epignathus

An unusual
cause of inspiratory stridor in the newborn: congenital pharyngeal teratoma – a case report. Intraoperative view of the pharynx prior to laser resection. When examining the surgical site, a protruding pediculated mass covered by mucous membranes (indicated by arrow) is clearly visible below the uvula. The endotracheal tube is still in position at that time, but cannot be readily seen due to a space-occupying effect of the tumor
bmcpediatr.biomedcentral.com • CC-by-4.0
An unusual
cause of inspiratory stridor in the newborn: congenital pharyngeal teratoma – a case report. Magnetic resonance imaging of the head and neck region. Sagittal magnetic resonance image (T1-TSE) after contrast showing a pediculated 6.6 x 17.4 x 10 mm muscle-isointense lesion with a moderate uptake of contrast agent at the level of the second and third cervical vertebrae
bmcpediatr.biomedcentral.com • CC-by-4.0
Radiopaedia • CC-by-nc-sa 3.0 • de

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

Epidemiology

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

Clinical presentation

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

Pathology

It is often classified under the broader umbrella of cervical teratomas. The tumors 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 tumor 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
  • raised maternal serum alpha feto protein (MSAFP)

Radiographic features

Antenatal ultrasound

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

MRI

Useful for better delineation of anatomy.

Treatment and prognosis

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 tumor may also demonstrate rapid growth. An EXIT procedure can be offered in selected cases .

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

Differential diagnosis

In general consider other tumors 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)

See also

Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu Teratom des Pharynx:
Teratom
 
Wikipedia • CC BY-SA 3.0
Rhabdomyosarkom
 
pediatricimaging.org • CC-by-nc-sa 4.0
Teratom der
Nase
pediatricimaging.org • CC-by-nc-sa 4.0
kongenitales
Teratom des Pharynx
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