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Achalasia is a rare disorder of esophageal dysmotility in which there is absent or incomplete relaxation of the lower esophageal sphincter (LES) in addition to generalized abnormal peristalsis of the esophageal body. The pathophysiologic basis of achalasia is thought to be related to the degeneration of the inhibitory myenteric nerve plexus of the LES and esophageal body. The incidence in adults is 1 per 100,000, but it is much less in children, estimated to be 0.1 per 100,000. Although the prevalence of achalasia is 80 per million, only 5% of achalasia patients are diagnosed at younger than 15 years of age.
Idiopathic disease predominates, but achalasia has been associated with Chagas disease, trisomy 21, eosinophilic esophagitis, familial dysautonomia, congenital hypoventilation syndrome, and alacrima, achalasia, and adrenocorticotropic hormone (ACTH) insensitivity (AAA) syndrome. Various nonsurgical therapies for achalasia have been utilized over the years but have largely produced only temporary results and/or required repeated interventions. These include medical therapies such as calcium channel blockers, sildenafil, and isosorbide dinitrate, and endoscopic treatments including pneumatic balloon dilation, esophageal stenting, and botulinum toxin (Botox) injection into the LES.
Definitive surgical management of achalasia has evolved over time to less invasive approaches. The two primary techniques utilized today are the laparoscopic esophagomyotomy (LEM) (with or without partial fundoplication) and the peroral endoscopic myotomy (POEM) procedure.
Indications for Workup and Operation
Indications for operation include ineffective treatment results from alternatives to surgical myotomy such as esophageal dilation, LES Botox injection, or pharmacologic therapies. Preoperative studies documenting the diagnosis of achalasia should include an esophagram demonstrating a dilated proximal esophagus and “bird beak” tapering at the esophagogastric junction (EGJ) ( Fig. 3-1 ), an esophagogastroduodenoscopy (EGD) with or without pH-metry study to evaluate for gastroesophageal reflux or stricture, and esophageal manometry to document nonperistaltic esophageal body contractions and failure of LES relaxation after swallowing.
Laparoscopic Esophagomyotomy with Partial Fundoplication
The patient is positioned supine on the operating table with legs straight (older patients) or frog-legged at the end of the operating table (children < 5 years of age) with the table rotated into a reverse Trendelenburg position. The surgeon can stand on the patient’s right or left side with the assistant on the opposite side. The monitor will be situated at the head of the bed. Five ports are used (as for laparoscopic fundoplication) and range from 3.5 mm to 10 mm size depending on the size of the patient ( Fig. 3-2 ). Our preferred access is via a vertical umbilical incision using an open cutdown technique. After the initial umbilical port is inserted, we confirm its intra-abdominal positioning before initiating CO 2 insufflation.
Insufflation pressures will range from 10 to 15 mm Hg, again dependent on patient age and size. We routinely use flexible endoscopy intraoperatively for assessment and measurement of the location of the EGJ, and prefer to leave the endoscope in the stomach during the LEM (with the endoscope light off). In addition, we utilize the endoscopic functional lumen imaging probe (EndoFLIP, Medtronic, Minneapolis, MN) to calibrate the cross-sectional area of the EGJ prior to and following esophagomyotomy to assess the adequacy of the myotomy. A 5-mm liver retractor is inserted and positioned to elevate the left lobe of the liver off the stomach, esophagus, and hiatus ( Fig. 3-3A ). The esophagus is exposed by dividing the gastrohepatic and phrenoesophageal ligaments and the phrenoesophageal fat pad with hook electrocautery. This anterior hiatal dissection will expose the distal esophagus, and identification of the anterior vagus nerve will determine whether the esophagogastric myotomy will be more readily accomplished on either the patient’s right or left of this nerve. The esophagus and stomach are marked with electrocautery along the proposed course of the myotomy for a length of 5 to 7 cm on the esophagus and 2 to 3 cm onto the stomach ( Fig. 3-3B ). The myotomy is initiated on the esophagus by incising the longitudinal muscle with hook electrocautery. The longitudinal fibers can be pulled apart with blunt graspers proximally and distally to carry the dissection more deeply to expose the circular muscle layer ( Fig. 3-3C and 3-3D ). Once the circular muscle layer is encountered, the muscle is lifted away from the submucosal layer and divided with a hook electrocautery. Once this dissection plane is achieved, dissection proceeds cephalad ( Fig. 3-4A ) and then distally ( Fig. 3-4B ) by dividing the circular muscle layer completely to the submucosal level. Circular muscular division is enhanced by elevating these muscle fibers with graspers or a Maryland instrument (see Fig. 3-4B ). It is very important to carry this dissection into the gastric wall for 2 to 3 cm ( Fig. 3-4C ). One should periodically measure the length of the myotomy for reference (esophageal plus gastric myotomy length) ( Fig. 3-4D ). Repeat endoscopy can be performed at any time to assess the EGJ and ensure it has been opened adequately (2 to 3 cm on the gastric side and 5 to 7 cm on the esophageal side). If an antireflux procedure is added to the myotomy, we perform an anterior Dor fundoplication. The fundus is mobilized and attached to the anterior aspect of the myotomy. This is done with silk or other permanent suture by placing two to three sutures first on the patient’s left side of the myotomy to the fundus, followed by two to three more sutures from the fundus to the patient’s right side of the myotomy ( Fig. 3-5 ).
