Pyriform Aperture Stenosis: Difference between revisions
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=== Physical Examination === | === Physical Examination === | ||
Physical exam can be nonspecific aside from nasal congestion / narrow nasal cavities. Key findings can include: | |||
* Difficulty passing nasogastric tubes | |||
* Narrow anterior nasal cavity on flexible or rigid nasal endoscopy | |||
Other key features of midline defects should be considered, as this may present as a part of the holoprosencephaly spectrum. With midline defects, there can be endocrine dysfunction as a part of developmental issues related to the hypothalamic-pituitary axis (HPA). As many as 75% of cases may also present with a solitary median maxillary central incisor (SMMCI). | |||
=== Imaging === | === Imaging === | ||
Revision as of 19:01, 21 July 2025
Overview
Pyriform aperture stenosis is a congenital malformation resulting in a narrowed pyriform aperture and subsequent nasal obstruction.
History
The earliest described case of congenital pyriform aperture stenosis repair in the literature was by Douglas in 1952.[1]
Pathophysiology
Relevant Anatomy
Key anatomy includes the structures of the nasal cavity and midface.
-
Skull with highlighted pyriform aperture
-
Midface anatomy
Disease Etiology
Genetics
There is no consistent genetic variant that has been identified as causative for congenital pyriform aperture stenosis. One study was able to identify genetic variants in two cases: a 2p16.3 deletion and a 7q36 deletion, the latter of which resulted in alterations to the Sonic Hedgehog gene.[2]
Diagnosis
Patient History
It is important to take a focused history related to any airway or feeding symptoms, including:
- Prolonged feeding times
- Needing frequent breaks during feeds
- Cyanosis or increased respiratory effort, especially with feeding
- BRUE's
- Difficulty placing nasogastric tubes
- Family history of genetic conditions with anatomic malformations
Physical Examination
Physical exam can be nonspecific aside from nasal congestion / narrow nasal cavities. Key findings can include:
- Difficulty passing nasogastric tubes
- Narrow anterior nasal cavity on flexible or rigid nasal endoscopy
Other key features of midline defects should be considered, as this may present as a part of the holoprosencephaly spectrum. With midline defects, there can be endocrine dysfunction as a part of developmental issues related to the hypothalamic-pituitary axis (HPA). As many as 75% of cases may also present with a solitary median maxillary central incisor (SMMCI).
Imaging
CT maxillofacial will demonstrate decreased pyriform aperture diameter, especially on axial view. A pyriform aperture width of less than 11 mm is diagnostic for pyriform aperture stenosis.[3] MRI should be considered in these patients in order to assess for other midline variants as a part of the holoprosencephaly spectrum.
Differential Diagnosis
Management
Medical Management
Surgical Management
Outcomes
Complications
Prognosis
References
- ↑ DOUGLAS B. The relief of vestibular nasal obstruction by partial resection of the nasal process of the superior maxilla. Plastic and Reconstructive Surgery. 1952 Jan 1;9(1):42-51.
- ↑ Ruda J, Grischkan J, Allarakhia Z. Radiologic, genetic, and endocrine findings in isolated congenital nasal pyriform aperture stenosis patients. International Journal of Pediatric Otorhinolaryngology. 2020 Jan 1;128:109705.
- ↑ Belden CJ, Mancuso AA, Schmalfuss IM. CT features of congenital nasal piriform aperture stenosis: initial experience. Radiology. 1999 Nov;213(2):495-501.