Description, Causes and Risk Factors:
In our body we have different types of blood vessels. Most people are familiar with arteries and veins but there is another system of vessels called lymphatics. These drain not blood but a clear fluid called lymph from around the body. The lymphatic vessels join together to drain into the large veins in the neck.
Cystic hygromas are fluid-filled sacs that result from a blockage in the lymphatic system. The lymphatic system is a network of vessels that maintains fluids in the blood, as well as transports fats and immune system cells. Cystic hygromas are single or multiple cysts found mostly in the neck region. A cystic hygroma can be present as a birth defect (congenital) or develop at any time during a person's life. A cystic hygroma in a developing baby can progress to hydrops (an excess amount of fluid in the body) and eventually fetal death. Some cases of congenital cystic hygromas resolve leading to webbed neck, edema (swelling), and a lymphangioma (a benign yellowish-tan tumor on the skin composed of swollen lymph vessels). In other instances the hygroma can progress in size to become larger than the fetus.
Cystic hygromas can occur as an isolated finding or in association with other birth defect as part of a syndrome. They result from environmental factors, genetic factors, or unknown factors.
Environmental causes for cystic hygroma include:
Maternal substance abuse, such as abuse of alcohol.
Maternal viral infections, such as Parvovirus of Fifth's disease.
Genetic syndromes with cystic hygroma as a clinical feature:
Chromosome abnormalities such as trisomies 13, 18, and 21.
The majority of prenatally diagnosed cystic hygromas are associated with Turner syndrome, a chromosome abnormality in which a female has only one X chromosome instead of two.
The pattern of inheritance for these syndromes varies depending upon the specific syndrome. Isolated cystic hygroma can be inherited as an autosomal recessive disorder for which parents are “silent” carriers. Finally, a cystic hygroma can occur from an unknown cause.
In general, symptoms vary from one mere presence of apainless, enlarging mass to respiratory compromise,dysphagia and difficulty in feeding with regurgitation. Very rarely, massive hygroma may present withsymptoms of neural encroachment (brachial plexuses andrecurrent laryngeal nerve). Cystic hygroma vary from1.0 to 30.0 cm in size, the mean size in Stromberg'sseries was 8.0 cm.
The differential diagnosis may include encephalocele, meningomyelocele, and teratoma. Teratomas are uncommon and are generally solid. Encephaloceles have associated calvarial abnormalities and contain brain tissue. Meningomyelocelesare uncommon in the cervical region and have associated spine abnormalities.
An ultrasound diagnosis of cystic hygroma should be followed by a careful search forother anomalies and by fetal karyotyping. Serial follow up scans are essential to assessthe development of fetal hydrops. Genetic counseling is necessary to discuss thediagnosis, the possible need of karyotyping, and the potential risks to the fetus or child.
Other Tests May Include:
Cystic hygromas rarely go away on their own. Aspiration by puncturing with a needle is usually followed by prompt reaccumulation of fluid or development of infection. Injection of chemical agents to cause shrinkage is effective in certain hygromas where there are large cysts, but is not as effective when there are very small cysts. This is not without potential complications, and as yet there is no universal agent available that is totally safe.
A baby with a prenatally diagnosed cystic hygroma should be delivered in a major medical center equipped to deal with neonatal complications. An obstetrician usually decides the method of delivery. If the cystic hygroma is large, a cesarean section may be performed. After birth, infants with persistent cystic hygroma must be monitored for airway obstruction. A thin needle may be used to reduce the volume of the cystic hygroma to prevent facial deformities and airway obstruction. Close observation of the baby by a neonatologist after birth is recommended. If resolution of the cystic hygroma does not occur before birth, a pediatric surgeon should be consulted. Cystic hygromas that develop in the third trimester (after thirty weeks gestation) or in the postnatal period are usually not associated with chromosome abnormalities. There is a chance of recurrence after surgical removal of the cystic hygroma. The chance of recurrence depends on the extent of the cystic hygroma and whether the wall of the cyst was able to be completely removed.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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