Congenital cricopharyngeal achalasia in a 4.5-year-old managed by cervical myotomy: a case report.

INTRODUCTION: Congenital cricopharyngeal achalasia (CCA) is a rare disorder in children characterized by inappropriate contraction of the cricopharyngeus muscle, resulting in the inability to relax the upper esophageal sphincter during deglutition. We report the diagnostic process and management of a relatively older patient who underwent cricopharyngeal myotomy at the age of 4.5 years. METHODS: A retrospective review of the case and clinical follow-up was performed. RESULTS: This young patient had a long history of dysphagia, choking, nasal reflux and recurrent pneumonia and croup since birth and was diagnosed with CCA at 22 months of age. She underwent balloon dilation of the cricopharyngeus muscle shortly thereafter with only transient relief of her symptoms of feeding difficulty (choking and aspiration). The parents were reluctant for her to undergo further interventions until 2 years later when they consented to cricopharyngeal myotomy. She underwent transcervical myotomy at age 4.5 years and had complete relief of her symptoms. She had no post-operative complications and has done well for nearly 12 months following myotomy. DISCUSSION: Our patient is one of the oldest children reported to have undergone myotomy, recovered quickly, and had no difficulty swallowing at any time following surgery. We suggest transcervical cricopharyngeal myotomy as the preferred treatment due to its lasting effects and repeated success in relieving dysphagia in young patients with CCA.