Specialty: Pediatric Airway

Introduction:

Cricopharyngeal dysfunction (CPD) is a spectrum disorder encompassing multiple entities that ultimately result in dysphagia as a result of disruption of the normal anatomy or physiology of upper esophageal sphincter. It is a known and well described cause of dysphagia in adults, however, it’s role in pediatric dysphagia is less clear and limited to mostly small case series.1 Despite it’s relatively low prevalence, the complex pediatric otolaryngologist must be aware of this entity and it’s management. We discuss a complex case of CPD with an associated cricopharyngeal bar and pharyngeal diverticulum, as well as our successful endoscopic surgical approach highlighting the principles of CPD management in children.

Case Presentation:

We present a 21 month of female with a history of DiGeorge Syndrome and oropharyngeal dysphagia. Despite appropriate conservative measures including feeding therapy and diet thickening modification, as well as attempted Botox injection, the patient continued to demonstrate dysphagia. It was also noted on her swallow study that she had a posteriorly based pharyngeal diverticulum that potentially served as an aspiration reservoir. The decision was made to proceed with endoscopic cricopharyngeal division and diverticulum marsupialization.  

Technique:

With the patient intubated, a Lindholm laryngoscope was placed posteriorly into the hypopharynx, elevating the larynx and allowing visualization of the upper esophageal sphincter and isolation of the cricopharyngeal bar. A non- contact CO2 laser fiber at 2W continuous spray was then used to divide the cricopharyngeal bar layer by layer making sure to isolate the muscle and not create a pharyngotomy. Standard laser safety precautions were followed. Tension was maintained using a right-angle hook allowing for optimal laser division. This was continued until the entirety of the bar was divided. At this point, the posterior pharyngeal diverticulum was identified. Again, with the use of a right angle probe for traction and depth assessment, The anterior wall of the diverticulum was divided. This was continued until the diverticulum was fully marsupialized and in continuity with the posterior pharyngeal wall into the esophageal inlet.

Post operatively the patient was extubated and observed overnight in the hospital

Swallow study three weeks later demonstrated normalization of the flow of bolus through the UES as well as resolution of the previously seen diverticulum.

Conclusion:

Cricopharyngeal Dysfunction (CPD) is an uncommon but recognized cause of pediatric dysphagia with multiple treatment options of varying success. Endoscopic CO2 laser division is a viable and effective treatment option for this condition.