Congenital nasal pyriform aperture stenosis: Elaboration of a management algorithm from 25 years of experience
Introduction
Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of neonatal airway obstruction leading to respiratory distress. It was identified for the first time in 1952 by Douglas et al. [1], and later radiologically described in 1988 by Ey et al. [2]. However, the first clinical description was published by Brown et al. [3] in 1989.
Few publications in the literature have reported both clinical and radiological characteristics with anatomical measurements on patients with CNPAS. Belden et al. [4] estimated that the lowest pyriform aperture width is about 11 mm on a normal term-born neonatal CT-scan. Although this value is not consensual, all CNPAS patients found in the literature had a PA width measurement under this threshold.
CNPAS can occur as an isolated anomaly or as part of holoprosencephaly spectrum including solitary median maxillary central incisor and other midline anomalies.
Because CNPAS is a rare anomaly, it is mainly diagnosed in specialized pediatric otolaryngology centers. Most of the recent studies focus only on one of the aspects of this craniofacial abnormality, with particular emphasis on trying to find objective criteria for diagnosis and surgical management. However, no clear guidelines have been proposed.
The objective of this study was to present a complete management algorithm for the diagnosis of CNPAS, as well as the medical and surgical management. This algorithm was created based on the experience of a tertiary care center pediatric otolaryngology center between 1988 and 2014, reporting clinical and radiological characteristics, management and outcome of patients presenting with CNPAS.
Section snippets
Materials and methods
This is a review of all patients presenting with CNPAS and referred to The Department of Pediatric Otolaryngology Head and Neck Surgery of La Timone Children's Hospital in Marseille between 1988 and 2014. Charts were reviewed for details including patient demographics, radiological characteristics, coexisting anomalies, medical management, and, when necessary, surgical treatment and outcome.
CNPAS diagnosis was suspected based on clinical features of airway obstruction: difficult breathing, poor
Results
A total of 10 patients were diagnosed with CNPAS. 7 patients were female and 3 were male (Table 1). Three patients were born prematurely, with the lowest gestational age being 35 weeks LMP. Mean birth weight was 2.900 kg and mean term of pregnancy was 38.2 weeks LMP (Table 2). All patients were symptomatic at birth: 8 of the 10 patients presented with respiratory distress necessitating immediate management, and 2 patients presented with noisy breathing. Radiological diagnosis with CT-scanning
Discussion
Newborns and infants are obligate nasal breathers; oral ventilation appears between 3 and 6 months of age. Any nasal obstruction during this period can cause respiratory distress, life-threatening dyspnea and eventually a failure to thrive. Congenital nasal airway obstruction occurs in up to 1 in 5000 infants, and most of them are affected with choanal atresia. Nevertheless, other anomalies can occur, such as major septal deviations, nasal cavity masses (teratomas [5] and dacryocystoceles [6])
Conclusions
Prompt diagnosis and management of CNPAS is necessary and include complimentary examinations due to the high prevalence of associated abnormalities. Diagnosis is made by CT-scanning with pyriform aperture width narrowing usually under 11 mm. Medical treatment should always be performed, and may be associated with surgical intervention when needed. Both medical and surgical management offer good long-term prognosis. The algorithm we propose provides guidelines for all physicians faced with CNPAS.
Conflict of interest
The authors confirm that they have no conflict of interest.
Funding
Eric Moreddu wrote the first draft of the manuscript. No grant or payment was given to produce the manuscript.
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