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Epignathus
Paula J. Woodward, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Nasopharyngeal teratoma
                • Oropharyngeal teratoma
              • Definitions

                • Teratoma arising in oral/nasal cavity or pharynx

              IMAGING

              • General Features

                • Ultrasonographic Findings

                  • MR Findings

                    • CT Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Morlino S et al: Oropharyngeal teratoma, oral duplication, cervical diplomyelia and anencephaly in a 22-week fetus: A review of the craniofacial teratoma syndrome. Birth Defects Res A Clin Mol Teratol. 103(6):554-66, 2015
                                              2. Walz PC et al: Prenatal diagnosis of obstructive head and neck masses and perinatal airway management: the ex utero intrapartum treatment procedure. Otolaryngol Clin North Am. 48(1):191-207, 2015
                                              3. Deloison B et al: Atypical case of prenatal cystic epignathus teratoma. Ultrasound Obstet Gynecol. 44(4):495-6, 2014
                                              4. Santana EF et al: Prenatal diagnosis of a giant epignathus teratoma in the third trimester of pregnancy using three-dimensional ultrasound and magnetic resonance imaging. Case report. Med Ultrason. 16(2):168-71, 2014
                                              5. Al-Mahdi AH et al: Giant epignathus teratoma involving the palate, tongue, and floor of the mouth. J Craniofac Surg. 24(1):e97-9, 2013
                                              6. Laje P et al: Perinatal management of congenital oropharyngeal tumors: the ex utero intrapartum treatment (EXIT) approach. J Pediatr Surg. 48(10):2005-10, 2013
                                              7. Chung JH et al: Fetal epignathus: the case of an early EXIT (ex utero intrapartum treatment). Obstet Gynecol. 119(2 Pt 2):466-70, 2012
                                              8. Allen LM: Prenatal 3-dimensional imaging techniques in the sonographic evaluation of an oral mass: comparison with postnatal imaging modalities. J Ultrasound Med. 30(4):561-8, 2011
                                              9. Roby BB et al: Complete peripartum airway management of a large epignathus teratoma: EXIT to resection. Int J Pediatr Otorhinolaryngol. 75(5):716-9, 2011
                                              10. Tonni G et al: Cervical and oral teratoma in the fetus: a systematic review of etiology, pathology, diagnosis, treatment and prognosis. Arch Gynecol Obstet. 10;282(4):355-61,2010
                                              11. Dar P et al: First-trimester diagnosis of fetal epignathus with 2- and 3-dimensional sonography. J Ultrasound Med. 28(12):1743-6, 2009
                                              12. Maartens IA et al: Neonatal airway obstruction caused by rapidly growing nasopharyngeal teratoma. Acta Paediatr. 98(11):1852-4, 2009
                                              13. Tsitouridis I et al: Sonographic evaluation of epignathus. Hippokratia. 13(1):55-7, 2009
                                              14. Freitas Rda S et al: Epignathus: two cases. Br J Oral Maxillofac Surg. 46(4):317-9, 2008
                                              15. Kumar B et al: Neonatal oral tumors: congenital epulis and epignathus. J Pediatr Surg. 43(9):e9-11, 2008
                                              16. Fotopoulou C et al: Prenatal sonographic diagnosis of an oropharyngeal teratoma (epignathus) on a stillborn infant: a case report. Z Geburtshilfe Neonatol. 211(4):165-8, 2007
                                              17. Johnston JM et al: Giant intracranial teratoma with epignathus in a neonate. Case report and review of the literature. J Neurosurg. 106(3 Suppl):232-6, 2007
                                              18. Sherer DM et al: Prenatal 3-dimensional sonographic diagnosis of a massive fetal epignathus occluding the oral orifice and both nostrils at 35 weeks' gestation. J Ultrasound Med. 25(11):1503-5, 2006
                                              19. Woodward PJ et al: From the archives of the AFIP: a comprehensive review of fetal tumors with pathologic correlation. Radiographics. 25(1):215-42, 2005
                                              20. Hirose S et al: The ex utero intrapartum treatment procedure: Looking back at the EXIT. J Pediatr Surg. 39(3):375-80; discussion 375-80, 2004
                                              21. Morof D et al: Oropharyngeal teratoma: prenatal diagnosis and assessment using sonography, MRI, and CT with management by ex utero intrapartum treatment procedure. AJR Am J Roentgenol. 183(2):493-6, 2004
                                              22. Papageorgiou C et al: Prenatal diagnosis of epignathus in the first half of pregnancy: a case report and review of the literature. Clin Exp Obstet Gynecol. 27(1):67-8, 2000
                                              23. Gull I et al: Antenatal sonographic diagnosis of epignathus at 15 weeks of pregnancy. Ultrasound Obstet Gynecol. 13(4):271-3, 1999
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Top Differential Diagnoses

                                                    • Pathology

                                                      • Clinical Issues

                                                        • Diagnostic Checklist

                                                          TERMINOLOGY

                                                          • Synonyms

                                                            • Nasopharyngeal teratoma
                                                            • Oropharyngeal teratoma
                                                          • Definitions

