American Pediatric Surgical Association :: Resources : Neck Abnormalities

Cysts (fluid filled lumps), solid lumps, and sinuses (small openings) found about the head and neck are frequently residual structures from embryologic development that have failed to resorb completely or mature. Some lumps may be caused by inflammation, infection or even tumors affecting lymph nodes in the neck area.

Cystic Hygroma (lymphangioma)
Cystic hygromas are varying sized cystic abnormalities of the lymphatic system occurring in perhaps 1 in 10,000 births. The word is derived from the Greek meaning moist or watery tumor and can occur equally in boys and girls. Approximately 50 or 60 percent are present at birth and about 80-90 percent appear or are detected before the end of the second year of life.

Embryology: Their cause is related to lymph channel development of the lymphatic system and may be due to poor communication with developing veins, which result in isolated sacs or little blind ends. These sacs slowly enlarge (filled with fluid) and may seem to infiltrate into surrounding tissues or push other tissues aside.

Figure 1. Characteristic large neck cystic hygroma present since birth in 3-month-old boy

Cystic hygromas present generally as a multicystic mass with thin walls, and being fragile they may have areas of bleeding or accumulation of blood within them. They usually reside in close association with large veins and known lymphatic ducts in the neck, axillary area, groin area, or may infiltrate any areas of the skin along the trunk, extremities, or inside the chest or abdomen (Figure 1).

They present as soft, discrete, and nontender swellings that are often compressible. Cysts within the mass vary from a very small size of a few millimeters to one of several centimeters. In some cases the hygroma may involve two or more areas by direct extension and seem to occur on the left side of the neck twice as often as the right, possibly related to the development of a large lymphatic structure in the chest which empties into the left internal jugular vein (Figure 2).

Figure 2. Axillary cystic hygroma extending beneath the collar bone from a smaller cystic lesion in the neck.

Most are noted shortly after birth and seem to gradually enlarge as they fill. They may expand as a result of bleeding into them or infection, in which case they may become tender or hurt and the overlying skin will have an inflamed or red appearance. It is common for a bluish color to appear under the skin, even though the liquid is clear, and the color of ginger-ale. In the neck the cysts can extend downward into the chest and on rare occasion may produce breathing difficulty because of pressure on the airway or difficulty with swallowing with resultant failure of the child to grow because of his or her inability to eat without discomfort.

Infection in a cystic hygroma may cause rapid enlargement and pain and respond slowly to antibiotic therapy. When this occurs, there is some scarring in the cystic hygroma, as well as the tissue adjacent to it, which may complicate surgical therapy. Although usually recognizable by physical examination alone, sometimes the use of imaging studies, such as ultrasonography and computed tomography (CAT scan), are helpful in distinguishing cystic hygromas from solid masses.

Treatment
Cystic hygromas rarely go away on their own. Aspiration by puncturing with a needle is usually followed by prompt reaccumulation of fluid or development of infection. Injection of chemical agents to cause shrinkage is effective in certain hygromas where there are large cysts, but is not as effective when there are very small cysts. This is not without potential complications, and as yet there is no universal agent available that is totally safe.

When infection occurs, it is necessary for the child to be treated with antibiotics, yet definitive treatment may rest in surgical removal once the infection is controlled. Most pediatric surgeons defer surgery until approximately six months of age if the rate of enlargement does not exceed general body growth. Complete removal is challenging and reoccurrence may be 5 or 10 percent, even in favorable cases. Although all efforts are made to remove remnants of the cyst, because it is non-cancerous no major nerves or other important structures should be removed when removing the cysts.

Preauricular Pits, Sinuses and Cysts
Frequently there are small pits at the front of the upper part of the ear, which are unrelated to branchial cleft development, but do represent abnormal development of the outer ear. These pits are lined by skin cells and may contain hair and local swelling if a cyst is present. These pits often run in the family. They usually do not cause symptoms and removal is not routinely needed. However, when drainage from or infection of a cyst occurs, drainage of pus from an abscess may be needed and subsequent removal of the pit and cyst carried out.

Dermoid and Inclusion Cysts
Where bones and skin join together during development, pieces of skin may become buried beneath the skin surface leading to the formation of cysts. These are called dermoid cysts because they contain material that is skin-like. The most common location in the head and neck is along the outer part of the eyebrow. They can also occur in the middle of the neck near the collar bones. Other common areas in the skull are where pieces of the skull bones fuse together and also right down the middle or midline of the skull. Cysts that occur in the midline from the tip of the nose along the head to the spine in the back may have connections to the brain and CT scans or MRIs are required before their removal.

