Perinatal diagnosis and management of epignathus

Background: After the diagnosis and treatment by EXIT procedure of a case of epignathus in our centre, we have reviewed the management of this condition.

Methods: Systematic review of the bibliography in Medline and our case experience. Search terms: "oral teraoma" OR Epignathus; Limits for age (Newborn) and languages (English, Spanish, Italian and portugese) (57 articles) and without limits (121). We select articles cases reports about oropharyngeal teratomas including diagnosis and/or treatment, specially those with polyhidramnios and/or respiratoy distress at birth. Articles before 1990 were refused.

Results: Epignathus is defined as a teratoma that occupies the lumen of the craniopharyngeal canal causing an obstructive polyhydramnios. This is a potential cause of death due to upper airway obstruction at birth. Cardiac function can be compromised due to its size. No other lethal conditions are normally associated. Prenatal diagnosis and the strategy of the perinatal treatment seem to improve the prognosis of this condition.

Conclusions: Consensus exit about that prenatal diagnosis is the key to avoid the neonatal respiratory distress. Formo our experience and from others it is clearly defined that the proper treatment is the prevention of the preterm labour and delivery by EXIT procedure, in order to have the neonate airway control under foetal-placental circulation. Conclusions: The most frequent associated anomalies with epignathus are feasible to surgical correction. The prognosis of giant epignathus seems to improve with a proper prenatal diagnosis (US and MRI) and an adequate strategy of the airway management. With EXIT procedure, a potential life-threatening emergency can be managed like an elective procedure, improving the prognosis of this children.