Massive fetal cervical teratoma managed with the ex utero intrapartum treatment (EXIT) procedure

Abstract

Teratomas are rare congenital tumors typically comprising all 3 germ cell layers. Cervical teratomas arise in the neck and represent a minority of all teratomas. These are associated with high morbidity and mortality because of their propensity to cause airway obstruction. Demonstration on prenatal magnetic resonance imaging is uncommon, especially for a tumor of this size. Fetuses diagnosed with large neck masses are managed through cesarean section with the ex utero intrapartum treatment procedure to secure the airway, such as in our case of a large cervical teratoma in the female fetus of a 30-year-old mother who went into preterm labor.

Ex utero intrapartum treatment (EXIT) procedure: Indications and procedural considerations

“…There were 12 cases where tumours were resected, all in separate procedures in the days or weeks following the EXIT procedure. The case described by Olivares et al 20 did not have a resection, possibly due to the infant requiring high levels of ventilator support in NICU.…”

“…In 13 cases with neck tumour, teratoma was diagnosed -12 of these were cervical and one mediastinal. Cervical teratomas account for approximately three per cent of all teratomas 3 with Olivares et al 20 stating this number could be closer to nine per cent. Cervical teratomas often contain thyroid tissue or may replace part of the gland which has led to speculation that they arise from thyroid tissue 9 .…”

“…They most commonly occur in the sacrococcygeal region 11 , with the second most common area being mediastinal 19 and less than ten per cent occurring in the neck 11 . They contain tissue from the three primordial embryonic layers -mesoderm, ectoderm and endoderm 11,20 -are highly vascular and grow quickly due to cystic components 9 . Hochwald et al 11 explained that teratomas are histologically benign but have a high morbidity and mortality rate due to their size and location 20 .…”

“…They contain tissue from the three primordial embryonic layers -mesoderm, ectoderm and endoderm 11,20 -are highly vascular and grow quickly due to cystic components 9 . Hochwald et al 11 explained that teratomas are histologically benign but have a high morbidity and mortality rate due to their size and location 20 . Teratomas may compress the airway or pull the trachea and lungs superiorly against the thoracic inlet causing pulmonary hypoplasia 3 .…”

“…Two cases had the airway secured via tracheostomy. Olivares et al 20 explain this may be necessary when the airway is difficult to access, due to the mass obstructing the upper airway to a degree that oral intubation is not possible 9 . Brodsky et al 9 add that an inability to secure a tracheostomy due to the size or location of the mass rarely occurs; however, if it does, partial resection of the tumour may be necessary.…”