Received: 27 January 2020 | Revised: 3 April 2020 | Accepted: 13 April 2020

DOI: 10.1002/ags3.12344

REVIEW ARTICLE

Surgical management of achalasia

Kamil Nurczyk1,2 | Marco G. Patti1,3

1
Department of Surgery, University of North
Carolina at Chapel Hill, Chapel Hill, NC, USA Abstract
2
2nd Department of General and Esophageal achalasia is a primary esophageal motility disorder characterized by
Gastrointestinal Surgery, and Surgical
lack of peristalsis and by incomplete or absent relaxation of the lower esophageal
Oncology of the Alimentary Tract, Medical
University of Lublin, Lublin, Poland sphincter in response to swallowing. The cause of the disease is unknown. The goal
3
Department of Medicine, University of of treatment is to eliminate the functional outflow obstruction at the level of the
North Carolina at Chapel Hill, Chapel Hill,
NC, USA
gastroesophageal junction, therefore allowing emptying of the esophagus into the
stomach. They include the laparoscopic Heller myotomy with partial fundoplication,
Correspondence
Marco G. Patti, Department of Medicine and
pneumatic dilatation, and peroral endoscopic myotomy. Esophagectomy is consid-
Surgery, University of North Carolina, 4030 ered as a last resort for patients who have failed prior therapeutic attempts. In this
Burnett Womack Building, 101 Manning
Drive 7081, Chapel Hill, NC 27599-7081,
evidence and experience-based review, we will illustrate the technique and results of
USA. the surgical treatment of esophageal achalasia and compare it to the other available
Email: Marco_patti@med.unc.edu
treatment modalities.

KEYWORDS

dor fundoplication, esophageal achalasia, heller myotomy, peroral endoscopic myotomy,

pneumatic dilatation

1 | I NTRO D U C TI O N The lack of peristalsis and the impaired LES relaxation impair the
transit of the food bolus from the esophagus into the stomach, leading
Esophageal achalasia is a rare disease that affects approximately one eventually to dilatation of the esophageal body. Almost every patient
in 100 000 people, regardless of gender or race. However, in some experiences dysphagia, which often leads to weight loss. Regurgitation
geographical locations such as Brazil, it is much more common in of undigested food is also a common ailment, and may cause compli-
1
connection to the high prevalence of Chagas disease. The incidence cations such as hoarseness, coughing, wheezing, and pneumonia. Up
of achalasia increases with patient age. 2 In addition, it seems that the to 50% of patients with achalasia also experience heartburn, which
prevalence of this disease is increasing, probably due to improve- is caused by fermentation of retained food in the esophagus. Chest
ments in diagnostic modalities.3 discomfort or pain can also occur, and they are caused by esopha-
In normal conditions, the lower esophageal sphincter (LES) relaxes geal distension, which usually increases while eating.4 The severity of
in response to swallowing. This physiological mechanism is dependent achalasia symptoms is evaluated using the Eckardt score.
on neurogenic control of the esophagus and LES through the myenteric
plexus, combining excitatory acetylcholine neurons, inhibitor nitric
oxide, and VIP neurons. Idiopathic achalasia is due to the degeneration 2 | PR EO PE R ATI V E WO R K- U P
of inhibitory neurons, which are involved in the relaxation of LES. As a
result, the LES does not relax properly in response to swallowing, and it A comprehensive evaluation of every patient should be carried out
is often hypertensive. In addition, there is a lack of esophageal peristalsis. to confirm the initial diagnosis suggested by the symptoms, and

This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium,
provided the original work is properly cited.
© 2020 The Authors. Annals of Gastroenterological Surgery published by John Wiley & Sons Australia, Ltd on behalf of The Japanese Society of
Gastroenterological Surgery

Ann Gastroenterol Surg. 2020;4:343–351.  www.AGSjournal.com | 343

344 | NURCZYK and PATTI

should include: esophagogastroduodenoscopy (EGD), barium swal- 3 | S U RG I C A L TR E ATM E NT O F

