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A cervical teratoma is a rare tumor that develops around the neck of a fetus and may vary in consistency and size. These growths are usually benign, though can be cancerous in some cases.
The main concern with a cervical teratoma is obstruction of the baby's airway and esophagus, resulting in the accumulation of fluid that can affect growth and even result in preterm delivery.
At the Colorado Fetal Care Center, we work with families who've recently found out their baby has a cervical teratoma. From your first visit with our fetal experts to the day you deliver your baby, we'll use our advanced diagnostics and state-of-the-art facility to provide the best treatment in the region.
A cervical teratoma is a large tumor that develops in the neck of a baby. These masses are an extremely rare form of a germ cell tumor and they are usually benign (non-cancerous).
There are no known genetic connections to the formation of cervical teratomas. While they're the second most common form of fetal neck mass, there are only 150 cases reported.
Cervical teratomas can complicate a baby's development by blocking or narrowing his or her airway. The tumor can also compress the esophagus, making it difficult or impossible for your baby to swallow amniotic fluid, which can lead to polyhydramnios, or excess fluid in the amniotic sac. This, in turn, may lead to preterm delivery.
Cervical teratomas are equally dangerous after birth, since the obstruction of the airway may prevent the baby from breathing or delay the ability to secure the airway at birth. As a result, it's important to develop a delivery treatment plan soon after receiving a cervical teratoma diagnosis.
A fetal cervical teratoma is almost always discovered through a routine ultrasound. These masses are large, asymmetrical and have clearly demarcated lines. A diagnosis is usually obtained through additional ultrasounds or a fetal MRI. An MRI is helpful in defining the degree of airway distortion, which can help your fetal care team create a treatment plan during your pregnancy as well as immediately after birth.
While a cervical teratoma diagnosis is certainly stressful for you and your family, several treatment options have proven successful. Our fetal and pediatric surgery team has made great strides in the advancement of care and treatment for this condition.
The Colorado Fetal Care Center is one of the leading care centers for babies diagnosed with a cervical teratoma, and we’ve treated more than 35 patients with these masses. Depending on the size and severity of the mass, our team will develop a treatment plan that may include observation, in-utero procedures, EXIT procedures and postnatal treatments.
Observation is one of the treatment options for small cervical teratomas with no risk of airway obstruction or high output cardiac failure. These cases are usually followed during pregnancy with ultrasounds. Once the baby is born, definitive anatomical detail is obtained by CT scans and consultations with our multidisciplinary team of experts.
Aspiration of cysts, or removal of fluid using a needle, is another option available for certain cervical teratomas with a dominant large cyst at risk of obstruction or rupture. Aspiration of the cyst contents will relieve the pressure temporarily, then ultrasounds will be used until delivery to monitor the cyst.
The EXIT procedure is a special delivery technique where the head and neck of a baby are exposed through a limited incision in the uterus while maintaining pregnancy. Then, a breathing tube is placed, the cord is clamped and the baby is delivered. This technique allows the airway to be secured so the baby can transition normally from the womb, and provides an opportunity to perform resection of the mass. In some cases, a staged approach may be used to reduce the size of a mass until more imaging can be provided to plan for definitive removal.
On ultrasound examination, cervical teratomas are typically asymmetric, unilateral, mobile and well-demarcated. Most are multiloculated, irregular masses with solid and cystic components. As many as 50% have calcifications present (Gundry et al., 1983; Kelly et al., 1990). Calcifications may be difficult to appreciate on ultrasound examination and are more easily seen on plain radiographs (Goodwin et al., 1965; Hajdu et al., 1966; Suita et al., 1982). Calcifications, when present in a partially cystic and solid neck mass, are virtually diagnostic of cervical teratoma (Gundry et al., 1983).
