Cystic hygroma facts
- Cystic hygroma (CH) is a cluster of cysts in the lymph drainage system that usually affects the head and neck of children (fetuses and newborns to age 2 years).
- Cystic hygromas are congenital malformations in the lymph system.
- The cause is unknown but may be related to genetic changes in the fetus.
- Symptoms and signs include the appearance of clusters of cysts that are sometimes visible with the naked eye and may cause breathing difficulties, feeding problems, sleep apnea, and failure to thrive.
- If the cystic hygroma is not visible, it still can be diagnosed using CT scan, MRI scan, ultrasound and other techniques.
- Although medical treatment can be used, consisting of drugs that shrink down the cysts, many clinicians prefer surgical treatment to remove all of the cystic tissue.
- Although many patients have a good outcome, there are several indicators like abnormal fluid accumulation in two more fetal compartments like heart or lungs (fetal hydrops) or thickness of the lesion wall that suggest a poor outcome.
- Cystic hygromas are very infrequently seen in adults.
What is cystic hygroma?
The cystic hygroma is a lymphatic lesion composed of a cluster of cysts that can appear in almost any area of the body, although it usually affects the head and neck (about 75%), most often on the left side. Cystic hygromas (CH) are also termed cystic lymphangiomas or macrocystic lymphatic malformations. The majority (over 50%) are seen at birth and about 80% to 90% present by 2 years of age. The microcystic form is comprised of cysts that are less than 2 centimeters while the macrocystic form comprises cysts greater than 2 centimeters.
What causes cystic hygroma?
The cause of cystic hygromas is not known, but genetic abnormalities are present in about 25% to 75% of affected children. The disease is more common in individuals with Turner syndrome, Down syndrome, or Klinefelter syndrome.
What are the symptoms and signs of cystic hygroma?
The symptoms and signs of cystic hygroma vary with the location of the cluster of cysts. However, because many are present in the neck, they can produce symptoms that range from none to severe. Signs and symptoms of cystic hygroma are as follows:
- clusters of clear, black, or red vesicles on buccal mucosa or the tongue (microcystic form)
- large cysts with overlying bluish or normal-appearing skin (macrocystic form)
- sleep apnea, occasionally obstructive
- breathing difficulties
- feeding difficulties
- failure to thrive (especially in children who have involvement of the gastrointestinal tract)
- intra-lesional bleeding (rare)
How is cystic hygroma diagnosed?
Although some patients have visible lesions to make the diagnosis on examination, others do not. The soft tissue lesions can be visualized with MRI scan, CT scan, ultrasound, and occasionally even with X-rays. Other studies such as airway fluoroscopy and lymphoscintigraphy (lymph node mapping by radiographic imaging) have been used. Also, endoscopic biopsy has been used for diagnosis.
Ultrasound has been used to diagnose cystic hygromas in fetuses as early as the first trimester.
What is the treatment for cystic hygroma?
Some clinicians prefer to “watch and wait” in children who have a cystic hygroma and no symptoms. However, if symptoms develop, there are two main treatments -- medical, using chemicals to scar or obliterate the cysts and surgical excision of the cystic tissue. Some authors consider surgical removal as the preferred treatment. Other techniques, such as radiofrequency ablation and laser induced thermotherapy, have also been used to treat the lesions. Parents should discuss treatment options that would be best for their child with their child's treatment team, usually composed of the child's pediatrician and a surgical specialist and/or otolaryngologist.
What is the prognosis with cystic hygroma?
The prognosis of cystic hygromas is variable and can range from good to poor. For example, if most but not all cystic hygroma tissue is removed, there is a 15% chance of recurrence.
Prognosis decreases when the cystic hygroma is in fetuses and in those with excessive neck thickening. Also, if the fetus also has genetic abnormalities the prognosis is poorer.
Acevedo, J. L., et al. "Cystic Hygroma." Medscape. 11 Mar 2015.
Daily Health News
Subscribe to MedicineNet's Children's Health & Parenting Newsletter
Acevedo, J. L., et al. "Cystic Hygroma." Medscape. 11 Mar 2015.
Top Cystic Hygroma Related Articles
Birth DefectsBirth defects have many causes and currently, are the leading cause of death for infants in the first year of life. Some of the causes of birth defects include genetic or chromosome problems. Exposure of the mother to rubella or German measles during pregnancy, or using drugs or alcohol during pregnancy. The treatment for birth defects depends upon the condition of the effected child.
Breastfeeding: Common Breastfeeding ChallengesBreastfeeding an infant can cause common challenges both for the mother an infant. Some challenges include sore nipples, low milk supply, oversupply of milk, engorgement, plugged ducts, breast infection, fungal infections, nursing strike, inverted, flat, or very large nipples, breastfeeding a baby with health problems, and breastfeeding in special situations. Tips and helpful information can inform mothers how to manage and handles these challenges while continuing to breastfeed her baby.
