J Rhinol.  2019 Nov;26(2):117-121. 10.18787/jr.2019.26.2.117.

A Case of Congenital Nasal Pyriform Aperture Stenosis

• Lee J
• Choi SY
• Choi MS

• KMID: 2465544
• DOI: http://doi.org/10.18787/jr.2019.26.2.117

Abstract

Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of upper airway obstruction in the newborn. This affliction is due to bony overgrowth of the nasal process of the maxilla. CNPAS is diagnosed clinically and confirmed with CT scan. Patients can be managed conservatively or surgically. In this report, a male neonate had respiratory distress, cyclic cyanosis, and apnea after delivery. The patient underwent surgical correction of pyriform stenosis with a transnasal approach. During follow up, the patient showed recurred respiratory distress and cyanosis. Revision operation was necessary to reduce the bony inferior turbinate and pyriform aperture. CNPAS should be suspected in newborns with clinical signs of severe nasal obstruction associated with difficulty passing a small catheter through the anterior nares.

Keyword

Holoprosencephaly; Nasal obstruction; Newborn; Pyriform; Respiratory distress

MeSH Terms

Airway Obstruction
Apnea
Catheters
Constriction, Pathologic*
Cyanosis
Follow-Up Studies
Holoprosencephaly
Humans
Infant, Newborn
Male
Maxilla
Nasal Obstruction
Tomography, X-Ray Computed
Turbinates

Figure

• 

  Fig. 1. Radiology of congenital nasal pyriform aperture stenosis. (A) Axial view of facial CT image, note that narrow width of nasal pyr-iform aperture. a black arrowhead indicates nasal pyriform aperture (B) Coronal view of facial CT image, note that narrow width of nasal pyriform aperture. a white arrowhead indicates nasal pyriform aperture (C) MRI demonstrates associated anomalies with con-genital nasal pyriform aperture stenosis, a white arrow indicates a single median maxillary central incisor and (D) MRI demonstrates as-sociated anomalies with congenital nasal pyriform aperture stenosis, a black arrow indicates a monoventricle which can be seen in lobar holoprosencephaly.

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  Fig. 2. Intraoperative findings of pyriform aperture stenosis. A: A single median maxillary central incisor was seen in upper gingiva. B: Making an incision on the nasal vestibular skin above pyriform aperture. C: Lt. nasal process of the maxilla was identified and drilled out. Black arrows indicate remnant nasal process of the maxilla. D: Left inferior turbinate was identified and drilled out. White arrows indicate attachment of inferior turbinate on the lateral wall in the right side.

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  Fig. 3. Nostrils of newborn. A, B: Nasopharyngoscope images, which were taken 17days after first operation. Narrow pyriform aperture and hypertrophied inferior turbinates were seen. C, D: Nasopharyngoscope images, which were taken 40days after second operation. Note that widened anterior nares.

Reference

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