[size=20px]A 14-year old boy presents with progressive dysphagia and is found to have achalasia on manometric and contrast studies.

 

The family wants to pursue endoscopic therapy for his achalasia and wishes to discuss the best option for long term relief. You recommend

 

A balloon dilation.

 

B botulinum toxin injection.

 

C bougie dilation.

 

D radiofrequency ablation.

 

E submucosal myotomy.[/size]

A

think again my dear

E

E

correct

 

Achalasia is a rare esophageal disorder with an incidence of 0.11/100,00 in children, characterized by aperistalsis, high resting pressure and failure of receptive relaxation of the lower esophageal sphincter (LES). In adults, a meta-analysis of randomized controlled trials noted that the most robust treatment of this condition is laparoscopic Heller myotomy. Balloon dilation has a very high recurrence rate although a European trial suggested equal efficacy at two years. In 2010, the first per oral endoscopic myotomy (POEM) was performed in a human by creating a submucosal working plane and using endoscopic instruments. This led to rapid adoption and large trials establishing safety and initial short term equivalent efficacy to the Heller myotomy in adults. Small series in children (nine to 12 cases) have also demonstrated the ability to perform this procedure safely.

 

The technique involves insertion of an endoscope into the esophagus. A cut is made with an endoscopic knife through the mucosa of the lower esophagus in order to access the circular muscle in the submucosal plane. The circular muscle layer in the lower esophagus and a small part of the upper stomach is incised in order to relieve the functional obstruction from the nonrelaxing LES. Clips are then placed to close the incision in the mucosa.

 

Botulinum toxin provides relief only for a few months and bougie dilation is neither indicated or efficacious. Radiofrequency ablation actually leads to an achalasia like state. Balloon dilation has not been shown to have durable long term relief.