Symptomatic Congenital Vallecular Cyst in a Neonate

Cheng, Sylvia S.; Forte, Vito; Shah, Vibhuti S.

doi:10.1016/j.jpeds.2009.01.076

« Previous Next »The Journal of Pediatrics
Volume 155, Issue 3 , Page 446, September 2009

Symptomatic Congenital Vallecular Cyst in a Neonate

Pediatric Resident, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada

The Hospital for Sick Children, Department of Otolaryngology-Head and Neck Surgery, University of Toronto

Department of Paediatrics, Mount Sinai Hospital, Department of Paediatrics, Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada

A term infant was born by precipitous vaginal delivery to a multiparous woman. From birth, the infant presented with respiratory distress and an inspiratory stridor requiring commencement of continuous positive airway pressure. The stridor, respiratory distress, and cyanosis were more prominent when the infant was agitated. The clinical examination result was normal except for edema of the face and bruising. A direct laryngoscopy was performed and showed the presence of a vallecular cyst (Figure, A). The patient then underwent a formal laryngoscopic examination and cyst removal with CO₂ laser therapy (Figure, B). No other anomalies were noted. The infant was successfully extubated, feeding was established, and the patient was discharged by 3 weeks of age.

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Figure.
A, Laryngeal endoscopy view shows (A) vallecular cyst and (B) endotracheal tube. B, Surgical bed after removal of cyst and lining with CO₂ laser micro dissection (A) and (B) endotracheal tube.

Vallecular cyst is a rare cause of an upper airway obstruction in infants and children.¹ It usually presents with stridor, feeding difficulties, and respiratory distress, as well as rarely death caused by supraglottic obstruction that is due to mass effect.¹ The precise incidence is unknown.² It arises from the lingual surface of the epiglottis and histologically contains respiratory epithelium with mucous glands with an external lining of squamous epithelium.¹ It is believed to occur as a result of either ductal obstruction of mucous glands or an embryologic malformation. Vallecular cysts are rarely associated with fetal anomalies. However, polyhydramnios, pulmonary hypoplasia, trachea, cervical vessels, and hypoglossal nerve compression have been described.³ Antenatal diagnosis of this condition with ultrasound scanning and magnetic resonance imaging have been reported and would facilitate appropriate counseling and preparation at delivery.³ During birth, the placental cord can also be left undivided until the airway is secured.³ However, postnatally, direct laryngoscopy remains the best diagnostic tool.³ If the airway is compromised, cyst aspiration can improve access for intubation. Definitive treatment involves either wide marsupialization of the cyst or complete removal of the cyst lining with a transoral approach, with no long-term consequences.¹, ³

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References

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Ahrens B, Lammert I, Schmitt M, Wahn U, Paul K, Niggemann B. Life-threatening vallecular cyst in a 3-month-old infant: case report and literature review. Clin Pediatr. 2004;43:287–290
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