low, esophageal manometry, and in some cases ambulatory 24-hour E S O PH AG E A L AC H A L A S I A
pH monitoring.
Usually, the evaluation begins with an EGD to exclude other 3.1 | Evolution of surgery for esophageal achalasia
causes of dysphagia such as a peptic stricture or a tumor. Typical
findings are esophageal dilation and presence of retained food. In 1914, the first transabdominal extramucosal cardioplasty was de-
Sometimes candidiasis of the esophageal mucosa is present. It is scribed by Heller. He performed the myotomy both on the anterior
worth mentioning that gastroesophageal junction cancer infiltrat- and posterior walls of the cardia.12 Groeneveldt and Zaaijer simpli-
ing the LES may mimic achalasia. This misleading condition, called fied the procedure by performing one myotomy only.13 In the 1940s
pseudo-achalasia, should be ruled out in elderly patients, with short and 1950s, the transabdominal approach was the most commonly
duration of symptoms and marked weight loss.5 used, while few surgeons favored a left trans-thoracic approach.14
The barium swallow often shows the characteristic “bird beak” Until the 1960s, the focus of treatment was on the relief of the
sign (narrowing at the level of the gastroesophageal junction), de- dysphagia by the myotomy and no consideration was given to the
layed passage of the contrast into the stomach, an air-fluid level, and possibility of post-myotomy reflux. In 1956, Nissen popularized a
tertiary contractions of the esophagus. 360-degree fundoplication to control gastroesophageal reflux and
The gold standard for the diagnosis of achalasia is the high-res- this inspired Dor to propose a 180-degree anterior fundoplication
olution esophageal manometry (HRM). It enables the measurement in 1962 that could be added to the myotomy.15 In 1963, Toupet de-
of the pressure, length, and relaxation of the lower and upper scribed a partial posterior fundoplication.
esophageal sphincters and assessment of esophageal peristalsis. To At the beginning of the 1990s, minimally invasive techniques
confirm the diagnosis of achalasia, it is necessary to document lack were introduced for the treatment of esophageal diseases. The first
of esophageal peristalsis and partial or absent LES relaxation. The laparoscopic cardiomyotomy was performed by Cuschieri in 1991.16
6
Chicago classification introduced by Pandolfino and his colleagues In 1992, Pellegrini et al described the outcomes of myotomy per-
distinguishes three types of achalasia. Type I involves aperistalsis formed through a left thoracoscopic approach, showing excellent
and absence of esophageal pressurization; type II is associated results in about 90% of patients.17 However, it soon became evident
with aperistalsis and pan-esophageal pressurization in at least that the procedure led to abnormal gastroesophageal reflux in 60%
20% of swallows; and in type III there are premature spastic con- of patients.18 Their findings determined a switch to a laparoscopic
tractions (distal latency <4.5 seconds) in at least 20% of swallows. approach combined with a partial fundoplication.19 In 1993, Ancona
What makes the Chicago classification useful is that it can also help et al reported the technique of a laparoscopic esophageal myotomy
predicting treatment outcome. In fact, many studies have shown and Dor fundoplication developed at the University of Padua. 20
7,8
higher success rates in patients with type II achalasia. It has been The same year the laparoscopic and open approach were compared
speculated that type II achalasia is an initial phase of the disease showing that, while the outcomes were similar, the minimally in-
process with pan-esophageal pressurization, while type I rep- vasive approach was associated with a shorter hospital stay, less
resents a later phase with complete absence of any contraction.7 post-operative discomfort, and faster return to regular activities. 21
Type III achalasia, characterized by premature spastic contractions, Finally, at the end of 20th century, the laparoscopic Heller myotomy
is associated with decreased response to surgical treatment.7 It is (LHM) with fundoplication became the standard of care worldwide
speculated that it may represent a recognizably different patholog- (Table 1).
ical process which is not a part of the progression from type II to
type I achalasia, rather being a variant of distal esophageal spasm
which involves the LES.7 3.2 | Laparoscopic Heller myotomy
Ambulatory pH monitoring is not necessary in the work-up of
patients with achalasia. It should be performed only in patients with The treatment of esophageal achalasia is palliative, and it focuses
heartburn and dysphagia who are considered to have gastroesoph- on decreasing the outflow resistance of the GEJ caused by the dys-
ageal reflux refractory to medical treatment. In these patients, this functional LES. LHM has been the gold standard therapy for most
test will distinguish GERD from achalasia.9 Interestingly, up to 50% esophageal achalasia patients. 29,30 SAGES guidelines describe it as a
of patients who end up having a diagnosis of achalasia have been safe and low-risk treatment method for resolving symptoms and im-
treated for prolonged periods of time with proton pump inhib- proving quality of life.31 This statement is based on strong evidence
10
itors on the assumption that abnormal reflux was present. The showing excellent and durable results. 27,31,32,33
pH monitoring study should also be performed after treatment to The evolution of achalasia treatment clearly shows that a fundo-
rule out pathologic gastroesophageal reflux (GER), which is often plication is required to prevent postoperative GERD.34,35 In 2003,
asymptomatic.11 In patients who are asymptomatic, particularly Falkenback et al presented data from a prospective randomized
if young, we do prescribe acid-reducing medications. In patients trial in 20 open Heller myotomy patients comparing those with and
found to have erosive esophagitis, we also prescribe acid-reducing without total fundoplication, at more than 3-year follow-up.36 By pH
medications. monitoring evaluation, they documented pathologic GER in 13.1%
NURCZYK and PATTI | 345

TA B L E 1 Evolution of Minimally
Year Author Importance
Invasive Surgery for Esophageal Achalasia
1991 Shimi et al16 Dr Cuschieri's group from the University of Dundee in
United Kingdom performed the first laparoscopic Heller
myotomy
1992 Pellegrini et al17 Dr Pellegrini from the University of California described
the new technique of thoracoscopic Heller myotomy and
performed the first minimally invasive cardiomyotomy in
the USA
1993 Ancona et al20 The group from the University of Padua in Italy was first to
report the technique of laparoscopic Heller myotomy with
Dor fundoplication.
1995 Ancona et al21 Randomized trial comparing outcomes of laparoscopic and
open Heller myotomy demonstrating the benefits of a
minimally invasive approach
1998 Patti et al18 A comparison of thoracoscopic and laparoscopic Heller
myotomy indicating high rate of postoperative reflux in
patients after myotomy without fundoplication
1999 Patti et al19 Study showing long-term outcomes of laparoscopic and
thoracoscopic Heller myotomy indicating that laparoscopic
Heller myotomy with Dor fundoplication should be
considered the treatment of choice
2001 Melvin et al22 First case report of robotically assisted Heller myotomy
23
2004 Richards et al A randomized controlled study that confirmed the
importance of adding an antireflux procedure to
laparoscopic Heller myotomy in order to avoid
postoperative reflux
2006 Torquati et al24 A report that confirmed good long-term outcomes of
laparoscopic Heller myotomy with Dor fundoplication in
terms of symptom control and occurrence of postoperative
reflux
2008 Rebecchi et al25 A randomized controlled trial that compared laparoscopic
Heller myotomy with total and partial fundoplication
and indicated higher rate of dysphagia symptoms after
total fundoplication with no significant difference in
postoperative reflux rate
2012 Rawlings et al26 A randomized study demonstrating the equivalence
of anterior and posterior partial fundoplication after
laparoscopic Heller myotomy in terms of symptom control
and postoperative reflux
2019 Costantini27 A report of 25-y experience at a single surgical center
showing good long-term outcomes of laparoscopic Heller
myotomy with Dor fundoplication
2019 Werner et al28 First randomized controlled trial comparing outcomes of
laparoscopic Heller myotomy with Dor fundoplication and
peroral endoscopic myotomy demonstrating equivalence
of both techniques in symptom control but higher rates of
esophagitis after POEM

