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12 The Endoscopic Treatment ofEsophageal Motility Disorders
38. Lee Y, Brar K, Doumouras AG, Hong D. Peroral endoscopic myotomy (POEM) for the
treatment of pediatric achalasia: a systematic review and meta-analysis. Surg Endosc. 2019;33(6):1710–20. https://doi.org/10.1007/s00464-019-06701-5.
39. Tyberg A, Seewald S, Sharaiha RZ, etal. A multicenter international registry of redo per-oral
endoscopic myotomy (POEM) after failed POEM.Gastrointest Endosc. 2017;85:1208–11.
40. Tyberg A, Sharaiha RZ, Familiari P, etal. Peroral endoscopic myotomy as salvation technique
post-Heller: international experience. Dig Endosc. 2018;30:52–6.
41. Fernandez-Ananin S, Fernandez AF, Balague C, Sacoto D, Targarona EM.What to do when
Heller’s myotomy fails? Pneumatic dilatation, laparoscopic remyotomy or peroral endoscopic myotomy: a systematic review. J Minim Access Surg. 2018;14:177–84.
42. Repici A, Fuccio L, Maselli R, et al. GERD after per-oral endoscopic myotomy as com-
pared with Heller’s myotomy with fundoplication: a systematic review with meta-analysis. Gastrointest Endosc. 2018;87:934–943.e18.
43. Inoue H, Ueno A, Shimamura Y, et al. Peroral endoscopic myotomy and fundoplication: a
novel NOTES procedure. Endoscopy. 2019;51:161–4.
44. Rohof WO, Salvador R, Annese V, etal. Outcomes of treatment for achalasia depend on mano-
metric subtype. Gastroenterology. 2013;144:714–8.
45. Salvador R, Costantini M, Zaninotto G, etal. The preoperative manometric pattern predicts the
outcome of surgical treatment for esophageal achalasia. J Gastrointest Surg. 2010;14:1635–45.
46. Pratap N, Kalapala R, Darisetty S, etal. Achalasia cardia subtyping by high-resolution manom-
etry predicts the therapeutic outcome of pneumatic balloon dilatation. J Neurogastroenterol Motil. 2011;17:48–53.
47. Khan MA, Kumbhari V, Ngamruengphong S, et al. Is POEM the answer for management
of spastic esophageal disorders? A systematic review and meta-analysis. Dig Dis Sci. 2017;62:35–44.
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Redo Interventions inFailed Procedures
13
KellyR.Haisley andLeeL.Swanström

