Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of neonatal upper airways obstruction. It can occur as an isolated anomaly or be associated with other cranial-facial defects. Clinically, CNPAS manifests as apneic crises, episodic cyanosis, and difficulty in feeding. Here we report a newborn developing respiratory distress after birth that required oxygen support and was confirmed diagnosis of CNPAS by computed tomography (CT) scan. The newborn had successfully undergone surgical intervention via the nasolabial approach.
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