Congenital nasal pyriform aperture stenosis with holoprosencephaly: Diagnosis and surgical man-agement

Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of neonatal nasal obstruction that clinically mimics choanal atresia. It occurs as a result of abnormal de- velopment of primary palate and maxilla, either in isolation or in association with other anomalies. Differentiation between CNSPAS and choanal atresia is critical as manage- ment approachers are quite different from one another. Diagnosis can be made clinically and with characteristics CT findings. Management options include both conservative and surgical approaches depending on the patient’s initial condition. Here is reported a case of 33 days infant who was admitted to our NICU with a working diagnosis of CN- PAS after she presented with two days of fast breathing and failure to suckle, managed surgically and discharged improved.

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