Summary

 Background: Cricopharyngeal achalasia (CA) is a rare cause of dysphagia in children presenting with non-specific symptoms such as choking, food regurgitation, nasal reflux, coughing, recurrent pneumonia, cyanosis, and failure to thrive. It results from failure of relaxation of the upper esophageal sphincter (UES) and may appear either as an isolated lesion or in conjunction with other pathologies. Recognition and early diagnosis of this condition may minimize children's morbidity.

Objectives: To evaluate the clinical course of four children with cricopharyngeal achalasia presenting to our clinic.

Methods: We conducted a 5 year retrospective chart review in a tertiary referral center.

Results: Four children were diagnosed with primary cricopharyngeal achalasia between 2006 and 2010. Diagnosis was established by videofluoroscopy and all underwent uneventful cricopharyngeal myotomy. Three children recovered completely and one child showed partial improvement. For residual UES spasm in a partially improved patient, botulinum toxin was injected into the UES which led to further improvement. Dysphagia recurred in one child who was successfully treated with botulinum toxin injection.

Conclusions: Cricopharyngeal myotomy is a safe procedure in infants and young children. Botulinum toxin injection of the UES was found to be effective in refractory cases.