Alan T L Cheng1, Edward J Beckenham. 1. The Children's Hospital at Westmead, Hawkesbury Road, Westmead NSW 2145, Sydney, Australia. atlcheng@bigpond.com
Abstract
UNLABELLED: Four children with severe congenital anterior glottic webs required surgical reconstruction of their laryngeal airway to either avoid a tracheotomy or allow tracheotomy decannulation. The technique of re-establishing a glottic inlet allows both normal respiration, good cough and a satisfactory voice outcome. It utilizes an autogenous graft with perichondrium acting as an overlay keel to minimize glottic web reformation. All children were found to have Shprintzen syndrome. OBJECTIVE: The objective of this case series was to document the method of surgically using this perichondrial keel for airway reconstruction. It also examined the subsequent outcome and associated complications that were encountered. METHODS: A prospective analysis of four cases from 2001 to 2008 created a database of information. All cases were classified using the Cohen staging system. They were treated with the same surgical technique using auricular or costal cartilage graft with attached perichondrium, but the postoperative course was tailored to each individual case. RESULTS: All four children were successfully treated with removal or avoidance of a tracheostomy. All had an associated subglottic stenosis treated, and had no major complication requiring revision tracheotomy. They did not have any respiratory complications, and they produce a satisfactory voice albeit still slightly husky. CONCLUSION: All four cases had Shprintzen syndrome, and confirms the need to screen for VCFS in children with an anterior glottic web. Tracheotomy is still the gold standard of treatment in severe congenital anterior glottic webs. The described technique offers another good option to the paediatric airway surgeon in managing this condition.
UNLABELLED: Four children with severe congenital anterior glottic webs required surgical reconstruction of their laryngeal airway to either avoid a tracheotomy or allow tracheotomy decannulation. The technique of re-establishing a glottic inlet allows both normal respiration, good cough and a satisfactory voice outcome. It utilizes an autogenous graft with perichondrium acting as an overlay keel to minimize glottic web reformation. All children were found to have Shprintzen syndrome. OBJECTIVE: The objective of this case series was to document the method of surgically using this perichondrial keel for airway reconstruction. It also examined the subsequent outcome and associated complications that were encountered. METHODS: A prospective analysis of four cases from 2001 to 2008 created a database of information. All cases were classified using the Cohen staging system. They were treated with the same surgical technique using auricular or costal cartilage graft with attached perichondrium, but the postoperative course was tailored to each individual case. RESULTS: All four children were successfully treated with removal or avoidance of a tracheostomy. All had an associated subglottic stenosis treated, and had no major complication requiring revision tracheotomy. They did not have any respiratory complications, and they produce a satisfactory voice albeit still slightly husky. CONCLUSION: All four cases had Shprintzen syndrome, and confirms the need to screen for VCFS in children with an anterior glottic web. Tracheotomy is still the gold standard of treatment in severe congenital anterior glottic webs. The described technique offers another good option to the paediatric airway surgeon in managing this condition.