of patients with no fundoplication and 0.15% in the fundoplication that a fundoplication is necessary to control pathologic GER after
23
group. In addition, Richards and colleagues, in a prospective and myotomy.
randomized double-blind trial with 6-month follow-up, proved the Determining whether to perform a total or partial fundoplication
superiority of LHM and anterior partial fundoplication versus LHM was not clear from the start. Topart et al,38 in a 10-year follow-up
alone in terms of postoperative GER, by reporting 47.6% pathologic evaluation of patients after LHM with total fundoplication, showed
reflux after LHM alone, and only 9% after LHM with Dor fundopli- that 82% of the patients had recurrence of symptoms. In contrast,
cation. Campos et al,37 in a large meta-analysis, showed a higher rate Rossetti et al39 described excellent outcomes regarding dysphagia
of pathologic postoperative GER in patients after LHM alone versus symptoms relief in more than 90% of patients, showing no patho-
LHM with fundoplication (32% vs 9%). These findings helped confirm logic GER at mean follow-up of 83 months. In 2008, Rebecchi and
346 | NURCZYK and PATTI

colleagues25 published data from their prospective randomized trial studies from the United States, Asia, and Europe confirmed the
comparing the outcome of a LHM with a Dor or Nissen fundoplica- initial experience, showing excellent relief of symptoms but a very
tion. They found that at 5-year follow-up the postoperative patho- high rate of post POEM pathologic reflux.65,66,67 Schlottmann et al,61
logic GER ratio was similar in both groups. However, patients after in a meta-analysis of 54 studies, compared 5834 patients who un-
total fundoplication had increased dysphagia rate when compared derwent a LHM with 1958 patients treated with POEM, with an
to those after Dor (15% vs 2.8%). Based on these findings, it is clear average follow-up of 24 months. Their study indicated that POEM
that a total fundoplication should not be performed in patients with was slightly more effective than LHM, since the improvement rate
achalasia after LHM, and LHM with partial fundoplication should be of dysphagia was described in 92.7% of patients after POEM, and
the treatment of choice. 27 90.0% of patients from LHM group. However, a significant differ-
The best type of partial fundoplication (anterior or posterior) ence was found in terms of pathologic GER. Ambulatory pH moni-
after LHM remains undetermined. A multicenter prospective trial toring showed pathologic reflux in 48% of patients after POEM, but
by Rawlings et al26 indicated that at 1-year follow-up both proce- in only 11% of patients after LHM. Esophagitis was present in 22% of
dures were equivalent in terms of symptom control and rates of patients after POEM and in 12% after LHM. Kumbhari et al reported
40
pathologic GER. Kumagai and colleagues compared outcomes of a higher rate of clinical response to POEM in patients with type III
LHM with Dor and Toupet fundoplication, finding no significant achalasia when compared to LHM with partial fundoplication (98.0%
difference in postoperative pathologic GER and Eckardt score at vs 80.8%).68 The reason for these different outcomes is probably
1-year follow-up. Since there is no evidence for the superiority due to the fact that POEM allows a proximally extended myotomy.
of one type of partial fundoplication over the other, the choice At the end of 2019, the results of a prospective European mul-
should belong to the surgeon. Some prefer the partial anterior ticenter randomized trial comparing 109 patients who underwent
Dor fundoplication, which requires limited hiatal dissection and LHM with 112 patients after POEM were published. 28 At a 3-month
allows coverage of the exposed mucosa, 33,41,42 while others be- follow-up, the rate of reflux esophagitis was 20% after LHM but 57%
lieve that a partial posterior fundoplication may keep the edges after POEM. The study indicated the equivalence of the two proce-
of the myotomy separated, reducing the probability of recurrent dures in terms of symptom relief at 2-year follow-up, which was not
dysphagia.43,44 surprising as POEM allows an excellent division of the muscle fibers.
Overall, GER remains a major concern for POEM, particularly since
there are data showing the onset of denovo Barrett's esophagus and
3.3 | Technical aspects of LHM reflux stricture after treatment.69 In addition, in 2019 the first case
of esophageal cancer following POEM was reported.70
Our technique for a laparoscopic Heller myotomy has been previ- In patients with end stage of achalasia, many experts recommend
45
ously described in the literature. It consists of a 8 cm myotomy an esophagectomy as primary treatment.71,72 However, esophagec-
extending for 2.5 cm onto the gastric wall and a Dor fundoplication. tomy is associated with longer hospitalization, risk of pneumonia,
anastomotic leak, recurrent laryngeal nerve injury, bleeding, chy-
lothorax, and death.72,73 Considering the satisfactory results of a
3.4 | LHM vs other treatment options myotomy, and the high morbidity and mortality associated with an
esophagectomy, LHM should always be considered as the first-line
Medical therapy and endoscopic botulin injection have limited effect treatment option even in end-stage achalasia, reserving esophagec-
and are indicated for patients who are not fit for other treatment tomy for patients who have failed other treatment options.
46
modalities. Other options commonly used are pneumatic dilatation
(PD) and the peroral endoscopic myotomy (POEM) (Table 2).
In 2015, a large European randomized controlled trial comparing 4 | FO LLOW- U P
LHM and PD was published.58 It showed no significant difference in
success rate between the two treatments, with 84% and 82% suc- Achalasia patients have an increased risk of squamous cell cancer
cess after 5 years for LHM and PD, respectively. However, 25% of after treatment, usually 10 to 50 times higher than the general popu-
patients treated with PD required additional dilatations. It is in fact lation.74,75,76 In addition, some studies have shown that adenocarci-
known that patients treated with PD eventually require additional noma can occur after treatment due to pathologic gastroesophageal
dilatations over time to control the symptoms. This was well shown reflux77,78. Interestingly the group that designed the 2018 ISDE
64
in this randomized trial. In 2017, Ehlers et al also showed that LHM achalasia guidelines specifically said: “We make no recommenda-
was associated with a lower rate of reintervention and readmission. tion about routine endoscopy surveillance or endoscopy intervals
In 2010, Dr Inoue from Japan described a novel endoscopic tech- after any treatment”.79 In our center, we do recommend routine EGD
47
nique – POEM. The myotomy was performed endoscopically by every 3 years or when symptoms recur. Unfortunately, there are no
the creation of a long submucosal tunnel (mean length about 12 cm), precise guidelines regarding the timing and frequency of follow-up
followed by transection of the circular fibers for about 8 cm-6 cm on EGD after intervention for achalasia. Even the 3-year time frame is
the esophagus and 2 cm onto the gastric wall. Many retrospective an arbitrary number that most but not all the insurance companies
TA B L E 2 Studies Comparing Different Treatment Modalities for Esophageal Achalasia

