Series of congenital vallecular cysts: a rare yet potentially fatal cause of upper airway obstruction and failure to thrive in the newborn

Abstract

Objectives: Stridor is a relatively common symptom during the neonatal period. The most probable cause of inspiratory stridor and supralaryngeal airway obstruction in infancy is laryngomalacia. Laryngeal cysts are known to be found in association with supraglottic prolapse and are a rare yet potentially lethal cause of respiratory distress in the newborn. In the absence of more alarming presenting signs, the vallecular cyst, a form of laryngeal cyst, frequently defies diagnosis. We present a series of illustrative cases to raise awareness of vallecular cyst to help reduce the therapeutic delays that are currently encountered clinically.

Methods: A retrospective case series of four patients was reviewed. Each patient presented initially with stridor and additional signs of upper airway obstruction shortly after birth and was later diagnosed with vallecular cyst.

Results: The predominant presenting signs were stridor (four cases), signs of respiratory distress (three cases), failure to thrive (three cases), poor feeding (two cases), and cyanotic spells (one case). Age at presentation ranged from 16 days to 8 months. A definitive diagnosis was achieved by flexible laryngoscopy in all four cases. Coexistent laryngomalacia was found in three of the four cases. Primary outcomes at 1 month following marsupialization were complete remission in all four cases.

Conclusions: The challenge in making an early diagnosis of vallecular cyst, especially when laryngomalacia is comorbid, has been reaffirmed in our case series. Diagnosis requires a high index of clinical suspicion in combination with careful inspection of the tongue base with direct examination and/or appropriate imaging modalities.