Achalasia, Studies on Long-Term Outcome

textabstractAchalasia is a rare motility disorder of the esophagus with evidence for an auto-immune etiology as auto-immune thyroid diseases appear more common in patients with achalasia. However this observation has not led to causative treatment. Treatment is still purely symptomatic at lowering the lower esophageal sphincter pressure. Pneumatic dilatation is an effective LES pressure lowering treatment module, however young age, classic achalasia, high LES-pressure 3 months after PD and incomplete obliteration of the balloon's waist are important predictors for the need of repeated treatment and alternative treatment as surgery should in these cases be considered. Patients with achalasia have a considerable risk to develop esophageal carcinoma (HR 28), which is often detected in a late incurable state. Efforts should be made to define those patients with the highest risk, who could benefit from a more intense surveillacne protocol. Long lasting disease, p53 overexpression and inflammation in esophageal surveillance biopsy samples, food stasis at endoscopy and development of Barrett's metaplasia appeared to be independent risk factors. Future research should focus on the best surveillance interval and strategy. The patients with risk factors should be offered a, probably annual, surveillance endoscopy starting 10 years after onset of symptoms. To improve the yield of surveillance endoscopy the esophagus should be properly cleaned by prescribing the patients with food stasis a liquid diet 2-3 days before endoscopy. In case of severe food stasis at endoscopy or severe inflammation in the surveillance biopsy samples retreatment should be considered even in the absece of deterioration of symptoms as these are important risk factors. To prove this strategy, a prospective randomized study is needen but will be hard to perform. Therefore it is important to cluster and follow achalasia patients by a strict protocol, to study the cancer risk as follow-up lengthens and to study the outcome of a more intensive surveillance and treatment protocol