Cervical teratomas are usually large and bulky, typically measuring 5 to 12 cm in diameter (Crombleholme and Albanese, 2001; Liechty and Crombleholme, 1999; Liechty et al., 1997; Batsakis, 1964; Silberman and Mendelson, 1960). Tumor masses greater than the size of the fetal head have also been reported (Batsakis et al., 1964; Jordan et al., 1988; Owor and Master, 1974). These tumors usually extend to the mastoid process and body of the mandible, superiorly displacing the ear. Inferiorly they can extend to the clavicle and suprasternal notch or extend into the mediastinum. Posteriorly, they can extend to the anterior border of the trapezius. Involvement of the oral floor, protrusion into the oral cavity (epignathus) and extension into the superior mediastinum have also been noted in cervical teratomas (Jordan et al., 1988).
Polyhydramnios will complicate 20 to 40% of the prenatally diagnosed cases and is more commonly observed in large tumors (Bale, 1949; Hajdu et al., 1966; Lloyd and Clatworthy, 1958; Trecet et al., 1984). Polyhydramnios is thought to be due to esophageal obstruction, as has been demonstrated by contrast amniography (Mochizuki et al., 1986; Rosenfeld et al., 1979). An empty stomach may be the first sonographic clue to esophageal obstruction from cervical teratoma (Rosenfeld et al., 1979; Suita et al., 1982). Other anomalies have been reported in association with cervical teratomas, including one case each of chondrodystrophia fetalis and imperforate anus (McGoon, 1952). Hypoplastic left ventricle and trisomy 13 have also been reported in association with cervical teratoma (Dische and Gardner, 1987; Gundry et al., 1983), as has agenesis of the corpus callosum (Goldstein et al., 2005). Mandibular hypoplasia may also be seen as a direct result of mass effect on the developing mandible (Liechty et al., 1997).
The fetus with a large cervical teratoma often has marked extension of the neck due to mass effect. The cervical teratomas being deep to the strap muscles of the neck cause severe compression of the larynx, trachea and esophagus. As noted above, esophageal compression results in polyhydramnios and compression of the larynx and trachea can result, not only in deviation and distortion of the airway, but also marked laryngotracheomalacia. The hyperextension of the fetal neck may also result in profound pulmonary hypoplasia. The hyperextended neck pulls the fetal trachea cephalad and, in severe cases, the carina can be found above the level of the thoracic inlet. This pulls the lungs into the cupola of the thoracic cavities preventing normal lung growth. Several cases of death due to profound pulmonary hypoplasia have been reported despite successful EXIT procedures to secure the airway (Crombleholme and Albanese, 2001; Liechty and Crombleholme, 1999).
The antenatal natural history of fetal cervical teratomas is not well defined. Although they are most often malignant in adults, the vast majority of cervical teratomas in fetuses and infants are benign (Mochizuki et al., 1986). However, rare cases of malignancies in this age group have been described with estimates of fewer than 10% of cases being malignant (Azizkhan et al., 1995; Baumann et al., 1993; Touran et al., 1989; Thurkow et al., 1983; Schoenfeld et al., 1982; Heys et al., 1967;). The true malignant potential of cervical teratoma is uncertain (Batsakis et al., 1964; Cunningham et al., 1987; Gundry et al., 1983; Owor and Master, 1974; Pupovac, 1986; Watanatittan et al., 1981). Despite the existence of primitive tissue types in the tumor and metastases to regional lymph nodes, many infants have remained free from recurrence following complete resection of a cervical teratoma. These cases suggest that malignant biologic behavior is uncommon in this population (Batsakis et al., 1964; Dunn et al., 1992; Gundry et al., 1983). Immature tissue seen on histologic examination may merely represent the immaturity of the host; thus, pathologic studies are not completely reliable in predicting prognosis (Batsakis et al., 1964; Tapper and Lack, 1983). This is an unusual tumor which may have “benign” metastases to regional lymph nodes detected after resection of the cervical teratoma (Oka et al., 2007; Azizkhan et al., 1995; Rothschild, 1994).
A fetus with a cervical teratoma is at increased risk for intrauterine fetal demise and stillbirth. It has been estimated that as many as 17% die in utero and 35% die prior to surgery (Berge et al., 2004, Berry, 1997; Mochizuki et al., 1986). Because of esophageal compression, the majority of fetuses with cervical teratoma will have polyhydramnios. The notable exceptions are small lesions and those that extend to one side without severe compression of the esophagus. The presence of a large cervical mass may result in compression of cranial nerves with resulting loss of function, most notably the mandibular branch of the facial nerve with attendant drop of the corner of the mouth, hypoglossal nerve with deviation of the tongue to the contralateral side with protrusion and the recurrent laryngeal nerve with paresis or paralysis of the ipsilateral vocal cord. The large cervical teratomas may compress or displace the mandible causing hypoplasia or flaring the angle of the mandible out from the face laterally causing marked facial asymmetry.