Complication rate Postoperative

Source Year Design Procedures Group size [n] F [%] LOS [d] Remission rate GERD

Ancona21 1995 RC LHMD vs OHMD 34 (17 + 17) 6 0% (LHMD) vs 0% 4 (LHMD) vs 10 94.2%(LHMD) vs by pH: 0% (THM) vs
(OHMD) (OHMD) 100%(OHMD) 5.8% (OHMD)
NURCZYK and PATTI

Patti19 1999 RC THM vs LHMD/T 168 (35 + 133) 28 8.6% (THM) vs 3 (THM) vs 2 85% (THM) vs 93% by pH: 60% (THM)
5.2% (LHMD/T) (LHMD/T) (LHMD/T) vs 17% (LHMD/T)
Richards 23 2004 RCT LHM vs LHMD 43 (21 + 22) 6 0% (LHM) vs 0% 1 (LHM) vs 1 LHM = LHMD (P = .79) by pH: 47.6% (LHM)
(LHMD) (LHMD) vs 9.1% (LHMD)
(P = .005)
Horgan48 2005 RC RAHM vs LHMD 121 (59 + 62) 18 0% (RAHM) vs 1.5 (RAHM) vs 92% (RAHM) vs 90% (LHMD) symptoms: 17%
16% (LHMD) 2.2 (LHMD) (P = .5) (RAHM) vs 16%
(LHMD) (P = .9)
Mikaeli49 2006 RCT PD vs EBTI + PD 54 (27 + 27) 12 0% (PD) vs 0% NA 62% (PD) vs 77% (EBTI + PD) NA
(EBTI + PD) (P = .1)
Kostic50 2007 RCT PD vs LHMT 51 (26 + 25) 12 8% (PD) vs 0% 0 (PD) vs 3 77% (PD) vs 96% (LHMT) NA
(LHMT) (LHMT) (P = .047)
Rebecchi25 2008 RCT LHMD vs LHMN 144 (72 + 72) 60 97% (LHMD) vs 3.2 (LHMD) vs LHMD > LHMN (P = .001) symptoms: 5.6%
85% (LHMN) 3.6 (LHMN) (LHMD) vs 0%
(LHMN)
by pH: 2.8% (LHMD)
vs 0% (LHMN)
(P > .05)
Bakhshipour51 2009 RCT EBTI + PD vs PD 34 (16 + 18) 12 0% (EBTI + PD) vs NA 87.5% (EBTI + PD) vs. 55.5% NA
0% (PD) (PD) (P = .53)
Novais52 2010 RCT PD vs LHMD 94 (4 + 47) 3 4% (PD) vs 0% NA 73.8% (PD) vs 88.3% (LHMD) by pH: 31% (PD)
(LHMD) (P = .08) vs 4.7% (LHMD)
(P = .001)
Boeckxstaens53 2011 RCT PD vs LHMD 201 (95 + 106) 24 4% (PD) vs 12% NA 86% (PD) vs 90% (LHMD) NA
(LHMD) (P = .46)
Rawlings26 2012 RCT LHMD vs LHMT 60 (36 + 24) 12 5.6% (LHMD) vs NA LHMD = LHMT (P > .05) by pH: 41.7%
8.3% (LHMT) (LHMD) vs 21.1%
(LHMT) (P = .152)
Shaligram54 2012 RC RAHM vs LHM vs 2683 (149 + 2116 1 4.02% (RAHM) 2.42 (RAHM) vs NA NA
OHM + 418) vs 5.19% (LHM) 2.70 (LHM) vs
vs 9.08% 4.42 (OHM)
(Open-HM)
Borges55 2014 RCT PD vs LHMD 92 (48 + 44) 24 4% (PD) vs 0% NA 54% (PD) vs 60% (LHMD) by pH: 27.7% (PD)
(LHMD) (P = NS) vs 4.7% (LHMD)
(P = .003)
|
347

