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Cervical teratomas are rare congenital neck masses that are most commonly benign but can compromise the fetal and neonatal airway. Most cervical teratomas are noted on prenatal ultrasound and require extensive perinatal planning and a multidisciplinary team for airway management at delivery. Once the airway is secured and the infant stabilized, complete surgical excision is the standard of care.
Congenital cervical teratomas are rare tumors that are increasingly found on prenatal ultrasound. The incidence of teratomas of the head and neck ranges from 1:20,000–1:40,000 births and some sources cite a female predominance.
Plastic reconstruction of fetal anatomy using three-dimensional ultrasound and magnetic resonance imaging scan data in a giant cervical teratoma. Case report.
The term “teratoma” is derived from the Greek word “teraton,” meaning “a monster” which speaks to the immense size and disfiguring effects of some tumors. Most teratomas occur as an isolated lesion, but sometimes are part of a syndrome, such as Klinefelter syndrome, trisomy 13, trisomy 21, or Beckwith-Wiedemann syndrome.
Teratomas form around the fourth to fifth week of gestation. Although the cause is not completely understood, the most commonly accepted theory suggests teratomas evolve from ectopic pluripotent cells that fail to migrate.
It is generally accepted that most cervical teratomas attach to the thyroid gland with some tumors infiltrating or completely replacing the thyroid gland.
The tumor can displace or infiltrate structures throughout the anterior neck, but most tumor is usually well encapsulated.
Histology
Teratomas are comprised of the following 3 embryonic germ layers: the ectoderm, mesoderm, and endoderm. The ectoderm produces skin, teeth, nails, ependymal, choroid plexus, neuroglia, and neurons. Mesoderm produces adipose tissue, bone, cartilage, and muscle. The endoderm creates the respiratory and intestinal epithelium, exocrine glands, and solid organs. Teratomas are classified as mature or immature depending on the degree of cell differentiation. Mature teratomas are the most common form of teratoma and contain skin, hair, fat, cartilage, bone, and glands, whereas immature teratomas contain neuroepithelial tissue and immature mesenchyme.
Mature or immature teratoma can sometimes be found in a nearby regional lymph node, but this tissue is generally considered a “deposit” and not true metastatic disease, as this involvement in cervical lymphadenopathy does not alter the prognosis.
Large teratomas can compress and deform developing fetal structures, such as the mandible and neck musculature, causing torticollis and nerve dysfunction.
When not found prenatally, cervical teratomas present as a neck mass that is usually firm, mobile, multilobular, and cystic with distinct margins. Patients often suffer from a degree of respiratory distress due to either compression of the airway or pulmonary hypoplasia.
Invasion or obstruction of the hypopharynx in utero can prevent fetal swallowing and lead to microgastria, which is associated with poor feeding tolerance, gastroesophageal reflux, and failure to thrive.
If the cervical teratoma is noted on a prenatal ultrasound, often a fetal magnetic resonance imaging (MRI) is performed to gather more details about the airway, characteristics of the mass, and surrounding anatomy (such as proximity to the great vessels). This information can help to establish a plan for delivery. If the cervical teratoma is not discovered before delivery, a postnatal computed tomography or MRI can be performed for radiologic evaluation of the mass and surgical planning. An MRI is preferred because it does not require ionizing radiation and provides better soft tissue detail (Figure 1, Figure 2, Figure 3). There is no radiologic characteristic that defines a teratoma. However, most cervical teratomas contain adipose tissue, and calcifications within a heterogeneous mass, which are suggestive of the diagnosis.
The usage of alpha fetoprotein (AFP) at the time of diagnosis is debatable, as the AFP level at birth or initial presentation can vary widely and is influenced by birth weight and age. Therefore, premature infants tend to have higher levels of AFP. The AFP level for an infant decreases after birth and generally is difficult to interpret. However, a teratoma should be suspected if the AFP level continues to stay elevated or increases.
For those patients who present with a cervical teratoma after birth, a detailed physical examination should be performed. It is important to assess the location, integrity of the overlying skin, presence of pulsations, and a thorough examination of the cranial nerves. One should take a detailed history and ask the patient’s family about other known pathologies or syndromes. A bedside flexible laryngoscopy should be performed to assess the nasopharynx, oropharynx, and larynx, as well as vocal cord function. It is also important to inquire about dysphagia and symptoms of aspiration.
