Pyriform Aperture Stenosis Repair in Infants

Publication date: Available online 9 March 2018
Source:Operative Techniques in Otolaryngology-Head and Neck Surgery
Author(s): Johnathan E. Castaño, David H. Chi
Congenital nasal pyriform aperture stenosis is a rare developmental anomaly, which is considered a variant of holoprosencephaly. It is characterized by a pyriform aperture width of 11mm or less on maxillofacial computed tomography, and is frequently associated with a solitary median maxillary central incisor. It presents with cyclical cyanosis, feeding difficulty, and respiratory distress. Conservative management includes nasal decongestant, saline and steroid drops, or a McGovern nipple. When conservative management fails, surgical intervention is indicated. Various methods of surgical repair have been described, two of which – nasal dilation and bone resection via a sublabial approach - are described in this article. The role of postoperative stenting is also discussed.



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