Achalasia is a rare esophageal disorder, affecting about one in every 100,000 individuals. Uncommon as it is, achalasia is well-known to surgeons within the Froedtert & the Medical College of Wisconsin health network, who offer patients and their providers the latest knowledge and experience in treating it.
“We treat about 25 achalasia patients a year,” said Andrew Kastenmeier, MD, general surgeon and MCW faculty member. “Because achalasia is rare, patients tend to get referred to centers like ours that treat higher volumes of patients with this disorder.”
Medical and Social Impacts
With achalasia, the nerves in the esophagus degenerate, causing two significant problems. “The first is that the esophagus does not squeeze food or fluids down appropriately when the person swallows,” Dr. Kastenmeier said. “The second problem is that the sphincter or valve at the bottom of the esophagus does not open to allow food to pass through.”
This results in regurgitation of fluid or food, vomiting, chest pain, heartburn and weight loss due to the inability to eat.
“The implications go further because eating is a social activity, and people with achalasia often get marginalized because they cannot participate in gatherings due to worries about regurgitation or throwing up,” Dr. Kastenmeier said.
None of this happens immediately. The onset of achalasia is slow and insidious. Patients may struggle for years with mild to moderate symptoms. They’re often treated for reflux with minimal improvement in their symptoms. The rarity of achalasia complicates matters and delays diagnosis because it may be confused with diseases that have similar symptoms.
“A simple screening test — an upper GI esophagram — often reveals achalasia,” Dr. Kastenmeier said. “But the gold standard for confirming the diagnosis is called esophageal manometry, which involves measuring the pressure with which the esophagus can squeeze and measuring the ability of the lower esophageal sphincter to relax.”
Once a diagnosis is confirmed, patients and their providers can choose from a range of treatment options:
Botox® injections of the lower esophageal sphincter delivered endoscopically
“While Botox injections work reasonably well, they only last a few weeks or months. They usually can only be repeated once or twice because the body forms antibodies to Botox, so it is not a long-term solution,” Dr. Kastenmeier said.
Endoscopic pneumatic dilation
“Mechanical expansion of the lower esophageal sphincter also works reasonably well but often requires multiple sessions of stretching the esophageal sphincter,” Dr. Kastenmeier said. “It needs to be repeated, so durability is one issue. The other is the risk of stretching too far and perforating the esophagus, so many physicians are hesitant to do that.”
Laparoscopic Heller myotomy
During this surgical procedure, the muscle fibers of the lower esophageal sphincter are cut. “Think of the sphincter as a circular valve,” Dr. Kastenmeier said. “We cut the valve so it opens from a circle to a U shape and can’t restrict the bottom of the esophagus. This procedure has the best durability and the longest-term outcomes. More than 90 percent of patients experience significant relief of symptoms.” The procedure is usually performed through five small incisions in the abdomen and typically requires one to three nights in the hospital.
Peroral endoscopic myotomy (POEM)
This is a less invasive approach and the latest advancement in achalasia treatment. “We cut the valve without making any incisions externally and make the incision with the endoscope while the patient is under general anesthesia,” Dr. Kastenmeier said. “It takes about 90 minutes, and the patient can leave the hospital the same day or after one night. With the POEM procedure, there’s less pain and a quicker recovery.” POEM is also preferred for patients who have had prior abdominal surgery because surgeons can avoid surgery in a reoperative area.
POEM was pioneered in Japan and is available at only a small number of centers in the United States. “I offer the Heller myotomy and POEM, but most patients are choosing POEM,” Dr. Kastenmeier said. “They’re excited about the option of a surgery without external incisions. Ultimately, we’re doing the same thing with the sphincter, but the path from inside is less risky and it’s less traumatic to the body.” Dr. Kastenmeier expects long-term outcomes from POEM to be at least as favorable as those for the Heller myotomy.
After the POEM procedure, patients usually eat a soft diet for about two weeks and take an acid suppression medication for several months.
Feeling Back to Normal
“None of these treatments are a cure for achalasia; however, patients who have the POEM procedure report that their symptoms resolve and swallowing goes back to their baseline,” Dr. Kastenmeier said. “If they felt marginalized by the disorder, they can go out to eat again without fear of regurgitation or vomiting, and they can maintain their weight. Patients realize a dramatic improvement immediately after surgery.”
If untreated, patients with achalasia face a difficult future. “The esophagus can continue to expand,” Dr. Kastenmeier said. “It becomes sigmoidal and develops curves. Patients who have not had treatment have reduced response to standard treatments when they get to end-stage achalasia. They may eventually need a feeding tube to maintain nutrition.”
Dr. Kastenmeier advocates awareness of potential symptoms and early intervention. “In patients who display symptoms of dysphagia or regurgitation, an upper GI esophagram is an inexpensive, noninvasive method of assessing for an esophageal motility disorder,” he said. “If they have achalasia, it warrants a discussion with someone who can offer treatment options and tailor treatment for the patient.”
Physicians who encounter this rare disorder in their patients can expect an uncommon level of support from Dr. Kastenmeier and his team. “If they suspect the disease and need assistance in determining a diagnosis or how to proceed, we’re available to consult,” Dr. Kastenmeier said. “We can help them determine the most effective treatment for their patients.”
For Our Referring Physicians:
Academic Advantage of POEM
The Froedtert & MCW health network gives patients and their referring physicians a distinct advantage.