The surgical management of cricopharyngeal achalasia in children

Objective: The objective of this study was to evaluate the clinical present ation of children with cricopharyngeal achalasia and to document the diagno stic process followed and evaluate the immediate and long-term results of t hose children treated with a cricopharyngeal myotomy. Methods: Five childre n who underwent cricopharyngeal myotomies since 1976 were identified and th e 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 establ ishing the diagnosis ranging from 11 to 138 months. Two children had pre- a nd post-operative manometry of the upper esophageal sphincter. Post-operati ve upper esophageal sphincter pressures were reduced to 29 and 47% in relat ion to pre-operative values. Nissen fundoplications were performed in two p atients to control documented gastro esophageal reflux. No post-operative c omplications 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 chil dren remaining free of symptoms. Conclusion: Cricopharyngeal achalasia is a n 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 a spiration, may result. Cricopharyngeal myotomy is a safe and effective oper ation with excellent results. Symptomatic relief is immediate and complete with no long-term recurrence documented in this series. (C) 2000 Elsevier S cience Ireland Ltd. All rights reserved.