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Radiology Quiz Case 1: Diagnosis

Radiology Quiz Case 1: Diagnosis Diagnosis: Congenital nasal pyriform aperture stenosis (CNPS) Congenital nasal pyriform aperture stenosis, which was first described in 1989, is a rare cause of upper airway obstruction that occurs in newborns.1The pyriform aperture is bounded superiorly by the nasal bones, laterally by the nasal process of the maxilla, and inferiorly by the horizontal process of the maxilla.2The anatomical obstruction created by CNPS is due to excessive growth of the medial nasal process of the maxilla, which leads to narrowing of the bony nasal cavity.3Embryologically, nasal development occurs during the fifth to eighth week of gestation. While the medial nasal prominences are fusing to form the primary palate, the developing maxilla meets the lateral prominence to form the lateral nasal wall and pyriform aperture. It is believed that overgrowth of maxillary ossification results in narrowing of the pyriform aperture.1 Although the clinical data are limited, CNPS is considered to be a mild form of holoprosencephaly, a malformation complex that is believed to result from abnormal cleavage of the prosencephalon during embryonic development. Holoprosencephaly involves a spectrum of craniofacial anomalies that may include median cleft lip, maxillary agenesis, cyclopia, hypotelorism, and solitary maxillary incisor (SMCI).4An important radiographic clue in this case was the presence of SCMI. While SCMI can occur in isolation, it should be recognized that it can occur in association with autosomal dominant holoprosencephaly, growth retardation, and midline developmental anomalies.5While the initial description of CNPS did not include reports of SMCI, subsequent case series have noted its association with CNPS in up to 67% of cases.6,7This finding is generally noted on initial CT scans that are performed when the diagnosis is suspected. Other associated developmental field defects include bifid uvula and pituitary insufficiency. Choanal atresia was also seen in 40% of patients in one series.6 Because newborns are obligate nasal breathers, CNPS generally presents in the first hours of life with respiratory distress, feeding difficulties, cyclic apnea that is relieved by crying, and noisy breathing. Children with severe obstruction may require transfer to the NICU and endotracheal intubation. Congenital nasal pyriform aperture stenosis may not initially be recognized as the cause of the nasal obstruction, especially since choanal atresia and midnasal stenosis have similar presentations. The diagnosis of CNPS is suggested by the inability to pass a pediatric flexible nasopharyngoscope (2.8-mm outside diameter) or a small feeding tube or suction catheter past the pyriform aperture. It is confirmed and distinguished from choanal atresia and midnasal stenosis by CT scans demonstrating substantial narrowing of the pyriform aperture on axial views. In one series of 6 patients aged 0 to 3 months with CNPS,8the average width of the bony pyriform aperture was found to be 4.8 mm compared with 13.4 mm (P < .001) in normal controls. Among older infants with CNPS up to 12 months of age, this same study found that the width of the pyriform aperture was no greater than 6.0 mm. None of the normal controls had widths of less than 11 mm.8Two studies have used 3-dimensional CT scans to assess the width of the pyriform aperture in infants with CNPS. These studies found the average width of the pyriform process in infants with CNPS younger than 4 months to be 4.49and 4.510mm at the narrowest location. Mild cases of CNPS can be managed conservatively using some combination of topical nasal steroids, nasal stenting, upright positioning, and a McGovern nipple for feeding. In infants who continue to be symptomatic, surgical enlargement of the pyriform aperture can be performed through a sublabial approach.1In one review, surgery was required in 43% of the patients in the first week of life. By 1 year, 86% of the paitents had undergone surgery.6 Return to Quiz Case 1. Disclaimer:The views expressed herein are those of the authors and do not necessarily reflect the official policy or position of the Department of the Navy, the Department of Defense, or the United States government. References 1. Brown OEMyer CM IIIManning SC Congenital nasal pyriform aperture stenosis. Laryngoscope 1989;99 (1) 86- 91PubMedGoogle ScholarCrossref 2. Ramadan HHOrtiz O Congenital nasal pyriform aperture (bony inlet) stenosis. Otolaryngol Head Neck Surg 1995;113 (3) 286- 289PubMedGoogle ScholarCrossref 3. Osovsky MAizer-Danon AHorev GSirota L Congenital pyriform aperture stenosis. Pediatr Radiol 2007;37 (1) 97- 99PubMedGoogle ScholarCrossref 4. Krol BJHulka GFDrake A Congenital nasal pyriform aperture stenosis in the monozygotic twin of a child with holoprosencephaly. Otolaryngol Head Neck Surg 1998;118 (5) 679- 681PubMedGoogle Scholar 5. Yassin OMEl-Tal YM Solitary maxillary central incisor in the midline associated with systemic disorders. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1998;85 (5) 548- 551PubMedGoogle ScholarCrossref 6. Losken ABurstein FDWilliams JK Congenital nasal pyriform aperture stenosis: diagnosis and treatment. Plast Reconstr Surg 2002;109 (5) 1506- 1512PubMedGoogle ScholarCrossref 7. Lo FSLee YJLin SPShen EYHuang JKLee KS Solitary maxillary central incisor and congenital nasal pyriform aperture stenosis. Eur J Pediatr 1998;157 (1) 39- 44PubMedGoogle ScholarCrossref 8. Belden CJMancuso AASchmalfuss IM CT features of congenital nasal piriform aperture stenosis: initial experience. Radiology 1999;213 (2) 495- 501PubMedGoogle ScholarCrossref 9. Lee JCYang CCLee KSChen YC The measurement of congenital nasal pyriform aperture stenosis in infant. Int J Pediatr Otorhinolaryngol 2006;70 (7) 1263- 1267PubMedGoogle ScholarCrossref 10. Lee KSYang CCHuang JKChen YCChang KC Congenital pyriform aperture stenosis: surgery and evaluation with three-dimensional computed tomography. Laryngoscope 2002;112 (5) 918- 921PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Otolaryngology - Head & Neck Surgery American Medical Association

