By Rahul V. Pawar, MD
Newborn infant, respiratory distress, inability to pass nasogastric (NG) tube.
Nasal bone CT reveals narrowing of the nasal piriform aperture (PA). As measured in the axial plane, PA width is 5.2 mm. At the posterior nares, the choanae were patent.
Congenital nasal piriform aperture stenosis (CNPAS).
Compared with choanal atresia, CNPAS is far less common. Shortly after birth, infants may develop difficulty breathing, cyanosis, and, potentially, upper respiratory tract infections. Passage of an NG tube may be difficult, if not impossible. CNPAS is anterior nasal cavity stenosis, whereas as choanal atresia refers to posterior nasal cavity stenosis, the latter usually a function of osseous and/or membranous obstruction.
From an imaging standpoint, a variety of features may be seen with CNPAS, including a stenotic bony inlet, triangular-shaped palate, and maxillary nasal process bony overgrowth +/- abnormal dentition.
In a comprehensive review of patients by Belden et al, all patients with CNPAS had a PA width <8 mm.1 In our case, the PA width was 5.2 mm.
It seems that defect(s) in primary palate formation belies CNPAS, which would also potentially explain associated findings, principally a triangular-shaped palate and/or abnormal incisors. In some cases, the possibility of central nervous system anomalies and/or endocrine dysfunction may warrant consideration, additional workup, etc.
All in all, CNPAS has an excellent prognosis. In the short term, alternate, specialized feeding techniques usually suffice. Surgery is usually unnecessary as the child grows.
— Rahul V. Pawar, MD, Department of Radiology, Division of Neuroradiology, is Clinical Assistant Professor, UMDNJ—New Jersey Medical School.
- Belden CJ, Mancuso AA, Schmalfuss IM. CT features of congenital nasal piriform aperture stenosis: initial experience. Radiology. 1999;213(2):495-501.
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