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13 Management ofAchalasia: ADisease Hard toSwallow
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anterior myotomy with promising results in eight patients in 1923 [38]. This myot­omy was performed via an open approach, either trans-thoracic or trans- abdominal. The myotomy incision, as described by Ellis etal., extended to only 5mm on the gastric wall [38]. Through a “short” myotomy on the gastric wall, he was able to achieve an improvement rate of 89% with marked reux in 5% of patients. Notably, a trans-abdominal approach resulted in a signicantly higher incidence of postopera­tive reux, which is attributed to a longer myotomy on the gastric wall, division of the phrenoesophageal ligament, and greater mobilization of the esophagus. In 1962, Dor introduced a partial fundoplication to an extended myotomy to decrease postop­erative reux. Bonavina etal. supported this technique nding that in 206 patients who had an anterior myotomy (8cm on the esophagus and 2cm on the stomach) with Dor fundoplication 93.8% of patients had complete or near-complete resolution of symptoms [38]. The addition of a Dor fundoplication after a Heller myotomy reduced pathologic reux demonstrated by Richards et al. nding a 47–9% measurable decrease in 24-h pH studies without impact on dysphagia [39].
The myotomy with fundoplication remained the tenet of surgical treatment for achalasia; however, the approach underwent modernization in the 1990s with the introduction of laparoscopic techniques to esophageal surgery. The rst laparo­scopic cardiomyotomy was performed by Dr. Cushieri in 1991 [40]. In 1992, Dr. Pelligrini described the results of 17 patients who underwent a thoracoscopic 7cm myotomy on the left side of the esophagus extending only 5mm on the gastric wall. The goal was to balance dysphagia relief with reux prevention. While the short­and long-term outcomes were excellent in 90% of cases, this approach had draw­backs, including intraoperative lung exclusion, a postoperative chest tube, and high incidence of reux given the absence of fundoplication [38]. The ability to perform a myotomy and a partial fundoplication could be achieved with a laparoscopic approach, making this the standard of care by the late 1990s. Comparisons of these approaches by Patti etal. found that the laparoscopic group had shorter median hospital stay (42hours laparoscopic vs. 84 hours thoracoscopic group), better reso­lution of dysphagia (90% laparoscopic vs. 87% thoracoscopic group), and improved 24h pH testing (10% laparoscopic vs. 60% thoracoscopic group) [38].
Regarding the type of fundoplication, Rawlings etal in 2012 found no signicant difference in terms of relief of dysphagia and reux control when comparing a Dor to a Toupet fundoplication. Albeit, Rebecchi, and colleagues found a signicant dif­ference in the dysphagia symptoms when a Nissen wrap was performed [38]. The advent of robotic surgery has rivaled the previous laparoscopic techniques arguing the absence of a tremor and 3D magnied view can reduce the incidence of esopha­geal perforation. In 2005, Horgan etal. demonstrated a perforation incidence of 0% in the robotic group versus 16% in the laparoscopic group. Huffman etal. supported these ndings in 2007 with a perforation rate of 0% in the robotic group versus 8% in the laparoscopic group. The development of cardiomyotomy, as envisioned by Heller over a century ago, performed minimally invasively with a partial fundopli­cation, remains the surgical treatment of choice for achalasia today [37].
The key component of selecting patients for surgical management of achalasia is to differentiate it from other motility disorders such as pseudoachalasia,
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malignancy, and mechanical obstruction. This should be performed in the preopera­tive setting by the surgeon reviewing the history of the patient’s symptoms and review of previous studies, including the results of esophageal manometry, endos­copy, and contrast esophagram. The patient needs to understand that this is a pallia­tive surgery meant to relieve the symptoms and is not a cure. A laparoscopic myotomy, when combined with an antireux procedure, provides better symptom relief (90%) than all endoscopic and other surgical approaches and carries a lower complication rate of 6.3% [40]. This information should be communicated to the patient during the preoperative clinic visit.
M. Belisle et al.

Heller Myotomy

Operative Steps
Anesthesia Induction andRobot Docking
Patients are advised to stay on a liquid diet preoperatively for 1week, especially if they have an end-stage esophagus, that is, sigmoid esophagus. During this time, they are placed on a high-protein shake. Preoperative enoxaparin is given prior to intubation. Care is taken by anesthesia to minimize the risk of aspiration by per­forming a rapid sequence intubation and the patient not laying completely at.