(Continues)
TA B L E 2 (Continued)
348
|

Complication rate Postoperative

Source Year Design Procedures Group size [n] F [%] LOS [d] Remission rate GERD

Hamdy56 2015 RCT PD vs LHMD 50 (25 + 25) 12 8% (PD) vs 4% 0 (PD) vs 3 76% (PD) vs 96%(LHMD) symptoms: 28% (PD)
(LHMD) (LHMD) (P = .04) vs 16% (LHMD)
(P = .3)
Persson57 2015 RCT PD vs LHMT 53 (28 + 25) 60 0% (PD) vs 7% NA 64% (PD) vs 92% (LHMT) NA
(LHMT) (P = .016)
Moonen58 2016 RCT PD vs LHMD 201 (96 + 105) 60 5% (PD) vs 11% NA 82% (PD) vs 84% (LHMD) by pH: 12% (PD)
(LHMD) (P = .92) vs 34% (LHMD)
(P = .14)
Chrystoja59 2016 RCT PD vs LHMD/T 50 (25 + 25) 60 4.5% (PD) vs 13% NA 77% (PD) vs 100% (LHMD/T) by pH: 10% (PD)
(LHMD/T) vs 0% (LHMD/T)
(P = .49)
Torres- 2018 RCT LHMD vs LHMT 73 (38 + 35) 24 2.6% (LHMD) vs 2.54 (LHMD) vs 100% (LHMD) vs 90% by pH: 10.5%
Villalobos60 0% (LHMT) 2.54 (LHMT) (LHMT) (LHMD vs 31.5%
(LHMT) (P = .111)
Schlottmann61 2018 M LHM vs POEM 7792 (5834 + 1958) 24 NA POEM (+1.03 d) 92.7% (POEM) vs 90% (LHM) by pH: 11.1% (LHM)
>LHMD (P = .01) vs 47.5% (POEM)
(P < .0001)
EGD: 11.5% (LHM)
vs 22.4% (POEM)
(P < .0001)
Ponds62 2019 RCT POEM vs PD 133 (67 + 66) 24 0% (POEM) vs 2% NA 92% (POEM) vs 54% (PD) by EGD: 41%
(PD) (P < .001) (POEM) vs 7% (PD)
(P = .002)
Costantini63 2019 CCS POEM vs LHMD 240 (140 + 140) 24 5% (POEM) vs 2 (POEM) vs 3 99.3% (POEM) vs 97.1% by pH: 38.4%
2.1% (LHMD) (LHMD) (LHMD) (P < .12) (POEM) vs 17.1%
(LHMD) (P < .01)
by EGD: 37.4%
(POEM) 15.2%
(LHMD) (P < .05)
Werner28 2019 RCT POEM vs LHMD 221 (109 + 112) 24 2.7% (POEM) vs POEM = LHMD 83% (POEM) vs 81.7% by pH: 30% (POEM)
7.3% (LHMD) (95% CI, (LHMD) (P = .007 for vs 30% (LHMD)
−0.12-0.63) noninferiority) by EGD: 44%
(POEM) and 29%
(LHMD) (95% CI
1.03-3.85)

Abbreviations: CCS, case control study; EBTI, endoscopic botulin toxin injection; EGD, esophagogastroduodenoscopy; F, months of follow-up; LHM, laparoscopic Heller myotomy; LHMD, laparoscopic
Heller myotomy with Dor fundoplication; LHMD/T, laparoscopic Heller myotomy with Dor or Toupet fundoplication; LHMN, laparoscopic Heller myotomy with Nissen fundoplication; LHMT, laparoscopic
Heller myotomy with Toupet fundoplication; LOS, length of stay; M, meta-analysis; NA, data nonavailable; OHM, open Heller myotomy; OHMD, open Heller myotomy with Dor fundoplication; PD,
Pneumatic dilation; pH, pH-monitoring; POEM, peroral endoscopic myotomy; RAHM, robotically assisted Heller myotomy; RC, retrospective cohort; RCT, randomized controlled trial; THM, thoracoscopic
Heller myotomy.
NURCZYK and PATTI
NURCZYK and PATTI | 349

