The surgical management of cricopharyngeal achalasia in children

Abstract

Objective: The objective of this study was to evaluate the clinical presentation of children with cricopharyngeal achalasia and to document the diagnostic process followed and evaluate the immediate and long-term results of those children treated with a cricopharyngeal myotomy.

Methods: Five children who underwent cricopharyngeal myotomies since 1976 were identified and the hospital records were reviewed in detail. Four patients were followed up to establish the long-term results of surgery.

Results: The age of initial presentation ranged from birth to 6 months with a universal delay in establishing the diagnosis ranging from 11 to 138 months. Two children had pre- and post-operative manometry of the upper esophageal sphincter. Post-operative upper esophageal sphincter pressures were reduced to 29 and 47% in relation to pre-operative values. Nissen fundoplications were performed in two patients to control documented gastro esophageal reflux. No post-operative complications were noted and complete symptomatic relief was obtained in all children. The long-term follow up was 2, 10, 12 and 14 years with all children remaining free of symptoms.

Conclusion: Cricopharyngeal achalasia is an important but relatively seldom diagnosed cause of dysphagia in children. The diagnosis is almost always delayed because the condition is not widely recognised amongst physicians. If the diagnosis and effective treatment is delayed significant morbidity or even mortality, mainly due to pulmonary aspiration, may result. Cricopharyngeal myotomy is a safe and effective operation with excellent results. Symptomatic relief is immediate and complete with no long-term recurrence documented in this series.