Özlem Boybeyi TÜRER, Numan DEMİR, Thomas CİECİERAGA, Rıza Önder GÜNAYDIN, Tutku SOYER

Assessment of pediatric cricopharyngeal achalasia with high resolution manometry

Cricopharyngeal achalasia (CPA) is an uncommon cause of oropharyngealdysphagia (OPD) which is the failure of upper esophageal sphincter (UES)to relax during bolus passage. The diagnostic challenges in OPD have beenovercome with the use of high resolution manometry (HRM) in childrenwhere a catheter based biomechanical evaluation testing of the oropharyngealswallowing is performed. Herein, we present a case with severe dysphagiadiagnosed as CPA utilizing HRM testing. An 8-year-old boy was seen in ourclinic with a two-year history of difficult swallowing, recurrent respiratorytract infections, hypoxia and seizure secondary to aspiration. Esophagographyrevealed an indentation of the cricopharangeal muscle (CPM) in the cervicalpart of the esophagus. Videofluroscopic swallow studies (VFSS) revealedcricopharyngeal bar at level of C5-6 and diffuse dysmotility in esophagus.Conventional esophageal manometry revealed absence of peristaltic activitythroughout the esophagus. Esophagogastroduodenoscopy revealed narrowingin upper esophagus that with applied force allowed passage of the endoscope.The patient underwent UES dilatation 6 times. He had temporary relief ofsymptoms. Since he did not have sustained response to dilatation, a botuliniumtoxin (5IU/each quadrant) injection (BTI) to CPM was performed twice. Hissymptoms recurred at the end of 3 months. HRM was performed revealingweakness of CPM and uncoordinated contractions and relaxations in UES. Wecould not reliably differentiate if HRM findings were a sign of primary illnessor secondary to BTI. Swallowing rehabilitation was adjusted based on HRMfindings. He is still under follow-up with mild dysphagia to certain solids.CPA is an uncommon cause of dysphagia with limited therapeutic options.HRM should be performed in OPD before considering any treatment modalityincluding esophageal dilatation and BTI. HRM has become gold standarddiagnostic tool in OPD that provides objective evaluation of pharyngeal andUES motility in children.

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