Abstract
Epignathus is a rare congenital oropharyngeal teratoma that arises from the oropharynx, especially the sphenoid, palatine, and ethmoid bones. Teratomas are benign tumors containing cells from ectodermal, mesodermal, and endodermal layers. The incidence of epignathus is between 1:35,000 and 1:200,000 live births with a female predominance. We reported an uncommon case of epignathus in a female newborn baby with an ill-defined oral mass protruding through a cleft in the hard palate. Computed tomography scan showed a contrast-enhanced solid mass with areas of calcification simulating a unique case of maxilla duplication. Surgery was performed, the mass was excised successfully, and microscopic analysis confirmed the diagnosis of mature teratoma. The patient evolved with good general health and showed no clinical signs of recurrence. Although epignathus is a rare condition, it should be diagnosed in the fetus as early as possible, especially to avoid fatal airway obstruction. In such cases, the treatment option is exclusively surgical, and complete resection is curative in most cases during the early neonatal period.
References
Feb 12, 2000·The Cleft Palate-craniofacial Journal : Official Publication of the American Cleft Palate-Craniofacial Association·B VandenhauteG Soto-Ares
Sep 1, 2001·Ultrasound in Obstetrics & Gynecology : the Official Journal of the International Society of Ultrasound in Obstetrics and Gynecology·K ClementA Molyneux
Jun 24, 2004·Journal of Pediatric Surgery·Hart Isaacs
Oct 8, 2004·Clinica Chimica Acta; International Journal of Clinical Chemistry·D BaderM David
Oct 24, 2007·Journal of Cranio-maxillo-facial Surgery : Official Publication of the European Association for Cranio-Maxillo-Facial Surgery·Wolfgang ZemannVesna Kozelj
May 3, 2008·The Cleft Palate-craniofacial Journal : Official Publication of the American Cleft Palate-Craniofacial Association·Kathy L ChapmanJulie Schulte
May 21, 2010·International Journal of Oral and Maxillofacial Surgery·S SumiyoshiA Kaetsu
Mar 8, 2011·International Journal of Pediatric Otorhinolaryngology·Brianne Barnett RobyRobert J Tibesar
Oct 11, 2012·The Cleft Palate-craniofacial Journal : Official Publication of the American Cleft Palate-Craniofacial Association·Yun-Ta TsaiJui-Pin Lai
Jan 26, 2013·The Journal of Craniofacial Surgery·Akmam H Al-MahdiAmeer Dhaher
Jun 5, 2013·Journal of Maxillofacial and Oral Surgery·Manjunath RaiUmesh M Devaraju
Jul 9, 2013·National Journal of Maxillofacial Surgery·Hari RamLaxman R Malkunje
Aug 3, 2013·Logopedics, Phoniatrics, Vocology·Tove B JohannissonChristina Persson
Nov 2, 2014·Birth Defects Research. Part A, Clinical and Molecular Teratology·Silvia MorlinoPaola Grammatico
May 12, 2017·Brazilian Dental Journal·Cyntia Helena Pereira de CarvalhoLeão Pereira Pinto