Introduction

Esophageal motility disorders can present with a wide array of clinical symptoms and dynamic differences in esophageal function, which makes recognition of treat­ment failures complicated. While achalasia is the best dened disorder of esopha­geal motility, there are other diagnoses that fall outside the achalasia denition, including esophageal outow obstruction, major disorders of peristalsis (diffuse esophageal spasm (DES), hypercontractile esophagus, absent contractility), and minor disorders of peristalsis (ineffective esophageal motility, fragmented peristal­sis) [1]. While esophageal relaxing medications such as calcium channel blockers or endoscopic approaches such as Botox or dilation are often applied for short-term symptom relief, surgical myotomy is the most commonly applied technique (thora­coscopic, laparoscopic, or endoscopic) for denitive management of esophageal motility disorders. Esophageal myotomy is effective for both hyper-contractile dis­orders (in which cutting the muscle decreases its ability to spasm) and hypocontrac­tile disorders (in which cutting the LES allows for easier bolus clearance either by weak peristalsis or gravity). With appropriate initial workup and a fastidious surgi­cal technique, myotomy can have an 80–90% initial clinical response rate when applied to appropriately selected patients [2].
Nevertheless, some patients will develop recurrent symptoms after myotomy. The recommended treatment course after a failed motility procedure is not well dened and no randomized trials have been conducted to date [3]. Management choices depend primarily on the patient’s clinical symptoms, severity of disease, and the cause of the treatment failure. Depending on individual factors, treatment options may include medical management, pneumatic dilation, surgical revision, or conversion to an alternate surgical procedure [3].
K. R. Haisley (*) · L. L. Swanström Gastrointestinal and Minimally Invasive Surgery, The Oregon Clinic, Portland, OR, USA
© Springer Nature Switzerland AG 2021 N. Zundel et al. (eds.), Benign Esophageal Disease,
https://doi.org/10.1007/978-3-030-51489-1_13
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Rates ofMotility Treatment Failure
Despite high rates of early treatment success, recurrent dysphagia or other symp­toms will return in approximately 10–20% of patients in the years following their index operation. Despite these relatively high rates of recurrent symptoms, the need for operative re-intervention remains low. In a retrospective analysis of more than 12,000 patients undergoing Heller myotomy over a 38-year period, Gouda and col­leagues showed a rate of reoperation of only 6.2% [4]. Of note, these re- interventions generally did not take place until 8–9years after the initial operation, indicating either a late recurrence or that many patients live with these symptoms for an extended period before getting treatment [3].
POEM (per-oral endoscopic myotomy) is a newer treatment for motility disor­ders, with less than 10years of outcomes data, and therefore less knowledge of the causes of treatment failures and rates of re-intervention in POEM patients. A recent 5-year follow-up by Teitelbaum etal. suggests that there is a small but signicant worsening of symptoms between 2 and 5years, though the clinical relevance of this remains to be seen. However, only three patients of 36 (0.8%) in this study required re-intervention in this period, two for recurrent dysphagia and one for the new onset of GERD (gastroesophageal reux disease) [5]. Longer term outcomes and rates of re-intervention remain to be dened.
In total, these data suggest a moderate rate of recurrent symptoms (20–30%) fol­lowing operative treatment for motility disorders, but a low rate of need for inter­vention following surgical myotomy (0.8–6.2%).
Symptoms ofMotility Treatment Failure
The clinical course after surgical myotomy can be challenging to follow as symp­toms and recovery are highly subjective. A well myotomized esophagus, in the setting of either primary aperistalsis due to achalasia or induced aperistalsis due to a long myotomy, will never truly function normally, and as such, some degree of swallowing abnormality is likely to persist for many patients who have had these procedures. However, failure to have any improvement after myotomy, or worsen­ing dysphagia after a period of improvement, should raise clinical concern for treat­ment failure. By far the most common presenting symptom of a failed motility procedure is dysphagia, with a mean time to recurrence of approximately 1.5years after the index operation [6]. Other less specic symptoms can also certainly develop, including persistent chest pain or gastroesophageal reux disease (GERD) symptoms such as heartburn or regurgitation. The presenting symptom will depend somewhat on the reason for treatment failure and should help guide the clini­cal workup.
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151
Table 13.1 Indications for re-intervention after motility procedures
Incomplete myotomy 50% Gastroesophageal reux disease 30% Megaesophagus 16% Others 4%
Reasons forMotility Treatment Failure
There can be a number of reasons for treatment failure after surgical myotomy (Table13.1), and the optimal treatment approach will ultimately depend on the eti­ology of the failure, the impact on quality of life, and the patient’s surgical risk prole. The specic reason for recurrence may also predict the likelihood of suc­cessful intervention. Veenstra and colleagues showed that while re-intervention for dysphagia related to an incomplete myotomy or a failed fundoplication will have long-term success rates of approximately 75%, those rates drop dramatically to 0–40% if the cause of the recurrent dysphagia is related to a mucosal stricture or signicant brosis [7]. For these reasons, a thorough investigation and understand­ing of the cause of treatment failure is essential in designing an appropriate treat­ment plan.
Incorrect Indication forInitial Surgery
Given the challenges in diagnosing motility disorders, it sometimes occurs that a treatment failure is related to a simple misunderstanding of the primary motility disorder prior to the index operation. Esophagogastric junction outow obstruction (EJGOO) can be a particularly challenging clinical entity. While an elevated IRP is characteristic of both EGJOO and achalasia, EGJOO is a distinctly different clinical entity than achalasia, as peristalsis is typically preserved and the LES failure may be intermittent. EGJOO can be caused by a number of different clinical entities, such as GERD, PEH, or even cancer. Recent reports suggest that in the setting of EGJOO, in spite of traditional approaches focusing on relieving the obstruction with endo­scopic dilation, Botox, POEM, or laparoscopic myotomy, relatively few patients respond well in terms of symptom resolution and surgical treatment should be con­sidered with caution [1]. In fact, a myotomy in EGJOO can even cause worsening symptoms if, for example, their true underlying disease process was GERD.For this reason, review of the initial workup and motility is an important key in treating these patients.

