Congenital partial arhinia with no associated malformations

Summary 

Congenital absence of the nose is an extremely infrequent congenital malformation with less than 40 cases reported previously in the literature. Only three cases of partial arhinia have been reported. It is commonly seen associated with other central nervous system and craniofacial malformations. Somatic anomalies have been reported in more than 50% of cases. Severe airway, feeding and phonetic problems may accompany this pathology in children. We describe a case of complete heminose agenesis and absence of the ipsilateral nasal fossae, without other congenital malformations.

Keywords: Arhinia, Nose defects, Midface hypoplasia, Congenital malformation, Nasal agenesis