Congenital epiglottic cyst presenting with severe airway obstruction at birth

Abstract

We report severe airway obstruction and distress at birth, in aterm newborn, caused by a congenital epiglottic cyst.Case reportA 3751 g female infant was born at 41 weeks to a 22-year-oldmother following an uneventful pregnancy. Antenatal ultrasoundscans at 18 and 22 weeks were reported normal. There wasevidence of respiratory distress and severe upper airway obstructionimmediately upon delivery. Direct laryngoscopy for intubationrevealed a white cystic mass in the oropharynx, obstructing thevisualization of the pharynx and, therefore, the glottis as well. Thepediatrician managed to move the mass out of the way, intubateorally and stabilize the airway. The infant was transferred to atertiary center for further management. Examination of the oralcavity revealed a white cystic mass filling the oropharynx.Transnasal fibro-optic laryngoscopy revealed the same but theexact site of origin could not be well delineated. Computedtomography (CT) was performed to rule out an intracranialconnection. The infant underwent surgery at 7 h of life.Examination of the oral cavity was undertaken in the operatingroom to try and establish exactly where the cyst attached andwhether it was broad based or on a stalk. This was difficult due tothe size of the cyst (Figure 1). The cyst was decompressed tofacilitate exposure and a revealed a viscous yellowish fluid. It wasestablished that the cyst was attached in a broad-based fashion tothe left lateral aspect of the epiglottis, into the vallecula, along thefree margin and lateral surface of the aryepiglottic fold and ontothe pharyngo-epiglottic fold.The covering of the cyst was then injected with 1% xylocainewith 1 in 200 000 epinephrine and deroofed. The sidewalls andbase of the cyst were dissected off of their respective underlyingstructures with microlaryngeal instruments utilizing high powermagnification of the microscope.The infant received broad-spectrum antibiotic coverage anddexamethasone in the perioperative period. Postoperative periodwas uneventful and the infant was successfully extubated after48 h. There was no evidence of upper airway obstruction or distresspostextubation and the infant was discharged home on day 4 oflife. At follow-up examination, 1 month postexcision, no evidenceof a recurrence was noted.Histological examination showed soft tissue lined by squamousepithelium, consistent with an epiglottic cyst.DiscussionCongenital laryngeal cysts are rare but may be fatal.