Cervial Thymic Cyst
Cysts arising from the thymus gland in the chest may present as soft swellings in the lower part of the neck. They can be mistaken for cystic hygromas, branchial cleft cysts, or even low-lying thyroglossal duct cysts. Thymic cysts generally can be excised through a low neck incision. When there is extension into the chest, the upper part of the breast bone may need to be opened for adequate removal.

Cervical Lymphadenopathy
Anterior cervical lymph nodes drain into the mouth and pharynx, so almost all upper respiratory infections have some effect on these lymph nodes causing swelling and tenderness. Lymph nodes are palpable in 80-90 percent of children 4-8 years old. They are uncommon to be felt in infants unless lingering infection occurs or there is a tumor process.

Suppurative Cervical Lymphadentitis
The function of lymph nodes is, effectively, to act as filters or traps for bacteria or viruses that have invaded into the body and, as such, are one of the first lines of the immune defense for the body. When lymph nodes are involved in fighting the infection or foreign material, they swell, and when bacteria are involved, may become filled with pus or develop an abscess. Nodes that continue to enlarge and are quite firm may be involved in a tumor process.

The most frequent cause of infected neck lymph nodes is infection of the throat and tonsils with either the streptococcal or staphylococcal germ. Typically, rapid enlargement of one or more neck lymph nodes occurs with tenderness, swelling, and redness of the surrounding area. Fever and elevation of the white blood cell count are common. Without treatment the lymph node usually continues to enlarge and becomes involved with the overlying skin. Spontaneous rupture or drainage of the pus often follows.

When lymph nodes are swollen on both sides of the neck, a viral infection is generally the cause and usually goes away within a few weeks. Enlarged lymph nodes that are present for longer than 4-6 weeks and are hard may indicate a tumor process.

Early antibiotics may prevent the formation of abscess. When softening of the lymph node occurs, drainage by an incision is generally necessary, although removing the pus by a needle combined with antibiotic therapy may be sufficient. When drainage is done surgically, the wound is left open and packed until the process totally resolves.

Mycobaterical Lymphadentitis
Persistent unilateral enlargement of nodes on one side of the neck, which look infected but don’t respond to the usual antibiotic therapy, may indicate mycobacterial infection. Mycobacteria are a group of bacteria including those of tuberculosis. Lymphadenitis caused by mycobacterial tuberculosis is considered an extension of lung infection and most commonly is in the lymph nodes just above the collar bone at the base of the neck. When mycobacterial tuberculosis infection is confirmed by a skin test, family history, or evidence of lung disease, antituberculous drugs are likely to make them better within several months.

When atypical mycobacteria infection that is not tuberculosis is identified, complete surgical removal of the involved lymph nodes is generally required, as standard antituberculous treatment appears to be of little value.

Cat Scratch Disease
Cat scratch disease is one of the more common causes of nonbacterial lymph node enlargement in the neck and other areas of the body. A likely cause seems to be a small germ known as Bartonella henselae. A scratch from a kitten usually causes the disease. The kitten or cat is not sick and the scratch is often not recognized.

The disease starts with a little pimple at the site of the scratch followed by enlargement of the lymph nodes in the area within two weeks. However, node enlargement may not occur for many weeks later, long after the scratch has occurred. Lymph node enlargement in the armpit area occurs in more than 60 percent of the patients, although lymph nodes may be involved in the neck, in front of the ear, under the jaw, or above the elbow. Diagnosis is frequently one of recognition based on a good story. At present there is no specific therapy for cat scratch disease. It is generally mild and goes away on its own. Treatment with Clarithromycin may offer some benefit.

Diagnosis may be confirmed by identification of cat scratch genes from some of the pus, however this is generally not needed. Rarely, patients may feel sick or have a headache, vomiting, and low-grade fever. Surgical removal is not indicated if only lymphadenopathy or lymph node swelling is present and resolves over 4 to 6 weeks. When the lymph node becomes filled with pus, surgical drainage is warranted. Enlarged lymph nodes which persist may indicate tumor and then biopsy is indicated.