In some cases, cervical teratomas may result in non-immune hydrops due to highly vascular nature of some of these lesions. In this rare circumstance, fetal demise would be anticipated without fetal surgical resection.
The ex utero partum treatment, or EXIT procedure, was specifically designed to provide time to secure an airway while preserving uteroplacental gas exchange. While Langer and Schwartz and their colleagues had each reported a case using this approach, the technique was not fully developed until it was applied in diaphragmatic hernia (Langer et al., 1992; Mychalishka et al., 1997; Schwartz et al., 1993). When tracheal-clip application was applied in human fetuses with diaphragmatic hernia, it was necessary to develop a technique to allow time for neck dissection, clip removal, bronchoscopy and intubation. Several individual case reports have described “operating on placental support” for fetal airway management, but none presented a systematic approach and correlated cord blood gases with time or placental support (Catalano et al., 1992; Langer et al., 1992; Schwartz et al., 1993; Skarsgard et al., 1996; Tanaka et al., 1994).
Recently Crombleholme et al. (1997) applied the lessons learned from use of the EXIT procedure in diaphragmatic hernia to the management of giant fetal neck masses in a small series of five patients, three with cervical teratomas and two with lymphangioma (Crombleholme et al., 1997; Liechty et al., 1997). The mean duration of the EXIT procedure was 28 minutes, with a range of 8 to 54 minutes. Direct laryngoscopy was performed in all five cases. Although orotracheal intubation was possible in two of the five, in one of the two, intubation was possible only after drainage of 1800 ml of cyst fluid. In one case, massive involvement of the chest, neck and face by the lymphangioma precluded orotracheal intubation and, in accordance with the family’s wishes, a surgical airway was not attempted. In the remaining two patients who had teratomas, there was severe distortion of the airway, rendering intubation by direct laryngoscopy impossible. In each of these cases, bronchoscopy and tracheostomy were required to secure the airway. In both cases, the mass effect of the tumor had pulled the thoracic trachea into the neck. In the first case, this resulted in a tracheostomy 1.5 cm above the carina and, in the second case, the tracheostomy was performed between the 8th and 10th tracheal rings, as this was the only available site due to mass effect of the tumor. This infant subsequently underwent resection of the mass closure of this distal tracheostomy and creation of a new tracheostomy between the 2nd and 3rd tracheal rings.
Unlike a conventional cesarean section, the EXIT procedure maintains uteroplacental blood flow and fetal gas exchange by keeping the uterus relaxed through the use of inhalational agents and the maintenance of uterine volume by only partially exposing the fetus (Marwan and Crombleholme, 2006; Bouchard et al., 2002). This is apparent in the relatively normal venous cord blood gases seen after up to 54 minutes of uteroplacental support (Liechty et al., 1997). By preserving uteroplacental blood flow, the EXIT procedure allows time to perform multiple procedures such as direct laryngoscopy, bronchoscopy, tracheostomy, surfactant administration and cyst decompression, some or all of which may be required to secure the airway.
There are a number of potential risks to the mother who undergoes an EXIT procedure. Because inhalational agents keep the uterus very relaxed, there is a risk of increased hemorrhage because of uterine atony. The risk of uterine atony and hemorrhage can be minimized through coordination between the surgeon and the anesthesiologist to decrease the concentration of inhalational anesthetic and administer oxytocin at the time of umbilical-cord ligation. The use of the uterine stapling device and coordination with the anesthesiologist kept the average intraoperative blood loss at 950 ml, well within the accepted range for traditional cesarean section (Hood and Holubec, 1990).