accept. Some insurance companies will allow an EGD only if a patient disease: a multicenter study. J Laparoendosc Adv Surg Tech A.
2016;26(7):548–50.
has recurrent symptoms.
11. Patti MG, Arcerito M, Tong J, De Pinto M, de Bellis M, Wang A,
et al. Importance of preoperative and postoperative pH moni-
toring in patients with esophageal achalasia. J Gastrointest Surg.
5 | TR E ATM E NT A LG O R ITH M FO R 1997;1(6):505–10.
12. Heller E. Extramukose Kardiaplastik beim chronischen kardio-
AC H A L A S I A M A N AG E M E NT
spasmus mit dilatation des oesophagus. Mitt Grenzgeb Med Chir.
1914;27:141–9.
POEM and LHM are equally effective and should considered in 13. Maingot R. Extramucous Oesophagocardiomyotomy in
every patient with achalasia. In our center, we do perform LHM Cardiospasm. Postgrad Med J. 1944;20(227):278–82.
for patients with type I and type II achalasia. These patients are 14. Kay EB. Surgical treatment of cardiospasm. Ann Surg.
1948;127(1):34–9.
often overweight and have a hiatal hernia so that the addition of a
15. Dor J, Humbert P, Dor V, Figarella J. L'interet de la technique de
fundoplication allows control of reflux in most patients. In patients Nissen modifiee dans la prevention de reflux apres cardio-
with type III achalasia, POEM should be considered as initial treat- myotomie extramuqueuse de Heller. Mem Acad Chir (Paris).
ment. In case of failure, we recommend PD as the second step 1962;88:877–83.
16. Shimi S, Nathanson LK, Cuschieri A. Laparoscopic cardiomyotomy
therapy. If pneumatic dilatation fails, it is reasonable to consider
for achalasia. J R Coll Surg Edinb. 1991;36(3):152–4.
POEM for those who underwent LHM initially and LHM for those 17. Pellegrini C, Wetter LA, Patti M, Leichter R, Mussan G, Mori
after POEM. Esophagectomy should be considered as a last resort T, et al. Thoracoscopic esophagomyotomy. Initial experience
for patients with persisting symptoms after failure of other treat- with a new approach for the treatment of achalasia. Ann Surg.
1992;216(3):291–6.
ment modalities.
18. Patti MG, Arcerito M, De Pinto M, Feo CV, Tong J, Gantert W, et al.
Comparison of thoracoscopic and laparoscopic Heller myotomy for
D I S C LO S U R E achalasia. J Gastrointest Surg. 1998;2(6):561–6.
Conflict of Interests: Authors declare no conflict of interests for this 19. Patti MG, Pellegrini CA, Horgan S, Arcerito M, Omelanczuk P,
Tamburini A, et al. Minimally invasive surgery for achalasia: an 8-year
article.
experience with 168 patients. Ann Surg. 1999;230(4):587–93.
20. Ancona E, Peracchia A, Zaninotto G, Rossi M, Bonavina L, Segalin
ORCID A. Heller laparoscopic cardiomyotomy with antireflux anterior fun-
Kamil Nurczyk https://orcid.org/0000-0002-1855-0401 doplication (Dor) in the treatment of esophageal achalasia. Surg
Endosc. 1993;7(5):459–61.
21. Ancona E, Anselmino M, Zaninotto G, Costantini M, Rossi M,
REFERENCES Bonavina L, et al. Esophageal achalasia: laparoscopic versus conven-
1. Stanaway JD, Roth G. The burden of Chagas disease: estimates and tional open Heller-Dor operation. Am J Surg. 1995;170(3):265–70.
challenges. Glob Heart. 2015;10(3):139–44. 22. Melvin WS, Needleman BJ, Krause KR, Wolf RK, Michler RE, Ellison
2. Boeckxstaens GE, Zaninotto G, Richter JE. Achalasia. Lancet. EC. Computer-assisted robotic Heller myotomy: initial case report.
2014;383(9911):83–93. J Laparoendosc Adv Surg Tech A. 2001;11(4):251–3.
3. Tebaibia A, Boudjella MA, Boutarene D, Benmediouni F, Brahimi H, 23. Richards WO, Torquati A, Holzman MD, Khaitan L, Byrne D, Lutfi
Oumnia N. Incidence, clinical features and para-clinical findings of R, et al. Heller myotomy versus Heller myotomy with Dor fundopli-
achalasia in Algeria: experience of 25 years. World J Gastroenterol. cation for achalasia: a prospective randomized double-blind clinical
2016;22(38):8615–23. trial. Ann Surg. 2004;240(3):405–15.
4. Ferguson MK, Little AG. Angina-like chest pain associated with 24. Torquati A, Richards WO, Holzman MD, Sharp KW. Laparoscopic
high-amplitude peristaltic contractions of the esophagus. Surgery. myotomy for achalasia: predictors of successful outcome after 200
1988;104(4):713–9. cases. Ann Surg. 2006;243(5):587–93.
5. Moonka R, Patti MG, Feo CV, Arcerito M, De Pinto M, Horgan S, 25. Rebecchi F, Giaccone C, Farinella E, Campaci R, Morino M.
et al. Clinical presentation and evaluation of malignant pseudoacha- Randomized controlled trial of laparoscopic Heller myotomy plus
lasia. J Gastrointest Surg. 1999;3(5):456–61. Dor fundoplication versus Nissen fundoplication for achalasia:
6. Kahrilas PJ, Bredenoord AJ, Fox M, Gyawali CP, Roman S, Smout long-term results. Ann Surg. 2008;248(6):1023–30.
AJPM, et al. The Chicago classification of esophageal motility disor- 26. Rawlings A, Soper NJ, Oelschlager B, Swanstrom L, Matthews
ders, v3.0. Neurogastroenterol Motil. 2015;27(2):160–74. BD, Pellegrini C, et al. Laparoscopic Dor versus Toupet fundopli-
7. Pandolfino JE, Kwiatek MA, Nealis T, Bulsiewicz W, Post J, Kahrilas cation following Heller myotomy for achalasia: results of a multi-
PJ. Achalasia: a new clinically relevant classification by high-resolu- center, prospective, randomized-controlled trial. Surg Endosc.
tion manometry. Gastroenterology. 2008;135(5):1526–33. 2012;26(1):18–26.
8. Salvador R, Costantini M, Zaninotto G, Morbin T, Rizzetto C, Zanatta 27. Costantini M, Salvador R, Capovilla G, Vallese L, Costantini A,
L, et al. The preoperative manometric pattern predicts the outcome Nicoletti L, et al. A thousand and one laparoscopic Heller myoto-
of surgical treatment for esophageal achalasia. J Gastrointest Surg. mies for esophageal achalasia: a 25-year experience at a single ter-
2010;14(11):1635–45. tiary center. J Gastrointest Surg. 2019;23(1):23–35.
9. Kessing BF, Bredenoord AJ, Smout AJ. Erroneous diagnosis of 28. Werner YB, Hakanson B, Martinek J, Repici A, von Rahden BHA,
gastroesophageal reflux disease in achalasia. Clin Gastroenterol Bredenoord AJ, et al. Endoscopic or surgical myotomy in patients
Hepatol. 2011;9(12):1020–4. with idiopathic achalasia. N Engl J Med. 2019;381(23):2219–29.
10. Andolfi C, Bonavina L, Kavitt RT, Konda VJ, Asti E, Patti MG. 29. Schlottmann F, Patti MG. Esophageal achalasia: current di-
Importance of esophageal manometry and ph monitoring in the agnosis and treatment. Expert Rev Gastroenterol Hepatol.
evaluation of patients with refractory gastroesophageal reflux 2018;12(7):711–21.
350 | NURCZYK and PATTI

