Current management of pediatric achalasia

Abstract

Achalasia is a rare condition affecting esophageal motility in children. In a manner similar to the disease found in the adult population, children experience symptoms of dysphagia, regurgitation, and chest pain due to a failure of relaxation of the lower esophageal sphincter. Standard diagnostic approaches include upper endoscopy and esophageal manometry. New developments in diagnosis include high-resolution esophageal manometry and the endoscopic functional lumen imaging probe. Therapies available include endoscopic balloon dilations and botulinum toxin injections into the lower esophageal sphincter, as well as surgical interventions. The Heller myotomy was first described in 1913; since then, there have been many modifications to the procedure to improve outcomes and lower morbidity. Currently, the most commonly performed surgical procedure is the laparoscopic Heller myotomy, in which the sphincter muscle is divided using longitudinal incisions with or without a partial fundoplication procedure. In recent years, per oral endoscopic myotomy (POEM) is gaining support as a viable natural orifice therapy for achalasia. Complications of POEM occur at a relatively low rate, and outcomes following the procedure have been promising. The treatment of end-stage achalasia however, may include partial or total esophagectomy with reconstruction if possible. Future research is focused primarily on increasing the efficacy, and lowering complications, of existing therapeutic modalities.

Keywords: Esophageal achalasia; heller myotomy; per oral endoscopic myotomy (POEM).

Figure 1

Esophageal achalasia. (A) Pre-operative contrast esophagram demonstrating achalasia. (B) Post-operative contrast esophagram demonstrating successful treatment of achalasia.

Figure 2

High-resolution esophageal manometry performed in a pre-operative setting, demonstrating a failure of lower esophageal sphincter relaxation.

Figure 3

Laparoscopic Heller myotomy. (A) At the start of the case, exposure of the phrenoesophageal ligament is performed using a Nathanson liver retractor. The phrenogastric and gastrohepatic ligaments are incised. (B) A myotomy is performed through the longitudinal and circular fibers. (C) A complete myotomy is carried 3 cm caudal to the gastroesophageal junction and 6 cm on the esophagus.

Figure 4

Peroral endoscopic myotomy. (A) A mucosotomy is made and the endoscope is introduced into the submucosal space. (B) A tunnel is extended distally in the submucosal space. (C) The myotomy is performed using an electrocautery blade. (D) The tunnel is visualized following the myotomy. (E) The mucosotomy is closed using endoscopic clips.

Figure 5

Endoscopic functional lumen imaging probe (EndoFLIP, Medtronic) measurements are shown, prior to (left) and following (right), the per oral endoscopic myotomy (POEM) procedure.