Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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May 16, 2011
Congenital Piriform Aperture Stenosis
- The piriform aperture is the most anterior and narrow part of the nasal airways. It is formed by the nasal and maxillary bones.
- Piriform aperture stenosis (PAS) results from early fusion and hypertrophy of the medial nasal processes.
- It is a rare cause of nasal airway obstruction in neonates and young children, presenting either at birth or in the first few months of life.
- Associated anomalies include central megaincisor, pituitary dysfunction, and semilobar and alobar forms of holoprosencephaly.
- Key Diagnostic Features: CT is the imaging modality of choice. Typical features include overgrowth and medial displacement of the nasal processes of the maxilla, hard palate hypoplasia which demonstrates a triangular shape, and piriform aperture stenosis. A piriform aperture width less than 11mm in a term infant is considered diagnostic. Abnormal dentition and a bone ridge along the underside of the palate are confirmatory imaging findings.
- Treatment: Conservative and use of decongestants. Surgical reconstruction is reserved for severe cases.