# Nasal Pyriform Aperture Stenosis in a Newborn: When to Operate

## TL;DR

A rare case of nasal stenosis in a newborn is described, highlighting the need for early diagnosis and surgical intervention when conservative treatments fail.

## Contribution

This case report emphasizes the importance of considering CNPAS in neonates with unexplained respiratory distress and demonstrates successful surgical treatment.

## Key findings

- CNPAS was diagnosed via maxillofacial CT showing a pyriform aperture width of 3.4 mm.
- Conservative treatments failed, necessitating surgical intervention via a sublabial approach.
- The child was successfully discharged on full oral feeds after surgery.

## Abstract

Congenital nasal pyriform aperture stenosis (CNPAS) is a very rare cause of neonatal respiratory distress and is often missed because of its rarity. It arises from the overgrowth of the nasal process of the maxilla. Maxillofacial CT scan findings of pyriform aperture width <11 mm in a full-term baby, median central incisor, triangular-shaped palate, and median palatal ridge confirm the diagnosis. We describe here a case of CNPAS admitted with respiratory distress that increased further on feeding. An infant feeding tube of size 6 was not negotiable through the nostrils. Resistance was appreciated at the inlet of the nostril. Maxillofacial CT showed pyriform aperture stenosis of 3.4 mm, suggesting CNPAS. The child could not be weaned off a high-flow nasal cannula despite conservative management with decongestants, steroids spray, dilatation, and stenting for 20 days. Subsequently, surgical widening of the nasal aperture by a sublabial approach was done. The child was discharged on the 10th postoperative day on full oral feeds.

It is important to suspect CNPAS in neonates with respiratory distress where other common causes have been ruled out, as it can be treated by surgery in cases refractory to conservative management.