Agenesis of the nose (arhinia, congenital nasal atresia) is a rare anomaly with about 20 surviving cases reported in the English literature in the last century. The embryological origin of the defect is thought to be maldevelopment of the paired nasal placodes. The respiratory distress is evident after the birth and requires immediate orotracheal intubation and mechanical ventilation. Herein we report a case of congenital absence of the nose in a male child. Immediately after birth, the baby had difficulty in breathing and developed cyanosis, for which he required orotracheal intubation and tracheotomy, posteriorly. On examination a, a flat bony area replaced the nose, the hard palate was highly arched, and bilateral microphthalmia. The child was also was evaluated by CT (computed tomography) and MRI (magnetic resonance imaging) studies. The CT scan revealed absence of the nose, anterior bony stenosis, absence of the nasal chamber and paranasal sinuses, a high-arched palate, bilateral microphtalmia, and bilateral mucocele of the lachrymal sac. The MRI demonstrated normal configuration and density of the brain.
agenesia of the nose; arhinia; computed tomography; magnetic resonance imaging