The patient is left nothing per os (NPO) following the LEM and an esophagram is performed on postoperative day 1. This study is obtained not so much to look for an esophageal leak (as this usually would have been identified on intraoperative endoscopy), but to document a baseline appearance of the postmyotomy EGJ. In the event of recurrent symptoms in the future (e.g., dysphagia, chest pain, or regurgitation), a new esophagram can be performed for comparison. Assuming a normal postoperative esophagram, a liquid diet is commenced and discharge typically occurs on postoperative day 1 or 2. A soft diet (no hard, solid food) is recommended for 2 to 3 weeks post-LEM (or until outpatient follow-up). Pre- and postoperative Eckardt scores are recorded to assess the results of the myotomy and these scores are monitored long term ( Table 3-1 ).
|Age at operation (years)||12.9||0.75-18|
|Operative time (minutes)||120||45-180|
|Length of stay (days)||1.9||1-6|
|LHM + DF||13||52%|
|∗ LHM + takedown DF||1||2%|
|Mucosal perforation||1||Laparoscopic repair|
|Recurrent dysphagia||5||Balloon dilation (1-3×)|
|Redo Heller myotomy||0|
|Normalization of Eckardt scores (< 3)||25||100%|
|Preoperative Eckardt score||9.7||(3-11)|
|Postoperative Eckardt score||0.9||(0-3)|
We routinely use endoscopy during our LEM to determine the proper myotomy length and to identify any unrecognized submucosal injury resulting from the dissection and myotomy.
Pre- and postmyotomy measurements at the EGJ using EndoFLIP will help guide the adequacy of the myotomy.
When possible, the use of a 10-mm telescope improves visualization in adolescent patients. If bothersome bleeding occurs above the submucosal layer (usually on the gastric aspect of the myotomy), an operative sponge can be inserted through this 10-mm port to help with hemostasis (see Fig. 3-4 ). This bleeding will usually stop spontaneously, and indiscriminate use of cautery should be avoided to prevent full-thickness injury.
We feel that using the L-hook electrocautery is superior to using the ultrasonic scalpel or other high-temperature energy device as it permits precise dissection and a reduced chance of heat contact from the energy source with the submucosal layer. Also, the circular fibers should be elevated away from the submucosa before using the cautery.
Dissection of the hiatus posteriorly should be avoided since it is unnecessary and can contribute to an increased incidence of reflux postoperatively. However, posterior dissection is indicated in cases where a Toupet partial fundoplication is performed.
An incomplete or inadequate myotomy will lead to recurrent dysphagia. This usually occurs due to incomplete muscle division on the gastric side of the myotomy. However, development of scar tissue and somatic growth can also predispose the patient to recurrent dysphagia.
Any antireflux procedure that is added to the LEM, such as a Dor fundoplication, should not be under tension or produce torque on the esophagus once it is created. Division of some of the upper short gastric vessels may be required to attain a tension-free fundoplication.
From 2011 until 2018, 25 patients underwent esophagomyotomy for achalasia at Children’s National Medical Center, Washington, DC (see Table 3-1 ). Patient age at operation ranged from 8 months to 19 years (mean, 12.8 years). Twenty-four patients (96%) underwent primary LEM with or without Dor fundoplication. Thirteen of 25 patients (52%) underwent Dor fundoplication in addition to the esophagomyotomy and the other 11 patients had only LEM. One patient underwent a redo LEM with takedown of the previous Dor fundoplication for recurrent achalasia (the initial procedure was performed in 2008). Follow-up has ranged from 8 to 94 months (mean, 32 months). The mean operative time for these procedures was 120 minutes (range, 92 to 180 minutes). The mean length of hospitalization has been 1.9 days (range, 1 to 6 days). All patients underwent a postoperative esophagram on the first postoperative day, and there were no leaks. There were no deaths or conversions to an open operation. Complications included one mucosal perforation (4%) (repaired during the primary procedure); five patients developed recurrent dysphagia following the LEM (6 months to 1 year following the initial myotomy) and required at least one balloon dilation; none have required operative revision of the esophagomyotomy.
The mean preoperative Eckardt score was 9.7 (range, 3 to 11) and decreased to 0.9 (range, 0 to 3) postmyotomy. All patients had normalization of their Eckardt scores (< 3) following their initial LEM procedure and/or balloon dilation (for those with recurrent dysphagia). Also, all had significant weight gain within the first year following their myotomy and are experiencing a good quality of life.
Diligent follow-up is mandatory for patients with achalasia since some patients will develop recurrent dysphagia or other symptoms following their esophagomyotomy.
Peroral Endoscopic Myotomy (POEM)
The indications for POEM are similar to those for LEM, although experience with advanced flexible endoscopic techniques is required. Consideration of patient size is important for POEM as an endoscope with a 2.8-mm working channel is necessary to accommodate the tools needed during POEM. This means that the outer diameter of the endoscope (e.g., GIF-H180 Olympus Endoscope) will be 8 mm. At Children’s National Medical Center, the smallest patient in whom we have achieved POEM thus far is a 2-year-old who weighed 9.7 kg. We used the standard endoscope but could not use the overtube. Patients should only consume liquids and use a nystatin swish and swallow for 3 to 4 days prior to POEM to ensure the esophagus is free of debris and to clear potential Candida esophagitis due to stasis.
Patient positioning is supine on the operating table with the abdomen exposed to detect possible pneumoperitoneum. Although our patients are placed on a liquid diet several days prior to surgery, an initial endoscopy is performed to completely eliminate debris from the esophagus and stomach prior to the procedure. Intravenous antibiotics are administered perioperatively. Decadron can be given on anesthesia induction to minimize mucosal swelling to make the closure easier. Necessary equipment is listed in Table 3-2 .