Pediatric cricopharyngeal achalasia: A systematic review

Pediatric dysphagia is a commonly encountered clinical problem, with primary cricopharyngeal achalasia being a rare etiology. Management options for this condition include observation, medical and surgical management. The goal of this review paper was to summarize the current literature on the topic with respect to patient presentation, diagnosis and surgical management options.

The inclusion criteria were as follows: pediatric patients less than age 18 years, case series featuring equal to or greater than two patients, and any article that described surgical interventions pertaining to primary cricopharyngeal achalasia. Exclusion criteria included patients greater than 18 years of age, those with lower esophageal sphincter pathology (achalasia), non-English articles and case reports consisting of only one patient. A search was run on the PubMed/Medline, OVID, EMBASE, Cochrane, and Web of Science databases on November 1, 2023. The validated bias tool from the Institute of Health Economics was used for bias screening. The results were synthesized using pooled analysis and descriptive statistics.

Ten case series were identified that met inclusion criteria. The most common symptoms at presentation were choking/regurgitation, aspiration and failure to thrive. The most utilized diagnostic test that identified cricopharyngeal achalasia abnormality was the video fluoroscopic swallow study. Management options are dictated by the patient's symptoms and include observation, treating concomitant co-morbidities, balloon dilation, botulinum injection, and endoscopic or open cricopharyngeal myotomies. While botulinum toxin injections are reported to be effective, they often need to be repeated and thus offer a temporizing strategy to allow the natural history of the condition to declare itself. Surgical myotomy represents a more definitive management strategy compared to balloon dilation but has associated risks.

There are limited studies available regarding the diagnosis and management of CPA in children. The studies that are available are mainly case series, with low sample sizes and heterogeneous data. This systematic review highlights the importance of keeping this diagnosis within the differential for infants and pediatric patients with dysphagia to minimize delays in diagnosis and provides updated data on the presentation and surgical management of this condition. There was a limitation of evidence in this systematic review, including the small number of articles that were identified, and limited sample size of patients within the articles themselves. Consequently, the analysis was not amenable to a meta-analysis.