Congenital nasal pyriform aperture stenosis: a report of 10 cases and literature review.

Background: Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of neonatal upper airway obstruction. This study is to review the presentation and management of this uncommon condition in a tertiary paediatric ENT centre in Scotland, United Kingdom.

Methods: Retrospective case note review between 2003 and 2011. A literature search was performed using MEDLINE and EMBASE between April and June 2011 using search terms 'pyriform aperture' in combination with diagnosis and treatment. Suitable references were also selected and reviewed.

Results: A total of 10 cases were identified and 50% of cases were managed surgically. Surgical widening of the pyriform aperture was performed at an average of 14 days at birth. Associated malformations include holoprosencephaly, hypopituitarism, cardiac and urogenital malformations.

Conclusions: CNPAS is a treatable cause of upper airway obstruction. Early recognition is vital to appropriate management. Treatment is either conservative or surgical and depends on the severity of symptoms.