This leaflet is to help you understand what an Lymphangioma is, what tests you need, and the implication of being diagnosed for you and your baby.
What is lymphangioma?
Lymphangiomas are benign malformations of the lymphatic system, composed of thin-walled, cystically dilated vascular channels.
How does a lymphangioma occur?
It is one of the developmental abnormalities of the lymphatic system which occurs at the site of the lymphatics and veins connect.
How can I know if my baby has lymphangiomas?
During the routine ultrasound, your doctor may find a multicystic mass, usually located near the neck but can be found in other places such as the armpit, abdomen, or lower limbs.
Should I have more tests done?
Once lymphangioma is suspected, your doctor will perform a series of ultrasonographic evaluations to rule out other tumors such as teratomas, hemangiomas, cervical meningoceles, thyroglossal duct cysts, esophageal diverticula, dermoid cysts, brachial cleft cysts, epignathus, and congenital goiter.
If it is an isolated lymphangioma, it is not necessary to carry out other genetic tests. If it is related to some other structural abnormality, your doctor will offer to carry out an invasive procedure for genetic analysis.
What are the signs and symptoms of alarm that I can present?
One of the major complications of lymphangioma is that it can, in some cases, occlude or interfere with swallowing of the fetus, therefore, amniotic fluid builds up and may cause polyhydramnios (too much amniotic fluid) which may increase the risk of preterm birth or difficult breathing in the newborn.
What are the things to watch for during pregnancy?
Regular follow-up with ultrasound is important to monitor the progress of the mass. Generally, this will occur about every two weeks, however, the period of time for follow-up depends on the evolution of the lymphangioma.
During the evaluations, your doctor will inform you about decisions related to the pregnancy, including expectant management or acceleration of labor, as well as to select an appropriate method of and place for delivery.
What does it mean for my baby after it is born?
The prognosis is related to the penetration or invasion of neighboring tissues, and whether there is a compromise of airways or other vital structures. Upper airway obstruction is life-threatening with subsequent perinatal mortality and morbidity, often associated with a delay or inability to obtain an airway at birth. Therefore, a delivery strategy known as EXIT has been designed to maintain the fetus on its placenta for a while after birth in order to give time to secure the baby’s airway. More recently, a fetal strategy known as Fetal Endoscopic Tracheal Intubation (FETI) has been used with success to improve this process. Your doctor will discuss with you the indications for these interventions and whether they are applicable to your baby.
The spontaneous resolution rate of lymphangioma has been reported as 8.9% during the gestational period and 17.7% in the postnatal period, before 2 years of age. Other therapies such as injection of the mass with special agents known as sclerosing agents have been used with success to treat this type of mass. Patients requiring sclerosing therapy have presented a rate of good to excellent results in 91.5%. However, the most common complications are recurrence, inflammation, persistent pain, and infection.
Will it happen again?
Recurrence in the patient who has been treated with surgical or sclerosing therapy may occur. Recurrence in subsequent pregnancies has not been reported in isolated lymphangioma.
What other questions should I ask?
- How often will I have ultrasound examinations done?
- What is the appropriate therapy in my case?
- Where should I deliver?
- Where will the baby receive the best care after it is born?
Last updated September 2022