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What is a cervical teratoma?
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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, that encircle essential structures, such as the esophagus, thyroid, and trachea, making it impossible for a newborn to breath upon birth.
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Advances in medical technology, early detection and careful monitoring during pregnancy, now make it possible for these babies to be saved, usually by a procedure that enables surgeons to create an airway for the baby as it's being delivered and is still attached to and sustained by the mother's placenta.
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What causes a cervical teratoma?
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The cause of a cervical teratoma is unknown. It has never been linked to maternal lifestyle, so it is likely that there is nothing an expecting mother can do to prevent it.
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An abnormality leading to a diagnosis is usually first discovered during a routine prenatal ultrasound, sometime around the 18th week of pregnancy. The first sign of a problem in the fetus could be an empty stomach indicating a blockage in the esophagus and/or polyhydramnios (excess amniotic fluid), or even discovery of the mass itself. The fetus's neck may also appear hyperextended.
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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 ultrasound and possibly Magnetic Resonance Imaging (MRI.)
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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. You're treatment will likely be handled by a multidisciplinary team of medical professionals including a perinatologist, general pediatric surgeon, radiologist, and ears, nose and throat surgeon and a nurse practitioner.
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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.
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The medical 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 (hydrops) which is a complication that can occur if the heart is overextending itself to supply blood to the tumor. For more information, see Hydrops Fetalis.
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In most cases involving cervical teratoma, surgery is done as the baby is delivered via Cesarean section and still attached to mother's placenta. This method, known as an EXIT (ex utero intrapartum treatment) procedure, gives surgeons time to perform multiple procedures to secure the baby's airway while the blood flow and exchange of gases that normally occurs in the womb between the fetus and the placenta is preserved.
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During an EXIT procedure, the fetus is only partially exposed during surgery, so that the volume of the uterus is maintained. Doctors may use inhalation agents to ensure relaxation of the mother's uterus. As with all prenatal surgeries there are risks to the mother, including excessive bleeding and infection. Be sure to discuss these risks fully with your doctor.
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At this stage, the main goal for surgeons is to secure the airway by inserting a breathing tube (endotracheal tube) into it, bypassing the obstruction. Some combination of the following procedures will be used to facilitate this:
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- 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 a tubular illuminated instrument (bronchoscope)
- 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.
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Once an airway is established, the umbilical cord is cut and while the mother's Cesarean section is completed and she is moved into recovery, doctors will surgically remove the tumor. This can be a long process since doctors must be extremely careful to avoid affecting any of the surrounding structures in the neck, including nerves, the larynx (voice box), trachea (windpipe) and 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.
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What happens after surgery?
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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.
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After surgery, doctors will want to monitor the 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.
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