Cervical Teratoma

What is a cervical teratoma?

A cervical teratoma is a very rare congenital tumor in the neck. These tumors tend to be large, disfiguring masses — partly solid and partly fluid. They make it impossible for a newborn to breathe upon birth. Advances in medical technology, early detection and careful monitoring during pregnancy, now make it possible for these babies to be saved.

How is cervical teratoma diagnosed?

It’s usually first discovered during a routine prenatal ultrasound, sometime around the 18th week of pregnancy. Signs of a problem in the ultrasound include:

  • an empty stomach indicating a blockage in the esophagus and/or excess amniotic fluid
  • the fetus's neck may appear hyperextended
  • your doctor may even discover the mass itself

If your obstetrician suspects a problem, you will be referred to an obstetrician who specializes in high-risk cases or a pediatric surgeon specializing in fetal and neonatal care for further ultrasounds and possibly magnetic resonance imaging (MRI).

How is a cervical teratoma treated?

The initial stages of treatment for a cervical teratoma involve careful monitoring of the mother and fetus along with the development of a surgical plan. Your treatment will likely be handled by a multidisciplinary team that includes the following medical professionals:

  • Perinatologist
  • General pediatric surgeon
  • Radiologist
  • Ear, nose and throat surgeon
  • Nurse practitioner

Prenatal care

Repeated ultrasounds will be used to monitor amniotic fluid volume, tumor size and the overall well-being of the fetus. The multidisciplinary team will be paying close attention to the tumor, which may not grow, but can grow rapidly, becoming large and bulky sometimes extending out of the fetus's mouth, displacing the fetus's ear and disfiguring the jaw. The team will be monitoring the lungs for hyperinflation, a sign that the airway is completely blocked by the tumor.

They will also be closely watching the heart, looking for signs of impending heart failure, which is a complication that can occur if the heart is overextending itself to supply blood to the tumor.

Surgery

In most cases, surgeons will perform an EXIT procedure as mentioned above. During an EXIT procedure, the fetus is only partially exposed during surgery, so that the volume of the uterus is maintained. Your doctors may use inhalation agents to ensure relaxation of your uterus.

Securing the airway

At this stage, the main goal for surgeons is to secure the airway by inserting a breathing tube into it, bypassing the obstruction. Your team will use some combination of the following procedures to facilitate this:

  • Laryngoscopy— Examination of the interior of the larynx (voice box) using an instrument called a laryngoscope.
  • Bronchoscopy— Examination of the bronchi (the two primary divisions of the trachea that lead to the lungs) using an illuminated tube.
  • Tracheostomy— The surgical formation of an opening into the trachea through the neck to allow the passage of air. This is done if surgeons cannot establish an airway with a breathing tube.

Birth

Once an airway is established, the umbilical cord is cut and your doctors will surgically remove the tumor while your Cesarean section is completed. This can be a long process since doctors must be extremely careful to avoid affecting any of the surrounding structures in the neck, including the following:

  • Nerves
  • Larynx
  • Trachea
  • Esophagus

Subsequent surgeries may be needed either for complete removal of the tumor or for reconstruction of the trachea or other neck structures that were distorted by the tumor.

What happens after the surgery?

Infants who undergo successful surgery have a high survival rate and grow up to live healthy, normal lives with perhaps one complication. These babies may develop transient or permanent hypothyroidism, both of which can be treated with medication.

These conditions develop when the cervical teratoma either completely or partially replaces the thyroid gland. In this case, a pediatric endocrinologist should be consulted.

After surgery, doctors will want to monitor your baby routinely to ensure that the cervical teratoma doesn't recur. Most cervical teratomas are benign; however there is the possibility that a recurring tumor could be malignant.