What are Lymphatic Malformations? (Lymphatic System Part 2)

Summary

Lymphatic malformations are congenital conditions where the lymphatic system develops abnormally, causing swelling in various parts of the body. These malformations can range from tiny, sponge-like cysts to larger fluid-filled bubbles, often appearing near the neck and armpits. While not cancerous, they can cause physical and emotional distress. Treatments include observation, sclerotherapy to shrink cysts, surgical removal, and medications to manage symptoms. The condition may change over time, and long-term monitoring is crucial. Children's Hospital Colorado’s Vascular Anomalies Center leads research to develop more targeted treatments for lymphatic malformations.

Takeaways

• 😀 Lymphatic malformations are congenital conditions where the lymphatic system forms incorrectly, causing swelling in different parts of the body.
• 😀 These malformations are present from birth and can be visible in early childhood, though sometimes not until later in life.
• 😀 Lymphatic malformations are caused by errors in the DNA blueprint, leading to the incorrect formation of the lymphatic system, and are not cancerous.
• 😀 The term 'cystic hygroma' or 'lymphangioma' is outdated, as these terms wrongly imply a link to cancer, which lymphatic malformations do not have.
• 😀 There are different types of lymphatic malformations, including 'microcystic' (small clusters of bubbles), 'macrocystic' (larger, smooth, translucent cysts), and 'mixed' types.
• 😀 Cystic lymphatic malformations are typically located near major lymphatic channels, such as the neck and armpits, but can form anywhere in the body.
• 😀 Central Conducting Lymphatic Anomalies (CCLA) involve malformed central lymphatics, leading to large lymphatic traffic jams and fluid buildup in the chest or abdomen.
• 😀 When the lymphatic system is blocked, it can lead to conditions like chylothorax (fluid in the chest) or chylous ascites (fluid in the abdomen), causing discomfort and breathing issues.
• 😀 The appearance of lymphatic malformations can change over time due to factors like injury, infection, or puberty, potentially causing swelling or rapid growth.
• 😀 Treatment options include observation, sclerotherapy (a procedure to shrink cysts), surgical removal, and medications to manage symptoms and improve lymphatic drainage.
• 😀 Long-term monitoring is essential, as flare-ups of swelling may occur, and ongoing research is aimed at developing more effective and personalized treatments.

Q & A

What is a lymphatic malformation?

A lymphatic malformation occurs when the lymphatic system doesn't form correctly, causing parts of the body to swell. It is a congenital condition that can be visible from early childhood, though it may appear later in life as well.

Are lymphatic malformations cancerous?

No, lymphatic malformations are not cancerous and do not become cancerous. Terms like 'cystic hygroma' and 'lymphangioma' are outdated and can be misleading, as they imply a relationship to cancer, which is incorrect.

What are the different types of lymphatic malformations?

There are several types of lymphatic malformations, including cystic lymphatic malformations (which can be microcystic or macrocystic), and Central Conducting Lymphatic Anomalies (CCLAs). Microcystic types are small and sponge-like, while macrocystic types are larger, smooth, and translucent. CCLAs affect the central lymphatics and can cause fluid buildup in areas like the chest or abdomen.

How do lymphatic malformations affect the body?

Lymphatic malformations cause swelling by disrupting normal lymphatic flow, which can lead to pressure on surrounding tissues. This can result in pain, difficulty breathing (if in the chest), and abdominal discomfort (if in the abdomen). Over time, it can lead to further health issues due to improper delivery of fats, proteins, and immune cells.

What is the difference between microcystic and macrocystic lymphatic malformations?

Microcystic lymphatic malformations consist of small, clustered bubbles that are sponge-like and may appear as small blisters on the skin. Macrocystic lymphatic malformations are larger, typically over 2cm, and appear as smooth, translucent bubbles under the skin. They can cause more noticeable swelling and discomfort.

What are CCLAs (Central Conducting Lymphatic Anomalies)?

CCLAs occur when the central lymphatic system, which transports chyle from the intestines, is malformed. This causes large traffic jams of lymph, and the body compensates by forming new, irregular lymphatic pathways. These pathways do not reach the heart, causing lymph to pool in areas like the chest or abdomen, leading to conditions like chylothorax and chylous ascites.

What are some complications of lymphatic malformations?

Complications can include infections, which can cause the cysts to swell or change in appearance. Lymphatic malformations can also grow larger over time, especially during puberty, and may lead to discomfort, pain, and other health problems like difficulty breathing or digestive issues.

How are lymphatic malformations treated?

Treatment options for lymphatic malformations include observation, sclerotherapy, surgery, and medications. Sclerotherapy involves injecting a medication that causes the cyst to shrink by creating scar tissue. Surgery may be an option for small, localized malformations. Medications may help reduce swelling and regulate lymphatic flow. Long-term monitoring is often required.

What is sclerotherapy and how does it work?

Sclerotherapy is a treatment for macrocystic lymphatic malformations. Under general anesthesia, a medication is injected into the cyst that irritates it, causing scar tissue to form. This leads to the cyst shrinking over time. Multiple treatments may be needed, and the cyst may initially swell before starting to shrink over a month.

Can lymphatic malformations disappear on their own?

In some cases, lymphatic malformations may shrink or deflate over time, especially if observed closely. However, many cases require medical intervention, such as sclerotherapy or surgery, to manage symptoms and reduce swelling. Long-term monitoring is often necessary to prevent flare-ups.