Fetal Surgery for Neck Masses

Updated: Sep 24, 2013
  • Author: S Christopher Derderian, MD; Chief Editor: Hanmin Lee, MD  more...
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Fetal cervical masses are a challenging congenital malformation to manage, as they can grow rapidly, resulting in hydrops fetalis, polyhydramnios, and fetal demise. Among affected fetuses who survive to delivery, asphyxia and death commonly result from airway compromise.

Fetal cervical masses are diagnosed with prenatal ultrasonography, and, when identified, a follow-up fetal MRI for further characterization is warranted.

Management has evolved over the past two decades, and, with the introduction of the ex-utero intrapartum treatment (EXIT) procedure, survival past the neonatal period has significantly improved.

Fetal cervical lesions can result from various congenital malformations, with cervical lymphangiomas and cervical teratomas as the two most common. The incidence of cervical cystic hygromas, a lymphatic malformation, is fairly high, affecting 1 in 1,000 births. [1] In fact, it has been observed in 1 in 300 spontaneous abortions. [2] Cervical teratomas, the second most common fetal neck mass, are far less common (see image below), with an unknown incidence. Only 150 cases have been reported in the literature.

Cervical Teratoma in an infant delivered via EXIT Cervical Teratoma in an infant delivered via EXIT procedure

Other rare neck masses include thyroid malignancies and cysts, brachial cleft cysts, vascular malformations, and neuroblastomas and are beyond the scope of this article.

Esophageal and tracheal obstruction are common complications, leading to polyhydramnios, hydrops fetalis, and in utero fetal demise (IUFD). Perinatal complications include asphyxia and death.{{Ref}{{Ref} The diagnosis of a fetal cervical lesions is not to be taken lightly, as it carries an estimated IUFD rate of 20%. [5]



Once a fetal cervical mass is identified with prenatal ultrasonography, fetal MRI should be performed to help characterize the mass and, if necessary, to assist with preoperative planning. Fetal MRI is valuable, as it aids in delineating solid and cystic components, as well as identifying the presence of fat. In addition, the tracheal anatomy can be scrutinized with MRI.

Difficulty arises in defining the severity of the lesion and determining the need for an EXIT procedure. Only recently has a classification been described to define the severity of cervical masses. The tracheoesophageal displacement index (TEDI), which was described by the Texas Children’s Hospital group, is based on a sum of the ventral and lateral displacement of the esophagus and the trachea from the cervical spine. In their series of 24 patients, 100% of patients with a TEDI score of more than 12 mm had a complicated airway, compared with 46% of those with a TEDI score of less than 12 mm. [6]

As this grading system has only recently been published, its use has not been universally accepted. In general, fetuses with lymphatic malformations do not have a compromised airway and do not require EXIT deliveries, likely because of the airway’s ability to displace the fluid-containing lesion.

Indications for fetal surgery are few, as the authors’ institution is the only to report a successful in utero resection. [7] An EXIT procedure, on the other hand, should be offered to viable fetuses with complicated airways, as it provides an opportunity to manage the airway while the fetus remains on uteroplacental support. If an EXIT procedure is not performed, a pediatric surgeon should be a present at the time of delivery in the event that a surgical airway is required.



Contraindications to fetal surgery can be maternal or fetal in origin. Maternal contraindications include frequent contractions, membrane rupture, short cervix, or uncontrolled comorbidities that may predispose the patient to preeclampsia or HELLP syndrome. Fetal contraindications include multiple fetal anomalies or chromosomal abnormalities.


Technical Considerations

Complication Prevention

Appropriate anesthesia and tocolytics are imperative to prevent uterine contractions and placental separation.

Uterine atony can lead to diffuse bleeding. Therefore, it is essential to minimize blood loss, to obtain a preoperative type and cross for 4 units of packed red blood cells, and to monitor hematocrits, if necessary.

A special fetal uterine stapler can reduce the risk of hemorrhage.



Outcomes vary. Early neonatal death after a secured airway during an EXIT procedure may result from pulmonary hypoplasia or prematurity. [8] Outcomes depend on the location, size, and underlying etiology of the cervical mass.

Cervical teratomas frequently displace rather than invade surrounding structures, allowing for feasible resection.

The malignant potential depends on the pathological findings, and screening for recurrence with alpha-fetoprotein levels should be routinely performed.

Lymphatic and vascular malformations have a much more protracted course and often require multiple operations.

Historically, developmental delay resulting from hypoxia in the perinatal period was common, but, with the advent of the EXIT procedure, obvious delays appear to be dramatically reduced. [9]