Abstract
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 11 mm 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, 2 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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