Pyriform Aperture Stenosis

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

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.^[2]

Differential Diagnosis

Choanal Atresia

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

[1] 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.

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

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