Abstract
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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