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Epignathus
Paula J. Woodward, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Synonyms

              • Nasopharyngeal teratoma
              • Oropharyngeal teratoma
            • Definitions

              • Teratoma arising from naso- or oropharynx

            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. Kirishima M et al: 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, 2018
                                            2. Butler CR et al: Ex utero intrapartum treatment (EXIT) for upper airway obstruction. Curr Opin Otolaryngol Head Neck Surg. 25(2):119-26, 2017
                                            3. 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
                                            4. 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
                                            5. Deloison B et al: Atypical case of prenatal cystic epignathus teratoma. Ultrasound Obstet Gynecol. 44(4):495-6, 2014
                                            6. 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
                                            7. 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
                                            8. 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
                                            9. 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
                                            10. 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
                                            11. 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
                                            12. 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
                                            13. Maartens IA et al: Neonatal airway obstruction caused by rapidly growing nasopharyngeal teratoma. Acta Paediatr. 98(11):1852-4, 2009
                                            14. Tsitouridis I et al: Sonographic evaluation of epignathus. Hippokratia. 13(1):55-7, 2009
                                            15. Kumar B et al: Neonatal oral tumors: congenital epulis and epignathus. J Pediatr Surg. 43(9):e9-11, 2008
                                            16. 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
                                            17. 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
                                            18. 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
                                            19. 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
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Clinical Issues

                                                    • Diagnostic Checklist

                                                      TERMINOLOGY

                                                      • Synonyms

                                                        • Nasopharyngeal teratoma
                                                        • Oropharyngeal teratoma
                                                      • Definitions

                                                        • Teratoma arising from naso- or oropharynx

                                                      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. Kirishima M et al: 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, 2018
                                                                                      2. Butler CR et al: Ex utero intrapartum treatment (EXIT) for upper airway obstruction. Curr Opin Otolaryngol Head Neck Surg. 25(2):119-26, 2017
                                                                                      3. 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
                                                                                      4. 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
                                                                                      5. Deloison B et al: Atypical case of prenatal cystic epignathus teratoma. Ultrasound Obstet Gynecol. 44(4):495-6, 2014
                                                                                      6. 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
                                                                                      7. 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
                                                                                      8. 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
                                                                                      9. 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
                                                                                      10. 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
                                                                                      11. 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
                                                                                      12. 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
                                                                                      13. Maartens IA et al: Neonatal airway obstruction caused by rapidly growing nasopharyngeal teratoma. Acta Paediatr. 98(11):1852-4, 2009
                                                                                      14. Tsitouridis I et al: Sonographic evaluation of epignathus. Hippokratia. 13(1):55-7, 2009
                                                                                      15. Kumar B et al: Neonatal oral tumors: congenital epulis and epignathus. J Pediatr Surg. 43(9):e9-11, 2008
                                                                                      16. 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
                                                                                      17. 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
                                                                                      18. 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
                                                                                      19. 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