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Laryngotracheoplasty for subglottic stenosis in Down Syndrome children: the Cincinnati experience
M E Boseley1, D T Link, S R Shott
1Department of Pediatric Otolaryngology, Children's Hospital Medical Center, Burnet Ave., Cincinnati, OH 45229, USA.
Insights
Children with Down Syndrome have a higher prevalence of subglottic stenosis (SGS). This study found an increased incidence of posterior glottic stenosis (PGS) in this population, though surgical outcomes are improving.
Area of Science:
- Pediatric Otolaryngology
- Genetics and Rare Diseases
Background:
- Subglottic stenosis (SGS) surgical repair presents challenges in children with Down Syndrome.
- Down Syndrome is associated with a higher prevalence of SGS compared to the general pediatric population.
Purpose of the Study:
- To update surgical outcomes for laryngotracheoplasty in children with Down Syndrome over 15 years.
- To compare these outcomes with the overall patient series.
- To report the incidence of posterior glottic stenosis (PGS) in this specific group.
Main Methods:
- Retrospective review of medical records for children with SGS and Down Syndrome (1982-1997).
- Data collected included prior intubation, tracheotomy, GERD, SGS grade, and decannulation status.
Main Results:
- Risk factors for SGS in Down Syndrome patients are similar to the general population.
- SGS remains more prevalent in children with Down Syndrome.
- A higher rate of posterior glottic stenosis (PGS) was observed in Down Syndrome patients compared to the overall series.
- Decannulation rates in Down Syndrome children are approaching those of the overall series.
Conclusions:
- SGS is a significant concern in children with Down Syndrome.
- The increased incidence of PGS requires specific attention in surgical planning.
- Surgical management for SGS in this population shows improving results, nearing those of the general pediatric population.
Abstract:
Previous studies from our institution have noted difficulties in the surgical repair of subglottic stenosis (SGS) in children with Down Syndrome. The objectives of this paper were to update our 15 year experience in the Down Syndrome patient population, compare our results with our overall series of laryngotracheoplasty for SGS, and to report on the increased incidence of posterior glottic stenosis (PGS) within this group of patients. Medical records of all children with SGS and Down Syndrome evaluated between 1982 and 1997 were reviewed for history of prior intubation, tracheotomy, gastroesophageal reflux disease (GERD), pre-operative SGS grade, and decannulation. From this review several conclusions have been drawn. First, the risk factors for SGS appear to be the same in the Down Syndrome group as the general population. Second, SGS continues to be more prevalent among children with Down Syndrome than among children in the general population. Third, we have now found a higher rate of PGS within these patients when compared to our overall series. Finally, it now seems that our decannulation rates in Down Syndrome children are approaching our overall series results.