Epignathus

Epignathus is a rare oropharyngeal teratoma.[1] This lesion is usually diagnosed antenatally with ultrasound or after birth.[2]

Epignathus

Pathology

The tumour arises from the palato-pharyngeal region around the basisphenoid.[3][4] Over time it grows and protrudes out of the mouth. This lesion may be associated with polyhydramnios by preventing the fetus from swallowing the liquor. It may also be associated with a cleft lip and/or palate. The tumour may spread into the cranial cavity and destroy the brain.[5]

Epidemiology

This lesion occurs in 1:35,000 to 200,000 pregnancies.[6] It is more common in females (male: female ratio 1:3) Up to 2004 70 cases had been described in the literature.

Diagnosis

This may be made antenatally with ultrasound. If the baby survives to birth the diagnosis is immediately apparent.

Other clinical features include dyspnoea, cyanosis, cough, difficulty in sucking and swallowing due to the presence of the tumour.[7]

Treatment

The priority is to establish a usable airway and then to feed the baby.[8][9] This is frequently not possible.

Prognosis

This is very poor with a mortality of 80-100% in the neonatal period.[10] Very few long term survivors have been reported so the prognosis past the neonatal period is unclear.[11]

References
  1. Kumar SY, Shrikrishna U, Shetty J, and Sitaram A (2011) Epignathus with fetiform features. J Lab Physicians 3(1): 56–58
  2. Sarioglu N, Wegner RD, Gasiorek-Wiens A, Entezami M, Schmock J, Hagen A, Becker R (2003) Epignathus: always a simple teratoma? Report of an exceptional case with two additional fetiforme bodies. Ultrasound Obstet Gynecol 21(4):397-403
  3. Todd DW, Votava HJ, Telander RL, Shoemaker CT (1991) Giant epignathus. A case report.Minn Med 74(7):27-28
  4. Moon NR, Min JY, Kim YH, Choi SK, Shin JC, Park IY (2015) Prenatal diagnosis of epignathus with multiple malformations in one fetus of a twin pregnancy using three-dimensional ultrasonography and magnetic resonance imaging. Obstet Gynecol Sci 58(1):65-68
  5. Kirishima M, Yamada S, Shinya M, Onishi S, Goto Y, Kitazono I, Hiraki T, Higashi M, Hida AI, Tanimoto A (2018) An autopsy case of epignathus (immature teratoma of the soft palate) with intracranial extension but without brain invasion: case report and literature review.Diagn Pathol 13(1):99
  6. Jadhav SS, Korday CS, Malik S, Shah VK, Lad SK (2017) Epignathus leading to fatal airway obstruction in a neonate. J Clin Diagn Res 11(1):SD04-SD05
  7. Moon NR, Min JY, Kim YH, Choi SK, Shin JC, Park IY (2015) Prenatal diagnosis of epignathus with multiple malformations in one fetus of a twin pregnancy using three-dimensional ultrasonography and magnetic resonance imaging. Obstet Gynecol Sci 58(1):65-68
  8. Chiu HH, Hsu WC, Shih JC, Tsao PN, Hsieh WS, Chou HC (2008)The EXIT (ex utero intrapartum treatment) procedure. J Formos Med Assoc 107(9):745-748
  9. Dakpé S, Demeer B, Cordonnier C, Devauchelle B (2014) Emergency management of a congenital teratoma of the oral cavity at birth and three-year follow-up. Int J Oral Maxillofac Surg 43(4):433-436
  10. Tsitouridis I, Sidiropoulos D, Michaelides M (2009) Sonographic evaluation of epignathus. Hippokratia 13(1):55-57
  11. Carvalho CHP, Nonaka CFW, Elias CTV, Matheus RCS, Dias RMB, Souza LB, Pinto LP (2017) Giant epignathus teratoma discovered at birth: A case report and 7-year follow-up.Braz Dent J 28(2):256-261
Classification
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