Definition

Lymphedema is swelling in various body areas when the lymphatic system is affected. The lymphatic system collects excess fluid, proteins, and waste products from cells and tissues and then returns them to the bloodstream. When the lymphatic system doesn't function properly, the body accumulates fluid, leading to swelling. This swelling typically affects the arms and legs but can also occur in other body areas.

Lymphedema can occur after certain surgeries or due to certain medical or genetic conditions. There are two types: primary and secondary. Primary lymphedema rarely occurs and is usually inherited from parents. It can manifest from infancy to adolescence to adulthood. This condition may appear in babies born with Milroy's disease, an inherited form of lymphedema.

Secondary lymphedema occurs when the lymphatic system is disrupted after surgery, trauma, or radiation therapy. Lymphedema commonly occurs in patients undergoing breast cancer treatment, but not all breast cancer patients experience it. It's important to note that lymphedema is not a form of cancer.

Causes

Some causes of lymphedema in children include:

• Minor trauma: Children with primary lymphedema have a reduced capacity to remove lymph fluid. This reduced capacity may only become apparent after minor trauma, such as a sprained ankle, causing acute inflammation and swelling of the lymphatic system due to fluid accumulation. Minor trauma can also initiate lymphedema by temporarily impairing the function of the lymphatic vessels.
• Medical procedures: Similar to minor trauma, minor medical procedures can cause swelling and inflammation, leading to the onset of lymphedema symptoms.
• Insect bites: Infections from insect bites can cause local swelling and inflammation, triggering lymphedema.
• Limited movement (sedentary periods): Prolonged immobility, such as during long flights, can lead to leg swelling.

Risk factor

Individuals who have had cancer and received radiation therapy involving the lymph nodes or tumor site have a higher risk of developing lymphedema.

Symptoms

The development of lymphedema in children follows similar patterns to adults, including the types of lymphedema (primary or secondary) and the location of the affected area. The stages of lymphedema are typically described using the Foldi scale.

Symptoms of lymphedema in children may include:

• Swelling in some or part of the arm or leg, including the toes or fingers
• Feeling of heaviness or tightness in the affected area
• Limited range of motion in the affected limb
• Recurring infections in the affected area
• Hardened and thickened skin over time

These symptoms can vary in severity from mild to severe. It's important to note that lymphedema caused by cancer therapy is usually temporary.

Diagnosis

Diagnosing lymphedema generally requires a thorough evaluation, which includes a medical interview, physical examination, and potentially some diagnostic tests. During the medical interview, the doctor will ask about the patient's current symptoms, medical history, family medical history, and any previous trauma or surgeries.

During the physical examination, the doctor will conduct a general assessment, which may include measuring blood pressure, pulse rate, respiratory rate, and body temperature. Additionally, the doctor will focus on examining the swollen parts of the body, assessing their size, shape, and texture, and checking for signs of inflammation or infection.

To support the diagnosis, doctors may conduct various diagnostic tests such as:

• Lymphoscintigraphy: This method is highly effective in confirming the presence of lymphedema in both children and adults and determining the location and extent of lymphatic damage. However, its use in children is still unclear, as it is divided between being allowed and not allowed.
• Genetic testing: Genetic testing helps determine the presence of genetic syndromes related to primary lymphedema in children.
• MRI (Magnetic Resonance Imaging): This examination is helpful in determining the presence of lymphedema in infants and young children when lymphoscintigraphy is not recommended.
• X-ray and ultrasound: These examinations help doctors identify the source of swelling in children. Ultrasound examination can help determine if the swelling is caused by fluid and what type of fluid it is, or to look for vascular clots as an alternative cause.

Management

The treatment approach for lymphedema depends on the child's medical condition, underlying cause, and stage of lymphedema. Physical therapy or other treatments that facilitate the movement of lymphatic fluid and reduce swelling and pain are typically recommended.

In addition, the doctor may prescribe antibiotics to address any underlying infections or pain medication to alleviate discomfort. While the treatment of lymphedema in children closely resembles that in adults, three crucial modifications ensure favorable outcomes: parental involvement, doctor supervision in monitoring and managing lymphedema symptoms, and encouraging the child's active participation in normal daily activities.

Parents and doctors should actively support the child in self-management, providing moral and physical support. As determined by the doctor, surgical intervention may be necessary if the swelling causes damage or if other conditions require surgical treatment.

Complications

In some cases, complications such as alterations in the skin and tissues may arise. Commonly observed complications include cellulitis, characterized by a soft, warm, red area that antibiotics can typically manage. Other potential complications may involve psychological issues, changes in the skin, and urinary tract disturbances.

Prevention

Some methods to mitigate the risk of lymphedema involve:

• Gently exercising the legs or arms, particularly after lymph node removal surgery.
• Cleansing the skin with warm water.
• Applying sunscreen before outdoor activities to minimize sun exposure.
• Avoid walking barefoot.
• Elevating the legs or arms above heart level using soft cushioning during recovery to promote rest.
• Maintaining good hygiene to prevent external infections from aggravating affected areas.

When to see a doctor?

Seek medical attention or contact a doctor if your child's hands or arms become swollen or show signs of possible infection.

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