After the first case of isolated agenesis of epiglottis was diagnosed 2 years ago, five more such cases were treated by the group. These formed the index cases of the cohort.

Decision for surgery was based on an arbitrary 3 ALTEs in 24 hours. All the patients were initially intubated. Supraglottic space was closed using interrupted unabsorbable sutures. Tracheostomy was added. At 3 to 6 months of follow-up, glottis was restored by Nd-YAG laser knife. Five out of six children are alive and thriving well. One child died before he could be actively managed.

Isolated agenesis of epiglottis with ALTE is extremely rare. Supraglottic closure saves lives and gives enough time for pharyngoesophageal coordination to develop.

Prasad RG. Congenital Isolated Agenesis of Epiglottis Presenting with Acute Life-threatening Event Successfully Managed by Temporary Supraglottic Closure and Tracheostomy. Int J Phonosurg Laryngol 2015;5(1):1-3.