Esophagectomy for end-stage achalasia: functional and quality of life outcomes
Original Article

Esophagectomy for end-stage achalasia: functional and quality of life outcomes

Nikhil R. Kundu1, Tanya Irvine1, David I. Watson1, Jennifer C. Myers2,3, Peter G. Devitt2, Sarah K. Thompson1,4 ORCID logo

1College of Medicine & Public Health, Flinders University, Bedford Park, South Australia, Australia; 2Discipline of Surgery, The University of Adelaide, Adelaide, Australia; 3Oesophageal Function, The Queen Elizabeth Hospital, Woodville, South Australia, Australia; 4Adelaide Gastrointestinal Specialists, Eastwood, South Australia, Australia

Contributions: (I) Conception and design: SK Thompson, DI Watson; (II) Administrative support: T Irvine, JC Myers; (III) Provision of study materials or patients: T Irvine, JC Myers; (IV) Collection and assembly of data: NR Kundu, T Irvine, JC Myers; (V) Data analysis and interpretation: NR Kundu, T Irvine, JC Myers, SK Thompson; (VI) Manuscript writing: All authors; (VII) Final approval of manuscript: All authors.

Correspondence to: A/Prof Sarah K. Thompson, MD, PhD, FRACS. College of Medicine & Public Health, Flinders University, Rm 5E221.3, Flinders Medical Centre, Sturt Road, Bedford Park, South Australia 5042, Australia. Email: sarah.thompson@flinders.edu.au.

Background: Achalasia is a primary motility disorder of the esophagus, with approximately 5% of patients progressing to end-stage disease, defined as a dilated, tortuous esophagus which does not empty. These patients often have issues with ongoing chest pain, dysphagia, and regurgitation. Esophagectomy for the treatment of end-stage achalasia is controversial and recommended as a last resort by recent guidelines. This study evaluated long-term outcomes for patients following esophagectomy for end-stage achalasia.

Methods: Questionnaires were mailed to patients who underwent esophagectomy for end-stage achalasia between 2001 to 2022 in South Australian hospitals. Outcome measures included symptoms at long-term follow-up, overall satisfaction measured on a 0–10 analogue score (0 = poor, 10 = highly satisfied), and level of function.

Results: Of the 11 patients who underwent esophagectomy, eight completed a questionnaire at a mean of 7.2 years post-esophagectomy. The study included 6 men and 2 women with a median age of 52.5 years (interquartile range, 40–69 years). Operative mortality was 0%, with a complication rate of 38%. All patients indicated that they considered the decision to operate was correct, with a mean satisfaction score of 7.6 out of 10. Of the six patients who were gainfully employed prior to esophagectomy, five returned to their normal vocation.

Conclusions: Esophagectomy can be considered as a treatment option for end-stage achalasia. All patients were positive about their decision to undergo major surgery, and all but one were satisfied with their long-term outcome. Despite recognition as an intervention of ‘last resort’, the decision to proceed to esophagectomy for end-stage achalasia should not be discounted.

Keywords: Esophagectomy; achalasia; quality of life; end-stage achalasia

Received: 12 April 2025; Accepted: 22 September 2025; Published online: 29 September 2025.

doi: 10.21037/aoe-25-15

Highlight box

Key findings

• Esophagectomy for end-stage achalasia is feasible and safe. Patients who underwent esophagectomy reported a mean satisfaction score of 7.6 out of 10.

What is known and what is new?

• Achalasia is a primary motility disorder of the esophagus, with approximately 5% of patients progressing to end-stage disease. These patients often have issues with ongoing chest pain, dysphagia, and regurgitation. Esophagectomy for the treatment of end-stage achalasia is controversial and long-term outcomes are unclear.

• All patients who underwent esophagectomy for end-stage achalasia considered their decision to proceed to be the correct one. Operative mortality was zero with a morbidity rate of 38%. The majority of patients returned to their normal vocation following surgical intervention.

What is the implication, and what should change now?

• Esophagectomy for end-stage achalasia is a reasonable option for many patients and leads to a high level of satisfaction. It should no longer be considered an option of last resort.

Introduction

Achalasia is a rare primary motility disorder of the esophagus that has an incidence of 2.3 to 2.9 per 100,000 people (1-3). Neurodegenerative, autoimmune and viral factors have been identified in the etiology of achalasia (1,4). The loss of ganglion cells of the myenteric plexus within the esophageal smooth muscle causes aperistalsis and impaired relaxation of the lower esophageal sphincter (1,5). Patients commonly present with dysphagia, aspiration, regurgitation, and chest pain (1,6,7).

