Cystic Hygroma

What is Cystic Hygroma?

Cystic hygroma refers to the abnormal lymphatic lesion that mostly develops at birth. It is a congenital defect that can affect any part of the human body but it in most cases affects the neck and head. It thereby affects babies and not the adults.

Picture 1 – Cystic Hygroma

Organs affected by Cystic Hygroma

In over 75% of the cases, it presents as predilection on the left side of neck.  If it has affected the neck, the greatest effect is felt and experienced on the posterior angle.

About 20% of the cystic hygroma cases affect the axilla. The least common Cystic Hygroma (less than 5%) occurs in the groin, mediastinum and the retroperitoneum.  This condition has also been reported to attack very rare locations like the middle ear.

Cystic hygroma was described for the first time in the year 1843 by Werenher. It is very synonymous to the macrocystic lymphatic malformation or cystic lymphangioma that was described for the first time by Redenbacker in the year 1828.

What causes Cystic Hygroma?

Cystic hygroma is believed to arise from the combination of several factors that include:

Failure of the connection between lymphatic and the venous system: This may cause the accumulation of fluid at the point in which the two systems have not properly connected to each other.

Failure of the lymphatic tissues to bud properly: If there is problem of budding of the lymphatic tissues, there may be some swelling and accumulation of fluid leading to cystic hygroma.

Lymphatic rests that are sequestered: In this case, there will be the retention of potential embryonic growth.  The lymphatic rests may sometimes penetrate the body structures that are adjacent or sometimes dissect along the plans of the face and they may become canalized in the ultimate. These spaces will make retention of those secretions that they produce and they may develop cystic components due to the lack of tract of venous outflow.

The nature of the tissues that surround these lymphatic rests determines the type of hygroma and it may be cystic hygroma, capillary hygroma or cavernous hygroma.  The cystic hygroma in most cases forms in the loose aerolar tissue while the cavernous and capillary hygroma in most cases form on the muscles.

Medical studies tend to show that the enlargement of cystic hygroma is directly related to engorgement instead of the actual proliferation of cells.  The molecular studies are to the effect that VEGF-C (Vascular Endothelial Growing Factor-C) together with its receptors can play a very vital role in the development of cystic hygroma malformations.

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

Karyotypic abnormalities: In between 25 and 70% of all the Cystic hygroma cases, the babies also suffer from Karyotypic abnormalities.  It is also very common in the babies that suffer from Down syndrome, Turner Syndrome, Trisomy 13, Trisomy 18 or the Klinefelter Syndrome. Even though the already mentioned conditions occur at the same time with cystic hygroma, they are not believed or proven to cause the condition.

Other conditions: Increased incidences of cystic hygroma have also been reported in the babies that suffer from nonchromosomal disorders such as Fryns syndrome, Noonan syndrome, achondroplasia and the multiple pterygium syndrome. Cystic hygroma may also be caused by 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 about the condition so there may be identified further causes or risk factors in the future.

What are the symptoms of Cystic Hygroma?

The symptoms of cystic hygroma vary depending on the part that has been attacked and infected by the condition. The cystic hygroma has a lot of predominance under the mylohyoid muscles and it may involve the posterior as well as the anterior neck triangles.

Blue hue over the skin: The cysts have thick wall and they are large in size. They have very little involvement with the tissue that surrounds it. The skin that overlies it may have a blue hue or in some other cases it may appear very normal.  In some other cases, the cystic hygroma will present as multi-cystic mass that has thin walls.

Bleeding: Because they are fragile they may have some areas that truly bleed. As stated earlier, they in most cases reside at the close association with the lymphatic ducts and the large veins in the groin, neck or axillary area. They may also infiltrate the areas of the skin that lie along the extremities of the trunk or even at the inside parts of the abdomen or chest.  They will present as discrete, soft and non-tender swellings which are in most cases, though not always compressible.

Enlargement of mass: The cystic hygroma will be clearly identifiable at the time of birth and it will seem to enlarge gradually as it gets filed with fluid.  It will be worse if the cystic hygroma point become infected by disease causing microorganisms like bacteria.  In this case, they may cause the cystic hygroma to be severe, very inflamed and reddish.  They may also become very tender and loll very hurt.

Breathing problems: Cystic hygromas that are located on the neck may cause breathing problems because of the pressure that they apply on the body respiratory airways. Kids suffer ring from this condition may also show retarded growth because they may have problems eating and swallowing. They may also have disinterest in play because of the pain or discomfort when they twist their neck or other part that the cystic hygroma may have affected.

