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Esophageal achalasia of unknown etiology in children Pedro M. FernandezI; Luiz A.G. LucioII; Fabíola PollachiII IAdjunct professor. Chief of the Service of Pediatric Surgery, Faculdade de Medicina do ABC, Santo André, SP, BrazilIIAssistant physician, Service of Pediatric Surgery, Faculdade de Medicina do ABC, Santo André, SP, Brazil ABSTRACT OBJECTIVE: To report a case of a 9-year-old female presented with esophageal achalasia and approached with surgery. The authors discuss the treatment and make a literature review on the topic.DESCRIPTION: Childhood esophageal achalasia is an unusual disease, often with unknown etiology. The main symptoms are esophageal vomits, dysphagia and weight loss. The diagnosis can be made by esophagogram and endoscopy, but the main examination is the esophageal manometry. Even though the surgical approach is a well-established therapy, some alternative treatments have been used, such as the endoscopy balloon dilatation and the use of botulinum toxin.COMMENTS: Esophageal achalasia is a rare disease in childhood, with unknown etiology. The presentation may be confused with gastroeshophageal reflux, sometimes causing a diagnosis delay. The surgical approach, as well as an antireflux procedure, is the treatment of choice. Introduction Esophageal achalasia of unknown etiology is a rare disease, affecting 1:100,000 of the general population and just 4 to 5% of these cases are described in children.1 Achalasia involves a failure of the lower esophageal sphincter (LES) in conjunction with reduced motility in esophagus itself. The passage of food via the esophagogastric junction is extremely difficult without true organic stenosis or extrinsic compression. The symptom spectrum is wide, varying from regurgitation to recurrent aspiration pneumonia. Older children describe symptoms of progressive dysphagia. For these reasons the disease is often confused with gastroesophageal reflux disease (GERD), delaying correct diagnosis.2,3 In response to