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World J Gastrointest Endosc. Apr 16, 2014; 6(4): 105-111
Published online Apr 16, 2014. doi: 10.4253/wjge.v6.i4.105
Childhood achalasia: A comprehensive review of disease, diagnosis and therapeutic management
Ashanti L Franklin, Mikael Petrosyan, Timothy D Kane
Ashanti L Franklin, Mikael Petrosyan, Timothy D Kane, Department of Pediatric General and Thoracic Surgery, Children’s National Medical Center, Washington, DC 20010, United States
Author contributions: All authors made substantial contributions to the conception and design, acquisition of data, or analysis and interpretation of data; all three authors participated substantially in the drafting and revising of the manuscript for intellectual content; and all authors approved the final version to be published.
Correspondence to: Timothy D Kane, MD, Department of Pediatric General and Thoracic Surgery, Children’s National Medical Center, Washington, DC 20010, 111 Michigan Ave NW, United States.
Telephone: +1-202-4762151 Fax: +1-202-4764174
Received: January 3, 2014
Revised: February 25, 2014
Accepted: March 11, 2014
Published online: April 16, 2014

Achalasia is an esophageal motility disorder characterized by failure of lower esophageal sphincter (LES) relaxation and is rare in children. The most common symptoms are vomiting, dysphagia, regurgitation, and weight loss. Definitive diagnosis is made with barium swallow study and esophageal manometry. In adults, endoscopic biopsy is recommended to exclude malignancy however; it is not as often indicated in children. Medical management often fails resulting in recurrent symptoms and the ultimate definitive treatment is surgical. Laparoscopic Heller myotomy with or without an anti-reflux procedure is the treatment of choice and has become standard of care for children with achalasia. Peroral endoscopic myotomy is a novel therapy utilized with increasing frequency for achalasia treatment in adults. More experience is needed to determine the safety, efficacy, and feasibility of peroral endoscopic myotomy in children.

Keywords: Achalasia, Pediatrics, Surgical Heller myotomy, Balloon dilatation, Lower esophageal sphincter

Core tip: Achalasia is a neurodegenerative disorder of the lower esophageal sphincter which occurs less commonly in children compared to adults and patients present with progressive dysphagia, vomiting, and weight loss. Medical therapy including botulinum toxin injection and endoscopic dilatation have been associated with only transient relief of dysphagia symptoms as is also seen in adults. While current evidence also suggests that the surgical approach of laparoscopic Heller myotomy provides lasting benefits for children with achalasia, future prospective evaluation will need to be conducted to ascertain whether peroral endoscopic myotomy is safe and equally effective in children.