Congenital epignathus associated with a cleft palate: a case report

Fabrice Lele Mutombo,Neil Wetzig,Médard Kabuyaya Kakule,Noé Kasereka Mwanamolo,Marc H Erickson,Jason Nzanzu Kikuhe

doi:10.1186/s13256-021-03007-w

Fabrice Lele Mutombo, Neil Wetzig + Show 4 more

Open Access

https://doi.org/10.1186/s13256-021-03007-w

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Abstract

BackgroundEpignathus is a rare, benign, congenital teratoma of the hard palate with an estimated incidence of 1 in 200,000 live births. Epignathus frequently leads to a high mortality (80–100%) due to airway obstruction in the neonatal period.Case presentationWe report a case of successful management of a rare oropharyngeal teratoma in a African newborn girl who was referred to our institute with a large protruding intraoral mass, combined with cleft palate, causing some respiratory compromise. The palatal mass was removed on the fifth day after birth, and a palatoplasty performed on day 30.ConclusionEpignathus is a life-threatening condition at the time of delivery. Appropriate management begins with securing the airway, followed by complete resection of the tumor.

Highlights

Epignathus is a rare teratoma that arises from the oral cavity, most commonly from the palate
Congenital tumors of the oral cavity frequently affect normal development of adjacent structures, and tumors developing during morphogenesis of the palate are commonly associated with a cleft palate [1]
We have reported a case of congenital teratoma arising from the hard palate in a newborn girl

Summary

Introduction

Epignathus is a rare teratoma that arises from the oral cavity, most commonly from the palate. Teratomas are uncommon malformations that arise in the three embryonic germ layers (ectoderm, mesoderm, and endoderm) They are felt to be the result of pluripotent cells that did not complete migration from allantois to the genital ridge during the fourth and fifth weeks of gestation [2]. If located in the upper aerodigestive tract, they are high risk, frequently causing life-threatening complications from compromise of the airway. Because of this, they have a high mortality rate, and prenatal diagnosis may allow for better preparation to help optimize care at the time of birth [3,4,5]. The palatal mass was removed on the fifth day after birth, and a palatoplasty performed on day 30

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