- 1 in 200,000 births.
- Solid tumor arising from the sphenoid bone, hard and soft palate, the pharynx, the tongue and jaw.
- The tumor grows into the oral or nasal cavity or intracranially.
- Differential diagnosis includes neck teratomas, encephaloceles, and other tumors of the facial structures.
- Polyhydramnios (due to pharyngeal compression) is usually present.
- The incidence of chromosomal abnormalities and genetic syndromes is not increased.
- Detailed ultrasound examination, including neurosonography.
- Follow-up scans every 4 weeks to monitor growth of the tumor and assess amniotic fluid volume. If polyhydramnios develops, monitor cervical length every 1 week to determine the need for amniodrainage.
- Fetal MRI at 32 weeks to assess the relation to adjacent structures.
- Place: hospital with neonatal intensive care and pediatric surgery.
- Time: 38 weeks.
- Method: cesarean section with EXIT procedure.
- Depends on the size of the lesion and the involvement of vital structures.
- Polyhydramnios has been associated with poor prognosis. The major cause of neonatal death is asphyxia due to airway obstruction.
- Surgical resection with a normal postoperative course is possible.
- No increased risk of recurrence.