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Case Reports
. 2012 Jun 26:38:28.
doi: 10.1186/1824-7288-38-28.

Congenital nasal pyriform aperture stenosis: diagnosis and management

Enrico Sesenna  1 , Massimiliano Leporati, Bruno Brevi, Gabriele Oretti, Andrea Ferri
Affiliations
Case Reports

Congenital nasal pyriform aperture stenosis: diagnosis and management

Enrico Sesenna et al. Ital J Pediatr. .

Abstract

Background: Congenital nasal pyriform aperture stenosis is a rare and potentially lethal form of airway obstruction in newborns. Immediate recognition and appropriate therapy are mandatory for this potentially life-threating condition. This anomaly may present as an isolated malformation or may be associated with other cranial-facial anomalies. Clinically, CNPS shows unspecific symptoms of nasal airway obstruction such as apnoic crisis, episodic cyanosis and inability to nurse. The purpose of this report is to present author's experience in the surgical management of this rare pathology in 3 patients.

Patients and methods: Three cases of congenital nasal pyriform aperture stenosis were reviewed for presentation of the disorder, management and effectiveness of the surgical treatment.

Results: All the patients underwent a surgical correction of the pyriform aperture stenosis using a sublabial approach followed by nasal stenting. During the follow-up no cases of restenosis, respiratory failure or cyanosis were detected.

Conclusions: Congenital pyriform aperture stenosis should be suspected in newborns with clinical signs of severe nasal obstruction associated with a difficulty to pass a small catheter though the anterior nasal valve. Timely recognition is mandatory to prevent a potential deadly outcome. Surgical correction of the stenosis though a sublabial approach followed by a nasal stenting revealed to be most effective treatment for these patients.

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Figures

Figure 1
Figure 1
Pre-operative CT scan showing the nasal pyriform aperture stenosis.
Figure 2
Figure 2
Intra-operative picture showing the enlargement of the pyriform aperture obtained with surgery.
Figure 3
Figure 3
Post-operative picture showing the nasal silastic stents applied fixed to the columella.
Figure 4
Figure 4
Pre-operative 3D-TC scan (a) showing the stenosis of the nasal pyriform aperture and the post-operative 3D-CT-scan (b) of the same patient showing the enlargement obtained.
Figure 5
Figure 5
Post-operative picture showing the application of nasal conformers routinely applied in cleft patients.
See this image and copyright information in PMC

References

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