Surgical resection is the standard of care for cervical teratomas. However, the top priority in a neonate with a cervical teratoma is airway management. For cases discovered prenatally with concerns for airway obstruction, a multidisciplinary team should be involved and should consist of an anesthesiologist, obstetrician, neonatologist, radiologist, and surgical team.
Stabilizing the airway and infant is the primary goal until surgical resection can occur. One group recommends intubation of all patients until surgical resection, even in the absence of respiratory distress, as hemorrhage within cystic components of the tumor can cause rapid enlargement, acute loss of airway and decompensation.
This should be considered on an individual basis and may not be necessary for small cervical teratomas. It is important to remember that acute needle decompression of large cystic components of the tumor can help to obtain an airway in urgent situations.
Patients with cervical teratomas often require an ex utero intrapartum procedure (EXIT) to secure the airway at delivery. Prenatal MRI imaging can assist with evaluating the extent of airway obstruction and determining the need for an EXIT procedure. The EXIT procedure relies on uterine relaxation to preserve the maternal-fetal circulation; this is facilitated by inhalational anesthetics for the mother.
This is opposite of the normal goal of uterine contraction after a cesarean delivery to minimize maternal bleeding; therefore, an EXIT procedure places the mother at increased risk of bleeding postoperatively. Uterotonic medications, such as oxytocin, can be administered after clamping of the umbilical cord to decrease bleeding. With successful EXIT procedures, the maternal-fetal circulation can be maintained for a reported 150 minutes.
In preparation for an EXIT procedure, a careful plan of initial as well as subsequent interventions to obtain an airway must be planned. Oftentimes, direct laryngoscopy and rigid bronchoscopy, followed by fiberoptic bronchoscopy with use of laryngeal mask airway for ventilation, retrograde intubation, and tracheotomy, are considered in a sequential manner until an airway is secured. Tumor resection is rarely performed during the EXIT procedure but can be performed to access the trachea for tracheostomy placement. The EXIT procedure allows the surgical team to secure an airway while maintaining the maternal-fetal circulation, avoiding hypoxia and brain injury at the time of delivery.
Early intervention is recommended to decrease the risk of infection, sepsis, ulcerations or skin breakdown over the tumor, coagulopathies, and hemodynamic instability.
Extensive counseling with the patient’s family should take place preoperatively, covering the chance for recurrence, infection, resection of surrounding structures (such as the thyroid), poor cosmesis, scarring, hypothyroidism, and a mortality rate estimated at 15% by some sources.
Preoperative antibiotics should be given before skin incision and continued while a surgical drain is in place. The patient should be placed in a supine position under general anesthesia with clear communication to anesthesia that no muscle relaxants are to be administered to allow for nerve monitoring. A shoulder roll should be placed for neck extension and the head rotated to expose the mass.
Electrophysiologic monitoring should be used for large tumors that may abut and displace the marginal mandibular nerve. Intraoperative nerve testing should be performed to confirm identification of the spinal accessory nerve and hypoglossal nerve if the dissection is in this vicinity, as these structures may be displaced by the tumor. The greater auricular nerve should be identified over the sternocleidomastoid (SCM) muscle and preserved during dissection.
With a short neck and no cervical wrinkles, the infant does not offer a natural skin crease for an incision. The incision should be placed over the mass and along the lines of a future natural skin crease if the tumor is not severely distorting the neck (Figure 4). If the tumor is large, an incision can be made over the mass and excess skin can be removed after resection is complete. If the tumor has caused a great deal of distortion, a plastic surgeon may be consulted to optimize cosmesis.
Figure 4The planned incision for cervical teratoma resection should be along a natural neck crease.
The skin, subcutaneous tissue, and platysma muscle are incised, and flaps are elevated superiorly and inferiorly if possible (Figure 5). The platysma may be thin and difficult to visualize because of the tumor. Teratomas are usually well-defined masses, and often careful, blunt dissection can be used to resect most tumor (Figure 6). However, they can infiltrate surrounding tissues requiring resection of local structures. One group recommends starting the dissection laterally and working medially to identify vital structures, as they are often severely distorted.