                                                            • Teratoma arising in oral/nasal cavity or pharynx

                                                          IMAGING

                                                          • General Features

                                                            • Ultrasonographic Findings

                                                              • MR Findings

                                                                • CT Findings

                                                                  • Imaging Recommendations

                                                                    DIFFERENTIAL DIAGNOSIS

                                                                      PATHOLOGY

                                                                      • General Features

                                                                        • Staging, Grading, & Classification

                                                                          • Gross Pathologic & Surgical Features

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Morlino S et al: Oropharyngeal teratoma, oral duplication, cervical diplomyelia and anencephaly in a 22-week fetus: A review of the craniofacial teratoma syndrome. Birth Defects Res A Clin Mol Teratol. 103(6):554-66, 2015
                                                                                          2. Walz PC et al: Prenatal diagnosis of obstructive head and neck masses and perinatal airway management: the ex utero intrapartum treatment procedure. Otolaryngol Clin North Am. 48(1):191-207, 2015
                                                                                          3. Deloison B et al: Atypical case of prenatal cystic epignathus teratoma. Ultrasound Obstet Gynecol. 44(4):495-6, 2014
                                                                                          4. Santana EF et al: Prenatal diagnosis of a giant epignathus teratoma in the third trimester of pregnancy using three-dimensional ultrasound and magnetic resonance imaging. Case report. Med Ultrason. 16(2):168-71, 2014
                                                                                          5. Al-Mahdi AH et al: Giant epignathus teratoma involving the palate, tongue, and floor of the mouth. J Craniofac Surg. 24(1):e97-9, 2013
                                                                                          6. Laje P et al: Perinatal management of congenital oropharyngeal tumors: the ex utero intrapartum treatment (EXIT) approach. J Pediatr Surg. 48(10):2005-10, 2013
                                                                                          7. Chung JH et al: Fetal epignathus: the case of an early EXIT (ex utero intrapartum treatment). Obstet Gynecol. 119(2 Pt 2):466-70, 2012
                                                                                          8. Allen LM: Prenatal 3-dimensional imaging techniques in the sonographic evaluation of an oral mass: comparison with postnatal imaging modalities. J Ultrasound Med. 30(4):561-8, 2011
                                                                                          9. Roby BB et al: Complete peripartum airway management of a large epignathus teratoma: EXIT to resection. Int J Pediatr Otorhinolaryngol. 75(5):716-9, 2011
                                                                                          10. Tonni G et al: Cervical and oral teratoma in the fetus: a systematic review of etiology, pathology, diagnosis, treatment and prognosis. Arch Gynecol Obstet. 10;282(4):355-61,2010
                                                                                          11. Dar P et al: First-trimester diagnosis of fetal epignathus with 2- and 3-dimensional sonography. J Ultrasound Med. 28(12):1743-6, 2009
                                                                                          12. Maartens IA et al: Neonatal airway obstruction caused by rapidly growing nasopharyngeal teratoma. Acta Paediatr. 98(11):1852-4, 2009
                                                                                          13. Tsitouridis I et al: Sonographic evaluation of epignathus. Hippokratia. 13(1):55-7, 2009
                                                                                          14. Freitas Rda S et al: Epignathus: two cases. Br J Oral Maxillofac Surg. 46(4):317-9, 2008
                                                                                          15. Kumar B et al: Neonatal oral tumors: congenital epulis and epignathus. J Pediatr Surg. 43(9):e9-11, 2008
                                                                                          16. Fotopoulou C et al: Prenatal sonographic diagnosis of an oropharyngeal teratoma (epignathus) on a stillborn infant: a case report. Z Geburtshilfe Neonatol. 211(4):165-8, 2007
                                                                                          17. Johnston JM et al: Giant intracranial teratoma with epignathus in a neonate. Case report and review of the literature. J Neurosurg. 106(3 Suppl):232-6, 2007
                                                                                          18. Sherer DM et al: Prenatal 3-dimensional sonographic diagnosis of a massive fetal epignathus occluding the oral orifice and both nostrils at 35 weeks' gestation. J Ultrasound Med. 25(11):1503-5, 2006
                                                                                          19. Woodward PJ et al: From the archives of the AFIP: a comprehensive review of fetal tumors with pathologic correlation. Radiographics. 25(1):215-42, 2005
                                                                                          20. Hirose S et al: The ex utero intrapartum treatment procedure: Looking back at the EXIT. J Pediatr Surg. 39(3):375-80; discussion 375-80, 2004
                                                                                          21. Morof D et al: Oropharyngeal teratoma: prenatal diagnosis and assessment using sonography, MRI, and CT with management by ex utero intrapartum treatment procedure. AJR Am J Roentgenol. 183(2):493-6, 2004
                                                                                          22. Papageorgiou C et al: Prenatal diagnosis of epignathus in the first half of pregnancy: a case report and review of the literature. Clin Exp Obstet Gynecol. 27(1):67-8, 2000
                                                                                          23. Gull I et al: Antenatal sonographic diagnosis of epignathus at 15 weeks of pregnancy. Ultrasound Obstet Gynecol. 13(4):271-3, 1999