It is an abnormal lymphatic lesion that develops mainly at birth.
It is a congenital disability that can affect any part of the human body, but it affects the neck and head in most cases.
Therefore, it affects babies and not adults. A common hygroma is a cystic type.
Organs affected by cystic hygroma
In more than 75% of cases, it appears as a preference on the left side of the neck. The most significant effect is felt and experienced in the posterior angle if it has affected the neck.
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 sporadic locations such as the middle ear.
Wernher described cystic hygroma for the first time in 1843. It is synonymous with a macrocytic lymphatic malformation or cystic lymphangioma first characterized by Redenbacker in 1828.
What causes cystic hygroma?
It is believed that cystic hygroma arises from the combination of several factors that include:
Failure to connect between the lymphatic and venous systems can cause fluid accumulation when the two systems have not connected correctly.
Failure of the lymphatic tissues to sprout correctly: if there is a budding problem of the lymphatic tissues, some swelling and accumulation of fluid may lead 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 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 large hygroma.
The cystic hygroma, in most cases, is formed in the loose areolar tissue, while the cavernous and capillary hygroma, in most cases, is included 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) and its receptors can play a vital role in developing cystic hygroma malformations.
Neck trauma: cystic hygroma may also develop due to neck trauma (which may even be caused by surgery), obstruction, or even inflammation of the lymphatic drainage pathway.
Karyotypic abnormalities: babies also suffer from karyotypic abnormalities: in between 25 and 70% of all cases of cystic hygroma.
It is also widespread in babies with Down syndrome, Turner syndrome, trisomy 13, trisomy 18, or Klinefelter syndrome.
Although the conditions above co-occur with cystic hygroma, it is not believed or proven to cause the disease.
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 intrauterine exposure to 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 more prevalent under the mylohyoid muscles and may involve posterior and anterior triangles.
Blue tone on the skin: the cysts have a thick wall and are significant. They have very little involvement with the tissue that surrounds them.
The skin that covers it may have a blue tone or seem very normal in other cases. In some other cases, cystic hygroma will appear as a multicystic mass with thin walls.
Bleeding: Because they are fragile, they may have some areas that bleed. As indicated above, in most cases, they reside in close association with the lymphatic ducts and prominent 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, not always compressible.
Enlargement of the mass: the cystic hygroma will be identifiable at birth and enlarge as it gradually 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 susceptible and very painful.
Breathing problems: the cystic hygromas found in the neck can cause respiratory problems due to the pressure exerted on the body’s respiratory tract.
Children who suffer from this problem may also show stunted growth because they may have difficulties eating and swallowing.
They may also be disinterested in the game due to pain or discomfort when they twist the neck or other parts that the cystic hygroma may have affected.
Tissue healing: Cystic hygroma infection can also lead to a slow response to antibiotic treatments.
If 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 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 respiratory system complications.
The 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, the baby may have a very high risk of death.
This only happens if the necessary medical intervention is not sought early.
Although babies of all races in the US UU are equally affected, there has been a specific 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 cystic hygroma be diagnosed in a fetus before birth?
Cystic hygroma can be visualized by gestation for ten 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 perfect because it will prepare the mother and the doctors to take the necessary intervention measures to save the baby’s life after delivery.
Ultrasonography: one of these medical procedures is known as abdominal ultrasonography. This is a medical image 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 particular 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 give obvious and accurate details about the cystic hygroma in the fetus. The drawback of the test is that it is costly.
Amniocentesis: doctors can also perform a fluid test or amniocentesis; the amniocentesis fluid surrounds the fetus and prevents it from experiencing a shock.
Doctors can collect this fluid with a special needle and then perform some tests on it. High alpha fetoproteins in this fluid may indicate that the fetus has a cystic hygroma.
In sporadic and exceptional cases, cystic hygroma can heal automatically after birth. There will be a need for medical treatment and intervention in most cases.
If the cystic hygroma is punctured with a needle, there may be some temporary relief, but it can be followed by an infection, complicating 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 tiny.
Injection with chemicals still faces other risks and complications for health. If there is an infection in cystic hygroma, the baby may need treatment with antibiotics.
At present, no agent is 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 months if the rate of cystic hygroma increases 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. Well-trained and experienced doctors should perform surgery to remove cystic hygroma altogether.
Pediatric surgeons admit that this surgery is usually complicated. Once the surgery has been carried out successfully, it is expected that there will be no severe 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.