A Patient With an Epignathus: Management of a Large Oropharyngeal Teratoma in a Newborn

Abstract

An epignathus is an oropharyngeal teratoma composed of cells from ectodermal, mesodermal, and endodermal layers. Epignathi that arise from the palate or pharynx and protrude from the mouth result in life-threatening airway obstruction and usually cause asphyxiation shortly after birth. In our reported case, an antenatal ultrasound diagnosis allowed for preparation of an ex utero intrapartum treatment (EXIT) procedure. A tracheostomy was performed at birth with maternal-fetal blood flow still intact through the umbilical cord. Debulking of the large extraoral portion of the tumor, followed by complete intraoral resection, was performed. Masseteric function and swallowing slowly improved over several weeks. Cleft palate repair is planned at 10 months of age. In the following report, the histology, classification, and pathogenesis of these "parasitic" tumors are reviewed.

Nasopharyngeal mature teratoma in the newborn

“…Surgically, it is aimed to remove the tumor completely. Recurrence may be observed in teratomas which have not been removed completely (1)(2)(3)(4)(5). In our patient, the teratoma was removed nearly completely and tumor was not found on histopathological examination of the biopsy samples obtained from the remnant tissue observed on radiologic follow-up examinations.…”

Oropharyngeal Tumor in the Newborn: A Case Report

“…They are very rare, and in the early literature [9,10] , epignathi and teratomas of the skull base have been considered incompatible with life. Some of the later literature reported the better outcome [6] . Epignathus is a misnomer, and its etymological meaning is 'upon the jaw'.…”

“…There was massive polyhydramnios noticed at the time of delivery in our case. A history of polyhydramnios is not uncommon in oropharyngeal tumors and has been suggested to be due to impaired ingestion of amniotic fluid in utero [1,2,6,7] . Most hairy polyps present as a single pedunculated mass with the stalk attached to the oropharyngeal wall.…”

“…Other reported signs have included snoring, rhinorrhea, recurrent cough, failure to gain weight and speech problems. In cases diagnosed antenatally with a mass large enough to cause life-threatening airway obstruction and asphyxiation, the ex utero intrapartum treatment (EXIT) procedure to secure the airway should be planned and performed as reported by Izadi et al [6] .…”

“…Epignathus is a misnomer, and its etymological meaning is 'upon the jaw'. However, it has been used for almost every teratoma of the oral cavity and pharyngeal tissues as well as for teratomas protruding from the mouth [1,6] . Some authors, employing Arnold's classification of teratomas, classify the lesion found in this case as a dermoid [2] .…”