30. Patti MG, Andolfi C, Bowers SP, Soper NJ. POEM vs Laparoscopic pneumatic dilatation in achalasia treatment: a randomized-con-
Heller myotomy and fundoplication: which is now the gold trolled trial. Aliment Pharmacol Ther. 2006;24(6):983–9.
standard for treatment of achalasia? J Gastrointest Surg. 50. Kostic S, Johnsson E, Kjellin A, Ruth M, Lönroth H, Andersson M,
2017;21(2):207–14. et al. Health economic evaluation of therapeutic strategies in pa-
31. Stefanidis D, Richardson W, Farrell TM, Kohn GP, Augenstein V, tients with idiopathic achalasia: results of a randomized trial com-
Fanelli RD, et al. SAGES guidelines for the surgical treatment of paring pneumatic dilatation with laparoscopic cardiomyotomy. Surg
esophageal achalasia. Surg Endosc. 2012;26(2):296–311. Endosc. 2007;21(7):1184–9.
32. Weber CE, Davis CS, Kramer HJ, Gibbs JT, Robles L, Fisichella 51. Bakhshipour A, Rabbani R, Shirani S, Soleimani HA, Mikaeli
PM. Medium and long-term outcomes after pneumatic dilation or J. Comparison of pneumatic dilation with pneumatic dilation
laparoscopic Heller myotomy for achalasia: a meta-analysis. Surg plus botulinum toxin for treatment of achalasia. Acta Med Iran.
Laparosc Endosc Percutan Tech. 2012;22(4):289–96. 2010;48(2):107–10.
33. Tomasko JM, Augustin T, Tran TT, Haluck RS, Rogers AM, Lyn-Sue 52. Novais PA, Lemme EM. 24-h pH monitoring patterns and clinical re-
JR. Quality of life comparing dor and toupet after heller myotomy sponse after achalasia treatment with pneumatic dilation or laparo-
for achalasia. JSLS. 2014;18(3):e2014.00191. scopic Heller myotomy. Aliment Pharmacol Ther. 2010;32(10):1257–65.
34. Wei MT, He YZ, Deng XB, Zhang YC, Yang TH, Jin CW, et al. Is Dor fun- 53. Boeckxstaens GE, Annese V, Varannes SBD, Chaussade S,
doplication optimum after laparoscopic Heller myotomy for achala- Costantini M, Cuttitta A, et al. Pneumatic dilation versus laparo-
sia? A meta-analysis. World J Gastroenterol. 2013;19(43):7804–12. scopic Heller's myotomy for idiopathic achalasia. N Engl J Med.
35. Andolfi C, Baffy G, Fisichella PM. Whose patient is it? The 2011;364(19):1807–16.
path to multidisciplinary management of achalasia. J Surg Res. 54. Shaligram A, Unnirevi J, Simorov A, Kothari VM, Oleynikov D.
2018;228:8–13. How does the robot affect outcomes? A retrospective review of
36. Falkenback D, Johansson J, Oberg S, Kjellin A, Wenner J, Zilling T, open, laparoscopic, and robotic Heller myotomy for achalasia. Surg
et al. Heller's esophagomyotomy with or without a 360 degrees Endosc. 2012;26(4):1047–50.
floppy Nissen fundoplication for achalasia. Long-term results from a 55. Borges AA, Lemme EM, Abrahao LJ Jr, Madureira D, Andrade MS,
prospective randomized study. Dis Esophagus. 2003;16(4):284–90. Soldan M, et al. Pneumatic dilation versus laparoscopic Heller my-
37. Campos GM, Vittinghoff E, Rabl C, Takata Mark, Gadenstätter M, otomy for the treatment of achalasia: variables related to a good
Lin F, Ciovica R. Endoscopic and surgical treatments for achalasia: a response. Dis Esophagus. 2014;27(1):18–23.
systematic review and meta-analysis. Ann Surg. 2009;249(1):45–57. 56. Hamdy E, El Nakeeb A, El Hanfy E, El Hemaly M, Salah T, Hamed
38. Topart P, Deschamps C, Taillefer R, Duranceau A. Long-term effect H, et al. Comparative study between laparoscopic Heller myot-
of total fundoplication on the myotomized esophagus. Ann Thorac omy versus pneumatic dilatation for treatment of early achalasia:
Surg. 1992;54(6):1046–52. a prospective randomized study. J Laparoendosc Adv Surg Tech A.
39. Rossetti G, Brusciano L, Amato G, Maffettone V, Napolitano V, 2015;25(6):460–4.
Russo G, et al. A total fundoplication is not an obstacle to esopha- 57. Persson J, Johnsson E, Kostic S, Lundell L, Smedh U. Treatment
geal emptying after Heller myotomy for achalasia: results of a long- of achalasia with laparoscopic myotomy or pneumatic dilatation:
term follow up. Ann Surg. 2005;241(4):614–21. long-term results of a prospective, randomized study. World J Surg.
40. Kumagai K, Kjellin A, Tsai JA, Thorell A, Granqvist S, Lundell L, et al. 2015;39(3):713–20.
Toupet versus Dor as a procedure to prevent reflux after cardiomy- 58. Moonen A, Annese V, Belmans A, Bredenoord AJ, Bruley des
otomy for achalasia: results of a randomised clinical trial. Int J Surg. Varannes S, Costantini M, et al. Long-term results of the European
2014;12(7):673–80. achalasia trial: a multicentre randomised controlled trial compar-
41. Patti MG, Fisichella PM. Laparoscopic Heller myotomy and Dor ing pneumatic dilation versus laparoscopic Heller myotomy. Gut.
fundoplication for esophageal achalasia. How I do it. J Gastrointest 2016;65(5):732–9.
Surg. 2008;12(4):764–6. 59. Chrystoja CC, Darling GE, Diamant NE, Kortan PP, Tomlinson GA,
42. Simić AP, Radovanović NS, Skrobić OM, Raznatović ZJ, Pesko PM. Deitel W, et al. Achalasia-specific quality of life after pneumatic
Significance of limited hiatal dissection in surgery for achalasia. J dilation or laparoscopic heller myotomy with partial fundoplica-
Gastrointest Surg. 2010;14(4):587–93. tion: a multicenter. Randomized clinical trial. Am J Gastroenterol.
43. Hunter JG, Trus TL, Branum GD, Waring JP. Laparoscopic 2016;111(11):1536–45.
Heller myotomy and fundoplication for achalasia. Ann Surg. 60. Torres-Villalobos G, Coss-Adame E, Furuzawa-Carballeda J,
1997;225(6):655–65. Romero-Hernández F, Blancas-Breña B, Torres-Landa S, et al. Dor
44. Tatum RP, Pellegrini CA. How I do it: laparoscopic Heller myot- vs Toupet fundoplication after laparoscopic Heller myotomy: long-
omy with Toupet fundoplication for achalasia. J Gastrointest Surg. term randomized controlled trial evaluated by high-resolution ma-
2009;13(6):1120–4. nometry. J Gastrointest Surg. 2018;22(1):13–22.
45. Schlottmann F, Allaix ME, Patti MG. Laparoscopic Heller myotomy 61. Schlottmann F, Luckett DJ, Fine J, Shaheen NJ, Patti MG.
for achalasia technical aspects. Am Surg. 2018;84(4):477–80. Laparoscopic Heller myotomy versus peroral endoscopic myotomy
46. Yamaguchi D, Tsuruoka N, Sakata Y, Shimoda R, Fujimoto K, (POEM) for achalasia: a systematic review and meta-analysis. Ann
Iwakiri R. Safety and efficacy of botulinum toxin injection ther- Surg. 2018;267(3):451–60.
apy for esophageal achalasia in Japan. J Clin Biochem Nutr. 62. Ponds FA, Fockens P, Lei A, Neuhaus H, Beyna T, Kandler J, et al. Effect
2015;57(3):239–43. of peroral endoscopic myotomy vs pneumatic dilation on symptom se-
47. Inoue H, Sato H, Ikeda H, Onimaru M, Sato C, Minami H, et al. Per- verity and treatment outcomes among treatment-naïve patients with
oral endoscopic myotomy: a series of 500 patients. J Am Coll Surg. achalasia: a randomized clinical trial. JAMA. 2019;322(2):134–44.
2015;221(2):256–64. 63. Costantini A, Familiari P, Costantini M, Salvador R, Valmasoni M,
48. Horgan S, Galvani C, Gorodner MV, Omelanczuck P, Elli F, Moser F, Capovilla G, et al. Poem versus laparoscopic Heller myotomy in
et al. Robotic-assisted Heller myotomy versus laparoscopic Heller the treatment of esophageal achalasia: a case-control study from
myotomy for the treatment of esophageal achalasia: multicenter two high volume centers using the propensity score. J Gastrointest
study. J Gastrointest Surg. 2005;9(8):1020–30. Surg. 2020;24(3):505–15.
49. Mikaeli J, Bishehsari F, Montazeri G, Mahdavinia M, Yaghoobi M, 64. Ehlers AP, Oelschlager BK, Pellegrini CA, Wright AS, Saunders
Darvish-moghadam S, et al. Injection of botulinum toxin before MD, et al. Achalasia treatment, outcomes, utilization, and costs: a
NURCZYK and PATTI | 351

