Pyriform Aperture Stenosis - Revision history

Jack.Dewey: /* Disease Etiology */

2025-07-21T19:11:00Z

Disease Etiology

← Older revision		Revision as of 19:11, 21 July 2025
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	=== Disease Etiology ===		=== Disease Etiology ===
			Congenital pyriform aperture stenosis is a consequence of failed recanalization of the nasal cavity during development. After the anterior nares are formed from fusion of the lateral and medial nasal processes (at approximately week 8 of gestation), the nasal cavity is blocked by an epithelial plug. After approximately 24 weeks, this epithelial plug is supposed to resorb, resulting in a patent nasal airway. It is though that failure of this process results in a stenotic pyriform aperture at birth.<ref>Papesch E, Papesch M. The nasal pyriform aperture and its importance. Otorhinolaryngol Head Neck Surg. 2016;1(4):89-91.</ref>

	=== Genetics ===		=== Genetics ===

Jack.Dewey: /* Physical Examination */

2025-07-21T19:04:12Z

Physical Examination

← Older revision		Revision as of 19:04, 21 July 2025
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	* Narrow anterior nasal cavity on flexible or rigid nasal endoscopy		* 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~~).		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 maxillary central incisor (SMCI).<ref>Lo FS, Lee YJ, Lin SP, Shen EY, Huang JK, Lee KS. Solitary maxillary central incisor and congenital nasal pyriform aperture stenosis. European journal of pediatrics. 1998 Dec;157(1):39-44.</ref>

	=== Imaging ===		=== Imaging ===

Jack.Dewey at 19:01, 21 July 2025

2025-07-21T19:01:47Z

← Older revision		Revision as of 19:01, 21 July 2025
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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 ===

Jack.Dewey at 13:24, 21 July 2025

2025-07-21T13:24:21Z

← Older revision		Revision as of 13:24, 21 July 2025
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	=== Genetics ===		=== 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.<ref>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.</ref>

	== Diagnosis ==		== Diagnosis ==
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	=== Imaging ===		=== 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.<ref>Belden CJ, Mancuso AA, Schmalfuss IM. CT features of congenital nasal piriform aperture stenosis: initial experience. Radiology. 1999 Nov;213(2):495-501.</ref>		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.<ref>Belden CJ, Mancuso AA, Schmalfuss IM. CT features of congenital nasal piriform aperture stenosis: initial experience. Radiology. 1999 Nov;213(2):495-501.</ref> MRI should be considered in these patients in order to assess for other midline variants as a part of the holoprosencephaly spectrum.

	=== Differential Diagnosis ===		=== Differential Diagnosis ===

Jack.Dewey at 13:14, 21 July 2025

2025-07-21T13:14:44Z

← Older revision		Revision as of 13:14, 21 July 2025
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	{{infobox Disease		{{infobox Disease
	\|Title =		\|Title =
	\|Aliases =		\|Aliases = Congenital Nasal Pyriform Aperture Stenosis (CNPAS)
	\|Image = [[Human skull with piriform aperture circled.png]]		\|Image = [[Human skull with piriform aperture circled.png]]
	\|Caption =		\|Caption =
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	=== History ===		=== History ===
	---		The earliest described case of congenital pyriform aperture stenosis repair in the literature was by Douglas in 1952.<ref>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.</ref>

	== Pathophysiology ==		== Pathophysiology ==

Jack.Dewey: /* Differential Diagnosis */

2025-07-21T02:15:33Z

Differential Diagnosis

← Older revision		Revision as of 02:15, 21 July 2025
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	=== Differential Diagnosis ===		=== Differential Diagnosis ===
	* Choanal Atresia		* [[Choanal Atresia\|Choanal Atresia]]

	== Management ==		== Management ==

Jack.Dewey: Created page with "{{infobox Disease |Title = |Aliases = |Image = Human skull with piriform aperture circled.png |Caption = |ICD-9 = 748.0 |ICD-10 = Q30.8 |MeSH = |Gene = |Locus = |OMIM = |EyeWiki = |Radiopaedia = [https://radiopaedia.org/articles/pyriform-aperture-stenosis?lang=us Pyriform Aperture Stenosis] |Pathology = }}..."

2025-07-21T02:13:48Z

Created page with "{{infobox Disease |Title = |Aliases = |Image = Human skull with piriform aperture circled.png |Caption = |ICD-9 = 748.0 |ICD-10 = Q30.8 |MeSH = |Gene = |Locus = |OMIM = |EyeWiki = |Radiopaedia = [https://radiopaedia.org/articles/pyriform-aperture-stenosis?lang=us Pyriform Aperture Stenosis] |Pathology = }}..."

New page

{{infobox Disease
|Title =
|Aliases =
|Image = [[Human skull with piriform aperture circled.png]]
|Caption =
|ICD-9 = 748.0
|ICD-10 = Q30.8
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki =
|Radiopaedia = [https://radiopaedia.org/articles/pyriform-aperture-stenosis?lang=us Pyriform Aperture Stenosis]
|Pathology =
}}

== Overview ==
'''Pyriform aperture stenosis''' is a congenital malformation resulting in a narrowed pyriform aperture and subsequent nasal obstruction.

=== History ===
---

== Pathophysiology ==
=== Relevant Anatomy ===
Key anatomy includes the structures of the nasal cavity and midface.
<gallery>
Human skull with piriform aperture circled.png | Skull with highlighted pyriform aperture
Slide2hal.JPG | Midface anatomy
</gallery>

=== Disease Etiology ===

=== Genetics ===

== 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 ===

=== 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.<ref>Belden CJ, Mancuso AA, Schmalfuss IM. CT features of congenital nasal piriform aperture stenosis: initial experience. Radiology. 1999 Nov;213(2):495-501.</ref>

=== Differential Diagnosis ===
* Choanal Atresia

== Management ==
=== Medical Management ===
=== Surgical Management ===

== Outcomes ==
=== Complications ===
=== Prognosis ===

== References ==
<references />