CONGENITAL NASAL PYRIFORM APERTURE STENOSIS AS A PRESENTING FEATURE OF HOLOPROSENCEPHALY

Congenital nasal pyriform apertures stenosis (CNPAS), a recently recog nized uncommon cause of nasal airway obstruction, can be a life-threat ening circumstance in the neonate. This study's experience with six ca ses 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 obstruc tion. Patients with poor overall prognosis should be managed conservat ively with an oropharyngeal airway. In patients with a good outlook, t he choice of treatment is conservative for those with less severe obst ruction and surgical correction for those with complete obstruction. T he 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 otolaryngolog ists on the nature of CNPAS is necessary to improve overall management of such patients.