Congenital lymphedema, which is the rarest form of primary lymphedema, appears early in life. It generally involves more than one limb, but rarely extends above the knee.

Swelling enlarges at a slower rate than body growth. It becomes less pronounced with age, and only supportive therapy is required in two-thirds of cases. The remaining one-third of patients have a poor prognosis and frequently require interventions.

Lymphedema praecox, which is the most common form of primary lymphedema, is seen mostly in females. It occurs in older children and teens, and swelling usually begins at the same time as a child’s growth spurt.

The swelling goes all the way up the leg to the groin and is sometimes linked with skin and nail changes. The swelling may increase over time, resulting in limbs that are very enlarged. 

A doctor will ask questions about the child’s medical history as well as do a physical exam. A complete evaluation of the blood vessels is done to rule out other vascular abnormalities.  A variety of diagnostic testing can be used:

• Doppler ultrasonography looks at the blood flow and pressure and is helpful in finding obstructions of the blood flow.
• Computed tomography (CT) scan reveals areas of the lymphatic system that may be blocked.
• Magnetic resonance imaging (MRI) takes a better look at the tissues and provides the ability for identification of the characteristics of lymphedema.
• Lymphangiogram / lymphangiography is a special X-ray of the lymph nodes and lymph vessels.

• Lymphangitis (inflammation of the superficial lymphatics)

• Cellulitis (skin and soft tissue infection)

• Functional disabilities (such as difficulty in wearing shoes)

• Cosmetic and psychological problems

Management of lymphedema consists primarily of supportive care. Although elevation of a limb is difficult to enforce in a child, it is often quite useful.

Supportive stockings and elastic garments worn throughout the day may be effective.  Treatment may also include complete decongestive therapy (CDT), which consists of manual lymphatic drainage, compression, exercises and skin care.

Skin care is extremely important for the prevention of infection. In the case of an infection, it should be treated right away with antibiotics and bed rest.

When supportive measures fail, and a limb becomes too heavy to maneuver during daily activities and/or when normal clothing or shoes cannot be worn, surgical options should be considered. Patients with recurrent cellulitis and lymphangitis may also benefit from surgical debulking procedures.