What Is Cystic Hygroma?
A cystic hygroma is a fluid-filled sac, commonly found on a baby's neck, resulting from malformations or blockages in the lymphatic system. The lymphatic system, responsible for circulating lymph fluid containing white blood cells to eliminate waste, is crucial for the body's detoxification. Detection of cystic hygromas can occur through prenatal ultrasounds, and they may manifest post-birth as a noticeable bump under the baby's skin
Who Gets Affected by Cystic Hygroma (CH)?
Cystic hygroma is a rare benign tumor, often seen in babies, although adults with previous trauma or respiratory infection have also been known to develop CH. It is most commonly seen in the cervicofacial (neck and face), armpit, midriff, groin, and below the tongue. Occasionally, it has been known to occur in the liver, kidney, spleen, and intestine.
What Causes a Cystic Hygroma?
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The precise cause of cystic hygromas remains unknown. The cysts develop due to damage to the lymphatic system during fetal development or can result from physical trauma or respiratory infections in adults.
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Damage to the lymphatic system disrupts the normal flow of lymph fluid, leading to its accumulation under the skin and the formation of a sac-like growth or bulge.
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Research suggests that environmental factors such as smoking or alcohol consumption during pregnancy may contribute to the development of cystic hygromas.
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Studies propose that genetic changes or mutations affecting lymphatic system development could be implicated in the occurrence of cystic hygromas.
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While cystic hygromas often form independently, some cases are associated with genetic conditions like Down syndrome, Turner syndrome, or Noonan syndrome.
What Are the Symptoms of a Cystic Hygroma?
The tumor often appears as a bulge under the skin; often on the neck, it is soft to touch and has a bluish tint. It could increase in size as the baby grows. Severe symptoms include;
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Disfigurement around the head and neck.
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Organ damage.
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Obstructed airway.
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Deformed teeth and bones.
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Feeding difficulties.
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Webbed neck.
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Infection may occur through the skin or by the respiratory system.
If the cysts are present during pregnancy, there is a risk of miscarriage or stillbirth. It can be visualized using prenatal ultrasonography by ten weeks gestation. The sonography will show a thin-walled cystic mass, usually on the posterior part of the neck; the clinician will prescribe further investigations to rule out other developmental abnormalities.
What tests are used to diagnose cystic hygroma?
The diagnosis of cystic hygroma typically involves various tests and imaging studies to assess the extent and characteristics of the condition. Common diagnostic approaches include:
Cystic Hygroma Ultrasound - Cystic hygroma ultrasound employs high-frequency sound waves to non-invasively visualize the presence, location, and size of fluid-filled sacs in the fetus. Cystic hygroma ultrasound is crucial for early detection during prenatal examinations, providing detailed images that aid in assessing the severity and potential impact on surrounding structures. It serves as a primary method for monitoring cystic hygromas, offering valuable insights into fetal abnormalities and guiding healthcare decisions. While cystic hygroma ultrasound is essential, complementary diagnostic methods are also used.
Fetal MRI - Magnetic Resonance Imaging (MRI) provides detailed images and is particularly useful in assessing the size and location of cystic hygromas in a fetus.
Amniocentesis - This procedure involves the removal of a small amount of amniotic fluid from the womb, which can be tested for chromosomal abnormalities. While not a direct test for cystic hygroma, it helps identify associated conditions.
Chorionic Villus Sampling (CVS) - Similar to amniocentesis, CVS is a prenatal test that involves the removal of a small sample of tissue from the placenta to analyze for genetic disorders.
Blood Tests - Certain blood tests may be conducted to assess markers associated with genetic conditions and to provide additional information for diagnosis.
How Is a Cystic Hygroma Treated?
The most preferred way of treating cystic hygroma is complete surgical excision; in a few cases, surgery might not be possible when it involves deep and vital structures. In those patients, alternative treatments are available; they are-
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Aspiration or Percutaneous Drainage- Aspiration (drawing of fluid) can be performed as a temporary measure to reduce the size, thereby reducing the pressure on the respiratory and feeding passages.
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Sclerotherapy- It involves rerouting the cyst’s fluid throughout the body by using agents like boiling water, quinine, sodium morrhuate, urethane, iodine tincture, Doxycycline, and Nitromin.
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Bleomycin- It is used in chemotherapy to treat different cancers. It works by converting the fluid-filled tumor into a fibrous cyst and eventually reduces its size; in some cases, it is even known to completely resolve the tumor.
Another agent which has shown good results is OK-432, also known as Picibanil, which produces an inflammatory reaction and reduces the size of the tumor.
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Laser Therapy- Lasers have been increasingly used for different lymphangiomas, it causes point destruction of the tumor, avoiding damage to the vital organs.
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Radiofrequency Ablation- Recent technological advances have made it possible to use radiofrequency current to ablate lymphangiomas.
What Are the Complications of the Treatment?
In rare cases, cystic hygroma may get confused for nuchal translucency, which is a normal collection of fluid under the skin behind the fetal neck in the first trimester of pregnancy. This confusion might delay the diagnosis, and if combined with chromosomal abnormalities, it can be fatal and increase the risk of miscarriage or stillbirth. Surgical treatment may result in scarring, influenced by the cyst's size and location. Less invasive treatments, such as laser therapy, exist to minimize scarring.
Post-Treatment Care - Treat the cyst post-surgery like a wound, watching for signs of infection. Any treatment carries infection risks; monitor for leakage, color changes, size alterations, or tenderness. Contact your provider if infection is suspected.
Can Cystic Hygroma Be Prevented?
Prevention is challenging due to the unknown cause. Consider genetic testing before pregnancy to understand genetic risks. Avoid smoking or drinking during pregnancy, attend regular checkups, maintain a balanced diet, and stay healthy.
What Is the Outlook of This Condition?
Prognosis depends on when the cystic hygroma is diagnosed, its size, location, and underlying conditions. Successful surgery improves the outlook; if excess tissue is safely removed, the prognosis is good: If excess tissue cannot be safely removed, there is a minor chance of cyst recurrence.
Conclusion:
Cystic hygroma is a manageable tumor; with proper treatment customized to the patient, chances of recurrence are low. With recent advances like laser and radiofrequency, issues like scarring can be avoided. There are risks with any treatment; make sure to monitor the post-surgical site for any leakage or color change and contact the clinician if there are signs of infection.