CAT ScanA CT scan is an X-ray procedure that combines many X-ray images with the aid of a computer to generate cross-sectional and three-dimensional images of internal organs and structures of the body. A CT scan is a low-risk procedure. Contrast material may be injected into a vein or the spinal fluid to enhance the scan.
Down Syndrome OverviewGet the facts on Down syndrome, a genetic disorder caused by an additional set of chromosomes in a developing fetus. Down syndrome signs and symptoms include:
- distinctive facial features,
- growth retardation, and
- decreased mental function and IQ.
Klinefelter SyndromeKlinefelter syndrome, also referred to as XXY condition, is a genetic disease. Klinefelter syndrome affects mostly males. Symptoms include decreased testosterone levels, development of breasts, wider hips, and infertility. Some men with Klinefelter syndrome have no symptoms. Treatment includes educational, therapeutic, and medical therapy.
MRI ScanMRI (or magnetic resonance imaging) scan is a radiology technique which uses magnetism, radio waves, and a computer to produce images of body structures. MRI scanning is painless and does not involve X-ray radiation. Patients with heart pacemakers, metal implants, or metal chips or clips in or around the eyes cannot be scanned with MRI because of the effect of the magnet.
Signs and symptoms of pregnancy vary by stage (trimester). The earliest pregnancy symptom is typically a missed period, but others include
breast swelling and tenderness,
- nausea and sometimes vomiting,
- fatigue, and
Second trimester symptoms include
- weight gain,
- itching, and
- possible stretch marks.
Third trimester symptoms are
- additional weight gain,
- swelling of the ankles,
- fingers, and face,
- breast tenderness, and
- trouble sleeping.
Eating a healthy diet, getting a moderate amount of exercise, also are recommended for a healthy pregnancy. Information about the week by week growth of your baby in the womb are provided.
Pregnancy Planning (Preparing for Pregnancy)
Pregnancy planning is an important step in preparation for starting or expanding a family. Planning for a pregnancy includes:
- Taking prenatal vitamins
- Eating healthy for you and your baby
- Disease prevention (for both parents and baby) to prevent birth defects and infections
- Avoiding certain medications that may be harmful to your baby
- How much weight gain is healthy
- Exercise safety and pregnancy
- Travel during pregnancy
Pregnancy: Prenatal Care and TestsPrenatal care is important for the health of the mother and the baby. Common prenatal tests include ultrasound, amniocentesis, screening for group B strep, and chronic villus sampling. Prenatal care also helps detect health conditions related to pregnancy, such as preeclampsia and gestational diabetes. Prenatal care costs can be expensive, but services are available to help women get the care they need.
Prenatal UltrasoundA prenatal ultrasound test uses high-frequency sound waves, inaudible to the human ear, that are transmitted through the abdomen via a device called a transducer to look at the inside of the abdomen. With prenatal ultrasound, the echoes are recorded and transformed into video or photographic images of your baby. An ultrasound examination is also known as a sonogram.
Sleep ApneaSleep apnea is defined as a reduction or cessation of breathing during sleep. The three types of sleep apnea are central apnea, obstructive apnea (OSA), and a mixture of central and obstructive apnea. Central sleep apnea is caused by a failure of the brain to activate the muscles of breathing during sleep. OSA is caused by the collapse of the airway during sleep. OSA is diagnosed and evaluated through patient history, physical examination and polysomnography. There are many complications related to obstructive sleep apnea. Treatments are surgical and non-surgical.
Stages of PregnancySee pictures on the various stages of pregnancy. See and learn what changes a woman's body goes through and view fetal images of how her baby grows during the 1st, 2nd and 3rd trimesters.
Third Trimester Tests During PregnancyTesting is often recommended during the third trimester of pregnancy. These tests are designed to ensure the health and safety of both the child and mother. Common tests during the third trimester of a woman's pregnancy include:
- group B streptococcus screening,
- electronic fetal heart monitoring,
- nonstress test,
- contraction stress test, and
- a biophysical profile.
Turner SyndromeTurner syndrome is an inherited chromosomal condition affecting women. Women with Turner syndrome do not have ovarian function, and features of the syndrome include webbed neck, lymphedema of the hands and feet, heart defects, kidney problems, and skeletal abnormalities. The X chromosome is related to Turner syndrome. Treatment focuses on the symptoms of the syndrome.
UltrasoundUltrasound (and ultrasonography) is imaging of the body used in the medical diagnosis and screening of diseases and conditions such as:
- heart valve irregularities,
- carotid artery disease,
- heart disease,
- kidney stones,
- liver disease,
- diseases of the female reproductive, and
- diseases of the male reproductive organs.