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Lymphangioma (Cystic Hygroma)

What is a cervical lymphangioma/cystic hygroma?

A cervical lymphangioma is a malformation of the lymphatic (tissue fluid) system that occurs in the neck region. This benign fluid-filled cystic mass results in displacement of normal tissue. The term cystic hygroma refers to a lymphangioma located in the neck region. A small portion of these lesions present as a giant neck mass. Lymphangiomas may be macrocystic (consisting of larger, interconnected cysts), microcystic (consisting of multiple small cysts resembling a sponge) or mixed lesions.

It occurs approximately in 1 in every 6,000 live births. A cystic hygroma located in the anterior portion of the neck is usually not associated with a chromosomal abnormality.

What additional testing is recommended during my pregnancy?

Serial ultrasounds are used to follow the baby's growth, monitor the size of the mass, and detect other defects. Amniocentesis may be recommended. A fetal MRI is recommended to differentiate between a cystic hygroma and cervical teratoma and to assess the airway.

Are there special considerations during the delivery?

Airway management at birth is critical with giant neck masses. It is recommended that the delivery be planned at a hospital prepared for high-risk deliveries as well as pediatric and fetal surgical subspecialties.

Giant neck masses may compress the esophagus which causes polyhydramnios and may precipitate preterm labor. Giant cervical lymphangiomas are evaluated by ultrasound and fetal MRI to assess the concern for airway compromise at birth. If there is concern about establishing an airway, an EXIT Procedure (ex uterine intrapartum) is recommended. If an endotracheal tube cannot be inserted, a fetal tracheostomy is performed. Occasionally, partial resection of the mass on placental support is required to place a tracheostomy.

Is there fetal treatment for giant cervical lymphangioma?

The EXIT Procedure (ex utero intrapartum treatment) procedure was designed to provide a period of placental support while securing a tenuous airway. This is a type of modified C-section delivery in which the mother is under general anesthesia which promotes uterine relaxation and permits the placenta to continue supplying oxygenated blood to the baby while the airway is being secured. The need for an EXIT Procedure is evaluated on an individual basis.

What is the treatment for a cervical lymphangioma?

Lymphangiomas are treated with surgery or sclerotherapy. Macrocystic lesions may be treated with sclerotherapy whereas microcystic lesions usually require surgical excision. Since lymphangiomas are benign lesions, complete removal is not warranted if vital structures are involved. The risk of recurrence is low after therapy.

Are there other health problems that are more common among babies with lymphangioma?

Babies with an isolated cystic hygroma do very well. Effective surgical resection or sclerotherapy is possible in the majority of cases. The location of the mass and the potential need for additional surgeries places several of the cranial nerves at risk for injury. If the lymphangioma involves the tongue, floor of the mouth or airway structures, complete resection is unlikely.

Those babies with lymphangioma secondary to a chromosomal abnormality will have additional health issues dependent on the underlying diagnosis.

Can this happen again with another pregnancy?

An anterior lymphangioma is often an isolated event. A doctor and a genetic counselor will review the risk in your family.