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

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Abstract

Introduction

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.

Conclusion

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.

Introduction

Congenital nasal pyriform aperture stenosis (CNPAS) is a rare but recognised cause of upper airway obstruction in the newborn. It is secondary to bony overgrowth of the nasal process of maxilla and infants being obligate nasal breathers can present with life threatening upper airway obstruction.

Nasal pyriform aperture stenosis in adults was first recognised by Douglas [1] in 1952 and the first radiological description of CNPAS was described by Ey et al. [2] in 1988. It was not until 1989 that Brown et al. described the first clinical case of CNPAS [3].

CNPAS can occur as an isolated anomaly or as part of solitary median maxillary central incisor syndrome (SMMCI). We report a series of 10 patients with CNPAS managed in a tertiary paediatric care centre between 2003 and 2011 and discuss their presentation, associated malformations, diagnostic tests and clinical management.

Section snippets

Materials and methods

This is a retrospective review of all patients diagnosed and referred to the Department of Otolaryngology at the Royal Hospital for Sick Children, Glasgow with pyriform aperture stenosis (PAS). Case notes were reviewed for details including patient demographics, coexisting anomalies, initial management, surgical treatment and outcome.

A diagnosis of PAS was suspected based on clinical features of airway obstruction such as tachypnoea, poor feeding, nasal congestion, episodes of apnoea/cyanosis

Results

All operations were performed in a similar manner and these patients are clinically asymptomatic with no evidence of recurrence at an average 2.5 years following surgery. Details of each case are summarized in Table 1. One child underwent surgery for unilateral choanal atresia along with widening of the pyriform aperture. 4 children underwent a single procedure whilst one patient had persistent symptoms following surgery and was noted to have granulation tissue in the nasal cavity which was

Discussion

Newborns are obligate nasal breathers and any cause of upper airway obstruction can cause life threatening dyspnoea. CNPAS is a relatively new medical entity that is gaining rapid recognition. Reports suggest that CNPAS is an anomaly where the pyriform aperture is narrowed secondary to bony overgrowth of the nasal process of the maxilla [5], [6]. The pyriform aperture is bordered superiorly by the nasal bones, inferiorly by the junction of the horizontal process of the maxilla and laterally by

Conclusion

Congenital nasal pyriform aperture stenosis is a rare but treatable cause of upper airway obstruction in neonates and infants. It is most commonly associated with holoprosencephaly. Once identified screening should rule out other congenital abnormalities involving the pituitary gland, cardiac and urogenital systems. Treatment is either conservative or surgical and depends on the severity of symptoms. Surgical treatment involves widening the pyriform aperture width through a sublabial approach.

Conflict of interest

None.

References (16)

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