Congenital nasal pyriform aperture stenosis: A report of 10 cases and literature review
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.
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Congenital nasal pyriform aperture stenosis; our experience of 34 cases
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2022, International Journal of Pediatric OtorhinolaryngologyCitation Excerpt :The case number of the original Belden series was small and heterogenous (age range 0–12 months), with 6 children with PAS compared with 23 controls. Subsequent cases series report a mean piriform aperture width between 4.5 and 6.6 mm.[5,6,8,10–12] The average piriform aperture width in cases with a clinical and radiological diagnosis of piriform aperture stenosis in the present study was 5.6 mm.
Congenital pyriform aperture stenosis: ten years experience
2021, Acta Otorrinolaringologica EspanolaCongenital nasal pyriform aperture stenosis repair: A case series and discussion of postoperative care
2021, Otolaryngology Case ReportsCitation Excerpt :Nasal cannulas and masks require fixation to the neonate and therefore cannot be used effectively following CNPAS repair [26] [Fig. 2]. Length of stenting is highly debated in literature, some publications recommending up to 28 days [2,8,10,22,27–29]. With use of this rigorous postoperative care regimen, we recommend stent removal as soon as POD 5 with an additional 48 hours of inpatient observation before discharge to ensure that restenosis and mucosal dehiscence do not occur.