Abstract
INTRODUCTION AND IMPORTANCE: Congenital teratomas arising from the oropharynx, also known as epignathus, are exceedingly rare tumours occurring in 1:35,000-200,000 live births. Although histologically benign, these tumours are often difficult to diagnose antenatally and can cause life-threatening airway obstruction in the newborn. CASE PRESENTATION: We present a case of a full-term female infant presenting at birth with respiratory distress and stridor. Clinical examination and magnetic resonance imaging showed a large multilobulated nasopharyngeal mass causing severe airway compromise. The infant underwent excision biopsy with partial debulking of the tumour surrounding the airway, and was successfully extubated thereafter to room air. Histopathological examination confirmed the diagnosis of mature teratoma. CLINICAL DISCUSSION: The pathophysiology of epignathus is thought to be aberrant proliferation of pluripotent cells in the notochord during early embryogenesis. Antenatally, elevated maternal alpha-fetoprotein or polyhydramnios may be clue to diagnosis. The mainstay of treatment is airway clearance with surgical resection, which can be curative or at least minimizes the risk of malignancy. CONCLUSION: This case highlights the importance of early recognition and multidisciplinary management of the neonatal airway, in order to improve outcome.