Epignathus como hallazgo incidental ecográfico: Reporte de un caso

[Epignathus as an incidental ultrasound finding: A case report]

Jaime Andrés Machado Bernal1, Mario A. Arteaga-Zarate2, Ricardo A. Torrents-González2, Arturo A. Montaño-Mendoza2

1. Universidad Libre. Barranquilla, Atlántico, Colombia.; 2. Universidad Libre. Barranquilla. Atlántico. Colombia.

Publicado: 2022-08-24

Descargas

Archivos adicionales

Resumen

Concepto: El epignathus consiste en una forma poco frecuente de teratoma orofacial congénito raro que puede surgir de la región del esfenoides, el paladar o la faringe. Esta entidad conlleva a una alta tasa de mortalidad secundario a una importante obstrucción de las vías aéreas en el periodo neonatal. El objetivo es reportar un caso de diagnóstico antenatal ecográfico de epignathus como hallazgo incidental.

Presentación de caso: Primigestante de 17 años, quien acude a control ecográfico para realización de primera ecografía, en donde se evidencia en los 2/3 inferiores de la cara una tumoración solida de ecogenicidad heterogénea donde se observan zonas ecogénicas que se alternan con zonas isoecogénicas la cual se extiende desde el borde inferior de las orbitas hasta el tercio superior del tórax (comprometiendo la región nasal y orofaríngea) de aproximadamente 61x50 mm sugestiva de epignathus.

Conclusión: El diagnóstico prenatal de esta entidad es ecográfico y es esencial para poder planificar un adecuado manejo en las primeras horas posnatales mejorando la tasa de sobrevida neonatal.


Abstract

Concept: Epignathus is an infrequent form of rare congenital orofacial teratoma that can arise from the sphenoid region, the palate or the pharynx, which can be related to different malformations. This entity leads to a high mortality rate secondary to significant airway obstruction in the neonatal period. The purpose is to report a case of antenatal ultrasound diagnosis of epignathus as an incidental finding.

Case presentation: 17-year-old primipregnant woman, who attends an ultrasound control to perform a first ultrasound, where a solid tumor of heterogeneous echogenicity is evidenced in the lower 2/3 of the face where echogenic zones are observed that alternate with isoechogenic zones. which extends from the lower edge of the orbits to the upper third of the thorax (compromising the nasal and oropharyngeal region) of approximately 61x50 mm, suggestive of epignathus.

Conclusion: The prenatal diagnosis of this entity is ultrasound and it is essential to be able to plan an adequate management in the first postnatal hours, improving the neonatal survival rate.

Citas

[1] Hirose S, Sydorak R, Tsao K y cols. Spectrum of intrapartum management strategies for giant fetal cervical teratoma. J Pediatric Surg 2003; 38(3): 446-450. https://doi.org/10.1053/jpsu.2003.50077

[2] Velasco S M, Conda H, Sandoval A, Balanzar J, Irra E, García P, Velasco Pérez M. Epignathus. Diagnosticojournal.com 12-15.

[3] Escobar GI, Poblete LA, Becker VJ, ZavalaB A, et al. Epignathus: Reporte de un caso y revisión de la literatura. Rev Chil Obstet Ginecol. 2005; 70(3):173-9. https://doi.org/10.4067/S0717-75262005000300009

[4] Kirishima M, Yamada S, Shinya 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. 2018;13:99. https://doi.org/10.1186/s13000-018-0776-y

[5] Vandenhuate B, Leteurtre E, Lecomte-Houcke M. Epignathus teratoma: report of three cases with a review of the literature. Palate Craniofac J 2000; 37(1): 83-91. https://doi.org/10.1597/1545-1569_2000_037_0083_etrotc_2.3.co_2

[6] Orrego Jaime, Jaramillo Martha Lucía, Ballesteros Adriana, López José Ignacio, Torres Martha Cecilia, Lasso Rubén Eduardo. Teratoma Cervical Congénito: Reporte De Casos. Ces Med. [Internet]. 2009 Jan [Cited 2022 May 07] ; 23( 1 ): 77-83.

[7] Vallejo, J., & Jimenez, G. (2015). Giant epignatus combined with cleft palate and macrostomy. International Journal of Oral and Maxillofacial Surgery, 44, e300. https://doi.org/10.1016/j.ijom.2015.08.360

[8] Clement K, Chamberlain P, Boyd P, Molyneux A. Prenatal diagnosis of an epignathus: a case report and review of the literature. Ultrasound Obstet Gynecol 2001; 18: 178-181. https://doi.org/10.1046/j.1469-0705.2001.00456.x

[9] Hart I. Perinatal (fetal and neonatal) germ cell tumors. J Pediatric Surg 2004; 39(7): 1003-1013. https://doi.org/10.1016/j.jpedsurg.2004.03.045

[10] Herath M, Siriwardena BSMS, Jayasinghe RD, et al. Oropharyngeal epignathus with partial facial duplication: report of a rare case. Int J Oral Maxillofac Surg. 2018;47:442-4. https://doi.org/10.1016/j.ijom.2017.08.011

[11] Araujo E, Hélio A, Guimaraes F, Saito M, Pires A. Prenatal diagnosis of a large fetal cervical teratoma by three-dimensional ultrasonography: a case report. Arch Gynecol Obstet (2007) 141-144. https://doi.org/10.1007/s00404-006-0180-9

[12] Tsitourdis I, Sidiropoulus D, Michaelides M: Sonographic evaluation of Epignathus, Hyppokratia, vol 13 2009

[13] Bouchard S, Johnson M, Flake A, Howell L, Myers L, Scott N. The EXIT procedure: experience and outcome in 31 cases. J Pediatric Surg 2002; 37 (3): 418-426. https://doi.org/10.1053/jpsu.2002.30839

[14] Wang AC, Gu YQ, Zhou XY. Congenital giant epignathus with intracranial extension in a fetal. Chin Med J (Engl) 2017;130:2386-7.

[15] Martino F, Avila L, Encinas J, Luis A, Olivares P, Lassaletta L, Nistal M, Tovar J. Teratomas of the neck and mediastinum in children. Pediatr Sug Int (2006) 22:627-634. https://doi.org/10.1007/s00383-006-1724-6

×