Lymphatic Malformations

Lymphatic malformations, also called or lymphangiomas, are benign (non-cancerous) vascular lesions made of fluid-filled spaces (cysts) thought to occur due to abnormal development of a baby’s lymphatic system while in the womb. 

In general, lymphatic malformations are categorized into macrocystic, microcystic, or combined depending on the size of the cysts. They are also classified in superficial lesions known as lymphangioma circumscriptum, and more deep-seated lesions (cavernous lymphangioma and cystic hygroma). 

Lymphatic malformations are present at birth, although they may be discovered later in life. They are most commonly located in the neck and axilla (armpit), but they also occur in the mouth, arm, mediastinum, lung, leg, abdomen and intestines. Some may appear as a tiny blue or red spot, while others are massive and affect an entire arm or leg. 

The signs and symptoms vary depending on the size and location of the mass; however, lymphangiomas can disfigure affected areas and disrupt the function of nearby organs or tissues. The exact underlying cause is unknown. Treatment options vary based on the signs and symptoms present in each person but may include surgery.

In general, the long-term outlook (prognosis) for people with lymphtic malformations (lymphangiomas) is considered good since it is a benign mass that does not advance to cancer. However, these masses can sometimes negatively affect quality of life. Depending on the size and location of the mass, they can be associated with significant disfigurement of affected areas and/or symptoms caused by the compression or blockage of nearby body parts or organs. Although treatment can alleviate certain symptoms, lymphatic malformations may regrow in some cases.

Why Choose Cooper for Lymphatic Malformation Treatment

The Children’s Regional Hospital at Cooper is committed to excellence in education, research, and high-quality, safe care for pediatric patients and their families. As the only state-designated acute care children’s hospital in South Jersey, Cooper provides exceptional pediatric primary care and comprehensive specialty care services for every patient, every day, in a child- and family-centered environment.

Parents and caregivers choose Cooper because of our:

  • 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.

Symptoms of Lymphatic Malformations

The signs and symptoms of a lymphatic malformation (or lymphangioma) vary depending on the type, size and location of the mass.

  • Cystic hygroma, which is also known as cystic lymphangioma or macrocystic lymphatic malformation, is a red or bluish swelling of the skin in the neck, armpit or groin region that consists of large, fluid-filled spaces. Some say it is a type of cavernous lymphangioma.
  • Cavernous lymphangioma is also considered a macrocystic lesion. It is a bluish or red rubbery swelling under the skin that can affect any area of the body, including the tongue.
  • Lymphangioma circumscriptum is a microcystic lymphatic malformation. It appears as a cluster of small, firm blisters most commonly on the shoulders, neck, underarm area, limbs, and in the mouth, especially the tongue. It consists of tiny, fluid-filled spaces and ranges in color from clear to pink, dark red, brown or black.

In general, lymphatic malformations are always present at birth, although they typically grow over time and may become more apparent with age. They are most commonly found in the head and neck region, but can affect almost any part of the body (except the brain). Some may appear as a tiny blue or red spot while others are massive and affect an entire limb (arm or leg). 

Symptoms usually develop if the lymphatic malformation compresses or blocks nearby body parts or organs. For example, lymphangiomas located in the mouth or neck region can cause dyspnea, speech problems, dysphagia and feeding problems. Certain complications can occur, including recurrent cellulitis, which can cause pain and disfigurement of the affected area, and bleeding, which may lead to pain, hardening or rapid enlargement of the mass.

Causes of Lymphatic Malformations

A lymphatic malformation (lymphangioma) is thought to occur due to abnormal development of the lymphatic system; however, the exact underlying cause is unknown in most cases.

Cystic hygroma (cystic lymphangioma) may occur as part of a genetic syndrome such as Noonan syndromeTurner syndrome and Down syndrome. In these cases, the lymphatic malformations are likely due to genetic causes, such as a change (mutation) in a gene or an abnormal number of chromosomes (a missing or extra chromosome).

Diagnosis of Lymphatic Malformations

Lymphatic malformations (lymphangiomas) can often be diagnosed before birth on a prenatal ultrasound. After birth, a diagnosis of a lymphatic malformation is usually made based on physical examination. Additional imaging studies such as magnetic resonance imaging (MRI)computed tomography scanning (CT scan) and/or ultrasound may be used to evaluate the lymphangioma in more detail. Other exams may be needed depending of the type of the lymphatic malformation and the location.

Treatment for Lymphatic Malformations

The best treatment options for lymphatic malformations (lymphangiomas) depend on many factors including the size and location of the mass, the type of mass, whether or not the mass is causing symptoms, and/or the affected person's tolerance of certain procedures.

Depending on their size and location, cystic hygromas and cavernous lymphangioma can be treated with:

  • Surgery to remove the mass
  • Sclerotherapy, a solution injected into the lymphatic malformation causing it to shrink or collapse
  • Laser therapy
  • Radiofrequency ablation, a procedure where a needle is used to deliver a high-frequency alternating current that destroys abnormal tissue

Unfortunately, some of these masses may be difficult to remove and they often regrow after surgery.

Lymphangioma circumscriptum usually require no treatment, although they can be removed with dermabrasion or laser therapy. Like other types of lymphatic malformations, they often recur after removal.

Make an Appointment With a Lymphatic Malformation Expert at Cooper

To learn more about the services available for treating lymphatic malformation at Cooper or to request an appointment, please call 800.8.COOPER (800.826.6737).