Neck masses in infants and children are common and sometimes confusing. Although most are of congenital or infectious origin, a few are neoplastic. The most common malignant tumors are of the lymph nodes. Nonlymph node masses are generally benign, with the exception of rhabdomyosarcoma and an occasional neuroblastoma presenting in the cervical ganglionic chain. Sonograms can often identify the solid nature of such masses, differentiating them from congenital cysts.
A nonsuppurative lymph node enlargement that fails to respond to a short course of antibiotics should be considered lymphoma until proven otherwise by biopsy. If this one rule is adhered to, little of major consequence will be missed.1
Generally, acute suppurative lymphadenitis is straightforward and easy to diagnose. Often there is an accompanying infectious illness, such as an upper respiratory infection or pharyngitis. The node or nodes enlarge rapidly, are tender, and are accompanied by erythema of the overlying skin; however, this will not be true of deeply located nodes. Fever and an elevated white blood count with a left shift are usually present. Fluctuant nodes may be aspirated with a large bore needle to obtain material for culture; aspiration of the central necrotic infected material sometimes hastens resolution and avoids formal incision and drainage.
The subacute inflammatory nodes are more problematic. These may appear suddenly or enlarge slowly over days and weeks. They are minimally tender, if at all, and no systemic signs are present. The most common inflammatory etiologies in the US are atypical mycobacterium and cat scratch disease,2,3 but lymph node hyperplasia can occur in response to any viral or bacterial infection.4
Mycobacterium tubercuhsis is currently an uncommon disease in the US, but if suspected as the source of lymphadenopathy it is usually accompanied by positive findings on chest roentgenogram, the common site of first entry of the organisms. Cervical lymph nodes are secondarily invaded. Atypical mycobacterial organisms, on the other hand, enter through the pharynx and are not associated with pulmonary findings. 5·6 Often, the first strength purified protein derivative of tuberculin (PPD) skin test is negative with atypical mycobacterium, but the intermediate strength test is positive due to weak cross-reactivity. In some institutions atypical antigens are available for dermal testing; cat scratch antigens are not widely available.
Figure 1. Capillary hemangioma is seen just lateral to the eye in the temporal area. Also illustrated is the "measuring tape and photograph" treatment that is the most appropriate therapy for these lesions under most conditions.
Therefore, the pediatrician is frequently faced with relatively asymptomatic lymph node enlargement and an inadequate diagnostic armamentarium. It is reasonable to prescribe a 5 to 10 day course of an antibiotic to which streptococcus and most nosocomial staphylococcal organisms are sensitive, and if the nodes fail to resolve in 2 to 3 weeks, refer the patient for an excisional biopsy. This is best done in the operating room under general anesthesia so that deep dissection to obtain the largest, most involved node is well tolerated. Obviously, excisional biopsies must be done by a surgeon trained and experienced in neck surgery in children; these are not office procedures. The specimens need to be handled by a pathologist familiar with preservation techniques to obtain tumor markers using electron microscopy to identify lymphomas properly. Cultures of the nodes should be sent for routine bacterial, viral, fungal, M tuberculosis, and atypical mycobacterial analysis. Nodes suspected of harboring atypical mycobacterial organisms must be completely excised or a draining fistula may result.
When a child presents with multiple enlarged and apparently nonsuppurative nodes, infectious mononucleosis, and other diseases associated with EpsteinBarr virus should be investigated by appropriate serum assays. If these are not diagnosed, Hodgkin's disease or non-Hodgkin's lymphoma must be strongly suspected.
The most common congenital lesions of the head and neck are hemangiomas, lymphangiomas (cystic hygromas), and cysts originating in the thyroglossal duct or branchial cleft.
Figure 2. A large cavernous hemangioma that is pushing the child's auricle forward and obstructing the ear canal. If this becomes totally occluding, some reduction of the lesion may be necessary. Careful follow-up is essential for such lesions.
Hemangiomas may occur anywhere on the head and neck, but common locations include the scalp, forehead, bridge of the nose, upper eyelid, temporal area just lateral to the eye (Figure 1), and the angle of the jaw or preauricular area of the face. When there is a significant dermal (capillary) component, the diagnosis is obvious; more difficult to diagnose are those that are primarily subcutaneous (cavernous). Any capillary component or telangiectasia helps to confirm that the underlying mass is a hemangioma. A sonogram may confirm that one lacking the telltale dermal component is a complex mass and not a cyst.