Lower-uterine-segment hysterotomy is preferred for the EXIT procedure, as it allows the possibility of future vaginal delivery. However, a low anterior placenta or extremely large neck mass may make lower uterine section impossible. In such cases, a classical hysterotomy is necessary. This approach necessitates cesarean delivery for all future deliveries because of the risk of uterine rupture during labor. Prior to an EXIT procedure, mothers should be counseled about the possibility that future pregnancies would require cesarean delivery. In rare cases, hydrops resulting from a cervical teratoma in a fetus less than 30 weeks’ gestation may necessitate open fetal surgery to resect the teratoma.
A fetal cervical teratoma can profoundly affect the course of pregnancy. Repeated ultrasound examinations are indicated to monitor amniotic fluid volume, tumor size, and fetal well-being. In cases followed by serial sonography, rapid tumor growth has been noted (Baumann et al., 1993; Hitchcock et al., 1987). There is a high incidence of preterm labor and preterm delivery, thought to be secondary to the increase in uterine size due to polyhydramnios and/or tumor. Because of the hyperextension of the neck, the so-called flying fetus sign and the often large tumor size, there is an increased incidence of malpresentation and dystocia (Gonzalez-Crussi, 1982; Owor and Master, 1974). Cesarean section is often recommended because of the abnormal fetal position (Chervenak et al., 1985; Kagan et al., 1983; Levine et al., 1990; Rempen et al., 1985). Stabilization of the newborn’s airway at delivery is facilitated by the assembly of a team qualified to obtain a bronchoscopic or surgical airway if orotracheal intubation is unsuccessful (Zerella et al., 1990). Currently, a fetus with cervical teratoma is best managed by the EXIT (ex utero intrapartum treatment) procedure, which provides time for laryngoscopy, bronchoscopy, tracheostomy or tumor resection, if necessary, to secure the airway (Marwan, 2006; Crombleholme and Albanese, 2001; Liechty et al., 1997).
Airway obstruction and respiratory compromise at birth can be life-threatening and accounts for up to 45% of the mortality seen with this abnormality. Consequently, delivery should occur at a tertiary-care center with significant experience with the EXIT procedure. There have been anecdotal reports of intrapartum laryngoscopy or bronchoscopy in cases of fetal neck masses in which the fetus is delivered but the cord is not clamped (Kelly et al., 1990). Unfortunately, this procedure offers no advantage over standard cesarean section, as the removal of the fetus from the womb results in uterine contraction and cessation of uteroplacental gas exchange (Marwan and Crombleholme, 2006; Bouchard et al., 2002; Crombleholme and Albanese, 2001; Liechty et al., 1997; McNamara and Johnson, 1995).
Airway obstruction at birth is life-threatening and associated with a high mortality rate. In giant fetal neck masses this mortality is usually associated with a delay in obtaining an airway and an inability to ventilate the infant effectively. This delay can result in hypoxia and acidosis and, if the delay is greater than 5 minutes, anoxic injury may occur (Dawes, 1968). This complication is all the more tragic as most of these children have an isolated benign tumor and do well after postnatal resection.
Mortality can be as high as 80% to 100% in untreated infants, regardless of the tumor size (Garmel and Crombleholme, 1994; Batsakis et al., 1964; Goodwin et al., 1965; Gundry et al., 1983; Hurlbut et al., 1967; Silberman and Mendelson, 1960). Delaying surgery can result in retention of secretions, atelectasis and/or pneumonia due to interference with swallowing (Batsakis et al., 1964; Gonzalez-Crussi, 1982). In addition, precipitous airway obstruction may occur due to hemorrhage into the tumor (Batsakis et al., 1964; Gundry et al., 1983; Hurlbut et al., 1967; Silberman and Mendelson, 1960) even in minimally symptomatic newborns. For this reason, orotracheal intubation is indicated in all patients regardless of the presence or absence of symptoms. Mortality decreases to between 9 and 17% in infants treated surgically (Batsakis et al., 1964; Goodwin et al., 1965; Gundry et al., 1983; Hajdu et al., 1966; Hurlbut et al., 1967; Silberman and Mendelson, 1960).
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Cardiology - Pediatric, Pediatrics
Cardiology - Pediatric, Pediatrics
Surgery - Pediatric, Surgery