Neonatal epignathus in the oral and pharyngeal regions: A case report

Abstract

Congenital teratoma that arises in the oropharyngeal region is called epignathus. Here, we report a case in which large epignathus extending from the pharynx to the oral cavity was resected. A female fetus was found to have a mass extending on her face to the pharyngeal region during a sonographic examination performed at 33 weeks’ gestation. On the 9th day after birth, the tumor extending from the left nostril (10 cm) was excised by the pediatric surgeons. The residual tumor protruded from the oral cavity day by day. Pediatric and oral surgeons resected the tumor (14.5 cm). A bifid uvula was noted and submucosal cleft palate was suspected. No recurrence has been seen after 4 years’ follow‐up.

Sequelae of congenital oropharyngeal teratoma in the oral cavity of the child

“…The presence of the larger tumor was found during the ultrasound exam in the 34th week of pregnancy, similar to other reports 9,10,12,16,19 in which the diagnosis also occurred in the third trimester. Other studies 7,8,20,23 reported the presence of teratoma in the second trimester of pregnancy, and in some 2,14,15 , only after birth.…”

“…Although congenital oropharyngeal teratomas are considered rare benign tumors, when present, they can cause several sequelae in the child's oral cavity, as observed in this case report, requiring long-term multidisciplinary follow-up to correct them. This clinical case has many similarities with others found in the literature 2,[6][7][8]10,11,[15][16][17][18][19] . Regarding gender, most studies reported the presence of oropharyngeal teratomas in female children.…”

“…In this case, the removal of the teratoma was performed early, o the 17th day of life, requiring removal of the residual teratoma two weeks later. The tumor described in this case was classified anatomically and pathologically as mature, as well as the tumors reported by most authors 2,4,6,9,11,12,14,16,19,21,22 . However, even though it is an uncommon occurrence, Kumar et al 7 and Chung et al 8 reported cases of immature teratoma.…”

“…Fetal manifestations are described in approximately 6% of cases, the most common being bifid tongues, left ventricular hypoplasia, pulmonary hypoplasia, hydrops, hypertelorism, imperforate anus, micrognathia, glossoptosis, cleft palate, and macrostomy 3,7,17,19 . In this clinical case, two alterations mentioned in the literature were observed, such as macrostomy 19 and cleft palate 11,[16][17][18][19][21][22][23] . The shape of the oral cavity corroborates with the suggestive diagnosis for macrostomy, a rare congenital anomaly understood as an enlargement of the mouth in the region of the oral commissures which may present itself unilaterally or bilaterally and the reported incident varies from 1:60,000 to 1:300,000 live births 26 .…”

“…This can be explained by the fact that this malformation occurs during intrauterine life, more precisely until the 12th gestational week, that is, at the end of the embryonic period, preventing the fusion of the nasal septum with the bilateral palatal processes 28 . In several reports 11,[16][17][18][19][21][22][23] , the presence of cleft palate was observed. Carreirão & Pitanguy 29 recommend that surgical intervention for the correction of cleft palate should occur at around 1 to 1.5 years of age, aiming to achieve better results due to the easier identification of anatomical structures and the age being the period acquisition of language function.…”