Reconstruction of a Large Palatal Defect by Two-Stage Palatoplasty after Excision of a Rare Giant Neonatal Pharyngeal Teratoma: A Case Report

Abstract

　　Neonatal oropharyngeal teratoma (=epignathus) is a markedly rare congenital malformation, with an incidence of 1 in 35,000-200,000 live births. Regarding deformities associated with this tumor, cleft palate is common. We report a rare case of epignathus in a male newborn with a huge oral mass protruding through a cleft in the hard palate.
　　For the large defect of the palate after surgical excision of the tumor, soft and hard palate closure were performed at 2 years and 11 months, and 5 years and 4 months, respectively. As a result, language development is age-appropriate and velopharyngeal insufficiency is mild, but the patient was able to produce monophonic words approximately 4 years after the second surgery. There is no malocclusion or rhinorrhea on ingestion at 9 years of age.
　　The two-stage operation resulted in insufficient velopharyngeal closure function in many reports; however, language training resulted in good language performance equivalent to that after the one-stage operation.
　　This report describes our procedure and postoperative course.