The tumor is then separated from the cervical fascia by sharp and blunt dissection. Finding landmark structures of the neck, such as the SCM muscle and great vessels, is crucial. The spinal accessory nerve may be located and preserved once the SCM is identified. Sharp or blunt dissection is used to take down fascial attachments and adhesions from the posterior and medial aspects of the tumor.
Figure 5Myocutaneous flaps are raised superiorly and inferiorly to assist exposure.
As mentioned previously, cervical teratomas can be intimately involved with, invade, or completely replace the thyroid, so a thyroid lobectomy or thyroidectomy may be necessary. The recurrent laryngeal nerve should be identified in the standard fashion, as done with a thyroidectomy.
Once the teratoma is completely resected, a closed suction drain is placed before closure and the platysma is closed with inverted interrupted 4–0 vicryl sutures (Figure 7). The skin is closed using a 5–0 monocryl in a running subcuticular fashion and reinforced with steri strips. A pressure dressing should be considered to help the drain hold suction and to prevent a seroma or hematoma. Any pressure dressing should be closely monitored to avoid skin breakdown.
Figure 7After resection of the teratoma, a closed suction drain is placed and then a layered closure is performed with reapproximation of the strap muscles. Next, the platysma is closed with inverted interrupted 4–0 vicryl sutures. The skin is closed using a 5–0 monocryl in a running subcuticular fashion and reinforced with steri strips.
Teratomas are usually well-defined masses. However, they can infiltrate surrounding tissues requiring further dissection and resection. One group recommends preserving normal structures and argues that residual disease is likely harmless and can be monitored closely with radiologic studies and future surgery, if needed.
Patients should be monitored closely in the pediatric intensive care unit postoperatively. Some patients will require continued airway support due to swelling, malacia, or injury to recurrent laryngeal nerves. If the teratoma was intimately involved with the thyroid, then thyroid and calcium levels should be monitored and an endocrine consult considered. There are reported cases of permanent hypothyroidism and temporary hypocalcemia.
Drains should be left in place until the output is less than 15 mL over a 24-hour time period and antibiotics should be continued until the drain is removed.
Long-term follow-up is crucial for these patients to monitor for recurrent disease. The AFP level is monitored and should decrease to adult levels by 1 year of age. Consistently high or increasing levels may indicate a recurrence or persistence. There is no current agreement on the proper frequency of AFP monitoring or length of follow-up; however, some recommend starting with drawing AFP levels every 3 months postoperatively.
Along with AFP, patients are monitored for recurrence with imaging. MRI is the modality of choice due to lack of ionizing radiation exposure.
Complications
The complications after resection of a cervical teratoma are related to the extent and location of the mass. Wound complications can occur due to infection, hematoma, or seroma. Transient hypothyroidism and permanent hypothyroidism have been reported and should be monitored.
Permanent or temporary nerve damage can involve the marginal mandibular branch of the facial nerve, spinal accessory nerve, recurrent laryngeal nerve, vagal nerve, or hypoglossal nerve.
Patients who survive the perinatal period generally do well, but can have difficulties related to cranial nerve dysfunction, most commonly with dysphagia and speech issues, as well as poor cosmesis.
A minority of patients will require a tracheostomy long-term. Overall, the prognosis for patients with a cervical teratoma is excellent, with most having normal function and cognition.
Poor cosmesis can result from scarring, difficult closure, or structural deformities from a mass effect during development. Rarely, a gastrostomy tube is needed for nutrition due to persistent dysphagia or aspiration.
Although most cervical teratomas are benign, they do carry a risk of perinatal mortality through airway or cardiovascular compromise. Proper imaging and surgical planning are crucial. Surgical resection is feasible in most cases with an excellent prognosis and minimal morbidity.
Disclosure
The authors reported no proprietary or commercial interest in any product mentioned or concept discussed in this article.
Plastic reconstruction of fetal anatomy using three-dimensional ultrasound and magnetic resonance imaging scan data in a giant cervical teratoma. Case report.
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