What is a fetal giant neck mass?

Fetal giant neck masses such as cervical teratoma and lymphangioma can grow to such large proportions that the fetal airway becomes distorted and obstructed. In a small number of patients with cervical teratomas, the condition can result in pulmonary hypoplasia, or underdeveloped lungs.

In addition to obstructing the airway, these fetal giant neck masses can compress the esophagus, resulting in polyhydramnios, which can lead to pregnancy complications and preterm labor. Unsuspected obstructive fetal giant neck masses often prove fatal because of an inability to secure an airway and ventilate the baby upon delivery. If the delay is longer than five minutes, brain injury may occur. This illustrates the importance of prenatal diagnosis and delivery planning, since most children with a fetal giant neck mass have an isolated anomaly and do well after postnatal resection.

Giant neck masses are typically detected by ultrasound. When patients are referred to the Center for Fetal Diagnosis and Treatment (CFDT) with a diagnosis of a fetal neck mass, an in-depth evaluation is performed by our specialized multidisciplinary team to confirm the diagnosis.

Monitoring and delivery

If the neck mass is small and does not compromise the airway, close ultrasound surveillance can follow the growth of the mass throughout the duration of your pregnancy.

For larger masses resulting in polyhydramnios, treatment options during pregnancy include bed rest, medication to decrease amniotic fluid, or regular procedures to remove excess fluid from the womb (amniocentesis).

If the polyhydramnios persists and the mass continues to grow, your baby may be delivered via the EXIT procedure. An EXIT procedure is a surgical procedure that is used to deliver babies who have airway compression due to cervical teratomas, cystic hygromas, or blockage of the airway such as congenital high airway obstruction (CHAOS) syndrome. It is similar to a caesarean section, but there are some important differences.

Treatment for giant neck masses

Giant neck masses are typically surgically removed after birth. The EXIT procedure allows time to perform procedures commonly used to treat babies born with giant neck masses, such as direct laryngoscopy, bronchoscopy, tracheostomy, surfactant administration, cyst decompression and tumor resection. Some or all of these procedures may be required to secure the airway and provide adequate ventilation.

Some babies need temporary help with breathing and eating. The compression of the windpipe can cause it to become soft, making it prone to collapse. For this reason, a temporary tracheostomy is sometimes necessary to allow your baby to breathe normally until the trachea hardens.

In addition, some babies may need to be partially or totally fed using a tube that goes directly into the stomach.

Central to achieving an excellent outcome for babies prenatally diagnosed with a giant neck mass is the coordination of an experienced team of pediatric surgeons, fetal and maternal anesthesiologists, obstetricians, neonatologists and obstetrical, neonatal and operating room nurses.