Cystic hygroma: Causes, Symptoms, Risk Factors, Diagnosis and Treatment

It is an abnormal lymphatic lesion that develops mainly at birth.

It is a congenital defect that can affect any part of the human body, but in most cases it affects the neck and head.

Therefore, it affects babies and not adults. A common hygroma is the cystic type.

Organs affected by cystic hygroma

In more than 75% of cases, it appears as a predilection on the left side of the neck. If it has affected the neck, the greatest effect is felt and experienced in the posterior angle.

About 20% of cases of cystic hygroma affect the axilla. Less common cystic hygroma (less than 5%) occurs in the groin, mediastinum, and retroperitoneum. It has also been reported that this condition attacks very rare locations such as the middle ear.

Cystic hygroma was described by Werenher for the first time in 1843. It is synonymous with a macrocytic lymphatic malformation or cystic lymphangioma that was first described by Redenbacker in 1828.

What causes cystic hygroma?

It is believed that cystic hygroma arises from the combination of several factors that include:

Failure of the connection between the lymphatic system and the venous system: this can cause the accumulation of fluid at the point where the two systems have not connected correctly to each other.

Failure of the lymphatic tissues to sprout correctly : if there is a budding problem of the lymphatic tissues, there may be some swelling and accumulation of fluid that leads to a cystic hygroma.

Lymphatic remains that are sequestered : in this case, there will be retention of potential embryonic growth.

Lymphatic remains can sometimes penetrate structures of the body that are adjacent or sometimes dissect along the planes of the face and can ultimately be channeled.

These spaces will maintain those secretions that produce and can develop cystic components due to the lack of venous exit routes.

The nature of the tissues surrounding these lymphatic remains determines the type of hygroma and can be cystic hygroma, capillary hygroma or cavernous hygroma.

The cystic hygroma in most cases is formed in the loose areolar tissue, while the cavernous and capillary hygroma in most cases is formed in the muscles.

Medical studies tend to show that the enlargement of cystic hygroma is directly related to congestion rather than the actual proliferation of cells.

Molecular studies indicate that VEGF-C (vascular endothelial growth factor C) together with its receptors can play a very important role in the development of cystic hygroma malformations.

Neck trauma : cystic hygroma may also develop as a result of neck trauma (which may even be caused by surgery), obstruction or even inflammation of the lymphatic drainage pathway.

Karyotypic abnormalities : between 25 and 70% of all cases of cystic hygroma, babies also suffer from karyotypic abnormalities.

It is also very common in babies suffering from Down syndrome, Turner syndrome, trisomy 13, trisomy 18 or Klinefelter syndrome.

Although the aforementioned conditions occur at the same time with cystic hygroma, it is not believed or proven to cause the condition.

Other conditions : A higher incidence of cystic hygroma has also been reported in infants suffering from non-chromosomal disorders such as Fryns syndrome, Noonan syndrome, achondroplasia and multiple pterygium syndrome.

Cystic hygroma can also be caused by the intrauterine exposure of alcohol.

Despite these identified causes, it is believed that there may be other causes or aggravating factors that are not yet known. There is ongoing research on the condition so there may be more causes or risk factors identified in the future.

What are the symptoms?

The symptoms of cystic hygroma vary according to the part attacked and infected by the condition. Cystic hygroma is much more prevalent under the mylohyoid muscles and may involve both posterior and anterior triangles.

Blue tone on the skin : the cysts have a thick wall and are large. They have very little involvement with the tissue that surrounds it.

The skin that covers it may have a blue tone or in other cases it may seem very normal. In some other cases, the cystic hygroma will appear as a multicystic mass that has thin walls.

Bleeding: Because they are fragile, they may have some areas that actually bleed. As indicated above, in most cases they reside in close association with the lymphatic ducts and large veins in the groin, neck or axillary area.

They can also infiltrate the areas of the skin that are along the extremities of the trunk or even in the internal parts of the abdomen or thorax.

They will present as discrete, soft and non-sensitive swellings that are in most cases, although not always compressible.

Enlargement of the mass: the cystic hygroma will be clearly identifiable at the time of birth and will appear to gradually enlarge as it fills with fluid.

It will be worse if the point of cystic hygroma is infected with microorganisms that cause diseases such as bacteria. In this case, they can cause the cystic hygroma to be severe, very inflamed and reddish. They can also become very sensitive and very painful.

Breathing problems : the cystic hygromas found in the neck can cause respiratory problems due to the pressure exerted on the respiratory tract of the body.

Children who suffer from this problem may also show stunted growth because they may have problems eating and swallowing.

