231 Congenital Anomalies of the Respiratory Tract

Pyriform aperture stenosis presents as unilateral or bilateral anterior nasal obstruction. The obstruction is firm and exists immediately posterior to the nares. The anatomic abnormality causing pyriform aperture stenosis is bony overgrowth of the nasal lateral process of the maxilla. Associated findings with pyriform aperture stenosis include a central incisor and may be considered a form of holoprosencephaly. Significant respiratory distress results from marked stenosis and establishing an airway may require placement of an oral airway or endotracheal intubation. The definitive diagnosis is made with a computerized axial tomogram (CAT) scan. With noncritical stenosis, the child may be managed expectantly by controlling nasal edema. Definitive management is surgical and involves the submucosal removal of bone with using either a transnasal or sublabial approach. Stenting of the nasal airway for a period of time may be necessary after surgery for pyriform aperture stenosis. Nasolacrimal duct cysts result from obstruction of the nasolacrimal duct as the duct enters the lateral nasal wall below the attachment of the inferior turbinate. The cysts can be unilateral or bilateral and present as compressible masses attached to the lateral nasal wall. Catheters can be passed beyond the cysts and may confuse the physical examination, as the child will have nasal obstruction despite the catheter placement procedure not being consistent with a fixed nasal obstruction. Confirmation of the suspected diagnosis of a nasolacrimal cyst is achievedwith a CATscan. Management can be via nasolacrimal duct probing by an