Primary Surgical Failure

If the patient fails to have the expected response from their procedure in the imme­diate postoperative period, a primary surgical issue should be considered. This may include incomplete myotomy or a problem with the fundoplication. Certainly a
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grace period to allow for resolution of postoperative swelling and general recovery should be allowed, though some authors prefer revisional surgery as the rst step in the treatment algorithm if dysphagia is early onset (<3 months) rather than any attempts at endoscopic dilation [3]. Later presentations of dysphagia are less likely to be due to a primary surgical failure and treatment will depend more on the deter­mined specic cause of the symptoms.

Incomplete Myotomy

Incomplete myotomy is the most common indication for re-intervention after a sur­gical myotomy, responsible for around 50% of all revisional operations [4]. This should certainly be the rst suspicion when evaluating a patient with recurrent dys­phagia after myotomy and can be related to either inadequate proximal or distal extent of the myotomy. Studies have shown that an extended myotomy at the initial procedure reduces relapse rates from 17% to 5% and the need for re-intervention from 7% to basically 0% [3]. When incomplete myotomy is suspected as the cause of symptoms, surgical extension of the myotomy, either laparoscopically or endo­scopically, is likely to be effective. While this can certainly occur in achalasia if the myotomy is not extended all the way through the high pressure zone, it is particu­larly likely in cases of non-achalasia motility disorders, such as diffuse esophageal spasm (DES) and Jackhammer esophagus that involve the entire esophagus. As the problematic portion of the esophagus may extend well above a traditional LES myotomy, a classic laparoscopic Heller myotomy (LHM) may result in an incom­plete myotomy being performed, simply due to the technical limitations of extend­ing a long myotomy from an abdominal approach. While VATS can be performed to lengthen this dissection, it does add some morbidity. POEM can be more effective in this regard, but incomplete myotomy is still possible if there is missed division of circular bers or incomplete proximal or distal extension due to misidentication of landmarks or measurements.

Gastroesophageal Reflux Disease (GERD)

New or recurrent GERD is always a concern after myotomy and is the second most common indication for late reoperation in this population, making up 30% of redo cases [3]. This is a particular concern in pneumatic dilation and POEM, which typi­cally do not include any fundoplication. Even in the case of a myotomy with fundo­plication, wraps do have a progressive rate of failure, which can lead to the development of post-myotomy GERD.Furthermore, although current approaches for LHM generally include a fundoplication, this was not always the case in previ­ous eras and its presence cannot necessarily be assumed [4].
The rates of increased acid exposure in the distal esophagus after POEM are consistently around 30% on objective testing, nearly twice that of the early rates reported for LHM patients with fundoplication [8]. It should be noted, however, that
13 Redo Interventions inFailed Procedures
quality of life scores seem to be fairly similar between the two groups and that most patients with post-myotomy GERD (POEM or LHM) are easily managed with med­ications alone [9]. Reux can also be the cause of dysphagia (due to esophagitis or peptic strictures), in which case aggressive PPI therapy is indicated as rst-line treatment before surgical re-intervention [3]. However, in rare cases, revisional sur­gery may be required if symptoms are severe or if patients have a contraindication to long-term acid suppression medication.
153

Failed Fundoplication

In patients undergoing LHM with fundoplication, wrap failures can cause signi­cant symptoms. A loose or dehisced fundoplication can lead to GERD, as men­tioned above, while a slipped, overly tight, or herniated fundoplication may be associated with dysphagia or pain. Wrap failure is the reason for reoperation in approximately 25% of failed Heller myotomies [7]. In these cases, treatment may need to be focused on the wrap rather than the myotomy.