Hemangiomas usually show florid growth during the first 6 to 12 months of life, then they stabilize and begin to recede. Capillary components change from fiery red to gray as epithelialization takes place, signaling the beginning of regression. Hemangiomas generally should be treated with "a measuring tape and photograph" (Figure 1) and reassurance to the family that resolution is probable. Serial visits are supportive to the parents and assure them that the occasional hemangioma that begins to interfere with function by closing the eyelid or obstructing the ear canal will be detected (Figure 2). Often, referral to a surgeon who will confirm the propriety of nonoperative treatment gives added reassurance to a concerned family. Those lesions that grow uncontrollably or impair function should be treated first with a course of steroids. An occasional massive lesion will be helped by low dose radiation treatments, but attention must be paid to the risk of subsequent thyroid malignancy from such treatments. If none of these modalities is successful, careful surgical excision may be required.
Figure 3. Typical location of a thyroglossal duct cyst in or just off the midline in the hyoid area of the neck.
The typical lymphangioma is similar to the hemangioma but is more likely to occur in the neck; cystic hygromas are basically lymphangiomas comprised of large fluid-filled sacs in contrast to the smaller grapelike clusters of sacs typical of other lymphangiomas. Cystic hygromas often are present at birth and occupy the submandibular or posterior cervical regions. They may be bilateral and rarely may compromise the airway by extrinsic pressure. Lymphangiomas are thought to occur because of failure of the lymphatic buds to establish insertion into the venus system.
Large disfiguring lymphangiomas and cystic hygromas should be removed, but it is essential that this be done by a surgeon experienced in head and neck lesions in children because of the close association of these lesions with the nerves and other important structures in the neck. Smaller lesions may be observed carefully, however, infection is a frequent complication of these entities.
Thyroglossal Duct Cysts
Thyroglossal duct cysts are said to be always in the midline, but "midline" includes the immediate paratracheal areas as well (Figure 3). These lesions arise from elements of the thyroglossal tract representing the descent of the thyroid gland from the foramen cecum at the base of the tongue.7·8 The tract usually passes through the hyoid bone but may be in front of or behind it. Much has been made of using tongueprotrusion to confirm the diagnosis of thyroglossal duct cysts. Because the tract connects to the foramen cecum at the base of the tongue, tongue protrusion should pull up the tract and cyst. This finding is inconsistent and, therefore, not dependable. Many have recommended a radioisotope thyroid scan for all thyroglossal duct cysts prior to excision to assure that the cyst does not contain the patient's only thyroid tissue. However, this rare condition does not mandate scanning every patient with a thyroglossal duct cyst. Excision of the cyst would still be indicated because infectious complications are likely and the dysgenetic thyroid tissue in the cyst is a premalignant lesion.9·10 For those patients who have thyroid tissue in their cysts, postoperative thyroid function studies will identify those who have no remaining thyroid and replacement therapy can be prescribed.
Successful excision requires removal of the cyst, its tract, and the central component of the hyoid bone and a 1.5 cm to 2.0 cm swath of tissue from the hyoid bone to the base of the tongue. These lesions may present in late infancy or early childhood; they are rarely apparent at birth. They may be asymptomatic or first present as an acutely infected lump. Their location in the midline - anywhere from the submental area to the upper trachea - is so characteristic as to make the diagnosis relatively certain. However, submental lymph nodes and epidermoid (sebaceous) cysts also occur in these locations. When acute infection is the initial presentation, antibiotic therapy using agents that cover "mouth flora" should be instituted. Trimethoprim-sulfamethoxazole is usually a good choice. Failure of resolution with antibiotics requires incision and drainage of the infected cyst followed by definitive excision when the infection has resolved.
Even proper surgical removal of a thyroglossal duct cyst may be followed by recurrence in about 10% of cases. Failure to perform the complete procedure results in recurrence rates of about 50%. If a proper excision has been done, subsequent infections in the neck should be treated initially with antibiotics; often complete resolution follows, obviating the need for a second surgical procedure.
BRANCHIAL CLEFT AND ARCH REMNANTS
Another fairly common etiology for neck lesions are the branchial vestiges. 7,n The early embryo has a series of "pseudogills" consisting of cartilaginous arches for support with internal pouches and adjacent external clefts. Cysts and sinuses can arise from persisting elements of these structures; remnants of the second cleft present along the anterior border of the sternocleidomastoid muscle (Figure 4). Second cleft remnants are the most common; those arising from the first pouch and cleft are much more rare and more likely to present as cysts in adult life than in infancy. These lesions occur in relation to the seventh cranial nerve at the angle of the jaw and may be confused with mumps and submandibular lymph nodes.
Figure 4. Typical location of a branchial cleft sinus tract along the anterior margin of rhe sternocleidomastoid muscle. (Reprinted with permission from Holder TM. Ashcraft KW: Pediatric Surgery. Philadelphia. WB Saunders Company !980. ? !065.)
