Congenital choanal atresia and pyriform aperture stenosis

Background: Choanal atresia (CA) has a reported incidence of 1 in 5000–8000 live births since its first description by Johann George Roederer in 1755. Nasal pyriform aperture stenosis (NPAS) was described more recently in 1989 by Brown et al. but its true incidence remained unknown. We describe a case of a term male infant with a unilateral left CA and bilateral NPAS that was managed successfully with surgical intervention. Methods: We performed a literature search with the keywords “choanal atresia” and “pyriform aperture stenosis” from Medline, Embase and PubMed databases. To our knowledge, there is no report where CA co-exists with NPAS in a term infant. Case: We present a case of a term male infant who presented with respiratory distress due to bilateral NPAS and inferior turbinates hypertrophy, and a left sided CA. He had none of the typical craniofacial abnormalities or syndromes. His only other associated condition is a peri-membranous ventricular septal defect and accessory tricuspid valve tissue. He was successfully treated with a midface degloving approach for NPAS repair and transoral endoscopic repair of CA. Conclusion: CA and NPAS may present with similar clinical signs and symptoms. The management of both conditions is dictated by the degree of stenosis and severity of symptoms, with the goal of establishing a normal nasal airway, as infants are obligate nasal breathers.