Ineffective Esophageal Motility/Pan-Aperistalsis

Many severe dysmotility disorders, aside from achalasia, are part of a progressive disease process that can lead to the subsequent deterioration of the motor function of other parts of the GI tract, including proximal esophagus, stomach, and small and large intestines. Therefore, recurrent dysphagia or regurgitation may develop even in the face of a successful LES myotomy [4]. Treatment options for generalized ineffective motility or pan-aperistalsis are less robust as there is no available method to restore esophageal motility once lost. Treatment goals in these cases should focus on esophageal emptying, chest pain mitigation, and controlling reux. In these cases, expectation management is extremely important. Despite aggressive and appropriate treatment, these complex cases can lead to progressive dilation with megaesophagus and end-stage failure, which eventually becomes the indication for surgery in 16.2% of patients who require revision after LHM [4]. When the esopha­gus progresses to this point, esophagectomy may be the best option for the patient.

Esophageal Cancer Development

Patients with achalasia have an elevated risk of squamous cell carcinoma due to chronic stasis, inammation, and increased exposure to carcinogens in the diet. This risk persists even in treated patients and may be as high as 3%—considerably higher than the general population. This stresses the importance of continued lifelong sur­veillance of all patients who have undergone myotomy for achalasia [3, 4]. In addi­tion, increased acid exposure in the distal esophagus from GERD after myotomy can also theoretically put the patient at risk of intestinal metaplasia (IM) and the
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development of adenocarcinoma. When identied, these patients should be treated along standard esophageal metaplasia/dysplasia/cancer protocols regardless of their concomitant motility disorder [10].
Other Causes ofTreatment Failure
Other possible causes of recurrent symptoms after surgical myotomy include but are not limited to diverticulum, healing/closure of the myotomy, and acute surgical complications such as bleeding and seroma/abscess.

Patient Workup

When a patient returns with persistent or recurrent symptoms after surgical myot­omy, the rst priority is to complete a thorough workup to identify the cause of the failure and to tailor a specic treatment plan for the patient moving forward [11].

Upper Gastrointestinal Series (UGI) +/− Barium Tablet

An UGI is an important starting point in understanding treatment failure after motil­ity surgery. Contrast evaluation provides valuable information about the anatomy of the esophagus, particularly if the preoperative lms are available for comparison. UGI can identify many fundoplication issues, hiatal hernias and will document ana­tomic evolution of the esophagus such as sigmoidization or end-stage dilation that could add to the difculty of any redo interventions, particularly POEM (Fig.13.1). It will further allow for the identication of any diverticulization that may have
Fig. 13.1 UGI series showing recurrent achalasia with signicant dilation and sigmoidization of the esophagus 15years after laparoscopic Heller myotomy
13 Redo Interventions inFailed Procedures
155
developed from an intrathoracic myotomy site and could be contributing to symp­toms. UGI may show also the presence of esophageal spasm, which can be a valu­able diagnostic tool, particularly if the patient is symptomatic at the time of the test.
A formal timed barium swallow in addition to the standard UGI is extremely helpful in the setting of motility disorders, as this will quantify the degree of delayed emptying and can be an objective follow-up tool for success or failure. A barium tablet may be particularly useful to assess the location of any transit delay if solid dysphagia is the main presenting symptom.
UGI may be particularly helpful in identifying which patients are likely to ben­et from redo myotomy, with those patients who have limited dilation and tortuosity being more likely to respond to redo myotomy, while those with massive esopha­geal dilation or signicant tortuosity being more likely to benet from esophageal resection [12].

Esophagogastroduodenoscopy (EGD)

Upper endoscopy (EGD) should be performed in all patients with recurrent symp­toms after motility surgery to evaluate for structural abnormalities or other unex­pected pathology (especially cancer) that could be causing symptoms. As discussed above, GERD may lead to esophagitis, ulcers, or strictures that may be better man­aged with medical therapy once diagnosed. Retained food or liquid in the esophagus or stomach on EGD is also helpful in understanding the degree of delayed esopha­geal or gastric emptying. If a fundoplication is present, its position and structure can be evaluated with visual inspection to determine if a wrap failure is present. Sometimes, an incomplete myotomy can be appreciated by feeling resistance to passage of the scope, hinting at the location of the failure. In cases where there is question, functional luminal imaging (Endoip, Medtronic, Ireland) measurements can be taken to help better dene any areas of narrowing and identify if an incom­plete myotomy is present based on a persistent waist of high resistance [13].