Radiology Quiz Case 1: Diagnosis

Archives of Otolaryngology - Head & Neck Surgery , Volume 137 (3) – Mar 21, 2011

Radiology Quiz Case 1: Diagnosis

Abstract

Diagnosis: Congenital nasal pyriform aperture stenosis (CNPS) Congenital nasal pyriform aperture stenosis, which was first described in 1989, is a rare cause of upper airway obstruction that occurs in newborns.1The pyriform aperture is bounded superiorly by the nasal bones, laterally by the nasal process of the maxilla, and inferiorly by the horizontal process of the maxilla.2The anatomical obstruction created by CNPS is due to excessive growth of the medial nasal process of the maxilla,...
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Publisher
American Medical Association
Copyright
Copyright © 2011 American Medical Association. All Rights Reserved.
ISSN
0886-4470
eISSN
1538-361X
DOI
10.1001/archoto.2011.26-b
Publisher site
See Article on Publisher Site

Abstract

Diagnosis: Congenital nasal pyriform aperture stenosis (CNPS) Congenital nasal pyriform aperture stenosis, which was first described in 1989, is a rare cause of upper airway obstruction that occurs in newborns.1The pyriform aperture is bounded superiorly by the nasal bones, laterally by the nasal process of the maxilla, and inferiorly by the horizontal process of the maxilla.2The anatomical obstruction created by CNPS is due to excessive growth of the medial nasal process of the maxilla, which leads to narrowing of the bony nasal cavity.3Embryologically, nasal development occurs during the fifth to eighth week of gestation. While the medial nasal prominences are fusing to form the primary palate, the developing maxilla meets the lateral prominence to form the lateral nasal wall and pyriform aperture. It is believed that overgrowth of maxillary ossification results in narrowing of the pyriform aperture.1 Although the clinical data are limited, CNPS is considered to be a mild form of holoprosencephaly, a malformation complex that is believed to result from abnormal cleavage of the prosencephalon during embryonic development. Holoprosencephaly involves a spectrum of craniofacial anomalies that may include median cleft lip, maxillary agenesis, cyclopia, hypotelorism, and solitary maxillary incisor (SMCI).4An important radiographic clue in this case was the presence of SCMI. While SCMI can occur in isolation, it should be recognized that it can occur in association with autosomal dominant holoprosencephaly, growth retardation, and midline developmental anomalies.5While the initial description of CNPS did not include reports of SMCI, subsequent case series have noted its association with CNPS in up to 67% of cases.6,7This finding is generally noted on initial CT scans that are performed when the diagnosis is suspected. Other associated developmental field defects include bifid uvula and pituitary insufficiency. Choanal atresia was also seen in 40% of patients in one series.6 Because newborns are obligate nasal breathers, CNPS generally presents in the first hours of life with respiratory distress, feeding difficulties, cyclic apnea that is relieved by crying, and noisy breathing. Children with severe obstruction may require transfer to the NICU and endotracheal intubation. Congenital nasal pyriform aperture stenosis may not initially be recognized as the cause of the nasal obstruction, especially since choanal atresia and midnasal stenosis have similar presentations. The diagnosis of CNPS is suggested by the inability to pass a pediatric flexible nasopharyngoscope (2.8-mm outside diameter) or a small feeding