Congenital Isolated Agenesis of Epiglottis Presenting with Acute Life-threatening Event Successfully Managed by Temporary Supraglottic Closure and Tracheostomy

Abstract

Isolated agenesis of epiglottis is extremely rare. The exact inci dence is unknown. Anecdotal presentations associated with Pierre Robbin syndrome maxillary hypoplasia have been reported. Age nesis of epiglottis has also been published in an adult. Iso lated agenesis of epiglottis with acute life-threatening event (ALTE) has not been specifically dealt with in the literature. Hence, this is the report of six contiguous cohort of isolated agenesis of epiglottis with ALTE. Materials and methods: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. Conclusion:Isolated agenesis of epiglottis with ALTE is extremely rare. Supraglottic closure saves lives and gives enough time for pharyngoesophageal coordination to develop.

Congenital absent epiglottis causing aspiration in infant

“…Prevention of reflux also plays an important role since aspiration of highly acidic gastric contents will result in further lung injury. Certain cases of severe aspiration may warrant surgical interventions 4 .…”