After induction of general anesthesia, the patient is placed on the operating table in supine position, with both arms tucked and a foot board in place. The arm is tucked to allow the robot to be docked with no interference with the arm board. Having the arm out is an acceptable practice. The patient is given prophylactic antibiotics, usu­ally a rst-generation cephalosporin, within 1h of the incision. An orogastric tube and an optional Foley catheter is inserted. Sequential compression devices are turned on and functioning throughout the case.
The procedure is commenced by making a stab incision in the left upper quad­rant at Palmer’s point. A Veress needle is inserted and pneumoperitoneum estab­lished to 15mmHg of CO2. The authors use the Davinci Xi platform. All trocars are lined in a horizontal line that is no more than 15cm below the xiphoid process. The abdominal cavity is then entered along the right mid-clavicular line using a 30-degree 5-mm laparoscope housed in a robotic 8-mm trocar, using the Optiview technique. Under direct visualization, an 8-mm trocar is then placed two ngerbreadths to the left of the umbilicus, approximately 15cm below the xiphoid process. Two addi­tional 8-mm trocars are inserted roughly 8cm apart along the left midclavicular and left anterior axillary lines. An optional additional 5-mm assistant port is placed in the right lower quadrant. The patient is then placed in reverse Trendelenburg posi­tion. The authors utilize two right hands and one left hand. The Davinci Xi robot is then docked with a Maryland bipolar in (right midclavicular trocar), camera in arm two, Vessel sealer/hook/syncroseal in (left midclavicular trocar), and Cardiere grasper in (left anterior axillary trocar). At this point, the orogastric tube is removed once the stomach is conrmed to be deated and suction is left on while it is being withdrawn slowly to suction all esophageal content.
13 Management ofAchalasia: ADisease Hard toSwallow
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Liver Retraction
Next, the left lateral segment of the liver is retracted. This has been described with a Nathonson retractor or pretzel retractor. Both of which require an additional inci­sional with an epigastric incision with the former and 5mm trocar in the latter. The Nathanson retractor should be positioned so it does not interfere with robot arms. Alternatively, a suture hammock with a #1 V-lock 18-inch suture as described by Dr. Havaleshko’s, “Dima stitch,” to suspend the left lateral segment of the liver anteri­orly in order to expose the hiatus.
Hiatal Dissection
The dissection toward the crus can be initiated with a right-rst approach or a left. If the surgeon plans to perform a Toupet fundoplication as the choice of partial wrap or the patient has a hiatal hernia (rare), then a left approach is more logical. This will allow for the placement of a penrose/umbilical tape for esophageal retraction from the patient’s left. This can be retrieved from the right side once the retroesophageal window is created. However, if the plan is to perform a Dor fundoplication or not to perform a wrap at all (in the case of sigmoid esophagus, i.e., end esophagus), it is better to start with the right-rst approach. A person may wonder why with a Dor wrap to start on the right side. When creating a Dor wrap, the retroesophageal attachment is kept intact. Also, if the fundus is very oppy, the short gastric arteries do not need to be taken down, although preserving the short gastrics with a Dor wrap is not the author’s preferred approach as it can create tension at the gastro­esophageal junction.
To begin the left-rst approach, the gastrocolic ligament is incised, and dissec­tion is continued toward the left crus, taking down the short gastric vessels and mobilizing the gastric fundus. This can be performed using the vessel sealer or Synchroseal. Care is taken to avoid avulsing the splenic capsule or splenic vessels by aiming the tip of the bipolar device toward the left crus and never toward the splenic hilum. To expose the left crus, the stomach should be grasped by the poste­rior wall and retracted toward the patient’s right side, that is, away from the spleen. This provides better exposure than grasping the anterior wall or the greater curve.
