A new procedure that blurs the boundaries between endoscopy and surgery has joined the armamentarium of treatments for achalasia, an esophageal disease that can have severe quality-of-life impacts for patients, and which, in its later stages, can be life-threatening.
Achalasia is a rare disorder that affects the nerve and muscle function of the esophagus and lower esophageal sphincter (LES), making it difficult for food and liquid to pass into the stomach. The hallmark features include chest pain, dysphagia and regurgitation. The dysphagia — difficulty swallowing — can manifest as a food-sticking sensation or a feeling of delay of food passage into the throat, chest or upper abdomen. Although the exact cause of achalasia is not known, the condition is believed to result from an immune-mediated response affecting the nerves that regulate muscle function of the esophagus and LES.
Diagnosis of achalasia can be challenging. “We get quite a few referrals of patients in whom it is not clear why they can’t swallow,” says Jeffrey Conklin, MD, medical director of the UCLA Center for Esophageal Disorders. Cancers and other motility disorders can mimic symptoms of achalasia, he notes. Patients with reflux can develop strictures at the bottom of their esophagus, which can also produce similar symptoms.
High-resolution esophageal manometry — a test during which a thin catheter is passed through the nose into the esophagus to record pressure changes during swallowing — is the gold standard for achalasia diagnosis. “On the manometry, the diagnosis is made by demonstrating that the lower esophageal sphincter does not relax and there is failure of peristalsis in the esophagus,” Dr. Conklin explains. This usually requires referral to a specialized center. The urgency of the diagnosis and treatment of the disorder goes beyond the quality-of-life impact. “At the end-stage of the disease, patients start to aspirate at night because the esophagus is full of food,” Dr. Conklin notes. “As the aspirated contents from the esophagus get into the lungs, this can become life-threatening, leading to pneumonia and chronic lung disease.”
Achalasia treatments aim to relax or disrupt the muscle of the esophagus in order to allow food to enter the stomach more easily. Dilating the esophagus with a large-bore balloon can be used as an alternative to surgery, but this approach has been found to be marginally less safe and less durable than other treatments, Dr. Conklin says. Similarly, botulinum toxin injections can provide relief, but given that the effects are temporary, it is offered only to patients who would be unable to tolerate other therapies.
The mainstay of treatment is myotomy, which cuts the abnormal LES muscle at the junction between the stomach and esophagus. It is accomplished by a laparoscopic surgery called Heller myotomy. The procedure typically involves adding a Dor fundoplication, in which a small piece of the stomach is partially wrapped around the esophagus to decrease the risk of reflux.
More recently, UCLA and a select number of centers have begun performing per-oral endoscopic myotomy, or POEM, which aims to recreate the Heller myotomy in a less-invasive way. POEM became possible through advances in endoscopic techniques, including submucosal endoscopy and natural orifice transluminal endoscopic surgery, or NOTES.
“This represents a paradigm shift in endoscopic intervention,” says Alireza Sedarat, MD, interventional endoscopist at the UCLA Center for Esophageal Disorders. “Traditionally, with endoscopy we stay within the lumen. In this case, an incision is made to enter the esophageal wall and expose the muscle. It’s blurring the boundaries between an endoscopic procedure and surgery, with great safety and success.”
Dr. Sedarat notes that POEM causes minimal disruption of the body’s anatomy in accessing the spastic muscle, and it results in a faster recovery and return to swallowing than the Heller myotomy. “This is one of the most gratifying procedures that I do,” he says. “Patients return with big smiles on their faces because they’re able to eat and drink what they want. It has a dramatic impact.”
Any patient with symptomatic achalasia who is a candidate for laparoscopic Heller is also a candidate for POEM, Dr. Sedarat says. The endoscopic procedure is particularly advantageous for patients with type III achalasia, in which there is an esophageal body spasm in addition to the lower esophageal sphincter relaxation impairment. For these patients, it can be more difficult to provide an adequate myotomy through laparoscopic surgery because of the physical limitations imposed by the diaphragm, whereas POEM allows precise tailoring of the myotomy length in the esophagus. For other types of achalasia, the anti-reflux component of the surgery may offer an advantage. At UCLA, patients see both the surgeon and the endoscopist before making a decision. Success rates for both procedures are greater than 90 percent.