What Is Lymphangioma?

Overview

Lymphangiomas, also known as lymphatic malformations, are benign (non-cancerous) sacs filled with fluid that develop in the lymphatic vessels. These vessels, along with the lymphatic system, contain a fluid called lymph, which plays a role in supporting the immune system in fighting infections. 

Approximately 1 in 4,000 births are affected by lymphangiomas, which can occur in various parts of the body but are most commonly found in the head or neck region, accounting for about 75% of cases. Half of these lymphangiomas in the head or neck are detectable at birth and around 90%become noticeable by the age of 2.³ 

Causes of lymphangioma

Lymphangiomas develop when there is a buildup of lymph in the lymphatic vessels that surround the body's tissues, resulting in swelling and accumulation of fluid. The exact cause of this condition is not fully understood, but experts believe it may be due to abnormalities in cell division during foetal development, leading to a malfunctioning lymphatic system. 

In some cases, lymphangiomas can occur in babies who have chromosomal abnormalities, such as Down syndrome and Noonan syndrome, where there are either too many or too few chromosomes. Most individuals with lymphangiomas are born with them, although they may not be immediately apparent at birth. As these children grow older, the lymphangiomas can become more noticeable. 

Signs and symptoms of lymphangioma

Lymphangiomas can manifest as either a single localized growth in a specific area or multiple growths distributed throughout the body. The symptoms experienced vary depending on the size and location of the lymphangioma. Initially, they may appear as small reddish or blue dots. However, as lymph fluid continues to accumulate, it can lead to significant and disfiguring swelling and masses. 

The severity of health problems associated with lymphangiomas depends on the specific location of the swelling and the tissues or organs involved. For instance, if lymphangioma affects the tongue, it can result in difficulties with speech and eating. In the case of lymphangiomas in the eye socket, double vision may occur. Lymphangiomas affecting the chest can cause breathing difficulties and chest pain. 

There are two types of lymphangiomas, primarily categorized based on their size:

1. Macrocystic lymphangiomas: These lymphangiomas are larger than 2 centimetres (cm) and have well-defined borders. They may also be referred to as cystic hygromas or cavernous lymphangiomas. These lymphangiomas often have a bluish-reddish colour and a spongy texture. 
2. Microcystic lymphangiomas: These lymphangiomas are smaller than 2 cm and have undefined borders. They may grow in clusters and appear as tiny blisters. They can also be known as lymphangioma circumscriptum and capillary lymphangiomas. 

Management and treatment for lymphangioma

If your child's lymphangioma only causes mild symptoms or no symptoms at all, their doctor may recommend against treatment. However, if the lymphangioma is large, causes discomfort, or impacts your child's well-being, the doctor will likely suggest various treatment options based on the type, size, and location of the lymphangioma.⁴ 

Treatment options may include: 

• Surgery: This involves surgically removing the cyst. However, if the mass is located near organs and nerves, the procedure can be challenging.
• Sclerotherapy: This therapy involves injecting a chemical directly into the mass to shrink and collapse it. Sclerotherapy is increasingly being used and has a similar success rate to surgery but with fewer complications.¹
• Laser therapy or radiofrequency ablation: This involves using a laser or a needle to deliver a current that destroys the mass.²

Since completely removing or destroying every cell of a lymphangioma is nearly impossible, they have a tendency to grow back. Multiple surgeries or treatments may be necessary over time to effectively manage the condition. 

The recurrence rates of lymphangiomas vary depending on the type, location, and whether they are present in one or multiple areas throughout the body. 

Diagnosis

Lymphangiomas can be detected before birth through ultrasound examinations if the abnormality is identified. If a doctor observes a lymphangioma after the child is born, they may request additional diagnostic tests such as an MRI scan, CT scan, or ultrasound to confirm the diagnosis and assess its size and impact.⁴ In cases where the lymphangioma is not visually apparent at birth, it typically becomes noticeable by the age of 2. 

FAQs

Can lymphangioma be prevented?

Currently, there are no known preventive measures to completely avoid the development of lymphangiomas. As the exact cause of lymphangiomas is not fully understood, it is challenging to implement specific strategies for prevention. Lymphangiomas can occur spontaneously and may be influenced by genetic and developmental factors. 

However, in cases where lymphangiomas are associated with certain genetic syndromes or chromosomal abnormalities, genetic counselling and prenatal testing may be considered for individuals with a family history or known risk factors. This can help provide information and support for families who may be at higher risk of having a child with a lymphangioma. It is important to consult with a healthcare professional for personalised advice and guidance regarding lymphangioma prevention, particularly in cases where there may be underlying genetic conditions or risk factors involved. 

How common is lymphangioma?

Lymphangiomas are uncommon occurrences. They make up about 4% of all vascular tumours and approximately 25% of benign vascular tumours found in children.³

Who is at risk of having lymphangioma?

Lymphangiomas can occur in individuals of any age, including infants, children, and adults. While the exact cause of lymphangiomas is not fully understood, certain factors may increase the risk of developing these growths: 

Congenital cases: Lymphangiomas can be present at birth or develop during foetal development. Some individuals may have a genetic predisposition to developing lymphangiomas. 

Chromosomal abnormalities: Certain chromosomal disorders, such as Down syndrome and Noonan syndrome, have been associated with an increased risk of lymphangiomas. 

It is important to note that while these factors may increase the risk of developing lymphangiomas, they do not guarantee their occurrence. Lymphangiomas can still develop in individuals without these risk factors, and the presence of risk factors does not necessarily mean that a person will develop a lymphangioma. 

What can I expect if my child has lymphangioma?

If your child has been diagnosed with lymphangioma, it is important to consult with healthcare professionals who can provide specific information and guidance tailored to your child's case. However, here are some general aspects to consider:

Medical Monitoring: Your child may require regular follow-up visits with healthcare providers to monitor the lymphangioma's size, growth, and any associated symptoms. The frequency of these visits will depend on the specific characteristics of the lymphangioma and its potential impact on your child's health. 

Symptoms Management: Treatment may be recommended if the lymphangioma is causing significant discomfort, functional difficulties, or affecting your child's quality of life. Treatment options can vary, including observation, medication, surgery, sclerotherapy, or other interventions, depending on the location, size, and impact of the lymphangioma.

Potential Complications: Lymphangiomas can sometimes cause complications depending on their location and size. These complications may include difficulty breathing, swallowing, or speaking if located in the throat or neck region. Eye-related complications, such as vision problems or double vision, can occur if the lymphangioma affects the eye area. It is important to be aware of potential complications and address them promptly with medical professionals. 

When should I see a doctor?

If you suspect that you may have a lymphangioma or notice any concerning symptoms, it is recommended to schedule an appointment with a healthcare professional. 

Here are some situations in which you should consider seeing a doctor:

• Visible or palpable mass under your skin
• Unexplained swelling or abnormal growth
• Symptoms such as pain, pressure, difficulty swallowing, breathing problems, changes in vision
• Family history or known risk factors

Summary

Lymphangiomas are benign sacs filled with fluid that develop in the lymphatic vessels. They can occur in various parts of the body and are most commonly found in the head or neck region. Diagnosis can be made through ultrasound examinations before birth or through imaging tests after birth. Treatment options include surgery, sclerotherapy, and laser therapy, but recurrence is possible. It is important to consult a healthcare professional if you suspect a lymphangioma or experience concerning symptoms such as visible masses, swelling, or abnormal growths.