Scarring of tissues: Infection of the cystic hygroma can also lead to slow response to the antibiotics treatments.  In case this happens, the cystic hygroma can have some scarring and the tissues that around may also become scarred.  This may cause a lot of complications for any medical interventional measures that may be undertaken in the form of surgical therapy.

What is the prevalence of Cystic Hygroma?

Globally, one child from every 6,000 to 16,000 babies that are born alive has a cystic hygroma.  It is sad top note that between 2 and 6% of the babies that are born with this condition die at the time of birth.  This death is in most cases not because of the cystic hygroma but because of the complications that arise from the condition including bronchiectasis, pneumonia and other complications of the airway system.

Mortality rate of Cystic Hygroma

The mortality is directly dependent on the cystic hygroma location.  If the condition has affected the vessels, nerves, airways and the lymphatics then the baby may be at very high risk of death.  This only occurs if necessary medical intervention is not sought early.

Who are at the risk of Cystic Hygroma?

Though babies of all races in the US are equally affected, there has been some decrease in the cystic hygroma incidences in the African Americans.  Both male and female babies seem to be equally affected by the condition so it does not vary by gender.

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

Can Cystic Hygroma be diagnosed in a fetus before birth?

Cystic hygroma can be visualized by gestation of 10 weeks through various medical procedures. These medical procedures vary in exactness, details and cost. Early diagnosis of the condition in a fetus before its birth is very good because it will prepare the mother and doctors to take the necessary interventional measures to save the life of the baby after delivery.

Diagnostic methods for Cystic Hygroma

Ultrasonography: One of these medical procedures is known as abdominal ultrasonography. This is medical imaging that is taken through the belly/ abdomen of the pregnant mother. These images will only show the location and possible size of cystic hygroma without any further details.

The transvaginal ultrasonography provides better details about the condition and is the most recommended.  In this examination, the medical images will be taken by a special device that is inserted through the birth canal of the pregnant woman’s vagina. It will take the image without any obstructions so it can give better details about the cystic hygroma.

MRI: To determine the extent of cystic hygroma in the fetus, the doctor may also use Fast-Spin Magnetic Resonance Imaging (MRI).   This test provides images of ultra high resolution and it can give very clear and exact details about cystic hygroma in the fetus.  The shortcoming of the test is that it is very expensive.

Amniocentesis: The doctors can also conduct an amniocentesis fluid test:  the amniocentesis fluid surrounds the fetus and it prevents it from experiencing shock. Doctors can collect this fluid suing a special needle and then perform some tests on it. High levels of apha fetoproteins in this fluid may indicate that the fetus is having a cystic hygroma.

How is Cystic Hygroma treated?

In very rare and exceptional cases, the cystic hygroma may heal automatically after birth. In most cases, there will be need for treatment and medical intervention.  If the cystic hygroma is punctured with a needle there may be some temporary relief but it may be followed by an infection and that may complicate the matters. There is also very high likelihood of the fluid accumulating again.

Injection of chemical agents: If the cysts are large special chemical agents may be injected soon as to cause shrinkage of the swelling. This treatment is however not very effective when the cysts are very small.  The injection with the chemicals is still faced by some other health risks and complications. If there is infection in the cystic hygroma, the baby may need to be treated with antibiotics.

At the present, there is no agent that is totally safe. The baby suffering from cystic hygroma will need to be put under constant and frequent medications and any changes of the condition should be addressed immediately.

Cystic hygroma surgery: Though still faced by risks and complications, the most promising treatment is surgery once the infection has been treated and cured.  Most of the pediatric surgeons will not undertake the surgery until the baby has attained six months.  The surgery may however be performed before the period of six months if the rate of increase of the cystic hygroma is faster than the rate of body growth of the baby.

Is Cystic Hygroma surgery safe?

Pediatric surgery is generally very sensitive and complicated than that of adults.  The surgery for the complete removal of cystic hygroma should be performed by very well trained and experience doctors. Pediatric surgeons admit that this surgery is usually very challenging. Once the surgery has been successfully undertaken, it is expected that there will be no very serious complications.  There will however be a risk of reoccurrence of cystic hygroma in 5 to 10 % of all the successful surgical treatments.

In doing the surgery, the pediatric surgeons make all the necessary efforts so as to remove the cyst remnants.  It should however be noted that the cysts is not cancerous and so there should be no interference with the major nerves or any other vital body structures when removing them.

Cystic Hygroma Pictures

Check out some of the pictures of Cystic Hygroma to know more about the condition.

Picture 2 – Cystic Hygroma Picture Picture 3 – Cystic Hygroma Image