A case report of congenital arhinia and literature review

Summary 

We report about a boy with congenital total arhinia and coloboma of the iris. The newborn had complete absence of external nose, nasal and paranasal cavities, with that area being flat with some elevation and firm on palpation. Congenital arhinia is a rare developmental abnormality characterised by lack of the formation of external and internal nasal structures. Since there were no life-threatening complications a tracheotomy was not performed on this newborn. Airway support with oropharyngeal tube was made and the feeding of the child was through an orogastral tube. The child learned to breathe and to eat through the mouth and at age of 3 years a reconstruction of the external nose was performed. Description of the treatment, embryological aspect and a literature review is made to suggest guidelines for the management of such cases.

Keywords: Congenital total arhinia, External nose, Nasal and paranasal cavities, Newborn infant, Abnormalities and anomalies