Attention is then carried to incising the pars accida along the lesser curvature, heading toward the right crus. Care should be taken as sometimes a replaced left hepatic artery can arise from the left gastric artery. The vessel can be clamped and the liver observed for color change. If none are observed, the vessel is transected. The phrenoesophageal membrane and peritoneum overlying the esophagus is incised, with care to not injure the esophagus or anterior vagus nerve. Then, the pil­lar of the left crus is separated from the esophagus. Dissection is then carried into the mediastinum. This is done mostly in a blunt fashion, anteriorly and laterally to the esophagus, so that at least 9cm of esophagus is exposed for esophageal myot­omy later, or dissected roughly to the level of the inferior pulmonary vein. The lat­eral esophageal dissection can help straighten the esophagus, whether this provides better emptying or not is unknown.
Next, the fat pad is excised from the anterior surface of the esophagus taking care not to injure the anterior vagus nerve. It is best to start taking the fat pad starting on
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the greater curve, heading toward the lesser curve with frequent, gentle pulling of the fat pad caudally to locate the vagus nerve.
Myotomy
The myotomy can be performed with or without a bougie in place. If utilized, a 56 French or similar-sized lighted Bougie is carefully inserted into the esophagus, to facilitate the myotomy. The lighted bougie can be seen entering the abdominal cavity both on white mode and near-infrared mode. The anesthesia team can per­form or the surgeon can insert the bougie to avoid the complication of an esopha­geal perforation. The myotomy can be performed in a blunt fashion (Fig.13.1a), using scissors, a hook (Fig.13.1b), or bipolar device (vessel sealer or Synchroseal). The magnication, stability of the robot platform, and 3D magnication allow this critical portion of surgery to be performed with precision. Initially, the longi­tudinal outer muscle of the esophagus is bluntly separated (Fig.13.2a). This facil­itates exposure of the circular muscle of the esophagus, which is then incised with the vessel sealer, which is our preferred approach (Fig.13.2b). The esophageal myotomy is then extended 7–9cm proximally along the esophagus, exposing the underlying mucosa (Fig.13.3). The lighted bougie helps with the mucosal transil­lumination to ensure myotomy completion (Fig.13.4). It is important to note there is currently no consensus on the length of the myotomy. Currently, SAGES rec­ommends a 4cm esophageal myotomy, extended on the stomach for 2cm. The 2018 International Society of Diseases of the Esophagus guidelines recommend at least 6cm proximal to the gastroesophageal junction, with 2cm distal to the junc­tion [41].
The myotomy is then extended distally along the stomach, for a length of 3cm (Fig.13.5). Wright etal. compared 52 patients with achalasia undergoing a Heller myotomy, extending for 1–2cm onto the gastric wall and Dor fundoplication to 63 patients who underwent an extended myotomy (3cm) with Toupet fundoplica­tion, and found that the extended 3cm myotomy gave patients better relief of dysphagia [42]. This is done with blunt dissection, exposing the underlying mucosa. The robot, with its articulating instruments, allows this portion of
Fig. 13.1 The myotomy can be performed using various techniques, including blunt dissection (a), or with the aid of instruments such as scissors, a hook (b), or a bipolar device like the Synchroseal or a vessel sealer
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13 Management ofAchalasia: ADisease Hard toSwallow
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Fig. 13.2 The procedure begins with blunt separation of the longitudinal outer muscle of the esophagus (a), allowing clear exposure of the underlying circular muscle. The circular muscle is then incised using the vessel sealer (b and c)
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Fig. 13.3 The esophageal myotomy is then extended 7–9cm proximally along the esophagus, exposing the underlying mucosa. In image “b” an epinephrine-soaked sponge is used to clear the blood and facilitate visualization. In image “c” you can see the anterior Vagus nerve crossing from left to become the anterior Vagus nerve in the abdomen. This should be preserved and retracted during the myotomy
Fig. 13.4 A lighted bougie helps with the mucosal transillumination to ensure myotomy completion
Fig. 13.5 The myotomy is further extended distally along the stomach, for a length of 3cm
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surgery, which is the most difcult due to the high risk of perforation on the gas­tric side due to the insertion of the oblique bers. Unlike during laparoscopy, there is no tactile feedback and the surgeon relies heavily on visual cues. Also, unlike laparoscopy, this portion can be performed with a hook in a top-down fashion from the esophagus toward the stomach because the instruments articulate. Completion of gastric myotomy should be conrmed by visualizing the cardinal vein, which can sometimes bleed during this portion of the procedure (Fig.13.6). Care should be taken not to use energy sources as to avoid gastric mucosal injury that can result in immediate versus delayed perforation. It is best to control any bleeding from the myotomy with soaked epinephrine gauze and gentle pressure (Fig.13.7). The length of both esophageal myotomy and gastric myotomy is mea­sured with a ruler that is inserted intra-abdominally.
