Lymphatic Malformations

Lymphatic malformations – also called lymphangiomas – are benign (noncancerous) vascular lesions made up of fluid-filled spaces (cysts). 

They are thought to occur because of abnormal development of a baby’s lymphatic system while in the womb. These malformations are classified by cyst size:

• Macrocystic: Large cysts.
• Microcystic: Small cysts.
• Combined: A mix of both.

They are also described as:

• Lymphangioma circumscriptum: Superficial, skin-level lesions.
• Cavernous lymphangioma or cystic hygroma: Deeper lesions.

Lymphatic malformations are always present at birth, though sometimes they are not found until later in life. They are most common in the neck and armpit (axilla) but can also appear in the mouth, arms, chest, lungs, legs, abdomen, or intestines. Some malformations look like small blue or red spots, while others are large and may affect an entire limb. Depending on their size and location, they can cause disfigurement or interfere with nearby organs or tissues.

The exact cause of lymphatic malformations is unknown, and treatment depends on symptoms and location.
 

Symptoms

Lymphatic malformation symptoms depend on the type, size, and location of the mass.

• Cystic hygroma (cystic lymphangioma): Large, fluid-filled spaces causing red or bluish swelling in the neck, armpit, or groin. Considered a type of macrocystic lesion.
• Cavernous lymphangioma: Another macrocystic lesion, appearing as a bluish or red rubbery swelling under the skin anywhere on the body, including the tongue.
• Lymphangioma circumscriptum: A microcystic lesion that looks like clusters of small, firm blisters on the shoulders, neck, underarm, limbs, or in the mouth (especially the tongue). These range in color from clear to pink, red, brown, or black.

Lymphatic malformations often grow larger over time. Symptoms occur if the mass presses on nearby organs, such as:

• Neck or mouth masses: Can cause trouble breathing (dyspnea), swallowing (dysphagia), feeding difficulties, or speech problems.
• Other complications: Recurrent infections (cellulitis) may cause pain and swelling. Bleeding can lead to hardening or sudden enlargement of the mass.

Causes of Lymphatic Malformations

Most lymphatic malformations are due to abnormal lymphatic system development, but the exact cause is unknown.
In some cases, cystic hygroma is linked to genetic syndromes such as:

• Noonan syndrome.
• Turner syndrome.
• Down syndrome.

These cases may involve genetic mutations or abnormal chromosome numbers.

Diagnosis

Lymphatic malformations can sometimes be diagnosed before birth with prenatal ultrasound. After birth, diagnosis usually involves a  physical exam and 
imaging tests such as MRI, CT scan, or ultrasound for more detail. Additional tests may be needed depending on the type and location of the mass.

Treatment Options

Treatment depends on the size, location, symptoms, and type of lymphatic malformation, as well as whether the mass affects quality of life.
The treatment for cystic hygromas and cavernous lymphangiomas may include:
•  Surgery to remove the mass.
•  Sclerotherapy, which involves injecting a solution into the mass to shrink it.
•  Laser therapy.
•  Radiofrequency ablation, in which a needle delivers electrical current to destroy abnormal tissue.

Because these masses may be difficult to remove completely, recurrence is common even after treatment.

Lymphangioma circumscriptum often requires no treatment unless symptoms develop. If symptoms develop, they may be removed with laser therapy or dermabrasion, although recurrence is possible.
 

Why Choose Cooper for Lymphatic Malformation Care

At the Children’s Regional Hospital at Cooper, we provide comprehensive care for children with lymphatic malformations. As the only state-designated acute care children’s hospital in South Jersey, we offer:

• Expertise: For complex medical or surgical needs, Children’s Regional Hospital at Cooper employs experts in 27 pediatric subspecialties. Many of our doctors are fellowship-trained, which means they receive advanced training to diagnose and treat children in their areas of expertise.
• Team approach: Children’s Regional Hospital at Cooper has 27 pediatric subspecialist programs – more than any other hospital in South Jersey. As our specialists collaborate together, you can be confident your child is receiving timely, expert care.
• Research and innovation: We strive to be on the leading edge of medical education and research. Our entire surgical staff stays on top of the most recent advancements in pediatric surgery techniques, ensuring all children receive the highest level of care.
• Advanced diagnostic and treatment options: Because of our affiliation with the Cooper Medical School at Rowan University, your child will benefit from the latest advances in medical care.