tube or suction catheter past the pyriform aperture. It is confirmed and distinguished from choanal atresia and midnasal stenosis by CT scans demonstrating substantial narrowing of the pyriform aperture on axial views. In one series of 6 patients aged 0 to 3 months with CNPS,8the average width of the bony pyriform aperture was found to be 4.8 mm compared with 13.4 mm (P < .001) in normal controls. Among older infants with CNPS up to 12 months of age, this same study found that the width of the pyriform aperture was no greater than 6.0 mm. None of the normal controls had widths of less than 11 mm.8Two studies have used 3-dimensional CT scans to assess the width of the pyriform aperture in infants with CNPS. These studies found the average width of the pyriform process in infants with CNPS younger than 4 months to be 4.49and 4.510mm at the narrowest location. Mild cases of CNPS can be managed conservatively using some combination of topical nasal steroids, nasal stenting, upright positioning, and a McGovern nipple for feeding. In infants who continue to be symptomatic, surgical enlargement of the pyriform aperture can be performed through a sublabial approach.1In one review, surgery was required in 43% of the patients in the first week of life. By 1 year, 86% of the paitents had undergone surgery.6 Return to Quiz Case 1. Disclaimer:The views expressed herein are those of the authors and do not necessarily reflect the official policy or position of the Department of the Navy, the Department of Defense, or the United States government. References 1. Brown OEMyer CM IIIManning SC Congenital nasal pyriform aperture stenosis. Laryngoscope 1989;99 (1) 86- 91PubMedGoogle ScholarCrossref 2. Ramadan HHOrtiz O Congenital nasal pyriform aperture (bony inlet) stenosis. Otolaryngol Head Neck Surg 1995;113 (3) 286- 289PubMedGoogle ScholarCrossref 3. Osovsky MAizer-Danon AHorev GSirota L Congenital pyriform aperture stenosis. Pediatr Radiol 2007;37 (1) 97- 99PubMedGoogle ScholarCrossref 4. Krol BJHulka GFDrake A Congenital nasal pyriform aperture stenosis in the monozygotic twin of a child with holoprosencephaly. Otolaryngol Head Neck Surg 1998;118 (5) 679- 681PubMedGoogle Scholar 5. Yassin OMEl-Tal YM Solitary maxillary central incisor in the midline associated with systemic disorders. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1998;85 (5) 548- 551PubMedGoogle ScholarCrossref 6. Losken ABurstein FDWilliams JK Congenital nasal pyriform aperture stenosis: diagnosis and treatment. Plast Reconstr Surg 2002;109 (5) 1506- 1512PubMedGoogle ScholarCrossref 7. Lo FSLee YJLin SPShen EYHuang JKLee KS Solitary maxillary central incisor and congenital nasal pyriform aperture stenosis. Eur J Pediatr 1998;157 (1) 39- 44PubMedGoogle ScholarCrossref 8. Belden CJMancuso AASchmalfuss IM CT features of congenital nasal piriform aperture stenosis: initial experience. Radiology 1999;213 (2) 495- 501PubMedGoogle ScholarCrossref 9. Lee JCYang CCLee KSChen YC The measurement of congenital nasal pyriform aperture stenosis in infant. Int J Pediatr Otorhinolaryngol 2006;70 (7) 1263- 1267PubMedGoogle ScholarCrossref 10. Lee KSYang CCHuang JKChen YCChang KC Congenital pyriform aperture stenosis: surgery and evaluation with three-dimensional computed tomography. Laryngoscope 2002;112 (5) 918- 921PubMedGoogle ScholarCrossref

Journal

Archives of Otolaryngology - Head & Neck SurgeryAmerican Medical Association

Published: Mar 21, 2011

Keywords: radiology specialty,nose,constriction, pathologic

References

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