Typical upper gastrointestinal series in a patient with achalasia.
Esophageal manometry is confirmatory when performed. Findings in achalasia demonstrate elevated resting lower esophageal sphincter (LES) pressure, absent or low-amplitude peristalsis, or non-relaxing LES upon swallowing. Since the LES is heterogeneous in children, the absence of these findings does not rule out the diagnosis of achalasia. Upper endoscopy with biopsy may be useful in ruling out esophagitis or other less common secondary causes of achalasia (Trypanosoma cruzi, malignancy) .
The indications for surgery for achalasia include:
Preoperative studies documenting achalasia (UGI, manometry, or endoscopy)
Trial and ineffective management by other approaches in treating achalasia including oral calcium channel blocker administration (nifedipine), pneumatic balloon dilatation, and endoscopic botulinum toxin injection therapy to relax the LES
For adults and children, nifedipine has not been shown to be a definitive treatment for achalasia but may palliate symptoms prior to another interventional therapy [6, 7]. Pneumatic dilatation has been effective in children; however, long-term follow-up is not available. Multiple dilatations are often required, and in adult studies, recurrence rates of 60 % are reported when only a single dilatation is performed . Endoscopic Botox® injection has also been shown to be effective in relaxing the LES in children and adults with achalasia. Optimal dosing and intervals of therapy have not been defined in children. However in adults, recurrent dysphagia occurs in 60–90 % of patients on an average of 4 months following treatment [9, 10].
Surgical intervention for achalasia may be considered as a primary treatment or after failed pneumatic dilatation or endoscopic Botox® injection of the LES. The most common surgical approach is the laparoscopic Heller myotomy with or without anterior fundoplication. An innovative and increasingly utilized surgical therapy for achalasia is the peroral endoscopic myotomy (POEM) procedure. Both techniques will be described here.
Laparoscopic Heller Myotomy with Dor Anterior Fundoplication
Flexible endoscopy is a useful adjunct both pre- and post-myotomy. It is advantageous to restrict patients to a clear liquid diet 3 days prior to procedure to reduce the possibility of retained food.
The pertinent anatomy includes the GEJ, longitudinal muscular layer of the esophagus, anterior (left) vagus nerve, circular muscular layer, and submucosal layer.
The patient is placed in supine position with arms tucked by sides. In larger patients, lithotomy and stirrups may be considered.
Laparoscopic 5-mm instruments are primarily used. This includes four 5-mm trocars, one 10- or 12-mm trocar for the umbilicus, 10-mm 30° telescope (5 mm in smaller patients), L-Hook cautery, Maryland grasper, 2 blunt Debakey-type graspers, suction irrigator, GIF 180 Olympus flexible upper endoscope (9.3 mm OD) (Olympus, Center Valley, PA), and Nathanson liver retractor:
Flexible endoscopy is first performed with assessment and measurement of location of gastroesophageal junction (GEJ) . The endoscope is left in the stomach with the light off.
The ports are placed in the same configuration as for a Nissen fundoplication.
The liver retractor is placed to elevate left lobe of the liver off of the stomach, esophagus, and hiatus.
The gastrohepatic and phrenoesophageal ligaments are divided. The phrenoesophageal fat pad is removed with hook cautery.
The anterior hiatal dissection is performed to expose the esophagus.
The anterior vagal nerve is identified. Dissection should stay to the patients’ right of this nerve.
First, mark the esophagus and stomach with cautery along proposed myotomy position and length (e.g., 5–7 cm on the esophagus and 2–4 cm on stomach) (Fig. 21.2).
Area of myotomy marked by cautery on esophagus and gastric wall.
Start the myotomy on the esophagus by incising the longitudinal muscle with hook cautery.
Pull the longitudinal fibers apart with blunt graspers proximally and distally and dissect more deeply to the expose the circular muscle layer (Fig. 21.3).
Laparoscopic Heller myotomy revealing deeper circular muscle layer of esophagus.
Identify and dissect the circular muscle layer by lifting this muscle layer away from the submucosal layer and divide with hook cautery (Fig. 21.4).
Extension of myotomy onto gastric wall.
Carry dissection proximally and distally by dividing circular muscular layer completely to the submucosal level by elevating the muscle fibers with graspers or a Maryland.
Measure the length of the myotomy for reference (esophageal plus gastric length).
Perform repeat endoscopy to assess the GE junction and assure it has been opened adequately (usually need 3 cm onto gastric side and 5–7 cm on esophageal side).
Anterior Dor fundoplication is performed by attaching the fundus to the left and right aspects of the myotomy with silk sutures.
Two-three sutures are placed on either side of the fundus and attached to the myotomy bilaterally (Fig. 21.5).
Dor fundoplication tacking stomach to open myotomy on the left side.
Endoscopy is helpful to ascertain proper myotomy and identify unrecognized submucosal injury from dissection.
Avoid extensive posterior hiatal dissection which is unnecessary when an anterior fundoplication is performed and may contribute to more reflux.
If a Toupet is performed, then posterior dissection is indicated.
A 10-mm telescope allows enhanced visualization of the dissection. When encountering troublesome bleeding above the submucosal layer (especially on the gastric side of the myotomy), a Raytec sponge can be inserted through the 10-mm trocar to help with hemostasis. Most of this bleeding will stop and indiscriminate use of electrocautery, which can lead to submucosal perforation, which should be avoided.
An L-hook is preferable to a Harmonic scalpel in performing a precise dissection and avoidance of contact of the energy source with the submucosal layer. Circular fibers should be elevated and then cauterized superficial to this layer.
An incomplete myotomy or recurrent dysphagia is usually due to an inadequate myotomy on the gastric side (similar to pyloric stenosis).
The Dor fundoplication should not be under tension or torque on the esophagus after it is secured. Some upper short gastric vessels may need to be divided to accomplish this.
Postoperative Care and Outcomes
An esophagram is obtained on postoperative day 1, primarily to document free flow of contrast across the GE junction. This image also provides a reference for comparison study for the future should symptoms of pain, dysphagia, or heart burn recur. Children begin a liquid and pureed diet postoperative day 1 following the contrast study and continue with a soft diet, avoiding large boluses of solid food and chewing well after each bite, for 2 weeks after the procedure. Patients can be discharged when tolerating a diet, typically on postoperative day 1 or 2. Early follow up at 2–3 weeks allows assessment of function and weight gain or loss. Pre- and postoperative Eckardt scores are documented in order to assess results and monitor long-term function. Complications include mucosal perforation, gastroesophageal reflux, incomplete myotomy with persistent dysphagia, and recurrent dysphagia, either in the short or long term. However, LHM with partial fundoplication is safe and very effective with a low incidence of complications or morbidity in the treatment of achalasia [2, 11, 12].