The lighted bougie is then removed and intraoperative upper endoscopy is per­formed. We like to perform a picture-in-picture endoscopy, that is, Tile Pro, where the surgeon can see both the laparoscopic view of abdomen and the endoscopic image at the console. The purpose of endoscopy is to
1. Examine the ease by which the scope passes the GE junction prior to and after
the wrap.
2. Conrm the GE junction and, therefore, conrm the length of gastric myotomy
and esophageal myotomy.
3. Examine the completion of myotomy by noticing mucosa bulging.
4. Examine for any bougie injury or mucosal perforation. The latter is performed
by emerging the mucosa underwater and performing a leak test.
Partial Fundoplication
Attention is then paid to the partial fundoplication; the type of partial fundoplication performed, Dor versus Toupet, is still under debate. Tomasko etal. retrospectively compared patient outcomes for laparoscopic Heller myotomy with either Dor ver­sus Toupet and found overall patient satisfaction was similar (93.8% vs. 87.5%)
Fig. 13.6 The completion of the gastric myotomy is conrmed by visualizing the cardinal vein
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13 Management ofAchalasia: ADisease Hard toSwallow
Fig. 13.7 Bleeding from the gastric myotomy is managed using epinephrine-soaked gauze and gentle pressure, which helps prevent delayed thermal injury to the mucosa
[43]. They found no difference in regards to the incidence or severity of postopera­tive heartburn, dysphagia, or bloating. The mean operative time for the Toupet fun­doplication was 137.3min while the mean time for the Dor fundoplication was only
111.5 min (p =0.006). Torres etal. had similar ndings with their randomized control trial comparing laparoscopic Heller myotomy with Dor versus Toupet fun­doplication in regards to patient symptom scores and high-resolution manometry long term [44]. These studies provide support that either option is acceptable.
The goal of surgical intervention of achalasia is to provide an adequate myotomy while reducing the risk of reux. This lies with an intraoperative assessment of the LES during the myotomy and fundoplication that can be done with EndoFLIP.Law etal. were able to report changes in EndoFLIP prior to and after myotomy and after fundoplication. There was a measurable difference in mean DI from 0.7 mm2/ mmHg prior to myotomy to 3.2mm2/mmHg after (p<0.001). Following fundopli­cation, the DI decreased to 2.2mm2/mmHg following Dor fundoplication, which is consistent with previous studies nding a decrease in DI to 3.3 from 4.5mm2/ mmHg. This distensibility has been correlated to an Eckardt score with a DI <3 associated with an Eckardt score of >3, indicating treatment failure. The median Eckhardt score in this study utilizing intraoperative EndoFLIP after myotomy and fundoplication was 0 from 4.5 prior to surgery, still with no reported symptoms of postoperative reux. Therefore, EndoFLIP can be a benecial adjunct during sur­gery to reduce the risk of reux symptoms and provide optimal patient outcomes [17]. In cases where the patient has an end-stage esophagus, that is, sigmoid esopha­gus, no wrap is performed. In these patients, we only reconstitute the angle of His to reduce reux without compromising the EGJ.Endoip is a great adjunct that is currently available at some institutions and can help tailor the choice of wrap; how­ever, the authors do not currently use this technology.
Our institution typically performs the Dor procedure. The initial and possibly most important step is to reapproximate the angle of His, by suturing the medial portion of the fundus to the left crus with a #2-0 Ethibond suture (Fig.13.8a, b). The fundoplica­tion is then performed by suturing the medial portion of the fundus to the left pillar of the crus and the left edge of the myotomy. Then, two additional sutures are placed distally, approximately 1cm apart, securing the medial portion of the fundus to the left
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edge of the myotomy (Fig.13.8c). The most lateral part of the fundus is then secured to the right edge of the myotomy and the right pillar of the crus. Two additional sutures are then placed distally, securing the lateral fundus to the right edge of the myotomy, ensuring no mucosa is incorporated. Finally, one last stitch is placed between the upper two stitches and the crus, completing the Dor fundoplication.
