Epignathus

Epignathus is an extremely rare congenital teratoma that arises from the oral cavity, most commonly from the hard or soft palate, but occasionally from the pharynx, jaw, or tongue. It is a type of oropharyngeal teratoma composed of tissues derived from all three embryonic germ layers (ectoderm, mesoderm, and endoderm), and may contain differentiated structures such as teeth, bone, cartilage, neural tissue, and skin. Epignathus is typically detected prenatally via ultrasound or at birth, presenting as a mass protruding from the mouth. The tumor is classified under ICD-10 as D37.0, reflecting its uncertain neoplastic behavior. The primary clinical concern with epignathus is airway obstruction, which can be life-threatening at birth. Large tumors may cause feeding difficulties, respiratory distress, and in severe cases neonatal asphyxia if not promptly managed. Prenatal complications may include polyhydramnios (excess amniotic fluid) due to impaired fetal swallowing, and very large masses can cause difficulty during delivery. Some cases are associated with cleft palate or other craniofacial anomalies. Rarely, intracranial extension of the tumor has been reported, which significantly complicates management. Treatment is primarily surgical excision of the mass, ideally performed as soon as the airway is secured. In cases diagnosed prenatally where significant airway compromise is anticipated, an EXIT (Ex Utero Intrapartum Treatment) procedure may be planned to establish the airway during delivery while the infant remains on placental circulation. The majority of epignathus tumors are histologically benign (mature teratomas), and complete surgical resection generally carries a favorable prognosis. However, outcomes depend on tumor size, location, degree of airway obstruction, and the presence of intracranial extension. Malignant transformation is exceedingly rare but has been reported. Long-term follow-up is recommended to monitor for recurrence and to address any residual craniofacial defects.

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