Health Alert:

See the latest Coronavirus Information including testing sites, visitation restrictions, appointments and scheduling, and more.

Fig 1 Esophograph
Figure 1: Barium esophagram in achalasia. Note esophageal dilatation and stenosis of the cardiac region of the esophagus with delayed evacuation of the barium meal.

Achalasia is a rare idiopathic motility disorder that manifests as hypertension and incomplete relaxation of the lower esophageal sphincter (LES) and aperistalsis of the esophageal body. The disorder is a result of impairment of the smooth muscle fibers, leading to failure of bolus transit through the esophagus. Symptoms include dysphagia, regurgitation, heartburn and chronic chest pain, with the consequent potential for weight loss, malnutrition and pulmonary sequelae.

Following diagnosis of esophageal achalasia by esophageal manometry and barium swallow esophagram, the standard surgical treatment is the Heller myotomy. First performed in 1914, Heller myotomy involves cutting the muscles of the LES to open the valve and permit food and liquids to pass into the stomach. Laparoscopic multi-port Heller myotomies are now the preferred approach. Post-operative complications may include infection, bleeding and rarely, esophageal or gastric perforation. The Heller procedure is often combined with fundoplication to prevent gastroesophageal reflux.

Alternatives to surgery for the treatment of achalasia include balloon dilation to expand the constricted sphincter and injections of botulinum directly into the esophagus to relax spastic muscle contractions. Both treatments are effective in the short term, but may require repeated administration to improve the symptoms of achalasia.

Fig 2. Esophageal achlasia
Figure 2: POEM: A 2 cm entry site is established in the mid-esophageal wall; a tunnel is then made in the submucosal space extending immediately beyond the esophagogastric junction to the lesser curve of the gastric cardia.

A more recent innovation, Peroral Endoscopic Myotomy (POEM) has been developed in Japan by Haruhiro Inoue, MD, PhD, who guided the introduction of the procedure at Penn Medicine. POEM involves the use of endoscopic tools to perform an intramural myotomy (as opposed to the extramural Heller procedure). A full description of the procedure can be found in the Case Study below.

In Dr. Inoue’s original series of 70 cases at Showa University Hospital, Yokohama, Japan, [1] POEM resulted in significant reductions in LES pressure (elevated in most patients with achalasia) and subjective symptom score. Marked improvement was noted in endoscopic appearance and esophageal emptying on barium swallow. Symptomatic post-POEM gastroesophageal reflux disease was observed in 11.4% of patients, but all were successfully treated with standard proton pump inhibitors. [1]

Case Study

Mr. Y, age 43 years, was referred to Penn Gastroenterology eight months after a botulinum toxin injection procedure for diagnosed achalasia at a hospital near his home in New Jersey.

Mr. Y’s symptoms at this time included protracted post-prandial pain, dysphagia to both solids and liquids and occasional vomiting.

The botulinum toxin injection procedure provided some immediate relief, but within three to six weeks, his symptoms began a slow and progressive return. Mr. Y was in otherwise good health.

At Penn, a barium esophagram revealed esophageal dilatation and stenosis of the cardiac region of the esophagus with delayed evacuation of the barium meal (Fig. 1). After a consultation to discuss his options, it was agreed that Mr. Y would have a POEM procedure. Mr. Y’s POEM was initiated by creating a 2 cm entry site into the mid-esophageal wall and then a tunnel in the submucosal space extending immediately beyond the esophagogastric junction to the lesser curve of the gastric cardia (Fig. 2).

Fig 3 Achalasia
Figure 3: Endoscopic observation following the POEM procedure reveals a successful myotomy with sufficient dilation of the lower esophageal sphincter and no loss of mucosal integrity following closure of the mucosal entry site.

Next, an inner circular muscle myotomy was performed by grasping and dividing the fibers. Following the myotomy, the endoscope was withdrawn from the submucosal tunnel and reinserted into the lumen to inspect the mucosa, ensure mucosal integrity and confirm easy passage of the endoscope through the LES consistent with an adequate myotomy. The mucosal entry site was then closed with endoscopic clips (Fig. 3).

Mr. Y was observed overnight in the Second-Stage Recovery Unit following his procedure. He received IV-hydration until post-operative day 1, when a barium esophagram was obtained.

Noting no loss of mucosal integrity or leakage, Mr. Y was started on a liquid diet and discharged home. Two days later, he began a solid diet, and his recovery thereafter was unremarkable. At his six-month follow-up, he reported a complete resolution of symptoms.

Access

Penn Gastroenterology
Perelman Center for Advanced Medicine
South Pavilion, 4th Floor
3400 Civic Center Boulevard
Philadelphia, PA 19104

Published on: May 19, 2016

References

[1] Enrolling Clinical Trials at Penn Gastroenterology
EndoFLIP Use in Upper GI Tract Stenosis (EndoFLIP) [NCT02354716]

[2] Familial Barrett’s Esophagus (FBE) [NCT00288119]

Penn Faculty Team

Daniel T. Dempsey, MD

Chief, Gastrointestinal Surgery

Assistant Director, Peri-Operative Services, Hospital of the University of Pennsylvania

Professor of Surgery at the Hospital of the University of Pennsylvania

Kristoffel R. Dumon, MD

Associate Professor of Surgery at the Hospital of the University of Pennsylvania

Gary W. Falk, MD, MS

Clinical Co-Director (Adult GI), Joint Center with CHOP

Co-Director, Esophagology and Swallowing Center

Professor of Medicine at the Hospital of the University of Pennsylvania

Gregory G. Ginsberg, MD

Director, Endoscopic Services

Professor of Medicine at the Hospital of the University of Pennsylvania

Professor of Surgery

Michael L. Kochman, MD

Development Officer, Department of Medicine

Director, Center for Endoscopic Innovation, Research and Training

Wilmott Family Professor

Professor of Surgery

Kristle Lee Lynch, MD

Director, GI Physiology Lab

Director, Advanced Esophagology Fellowship

Assistant Professor of Clinical Medicine

David C. Metz, MD

Co-Director, Esophagology and Swallowing Program

Director, Acid-Peptic Program

Co-Director, Neuroendocrine Tumor Center

Professor of Medicine at the Hospital of the University of Pennsylvania

Noel N. Williams, MD

Director, Penn Metabolic & Bariatric Surgery Program

Director, William T. Fitts Surgery Education Center

Rhoads-Harrington Professor in Surgery

Share This Page: