Congenital nasal pyriform aperture stenosis as a presenting feature of holoprosencephaly.

Congenital nasal pyriform apertures stenosis (CNPAS), a recently recognized uncommon cause of nasal airway obstruction, can be a life-threatening circumstance in the neonate. This study's experience with six cases confirmed the suggestion that CNPAS represents a manifestation of holoprosencephaly. Management of this condition depends firstly on the overall prognosis of the patient and secondly the severity of obstruction. Patients with poor overall prognosis should be managed conservatively with an oropharyngeal airway. In patients with a good outlook, the choice of treatment is conservative for those with less severe obstruction and surgical correction for those with complete obstruction. The ability to pass a No. 5Fr catheter (O.D. 1.67 mm) may be a guide to the choice of treatment. An increase in awareness among otolaryngologists on the nature of CNPAS is necessary to improve overall management of such patients.