population-based study from the United States. J Am Coll Surg. 74. Zaninotto G, Costantini M, Rizzetto C, Zanatta L, Guirroli E,
2017;225(3):380–6. Portale G, et al. Four hundred laparoscopic myotomies for
65. Swanstrom LL, Kurian A, Dunst CM, Sharata A, Bhayani N, Rieder E. esophageal achalasia: a single centre experience. Ann Surg.
Long-term outcomes of an endoscopic myotomy for achalasia: the 2008;248(6):986–93.
POEM procedure. Ann Surg. 2012;256(4):659–67. 75. Leeuwenburgh I, Scholten P, Alderliesten J, Tilanus HW, Looman
66. Familiari P, Gigante G, Marchese M, Boskoski I, Tringali A, Perri V, CW, Steijerberg EW, et al. Long-term esophageal cancer risk
et al. Peroral endoscopic myotomy for esophageal achalasia: out- in patients with primary achalasia: a prospective study. Am J
comes of the first 100 patients with short-term follow-up. Ann Surg. Gastroenterol. 2010;105(10):2144–9.
2016;263(1):82–7. 76. Zaninotto G, Rizzetto C, Zambon P, Guzzinati S, Finotti E, Costantini
67. Kumbhari V, Familiari P, Bjerregaard NC, Pioche M, Jones E, Ko W, M. Long-term outcome and risk of oesophageal cancer after sur-
et al. Gastroesophageal reflux after peroral endoscopic myotomy: a gery for achalasia. Br J Surg. 2008;95(12):1488–94.
multicenter case-control study. Endoscopy. 2017;49(7):634–42. 77. Leeuwenburgh I, Scholten P, Caljé TJ, Vaessen R j, Tilanus HW,
68. Kumbhari V, Tieu AH, Onimaru M, El Zein M, Teitelbaum E, Ujiki M, Hansen BE, et al. Barrett's esophagus and esophageal adenocar-
et al. Peroral endoscopic myotomy (POEM) vs laparoscopic Heller my- cinoma are common after treatment for achalasia. Dig Dis Sci.
otomy (LHM) for the treatment of Type III achalasia in 75 patients: a 2013;58(1):244–52.
multicenter comparative study. Endosc Int Open. 2015;3(3):E195–201. 78. Csendes A, Braghetto I, Burdiles P, Korn O, Csendes P, Henríquez A.
69. Werner YB, Costamagna G, Swanström LL, von Renteln D, Familiari Very late results of esophagomyotomy for patients with achalasia:
P, Sharata AM, et al. Clinical response to peroral endoscopic myot- clinical, endoscopic, histologic, manometric, and acid reflux stud-
omy in patients with idiopathic achalasia at a minimum follow-up of ies in 67 patients for a mean follow-up of 190 months. Ann Surg.
2 years. Gut. 2016;65(6):899–906. 2006;243(2):196–203.
70. Ichkhanian Y, Benias P, Khashab MA. Case of early Barrett cancer 79. Zaninotto G, Bennett C, Boeckxstaens G, Costantini M, Ferguson
following peroral endoscopic myotomy. Gut. 2019;68(12):2107–10. MK, Pandolfino JE, et al. The 2018 ISDE achalasia guidelines. Dis
71. Glatz SM, Richardson JD. Esophagectomy for end stage achalasia. J Esophagus. 2018;31(9):17–8.
Gastrointest Surg. 2007;11(9):1134–7.
72. Devaney EJ, Lannettoni MD, Orringer MB, Marshall B.
Esophagectomy for achalasia: patient selection and clinical experi-
How to cite this article: Nurczyk K, Patti MG. Surgical
ence. Ann Thorac Surg. 2001;72(3):854–8.
73. Aiolfi A, Asti E, Bonitta G, Bonavina L. Esophagectomy for end-
management of achalasia. Ann Gastroenterol Surg. 2020;4:
stage achalasia: systematic review and meta-analysis. World J Surg. 343–351. https://doi.org/10.1002/ags3.12344
2018;42(5):1469–76.