Achalasia rarely affects children and the clinical presentation, though often insidious, is distinctive: dysphagia, chest pain, and regurgitation of undigested food. Nevertheless, patients frequently present for surgical consultation only after many months of misery, failed interventions, ineffective medical therapy. This is unfortunate, not only because the patient suffers needlessly, but because there is a safe and effective operation available that becomes more arduous and more perilous when the patient presents late after having been dilated or injected. Primary physicians and gastroenterologists should be encouraged to refer these patients to an experienced surgeon early in the course of the disease rather than as a “last resort.”
Laparoscopic Heller myotomy is an advanced minimally invasive technique but is generally safe in the hands of an experienced laparoscopist. I feel the operation can be done safely with the patient in the supine position and the surgeon standing to the right, which avoids the inherent delay and added risk of placing the patient in stirrups. Some have touted the use of intra-operative adjuncts like manometry or endoscopy to assess the adequacy of the myotomy, but these are unproven and generally unnecessary. To protect the exposed submucosa and to avoid unnecessary dissection posterior to the esophagus, I always perform an anterior fundoplication, but this is certainly open to debate. A postoperative esophagram is admittedly overkill but avoiding even a single rare case of unrecognized perforation seems like adequate justification for this generally harmless exercise.
The key to the success of the Heller myotomy, like most complex operations, is proper management of expectations. Patients naturally, but inappropriately, expect instantaneous relief of their symptoms and the ability to eat anything and everything immediately after the operation. In most cases, this is unrealistic and patients should be counseled to expect that some degree of dysphagia will persist for some time after the operation. This is because the disease affects the motility of the entire esophagus, the chronically dilated esophagus is ineffective at peristalsis, and there is a partial functional obstruction at the LES after myotomy. The esophagus drains principally by gravity and pressure from the advancing bolus of swallowed food or liquid. These symptoms resolve gradually over the course of several weeks or months; but in the meantime patients should be encouraged to avoid food with large chunks (meat, bread crust), to chew their food well, and to drink fluid frequently during meals. Some patients can develop intermittent painful episodes of esophageal spasm that usually eventually cease. This has been treated with variable results using calcium channel blockers and medications that counter smooth muscle spasm.
Intra-operative perforations should be primarily repaired and then “patched” with the fundoplication, in which most patients should be able to tolerate oral intake after a brief (48–72 h) period of observation and a negative esophagram. The rare patient with an esophageal perforation noted on postoperative esophagram is treated initially with bowel rest, antibiotics, and careful observation. The leak usually seals spontaneously in 5–7 days but occasionally will require percutaneous drainage or reoperation for local or systemic sepsis. Perforation should be an extraordinarily rare event.