Prevalence:

• 1 in 200,000 births.

Ultrasound diagnosis:

• 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.

Associated abnormalities:

• The incidence of chromosomal abnormalities and genetic syndromes is not increased.

Investigations:

• Detailed ultrasound examination, including neurosonography.

Follow-up:

• 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.

Delivery:

• Place: hospital with neonatal intensive care and pediatric surgery.
• Time: 38 weeks.
• Method: cesarean section with EXIT procedure.

Prognosis:

• 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.

Recurrence:

• No increased risk of recurrence.