowing and are a discrete mass.   On initial presentation, they are seldom fixed to the lobe of the thyroid, extend upwards through or around the mid-portion of the hyoid bone and end at the base of the tongue.  Such a tract follows the embryologic descent of the primordial thyroid.  A cyst may occasionally contain the patient’s total thyroid tissue.

Rannulas are bluish cysts that appear in the anterior floor of the mouth but some extend into the neck usually just under the body of the mandible.  By manual palpation of the neck and the floor of the mouth, cyst will show continuity.  Those that present in the neck require external excision with removal of the offending sublingual salivary gland.  The etiology of these is cystic dilation of the sublingual gland duct. 

Hemangiomas:  Hemangiomas are reddish or purplish skin lesions that present at birth and tend to enlarge over the first six months of life.  In doing so, they usually produce a cosmetic deformity and may encroach on the mouth, eyelid, nose or ear causing a functional problem.  The parents of a child with a hemangioma should be reassured that they often regress before the second year of life and early aggressive treatment should be avoided in most cases.  Treatment when indicated, may take the form of excision, sclerosing agents, freezing or steroid injections.  Those with lumens (cavernous hemangiomas) are at risk for secondary infection and thrombocytopenia.  Any treatment will cause scarring, and care must be taken not to do more damage than the hemangioma would have done if left alone.  Therapy may now respond to L interferon (1995).

Preauricular Masses:  Preauricular masses can be caused from disease involving the scalp, nasopharynx, parotid and orbit.  Conjunctivitis caused by adenovirus or chlamydia is a common etiology.4    If they involve the parotid gland excision, it will always place the facial nerve at risk.  A small dimple with or without mucoid drainage that is located just in front of the ear is most likely a congenital pit formed by abnormal auricular development.  These are easily infected and if draining, require total excision.  However, this should not be taken lightly.  A first branchial cleft cyst may present in this manner with deep extension and possible facial nerve involvement.  Also, an occasional parotid tumor may present in this fashion.

Firm preauricular masses in the parotid gland have a completely different significance in a child than in an adult.  Fifty percent of the tumors in the parotid gland of children are vascular (hemangiomas or lymphangiomas).  Of the remaining solid tumors, fifty percent are malignant.  The vascular masses are often capillary hemangiomas that are not prone to spontaneous regression and often have no surface manifestations.

Postauricular Masses:  Postauricular masses may be a manifestation of either scalp, ear or systemic disease.  External otitis media or mastoiditis may be associated with postauricular lymphadenopathy.  Scalp disorders, especially those associated with scratching, are one of the most common causes of this disorder.  Fleas, ticks and lice must be carefully searched for and if found, the entire family should be treated.  Lymphomas and other systemic disease are rarely present in this area, but their existence should be ruled out if the nodes persist or increase in size.
 
 

References:

1.  Knight, P.J., Mulne, A.F., Vassy, L.E.  When is Lymph Node Biopsy Indicated in 
Children with Enlarged Peripheral Nodes?  Pediatrics.  1982; 69:391.

2.  Herzog, L.W.  Prevalence of Lymphadenopathy of the Head and Neck in Infants 
and Children.  Clinical Pediatrics.  22:485-487, 1983.

3.  Dietrich.  Die Palpation der Lymph Drusen.  Sitzvngsber Physikae Med Soc. Erlangen 1886, 18:52.

4.  Bedros, A.A., and Man, J.P.  Lymphadenopathy in Children.  Advances in Pediatrics.  28:341-376, 1981.