High-Resolution Manometry (HRM)

High-quality manometry is essential to the understanding of the relationship of motility disorders of the esophagus and symptoms. While motility studies are cer­tainly important before undertaking an initial operation, they are extremely valuable in understanding treatment failure, as they may detect a misdiagnosis in the initial surgery or a clinical change over time. Repeating manometry can allow for a com­parison of the LES pattern and pressure before and after surgery to assess for incom­plete myotomy and the degree of remaining peristalsis. In cases of Jackhammer esophagus or DES, motility will show whether hyper-contractile or spastic seg­ments remain proximal to the area of the previous myotomy, or if new proximal spasm has developed. High-resolution manometry with impedance in combination
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K. R. Haisley and L. L. Swanström
Fig. 13.2 High-resolution manometry performed 15 years after a laparoscopic Heller myotomy in a patient with recurrent dysphagia to solids with the catheter unable to be threaded past the LES with pan-esophageal pressurization from uid stasis
with the revised Chicago classication provides the most comprehensive evaluation of esophageal function and should be a part of a standard foregut workup (Fig.13.2) [14].

pH/Impedance

If post-myotomy GERD is suspected, it is important to conrm its presence through objective pH testing as many of the symptoms and even some manometric ndings of motility disorders can be caused by reux [1]. Contrarily, it is also well described that GERD symptoms, and even lack of symptoms, are extremely unreliable indica­tors of true gastroesophageal reux [15]. A 48-h Bravo pH can be placed at the time of EGD, or standard 24-h pH testing can provide similar information. Impedance testing can be included with a standard pH to evaluate for non-acid uid reux, though this data can be somewhat difcult to interpret in the setting of the esopha­geal stasis that is common in esophageal dysmotility disorders and the data should be interpreted with caution and by an expert.
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157

Gastric Emptying Study (GES)

Patients with esophageal motility disorders may have dysmotility in other parts of the GI tract as well. In the case of gastroparesis or delayed gastric emptying (DGE), backup in the stomach can cause overow reux into the esophagus. Patients with this problem typically complain of bloating, nausea, and early satiety in addition to their more clas­sic reux symptoms. If this is suspected or conrmed, treatment may be best focused on improving gastric emptying rather than simply esophageal interventions [16].

Medical Treatments

Medical options for failed motility procedures are somewhat limited, though in the setting of mild symptoms or high-risk patients, conservative treatment may be best in some scenarios. If spasm, as evidenced by noncardiac chest pain, is a primary issue, either new or persistent, esophageal relaxants such as calcium channel blockers (Nifedipine) may be effective in a partially myotomized esophagus, even if the patient had incomplete relief with these medications prior to their myotomy. Narcotics should be avoided whenever possible in the case of esophageal pain as they are unlikely to be effective and carry signicant side effects and risks and can actually worsen esopha­geal contraction strength or induce spastic peristalsis [17]. Botox is generally not rec­ommended after a myotomy due to high failure rates and development of brosis [3].

Pneumatic Dilation (PD)

Pneumatic dilation is a good initial treatment choice in patients with recurrent dys­phagia after myotomy, with 64–80% of recurrent patients (whether LHM or POEM) having good results with PD [3]. PD is reported to have a high short-term success rate of approximately 85% after failed Heller but requires a mean of 2.5 treatments (range 1–3) [3]. Presumably, some of this effect would deteriorate over time, as is the case in treatment naive achalasia patients who undergo pneumatic dilation, in whom long-term effectiveness is only around 60% [3]. If pneumatic dilation is to be under- taken after a myotomy, timing is important. The risk of perforation is highest in the rst 4months after surgery, and it should be avoided in this time frame if possible. If early dilation is needed, it can be still acceptable to dilate to 35–40mm, but it should be recognized that there is signicant scar tissue, which will increase the risk of esophageal perforation with more aggressive dilations [3]. After two dilations, if the patient is not improved, it should be considered a treatment failure and surgical revi­sion should be considered. Savary or wire guided dilations are also acceptable but are less effective and should be used with caution in cases of sigmoid esophagus.
Many patients who undergo PD will continue to progress to needing formal sur­gical re-intervention, whether by LHM or POEM. While there is no data to suggest that a previous PD complicates success rates of a redo LHM, there is some conict­ing data on whether PD may lead to decreased success rates of POEM, but this data is somewhat limited, particularly in the redo population [18, 19].