Congenital nasal pyriform aperture stenosis: retrospective case series, systematic review, and pooled analysis

Background: Congenital nasal pyriform aperture stenosis (CNPAS) is a rare and potentially life-threatening source of neonatal upper airway obstruction. Treatment can be medical or surgical. Our objective was to describe CNPAS presentation and management. Methods: Pooled data analysis of records of children identified with CNPAS from relevant publications and between 2014 and 2024 at our institution was performed. Published studies identified by a medical librarian were assessed independently by two reviewers. Results: From 210 abstracts, 170 were identified as relevant, and 106 studies were included. Data from 10 children with CNPAS from our institution and 310 from the medical literature were analyzed. The majority 74.2 % (n = 230) of our sample received a surgical intervention for CNPAS. Pyriform aperture diameter of children receiving only medical management (mean = 5.3 mm; SD = 1.6) was not different from that of children who received or were recommended surgery (mean = 5.1 mm; SD = 1.5) with a mean difference of 0.2 mm (95 % CI -0.4 to 0.7). Presence of apnea (adjusted odds ratio [aOR] = 9.1; 95 % CI 2.4 to 35) and stridor/noisy breathing (aOR = 3.9; 95 % CI 1.4 to 10.8) were associated with higher odds of having surgery performed or recommended. Conclusions: Management decisions in CNPAS have been driven by individual patient presentation rather than pyriform aperture diameter. Clinicians should use pyriform aperture diameter as a diagnostic rather than prognostic tool, as a narrow pyriform aperture diameter may not necessitate surgery in the absence of severe symptoms.