Epignathus

Epignathus is a term given to a very rare form of teratoid tumour that arises from the oropharyngeal region.

There may be a slight female predilection ref. The estimated incidence is ~ 1 in 35,000 to 200,000 births.

The tumour classically presents in utero or in the neonatal period. In extreme rare occasions, it may present in young children 9.

It is often classified under the broader umbrella of cervical teratomas. The tumours are not often consistent in origin, number, and differentiation of tissues. They usually have components from all three germ layers and can contain fat, cartilage and bone. 

Location

The tumour typically arises from the palato-pharyngeal region around the basisphenoid (Rathke's pouch) and with progressive growth fills the buccal cavity and finally protrudes out of the mouth. 

Associations
Markers
Antenatal ultrasound

Usually seen as a complex mass protruding from the fetal oral cavity (often with mixed echogenecity 11). Accompanying polyhydramnios may be present when there is swallowing impairment.

MRI

Useful for better delineation of anatomy.

  • development of fetal hydrops: often due to tumour shunting/using a lot of blood flow 
  • the tumour can spread superiorly destroying the skull base and brain tissue 12
  • neonatal death usually occurs due from airway obstruction and consequent asphyxia after birth

The prognosis is generally poor (often as a result of its location) and part of the high mortality rate is caused by severe airway obstruction in the neonatal period. The tumour may also demonstrate rapid growth. An EXIT procedure can be offered in selected cases 6.

In general consider other tumours from the oropharyngeal region:

For small lesions on a sagittal ultrasound scan also consider:

  • macroglossia
  • prominent philtrum/premaxillary tissue  from a midline facial defect (can also be an association)
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Article Information

rID: 13577
Section: Pathology
Tag: cases
Synonyms or Alternate Spellings:
  • Nasopharyngeal teratoma
  • Facial teratoma
  • Oropharyngeal teratoma

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