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Gardener-associated fibroma: an unusual cause of upper airway obstruction.

Authors :
Pinto RS
Simons A
Verma R
Bateman N
Source :
BMJ case reports [BMJ Case Rep] 2018 Sep 28; Vol. 2018. Date of Electronic Publication: 2018 Sep 28.
Publication Year :
2018

Abstract

We present the first case of upper airway obstruction secondary to a retropharyngeal Gardner-associated fibroma (GAF). A 16-month-old infant presented with a 3-month history of worsening dyspnoea and apnoeic episodes. Examination revealed stridor and left-sided retropharyngeal asymmetry. MRI demonstrated a mass in the retropharynx. Tracheostomy and pharyngeal biopsy under anaesthesia were performed, and histology confirmed a diagnosis of GAF. The mass was excised using a transcervical approach, and postoperative recovery was unremarkable. GAF is associated with Gardner's syndrome (GS) and familial adenomatous polyposis (FAP), both of which are associated with multiple colonic polyps and increased risk of colorectal malignancy. Subsequent testing for an APC mutation seen in GS and FAP was negative in our patient. The details of this unusual presentation of a rare disease are given in addition to a review of the literature.<br />Competing Interests: Competing interests: None declared.<br /> (© BMJ Publishing Group Limited 2018. No commercial re-use. See rights and permissions. Published by BMJ.)

Details

Language :
English
ISSN :
1757-790X
Volume :
2018
Database :
MEDLINE
Journal :
BMJ case reports
Publication Type :
Academic Journal
Accession number :
30269086
Full Text :
https://doi.org/10.1136/bcr-2018-225079