Massive facial teratoma managed with the ex utero intrapartum treatment (EXIT) procedure and use of a 3-dimensional printed model for planning of staged debulking

Abstract

Teratomas are the most frequent solid tumor found in neonates. However, only 1.5% of neonatal teratomas originate from facial structures. Neonatal facial teratomas are associated with polyhydramnios, preterm birth, pulmonary hypoplasia, cleft palate, cleft lip, and life-threatening airway compromise. The overall survival reported with these lesions has been between 17 and 87.5%; however survival in the setting of antenatally diagnosed facial teratomas has only been described anecdotally. We present a case of an antenatally diagnosed massive facial teratoma originating from the pterygomaxillary fossa, which was associated with polyhydramnios and pre-term birth. We managed this complex tumor with an ex utero intrapartum treatment (EXIT) procedure, multidisciplinary medical and surgical team, and staged excision and reconstruction aided by use of a 3-dimensional printed model. Here we review the surgical management of this rare and complex tumor.

Keywords

• Facial teratoma
• Ex utero intrapartum treatment (EXIT) procedure
• Neonatal teratoma
• Surgical management