What is Cystic Hygroma (Lymphangioma)?
Cystic hygromas, a common type of lymphangiomas, are birth defects involving the lymphatic drainage system. Frequently, these can develop in the areas of the neck, collarbone, and underarm. These malformations are mostly detected in newborns or during prenatal ultrasounds. Depending on where they occur in the body, they can potentially block the airway. Difficulty breathing, infection, and cosmetic concerns are the primary reasons for seeking treatment. The go-to method of medical management for cystic hygromas is usually surgical removal. This discussion aims to shed light on the symptoms, evaluation, and handling of cystic hygromas in infants, children, and adults.
What Causes Cystic Hygroma (Lymphangioma)?
Cystic hygromas are harmless fluid-filled lumps caused by problems with the development of the lymphatic system, a part of your body’s immune system. While their exact origin during fetal development isn’t fully known, professionals think that they happen because of defects in the formation of lymphatic channels, which are the passageways that help to carry lymph fluid around the body.
Cystic hygromas appear to come from leftover embryonic lymphatic tissue that still has the ability to grow. Research has linked them to certain medical conditions, such as abnormalities in the number of chromosomes, a severe condition in unborn babies known as hydrops fetalis, and death in an unborn baby.
Risk Factors and Frequency for Cystic Hygroma (Lymphangioma)
Cystic hygromas are unusual growths found more in infants and young children, making up 6% of all harmless growths in these ages. Despite its rarity, when it comes to lymphangiomas – a type of benign lump – cystic hygromas are the subtype that doctors see most often. Half of these growths are present from birth, with the other half developing by the time children turn two. Most cases (up to 90%) are identified in children under the age of two, but the majority are diagnosed between ages 3 and 5.
- – 75 to 90% of cystic hygromas are found in the neck (cervical region).
- – 20% are spotted in the armpit (axillary area).
- – The rest occurs in the groin (inguinal regions), the area behind the stomach and around the kidneys (retroperitoneal space), and chest (thoracic region).
Signs and Symptoms of Cystic Hygroma (Lymphangioma)
Cystic hygromas usually appear in children, with adults very rarely being affected. The individual or their guardian should provide a thorough health history to help the medical provider diagnose the condition. Often, the person will have a large, soft, painless lump, usually noticed around the time of birth or before the age of 2. It’s important to check if the person has any underlying genetics conditions that might increase their chances of having a cystic hygroma. Also, it’s necessary to rule out any symptoms that might indicate an infection, such as fever or feeling unwell.
The symptoms experienced by the person can differ depending on where the lump is located. If the lump is pressing on vital structures in the neck, the person might experience pain, voice changes, difficulty swallowing, or shortness of breath. Some may also experience restricted neck movement.
Cystic hygromas are often found in the neck region and are characterized by being soft, mobile, and able to transmit light. They do not typically have a pulse and may enlarge when the person coughs or cries. These lumps can vary in size, from 1-30cm, and may occur on one or both sides. The skin over the lump is usually normal in appearance. If the cystic hygroma is infected, it might be tender and have red skin over it.
Testing for Cystic Hygroma (Lymphangioma)
For a harmless, non-infected lump known as a cystic hygroma, standard physical checks and blood tests likely won’t show anything unusual. However, if the lump has developed into an abscess, or an infected area filling with pus, blood tests may reveal a higher white blood cell count and increased inflammation.
To confirm the presence of a cystic hygroma, doctors usually aspirate, or drain fluid from, the lump and combine this with different imaging methods. These imaging methods can include an ultrasound, CT scan, or MRI.
The least intrusive of these methods is the ultrasound, which typically shows an image of multiple small fluid-filled sacs within the lump. A CT scan or MRI can give a clearer picture of the lump and its location relative to nearby structures, which is helpful in planning for surgery. If the lump is aspirated, the fluid that comes out is often clear, light pink, or straw-colored.
In some cases, doctors might perform karyotyping, a test examining the structure and number of chromosomes in a cell, particularly if they suspect a chromosomal disorder.
Treatment Options for Cystic Hygroma (Lymphangioma)
Cystic hygromas are noncancerous growths, which do not require treatment if they aren’t causing any issues. They may need to be treated if they cause an infection, bleeding, breathing difficulties, trouble swallowing, or physical deformities. Surgery is the main way to manage these growths; however, other options like sclerotherapy (a procedure that shrinks the cyst), drainage, radio-frequency ablation (a procedure that uses heat to remove the cyst), or cauterization can also be used. The treatment chosen will depend on the size, location, and any complications of the cyst.
If a cystic hygroma turns into an abscess, or a pocket of pus, then medications that fight infections, reduce fever, and relieve pain will be needed. It may also require surgical treatment. If surgery is necessary, it is usually not done until at least three months after the course of antibiotics.
What else can Cystic Hygroma (Lymphangioma) be?
A lump in the neck is a common concern for both children and adults, and there could be many possible reasons behind it.
- Lumps that are present from birth are generally due to conditions present from before birth, like thyroglossal cysts, branchial cysts, dermoid cysts, or teratomas.
- Some lumps may be due to infections that cause swollen inflammation in the lymph nodes.
- They could also suggest more serious diseases like cancer, specifically lymphoma.
- Inflammatory diseases like sarcoidosis could also cause these lumps.
- Some lumps might appear after an event like an injury, which can cause a clot of blood (hematoma).
- Vascular issues, like carotid body tumors, are another possible reason.
Your healthcare provider will consider all these possible causes to find out the reason behind a neck lump.
What to expect with Cystic Hygroma (Lymphangioma)
The outlook for a patient with a cystic hygroma, a fluid-filled sac, greatly depends on the location of the sac and any additional complications that may arise. Cysts detected before birth often have a less favorable outcome compared to those identified after birth.
Surgical removal of the cyst generally leads to good results and complete recovery if the entire cyst can be removed. Still, in some cases, quite high, up to 53% of cases, may require surgery. This success rate can be improved with the help of additional therapies, such as sclerosing agents, which help to shrink the cyst.
If cystic hygromas are not treated, they are likely to continue to grow and lead to further complications.
Possible Complications When Diagnosed with Cystic Hygroma (Lymphangioma)
Complications may occur if the cystic hygroma, a fluid-filled sac, rapidly grows in size and spreads into the neck. This can cause a range of issues such as complete or partial blockage of the airways, difficulty swallowing, and sleep apnea, a sleep disorder where breathing repeatedly stops and starts. There can also be other complications like bleeding inside the cystic hygroma, which can lead to an infection and later the formation of an abscess, a collection of pus inside the body.
Possible complications include:
- Complete or partial blockage of the airways
- Difficulty swallowing
- Sleep apnea
- Bleeding inside the cystic hygroma
- Infections
- Abscess formation
Preventing Cystic Hygroma (Lymphangioma)
It’s essential to educate patients to help them avoid complications that can stem from not seeking medical help right away. Serious complications, like obstructions in the patient’s airway, can escalate quickly and cause death if they aren’t treated immediately.
