What Are Lymphatic Malformations?

A group of aberrant lymph veins that come together to produce a spongy, expanding cyst cluster is known as a lymphatic malformation.

Lymphatic malformations are benign (not malignant) masses that resemble odd growths.

How Do Lymphatic Malformations Develop?

The lymphatic system is a series of tubes that collects lymph (LIMF) from bodily tissue and maintains a healthy balance of body fluids. All around the body, blood vessels leak this transparent, light yellow fluid. The lymph is returned to the circulation through the lymphatic system. Because the lymph in lymphatic malformations’ abnormal vessels, which are typically distinct from the normal vessel system and have poor lymphatic drainage, grows. They flare as they swell as a result of this.

Lymphatic malformations expand as a result of the brittle, poorly-formed veins in their walls that bleed readily. It is possible for the lymphatic abnormality to bleed. The lymphatic malformation enlarges due to the stretched arteries and cysts caused by the pooled lymph or blood. It could appear bruised if it’s right next to the skin’s surface.

The trachea (windpipe), eyes, and blood vessels may come under strain when a lymphatic malformation develops. Skin, fat, connective tissue, joints, organs, or bones may develop lymphatic abnormalities. They are most prevalent in the head and neck region but can develop elsewhere in the body excluding the brain.

Lymphatic abnormalities mostly fall into two categories:

  • microcystic lymphatic abnormalities are spongy, include microscopic cysts, and have small veins.
  • known also as cystic hygromas or lymphangiomas, macrocystic lymphatic malformations contain huge, stretched arteries and cysts that are either filled with lymph, blood from internal bleeding, or both.

What Symptoms and Signs Indicate Lymphatic Malformations?

Typically, a lymphatic malformation presents as a swelling lump with a spongy sensation. Although a child may have multiple lymphatic malformations, they are typically located in the same region of the body.

Vesicles, pronounced “ves-ih-kuls,” are microscopic bubbles that frequently appear on the skin over lymphatic abnormalities. These resemble microscopic blisters. As in other types of lymphatic malformations, the fluid in them initially starts out clear and colorless but will turn dark red if blood leaks into it. These skin growths have the potential to bleed through the epidermis. Less frequently, the vesicles contain chyle (KYE-ul), a milky fluid.

A lymphatic abnormality in a kid might result in:

  • a tendency to harm the lymphatic malformation, producing bleeding, which can happen with very slight trauma or even with no known trauma;
  • overgrowth and swelling in the afflicted regions (lips, mouth, jaws, cheeks, arms, legs, fingers, or toes);
  • discomfort and swelling of the affected areas.

The damaged body part and some symptoms are connected.

Tongue, windpipe, and mouth lymphatic malformations may cause problems with:

  • speaking
  • breathing
  • swallowing
  • feeding

Orbital lymphatic abnormalities may result in:

  • double vision
  • bulging of the eye

Chest lymphatic abnormalities can result in:

  • wheezing
  • chest pain or pressure
  • trouble breathing
  • narrowing of the airways to the lungs

Lymphatic abnormalities in the pelvic or gastrointestinal system (gut) can result in:

  • bleeding from the rectum
  • constipation (trouble pooping and pain)
  • bladder obstruction (trouble peeing and pain)
  • infections
  • protein loss due to poor absorption and loss of lymph into the gut

The following symptoms of genitourinary tract lymphatic abnormalities (kidney and bladder):

  • blood in the pee
  • pain when peeing

Bones with lymphatic abnormalities may develop:

  • bone overgrowth
  • bone loss
  • rarely, a fracture

Why Do Lymphatic Malformations Occur?

It is unknown why lymphatic abnormalities develop so early in pregnancy. It is believed that most lymphatic abnormalities emerge at random throughout the baby’s development. Nothing a woman did or didn’t do while she was pregnant contributed to them.

There have been genetic mutations found in some of the more severe lymphatic abnormalities. However, they are also believed to occur at random and are not inherited.

Lymphatic abnormalities are more prevalent in kids with:

  • Down syndrome
  • Turner syndrome
  • an overgrowth syndrome (genetic disorders that cause an unusual increase in the size of the body or a body part)
  • Noonan syndrome

Lymphatic malformations: How Are They Diagnosed?

An ultrasound scan performed during pregnancy (before delivery) may reveal lymphatic abnormalities. It’s possible that the baby’s birth will reveal a lymphatic abnormality that was missed before.

By the time a child is two years old, the majority of serious lymphatic abnormalities have stretched their arteries through lymph fluid buildup or bleeding into them. However, some don’t surface until adolescence or later.

Doctors will perform an examination and arrange imaging tests like the following to be sure the lump is a lymphatic malformation and not another type of tumor:

  • ultrasound scan
  • X-ray
  • computed tomography (CT or CAT) scan
  • MRI scan

What is the treatment for lymphatic malformations?

To treat a child’s lymphatic malformation, pediatric doctors frequently collaborate as a team.

The participating physicians might be:

  • pediatricians
  • vascular and interventional radiologists
  • hematologists/oncologists
  • pathologists
  • genetic specialists
  • orthopedic surgeons
  • pediatric medical specialists (pulmonology, cardiology, etc.)
  • pediatric surgeons
  • neurosurgeons
  • ophthalmologists (eye surgeons)
  • otolaryngologists (ear, nose, and throat surgeons)
  • speech pathologists

In addition to taking into account the child’s age, health, and other medical issues, the team will investigate the nature and location of the lymphatic malformation. They will select a course of therapy that produces the best outcomes while posing the fewest risks and adverse effects.

Observations on available treatments:

  • Most lymphatic malformations that appear suddenly will decrease in size and pain without treatment, but very rarely go away on their own.
  • Incision (cutting into) and draining the lymph from a lymphatic malformation can temporarily reduce its size. This is usually only done for diagnosis or to treat an infection.
  • Sclerotherapy (injection of a medicine into the lymphatic malformation) makes the lymph vessels collapse and scar together, shrinking the lymphatic malformation.
  • Surgical removal is sometimes the best choice when the lymphatic malformation affects organs or interferes with speech, swallowing, or appearance. Minimally invasive surgery may be used, so the surgical scars are very small.
  • Laser therapy may be used for lymphatic malformations in the skin or mouth.
  • Radiofrequency ablation is sometimes used to treat lymphatic malformations in the tongue. A needle inserted into the lymphatic malformation sends out radio waves that ablate (destroy) the malformation.
  • Medicines are often used for large lymphatic malformations. These are low-risk, generally well-tolerated, and shrink most lymphatic malformations over time.

If it becomes infected, which is uncommon, antibiotic therapy and drainage of the lymphatic malformation may be required.

What Else Do I Need to Know?

Because lymphatic malformations can recur, children may require multiple treatments.

Due to the appearance of a lymphatic malformation, children may feel bashful or humiliated. Make sure your youngster has emotional support from you. Finding a local support group where other families may discuss what works for them might be helpful. Consult the care staff about recommendations. Also available online are:

If children or teenagers are experiencing sadness or anger over a lymphatic malformation or its treatment, talking to a therapist can help.

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