A 17 year old boy is seen in the pediatric surgery clinic after ongoing issues with dysphagia. Subsequent evaluation reveals a diagnosis of achalasia. Per-oral endoscopic myotomy (POEM) has been used in the adult population to manage this disease process. A contraindication to the POEM procedure is?

 

A Type I Achalasia

 

B Type II Achalasia

 

C Type III Achalasia

 

D Pseudoachalasia

 

E Nutcracker esophagus​

 

D

D

Correct answer

D Pseudoachalasia

 

The gold standard in the management of achalasia has been the laparoscopic Heller myotomy with or without fundoplication. A novel approach called the POEM procedure has been used for over a decade in the adult population since its first description in the Japanese literature. There is a steep learning curve as the procedure requires an advanced endoscopic technique to create the submucosal tunnel and subsequent myotomy. POEM has been used successfully to treat all three types of achalasia (described below), as well as other hypertensive lower esophageal sphincter pathologies such as nutcracker esophagus. Recent small studies in the pediatric population have shown a similar efficacy to laparoscopic Heller myotomy with a similar complication profile. As with adult data, there may be a shorter hospital length of stay and a lower cost associated with the procedure. Complications of the POEM procedure include esophageal perforation, bleeding, pneumothorax, pneumomediastinum, reflux, and incomplete myotomy, Contraindications to the procedure include portal hypertension, history of radiation, coagulopathy, pseudoachalasia, and erosive esophagitis. Further studies are needed in the pediatric population to continue to validate these data.(PJ)

• Type I (classic achalasia) - no significant pressurization within the esophageal body and impaired LES relaxation
  
• Type II (achalasia with compression or compartmentalization in the distal esophagus >30 mm Hg) - rapid panesophageal pressurization with water swallows
  
• Type III (spastic achalasia) - rapidly propagated pressurization attributable to an abnormal lumen obliterating contraction
  

​