Congenital arhinia – First published case in Malaysia

Congenital arhinia is one of the rare craniofacial malformation that may cause severe respiratory distress at birth due to upper airway obstruction. Our patient, whose abnormalities were only detected after delivery in our centre, is the first reported case of congenital arhinia in Malaysia. Contrary to popular belief that neonates are obligate nasal breather, our patient adapted well to breathing through mouth before an elective tracheostomy was performed on day four of life.

Congenital Complete Arhinia: Report of a Rare Case.

Congenital absence of the nose (arhinia) is extremely rare. Arhinia leads to severe airway impairment and poor feeding in the neonate. Th e degree of nasal absence varies from case to case. Here, we present a case of congenital complete arhinia associated with other craniofacial anomalies. Arhinia leads to severe respiratory distress and feeding problems in newborn warranting emergency management, but our case diff ers as the child was adapted to mouth breathing and naso-gastric tube feeding, giving us the opportunity to delay the defi nitive management for a few weeks yet avoiding the risk of operating in a small child to create nasal airway or tracheostomy on an emergency basis.