They may also have disinterest in the game due to pain or discomfort when they twist the neck or other part that the cystic hygroma may have affected.

Tissue healing: Cystic hygroma infection can also lead to a slow response to antibiotic treatments.

In case this happens, the cystic hygroma may have some scars and the tissues surrounding it may also heal. This can cause many complications for any medical intervention measure that can be performed in the form of surgical therapy.

What is the prevalence?

Worldwide, one child in every 6,000 to 16,000 babies born alive has a cystic hygroma. It is sad to note that between 2 and 6% of babies born with this condition die at the time of birth.

In most cases, this death is not due to cystic hygroma, but to the complications that arise from the condition, such as bronchiectasis, pneumonia and other complications of the respiratory system.

Mortality rate of cystic hygroma

Mortality depends directly on the location of the cystic hygroma. If the condition has affected the vessels, nerves, airways, and lymph vessels, then the baby may have a very high risk of death.

This only happens if the necessary medical intervention is not sought early.

Risk factor’s

Although babies of all races in the US UU They are equally affected, there has been a certain decrease in the incidences of cystic hygroma in African-Americans.

Both male and female babies seem to be equally affected by the condition, so it does not vary by sex.

Between 50% and 65% of all cases of cystic hygroma are diagnosed and can be seen at birth. In about 80 to 90% of all cases of cystic hygroma, the condition will be fully present when the baby reaches the age of 2 years.

Can a cystic hygroma be diagnosed in a fetus before birth?

Cystic hygroma can be visualized by gestation for 10 weeks through various medical procedures. These medical procedures vary in accuracy, detail and cost.

Early diagnosis of the condition in a fetus before birth is very good because it will prepare the mother and the doctors to take the necessary intervention measures to save the life of the baby after delivery.

Diagnostic methods

Ultrasonography: one of these medical procedures is known as abdominal ultrasonography. This is a medical image that is taken through the abdomen of the pregnant mother.

These images will only show the location and possible size of the cystic hygroma without further details.

Transvaginal ultrasound provides better details about the condition and is the most recommended. In this exam, the medical images will be taken with a special device that is inserted through the birth canal of the pregnant woman’s vagina.

It will take the image unobstructed so you can give better details about the cystic hygroma.

Magnetic resonance: To determine the extent of cystic hygroma in the fetus, the doctor can also use fast-spin magnetic resonance imaging.

This test provides ultra high resolution images and can provide very clear and accurate details about the cystic hygroma in the fetus. The drawback of the test is that it is very expensive.

Amniocentesis: doctors can also perform a fluid test of amniocentesis; the amniocentesis fluid surrounds the fetus and prevents it from experiencing shock.

Doctors can collect this fluid with a special needle and then perform some tests on it. High levels of apha fetoproteins in this fluid may indicate that the fetus has a cystic hygroma.

Treatment

In very rare and exceptional cases, the cystic hygroma can heal automatically after birth. In most cases, there will be a need for medical treatment and intervention.

If the cystic hygroma is punctured with a needle, there may be some temporary relief, but it can be followed by an infection and that can complicate things. There is also a very high probability that the fluid will accumulate again.

Injection of chemical agents : if the cysts are large, chemical agents can be injected early to reduce swelling. However, this treatment is not very effective when the cysts are very small.

Injection with chemicals still faces other risks and complications for health. If there is an infection in the cystic hygroma, the baby may need treatment with antibiotics.

In the present, there is no agent that is totally safe. The baby suffering from cystic hygroma will have to undergo constant and frequent medications and any change in the condition should be addressed immediately.

Cystic hygroma surgery : although it still faces risks and complications, the most promising treatment is surgery once the infection has been treated and cured.

Most pediatric surgeons will not perform surgery until the baby has reached six months.

However, surgery can be performed before the six-month period if the rate of cystic hygroma increase is faster than the baby’s body growth rate.

Is cystic hygroma surgery safe?

Pediatric surgery is generally more sensitive and complicated than that of adults. Surgery for the complete removal of cystic hygroma should be performed by very well trained and experienced doctors.

Pediatric surgeons admit that this surgery is usually very difficult. Once the surgery has been carried out successfully, it is expected that there are not very serious complications. However, there will be a recurrence risk of cystic hygroma in 5 to 10% of all successful surgical treatments.

When doing surgery, pediatric surgeons make every effort to remove the remains of the cyst.

However, it must be taken into account that the cysts are not cancerous and therefore there should be no interference with the main nerves or any other vital structure of the body when extracting them.