After the wrap is completed, repeat upper endoscopy is performed to ensure patency of the gastroesophageal junction and the scope is retroexed to assess the wrap (Fig. 13.9). Richards etal. performed a prospective, randomized, double­blind, control trial comparing surgical outcomes in patients who underwent Heller myotomy alone versus Heller myotomy plus Dor fundoplication [39]. They enrolled 43 patients and found pathologic reux, dened as distal esophageal acid exposure
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Fig. 13.8 Dor fundoplication is performed to re-establish the angle of His. This involves suturing the medial portion of the fundus to the left crus using a #2-0 Ethibond suture (a and b). Total of three sutures are placed separated 1 cm apart. The fundoplication is then completed by securing the the anteriolateral fundus to the right pillar of the crus and the right edge of the myotomy with three sutures separated 1 cm apart
Fig. 13.9 A repeat upper endoscopy with retroexion is performed to assess the integrity and positioning of the wrap after its completion. It’s important to make sure the wrap although partial is not too tight when the scope is traversing the GE junction. The latter can lead to postoperative dysphagia
13 Management ofAchalasia: ADisease Hard toSwallow
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time of more than 4.2% per 24-hr period 6months postoperatively, was signicantly reduced in the Heller plus Dor group (47.6% vs. 9.1%). There was no difference found in postoperative dysphagia. These ndings were investigated for long-term effects by Broman etal., who contacted the previous cohort 10 years later, and found the dysphagia scores were slightly worse for Heller alone than Heller plus Dor, but the ndings were not statistically signicant [45]. Interestingly, 96% of patients contacted stated they would undergo the operation again.

Intraoperative Complications

Esophageal Perforation
Iatrogenic esophageal perforation can occur while creating the myotomy. This should be noticed intraoperatively with the assistance of the lighted bougie (Fig.13.10a) or during upper endoscopy with or without a leak test. This can be repaired with a 5-0 PDS suture, in a gure-of-eight fashion (Fig.13.10b). A leak test should be performed after repair (Fig.13.10c).
Gastric Perforation
Gastric mucosal perforation is more common than esophageal mucosal perforation due to the oblique muscles. This can occur while extending the myotomy into the cardia of the stomach. This can be repaired with a 5-0 PDS suture, in a gure-of­eight fashion (Fig.13.11). A leak test should be performed after repair.
Vagal Nerve Injury
Care should be taken to identify anterior vagus nerve intraoperatively. If one vagal nerve is transected, this should be disclosed to the patient after completion of the case (Fig. 13.12). Unilateral vagal nerve injury can lead to delayed gastric emptying;
abc
Fig. 13.10 An iatrogenic esophageal injury is identied intraoperatively with the assistance of the lighted bougie (a). The injury is repaired using a 5-0 PDS suture (b), followed by a leak test to ensure the repair’s integrity (c)
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Fig. 13.11 An iatrogenic gastric perforation is repaired using a 5-0 PDS suture in an inter­rupted fashion
Fig. 13.12 Unilateral anterior vagal nerve injury is noted intraoperatively
M. Belisle et al.
however, Lindeboom et al. have shown that fundoplication itself may signicantly accelerate gastric emptying [46]. This may be due to alteration in gastric compliance. Postprandial accommodation of the proximal stomach is impaired, which may lead to more rapid transport of the meal to the antrum, thereby accelerating gastric emptying. Oelschlager etal. recently investigated the use of an intentional vagotomy to lengthen the esophagus during complex esophageal surgery [47]. They found no difference in the severity of abdominal pain, bloating, diarrhea, or early satiety between the vagot­omy and no vagotomy groups. They propose it as an alternative to a Collis gastroplasty when extensive esophageal mobilization fails to provide adequate esophageal length.

Postoperative Care

Postoperatively, patients are admitted to the surgical team and are started on a clear liquid diet immediately, even if the patient had a mucosal injury that was repaired intraoperatively. Scheduled antiemetics are given for 5days postoperatively. Patients also receive 10mg of Decadron every 8h for 48h. This helps reduce the swelling