Abstract

Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of respiratory distress in newborns. This paper reports a case of severe CNPAS that required endotracheal intubation immediately after birth, and eventually, surgical intervention. At birth, the width of the pyriform aperture was only 4 mm, and the patient was completely unable to breathe through his nose. We performed tracheostomy at 23 days of age and waited for the patient to grow, but at 56 days of age, the width of the pyriform aperture was not sufficient (6 mm) for the patient to breathe through his nose. Therefore, surgical dilation of the pyriform aperture by a sublabial approach was performed on day 79 after birth, and the width was increased to 14 mm. Postoperative stent placement was performed for two weeks. After the removal of the stents, the patient could finally breathe through his nose, and the postoperative course was uneventful, with no restenosis after four months. CNPAS is a rare cause of nasal obstruction, but it can cause respiratory distress in infants because they are dependent on nasal breathing. Conservative treatments are initially recommended for CNPAS; however, in severe cases where conservative treatments are ineffective, surgical treatment is recommended.

Highlights

  • Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of respiratory distress in newborns

  • This paper reports a case of severe CNPAS that required endotracheal intubation immediately after birth, and eventually, surgical intervention

  • Surgical dilation of the pyriform aperture by a sublabial approach was performed on day 79 after birth, and the width was increased to 14 mm

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Summary

Introduction

Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of respiratory distress in newborns. We decided to surgically dilate the stenosis of the nasal aperture In this period after tracheostomy, the patient received swallowing and physical therapies. Axial CT scan on day 7 after birth showed a narrow nasal pyriform aperture. The patient was provided with direct breast as well as bottle feeding His milk intake gradually increased, and he was discharged 32 days after surgery (111 days after birth). The width of the pyriform aperture was 11 mm. (b) Appearance of the nostrils one month after removal of the stents

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Dinwiddie R
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