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Congenital Familial Subglottic Stenosis
Author(s) -
Manickavasagam Jaiganesh,
Thevasagayam Ravi,
Bateman Neil D.
Publication year - 2012
Publication title -
otolaryngology–head and neck surgery
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.232
H-Index - 121
eISSN - 1097-6817
pISSN - 0194-5998
DOI - 10.1177/0194599812451426a332
Subject(s) - subglottic stenosis , medicine , stridor , stenosis , presentation (obstetrics) , congenital talipes equinovarus , pediatrics , cricoid cartilage , surgery , larynx , airway , radiology , clubfoot , deformity
Objective Congenital stenosis may be caused by either a small cricoid cartilage or a thick submucosa secondary to a failure of canalization of the subglottic lumen. Acquired subglottic stenosis is caused by trauma. Congenital subglottic stenosis is a well‐known cause of stridor in infancy. It usually occurs sporadically and familial occurrence is rare. Method Case series. Pediatric tertiary referral center.Three children who presented with subglottic stenosis born to consanguineous parents were identified. For the literature research, reports published from 1950 to January 2012 were retrieved. We also screened bibliographies of the collected articles to identify pertinent reports. Results Three Asian children were born with congenital subglottic stenosis for closely related consanguineous parents. Their presentation, family tree, genetic tests, associated features, and treatment will be discussed. All patients underwent laryngotracheal reconstruction. Conclusion Congenital familial subglottic stenosis in unrelated parents has been reported previously but not in consanguineous parents. Family genetic work up is recommended. Further genetic research is needed to assess the mode of possible heritage in these cases.

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