Congenital Nasal Bones Agenesis: Report of a Rare Malformation.

Abstract

Congenital arhinia and hyporhinia are rare facial anomalies whose knowledge usually comes from case reports. The severity of each case described in literature is variable; it also depends on associated malformations too. Since the newborns are obligate nasal breathers, babies with arhinia or hyporhinia usually have respiratory distress and need airway stabilization. In addition, most of these children present difficulties in feeding and this impairment must be managed early. We describe an unusual case of partial congenital arhinia, the baby did not have other anomalies or any specific complication such as respiratory and feeding issues, so the major problem was the aesthetic and psychological issues for the family. Even if the neonatal course was uncomplicated, a coordinated approach of the pediatrician with the pediatric otolaryngologist, the geneticists and the neurosurgeons was necessary because the management of these malformations is always very complex; due to the lack of reports described in literature, an univocal management and also the best timing and technique for reconstructive surgery are still not defined.