Congenital absence of the nose: a case report.

Congenital absence of the nose (arhinia, congenital nasal atresia) is a rare anomaly which is infrequently described in the literature. Herein we present a case of congenital absence of the nose recently evaluated and treated at Children's Hospital Medical Center (CHMC), Cincinnati, OH. CT and MRI studies were obtained. To our knowledge, no such radiographic evaluations have been described in patients with congenital absence of the nose. In addition to complete absence of the anterior soft tissues of the nose, thin anterior and thick posterior atretic plates were present. MRI was useful in defining the nature of the soft tissue mass which filled the single hypoplastic nasal cavity. The lip, alveolus, and palate were remarkably well developed. A thorough investigation revealed no other congenital defects. At 15 days of life, because of the airway support required and the associated feeding difficulties, a nasal airway was created using a combination of sublabial, transpalatal and percutaneous approaches. Frequent home dilation of the surgically created opening has been successful in maintaining nasal patency thus allowing the patient to go without continuous stenting.