A thirteen-year old girl has progressive difficulty swallowing. An esophagram and manometry are suggestive of achalasia.

 

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[size=20px]The most definitive management of achalasia is

 

A oral nitrates.

 

B pneumatic dilation.

 

C botulinum toxin injection.

 

D thoracoscopic myotomy.

 

E laparoscopic esophagocardiomyotomy.[/size]

E

E

correct

 

Achalasia is characterized by the degeneration of the inhibitory myenteric plexus that innervates the lower esophageal sphincter (LES) and esophageal body. This results in an imbalance of inhibitory and excitatory neurons which leads to a failure of the LES to relax with swallowing, an absence of peristalsis in the esophageal body and increased LES resting pressures. Children typically present with progressive dysphagia, vomiting and weight loss. Other symptoms that are also typical of gastroesophageal reflux disease may be present and this will often delay the diagnosis of achalasia. Achalasia has been associated with trisomy 21, congenital hypoventilation syndrome, glucocorticoid insufficiency, eosinophilic esophagitis, familial dysautonomia, Chagas disease and AAA syndrome (achalasia, alacrima, ACTH insensitivity).

 

The diagnosis of achalasia is made by contrast esophagram findings of a dilated esophagus with tapering at the distal end and manometric documentation of a hypertensive LES with absent peristalsis.

 

Medical treatment options include nitrates, calcium channel blockers and sildenafil. These medications are often not well tolerated by children. Botulinum toxin injection into the LES can provide transient relief. Pneumatic dilation can lead to relief of symptoms in 50-80% of children although recurrent symptoms are common and complications, including perforation, are not infrequent. Open or laparoscopic myotomy has provided the most reliable control of symptoms. Some of these patients will go on to develop recurrent symptoms (up to 25%) of dysphagia or reflux often due to problems with the intrinsic esophageal motility. The incidence of postmyotomy reflux may be as high as 40%. Most authors recommend an antireflux procedure, most commonly a Dor fundoplication, with myotomy. The most reliable myotomy (Heller) incorporates four to five cm of the lower esophagus and two to three cm of the proximal stomach. The thoracoscopic approach is less desirable because it may be challenging to extend the myotomy onto the stomach from this approach.

 

Peroral endoscopic myotomy (POEM) has been reported with success in adults. Recently, several series in children have been reported. With this technique, endoscopically the mucosa over the LES is opened. The circular muscle of the LES of the distal esophagus and proximal stomach is divided. Clips are then used to close the mucosa. Chen et al recently reported a series of 24 patients who underwent POEM successfully. During the series they modified their technique to perform a full-thickness myotomy. There were no major complications and no recurrent symptoms with an average follow up of 24 months. 19% of patients did develop symptoms of GERD. Although early data of the POEM procedure is promising it is unclear if the results longterm will be superior to the laparoscopic Heller myotomy. The concerns for GERD with the POEM procedure may outweigh the advantage of being less invasive than a laparoscopic approach.