Rare. Anomaly in the development of the laryngeal structures between the 32nd and 41st day of gestation. It can be isolated or associated with a syndrome in which an abnormality of other structures developping during the same gestational period (ear, hand) such as trichorhinophalangeal syndrome, lacrimo-auriculo-dento-digital syndrome, G syndrome, Goldenhar syndrome, acrofacial dysostosis, etc.

-   aplasia: no epiglottic structure is visible at laryngoscopy, absence of valleculae (70 % syndromic)

-   hypoplasia: remnants of a rudimentary epiglottis, visible valleculae (90 % syndromic)

Treatment: in case of major swallowing disorders, a tracheotomy and a gastrostomy are usually necessary.

The mortality is higher in case of aplasia than in case of hypoplasia.

Anesthetic implications:

difficult intubation (atypical laryngoscopy), risk of aspiration; sometimes associated subglottic stenosis; management according to the associated syndrome

References :

-        Rizk HB, Nassar M, Rohayem Z, Rassi SJ.
Hypoplastic epiglottis in a non-syndromic child : a rare anomaly with serious consequences.
Int J Pediatr Otorhinolaryngol 2010; 74: 952-5.

-        Dritsoula A, Thevasagayam MS.
Congenital aplasia/hypoplasia of the epiglottis: a case report and review of the literature.
Int J Pediatr Otorhinolaryngol 2015; 79: 1609-12.

-        Tay SY, Loh WS, Lim TC.
A case report of absent epiglottis in children with Nager syndrome: its impact on swallowing.
Cleft Palate-Craniofacial Journal 2017; 54: 754-7

Updated:  August 2020