Ffgure 5. Catheter injection of a second branchial cleft sinus tract showing the contrast material coursing upward to the tonsillar fossa and into the hypopharynx. (Reprinted with permission from Holder TM. Ashcraft KW: Pediatric Surgery. Philadelphia. WB Saunders Company. 1980. ? 1065.)
Cysts and sinuses of second branchial origin usually track upward through the carotid bifurcation to the tonsillar fossa (Figure 5). Contrast injected into the sinus will usually demonstrate its extent on neck radiographs; injection of methylene blue dye during the operative procedure allows close adherence to the tract, avoiding injury to important adjacent cervical structures. Failure to excise the tract completely may lead to recurrence.
The typical branchial arch remnant is a small cartilaginous horn presenting in the subcutaneous tissue along the lower anterior border of the sternocleidomastoid muscle; it is usually visible andpalpable, and bilateral occurrence is common. A sinus or cyst is rarely present and infection is unusual. Treatment is primarily for cosmetic reasons and may be delayed indefinitely.
Preauricular "sinuses," or, more accurately, preauricular "pits," are very common. Sinuses and cysts that become infected are less common. These lesions have been ascribed to first branchial cleft vestiges, but in reality they probably relate to the infoldings and fusions associated with formation of the auricle. No treatment is required for asymptomatic pits; draining sinuses and infected cysts mandate antibiotic treatment, incision and drainage for failure to resolve, and ultimately excision of the sinus and cysts.
A brief discussion of several other uncommon neck lesions will heighten awareness if they are encountered.
Epidermoid (sebaceous) cysts occur as small, usually midline, smooth, discrete masses. They are often low in the neck in the suprasternal area, but when higher may be confused with thyroglossal duct cysts. The presence of sebaceous material in the cyst confirms the diagnosis and obviates the need for further dissection (Figure 6).
Figure 6. An epidermoid cyst excised from the midline of the lower neck shows the typical sebaceous cheesy material characteristic of the sebaceous cyst.
Lesions that occur at the lateral aspect of the eyebrow are usually called dermoid cysts, but these, too, are sebaceous cysts and are more properly called epidermoids. True dermoids should contain elements of more than one primitive germ layer. These "dermoids" of the eyebrow may cause pressure erosion of the anterior table of the skull in this region, and occasionally extend into the adjacent orbit.
True teratomas of the cervical region are usually much larger and more complex masses. They are usually apparent at birth and are almost always benign; however, they may cause airway compression. Complete excision is usually possible and is curative.
Occlusion of sublingual salivary ducts may produce a sublingual cyst known as a ránula, named after the genus Rana (the frog), which has a nictitating membrane of similar appearance. The resulting cystic mass may dissect into the submental area, whence it is termed a plunging ránula.12
An unusual defect in Sibson's fascia, which is the barrier between the cervical and thoracic spaces, produces a suprasternal or manubrial herniation. 1 3 A soft ill-defined bulge occurs with any Valsalva-type pressure; these lesions must be differentiated from venous aneurysms, which also become apparent with increased intrathoracic pressure. The manubrial hernia can be confirmed if superior extension of the cupula of the lung can be demonstrated radiographically during a Valsalva maneuver.
Calcification noted in a cervical mass, often in the supraclavicular region, indicates epithelioma of Malherbe. 14
One final lesion to be aware of in the neck of the infant is the "torticollis tumor."7·15 Usually discovered at the 3 to 6 week well baby visit, it is a firm "fibrous" mass in the sternocleidomastoid muscle, which usually cants the head slightly toward the side of the lesion and prevents full rotation of the head toward that side. Debate persists as to whether this is a birth injury with formation of an organized hematoma in the muscle or a primary benign fibrous tumor. Early institution of stretching exercises usually obviates the need for surgery, but cervical radiographs are essential to rule out a cervical rib before beginning the exercise program.
Lesions of the thyroid are uncommon in childhood and very rare in infancy; nevertheless, masses in the thyroid location do merit the usual scans and thyroid function studies.
The majority of lesions in the neck of children will prove to be benign and of congenital origin arising from such structures as the thyroglossal duct and the branchial clefts in addition to hemangiomas and lymphangiomas. However, it is essential that lymphadenopathy be proven infectious and any asymptomatic lymph node enlargement must be considered Hodgkin's disease until proven otherwise. The occasional neuroblastoma or rhabdomyosarcoma presenting in the neck can usually be identified as solid by sonography, leading to early investigation and biopsy.
Because of the abundance of important structures that course through the neck, surgery should be conducted in an operating suite with sophisticated, modern anesthetic techniques and with a surgeon experienced in dealing with the full array of lesions that occur.
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