Literature Review of Various Treatments in CNPAS and a Proposed Novel Clinical Treatment Algorithm

Background: Congenital nasal pyriform aperture stenosis (CNPAS) is a rare disorder characterized by a narrowed pyriform aperture and nasal obstruction. Children with CNPAS often exhibit a bony ridge on the underside of the secondary palate, a solitary central incisor, and a triangular-shaped palate. Due to its rarity, limited research exists, and there is no established treatment algorithm, which complicates management, particularly in craniofacial treatments and long-term follow-up. Aims: This study aimed to develop a comprehensive treatment algorithm based on long-term follow-up, focusing on maxillofacial deformities, dental considerations, and upper airway assessment. Methods: We conducted a retrospective study of four CNPAS patients treated at our institute. We performed a literature review, and a comparison was executed with our data in order to introduce our novel, age-specific clinical treatment framework. Results: A literature review was conducted, and different clinical parameters were examined. Previously published data were compared to our patients-related findings to develop our clinical treatment algorithm based on patients' age. Patients were monitored for respiratory distress during the first two years of life. Children with cyclic cyanosis underwent surgical widening of the pyriform aperture through bony excess removal and nasal tubing to ensure airway patency. For those with ongoing desaturation events, neonatal palatal expansion was performed. At ages 10-12, additional evaluations using polysomnography and orthodontic assessments were conducted. Based on these findings, patients received surgically assisted rapid palatal expansion (SARPE) and further surgical widening of the pyriform aperture if obstructive sleep apnea (OSA) was present. Subsequent treatments included orthodontic care and restoration of the missing central incisor. Conclusions: We propose an age-based clinical treatment algorithm for CNPAS tailored to address individual patient needs throughout their growth.