Treatment of achalasia is palliative and aims to reduce the pressure gradient across the lower esophageal sphincter and improve esophageal emptying (8). Whilst pneumatic dilatation, laparoscopic cardiomyotomy, and per-oral endoscopic myotomy (POEM) demonstrate good long-term symptom control (9), approximately 5% of patients will have a poor outcome due to end-stage disease. In these patients, there is progressive dilatation and tortuosity of the esophagus, leading to sump formation and poor esophageal emptying (Figure 1) (10,11). For this subset of individuals, when other treatment modalities have been exhausted, the International Society for Diseases of the Esophagus (ISDE) guidelines recommend progression to esophagectomy as a last resort (12). The aim of esophagectomy is to improve nutritional deficiencies, restore alimentary transit and decrease the risk of long-term complications such as aspiration and carcinoma (13).

Figure 1 Barium swallow, anterior-posterior projection, of a very dilated, patulous esophagus in a patient with end-stage achalasia, prior to esophagectomy. The arrow points to the esophageal sump, and the star depicts the fundus of the stomach. Reprinted from Kundu et al. (10), with permission from AME Publishing Company.

However, esophagectomy for end-stage achalasia is controversial. The low incidence of achalasia has resulted in an inability to define an ideal management algorithm. Much of the literature contains retrospective studies, with small sample sizes (14-24). To date, there are no studies that have examined quality of life nor functional outcomes following esophagectomy for patients with end-stage achalasia (9). The aim of this study is to present this data. We present this article in accordance with the SURGE reporting checklist (available at https://aoe.amegroups.com/article/view/10.21037/aoe-25-15/rc).

Methods

Study design

This is a descriptive cohort study of end-stage achalasia patients who underwent esophagectomy in South Australia between January, 2001 to July, 2022. Patients were required to be over 18 years old, English-speaking and be available to complete follow-up questionnaires. Esophagectomies performed prior to 2000 were excluded as laparoscopic cardiomyotomy was only introduced in the mid-1990s and no patients proceeded to esophagectomy before 2005.

Participants

Participants received an invitation via mail with an attached questionnaire. Follow-up was conducted 1 to 3 weeks later to confirm participation and ensure completion of the questionnaire. Medical records and a surgical audit were also reviewed to determine baseline data such as demographic information, previous achalasia treatments, and perioperative details.

Study questionnaires

A 17-item questionnaire was used to assess long-term symptom follow-up, overall satisfaction, and level of function in daily life. The survey was structured using Urbach’s disease specific quality of life assessment for achalasia, and was modified for this cohort (25). A questionnaire specifically designed for post-esophagectomy patients was not used in our study as our patients’ underlying condition was achalasia, not cancer. Outcome measures included long-term symptom follow-up, overall satisfaction measured on a 0–10 analogue score (0 = poor, 10 = highly satisfied), and level of function (Appendix 1).

Ethical consideration

The study was conducted in accordance with the Declaration of Helsinki and its subsequent amendments. The study was approved by the Southern Adelaide Clinical Human Research Ethics Committee (No. LNR/21/SAC/188). Participation in this survey was voluntary and anonymous, and informed consent was obtained from all participants.

Results

Patient participation and demographics

Within the study duration, 350 laparoscopic cardiomyotomies with Dor fundoplication were performed for achalasia. During the same time period, 11 (3.1%) patients with end-stage achalasia underwent esophagectomy and received questionnaires. Of this cohort, 8 (72.7%) patients (6 men and 2 women) completed answers to all questions and were included in the analysis. The median age at time of surgery was 52.5 years [interquartile range (IQR), 40–69 years]. Questionnaires were completed at a mean of 7.2 years (range, 4.9–16.9 years) post-esophagectomy.

Perioperative details

All eight patients had previously undergone two or more interventions for achalasia (Table 1). One underwent 3 redo myotomies before progressing to esophageal resection. Two patients had undergone an experimental procedure, a laparoscopic stapled cardioplasty (26). Some patients also underwent pneumatic dilatation using 30 or 35 mm diameter balloons on at least 3 occasions before considering esophagectomy (Table 1). Esophagectomies were performed across two centers. Seven patients underwent a transthoracic approach (Ivor-Lewis esophagectomy), whilst one patient underwent an open transhiatal esophagectomy (due to prior thoracic surgery) (Table 2).

Table 1

Demographic data for 8 patients with end-stage achalasia who underwent esophagectomy

Patient Age at esophagectomy (years) Sex Type of achalasia # of interventions Type of intervention
1 45 M I 2 Myotomy†; laparoscopic cardioplasty (26)
2 69 F I 3 Myotomy; redo myotomy ×2
3 55 M Unknown 3 Myotomy; redo myotomy; cardioplasty
4 55 F II 4 Myotomy; dilatation ×3‡
5 49 M III 6 Myotomy; redo thoracoscopic myotomy; dilatation ×3; cardioplasty
6 63 M II 4 Open myotomy; dilatation ×3
7 50 M II 2 Myotomy; redo myotomy
8 40 M I 2 Myotomy; takedown fundoplication

†, myotomy = laparoscopic cardiomyotomy; ‡, dilatation =30 or 35 mm balloon. F, female; M, male.

Table 2

Operative data for 8 patients with end-stage achalasia who underwent esophagectomy

Patient Time from achalasia diagnosis to esophagectomy (years) Type of esophagectomy Length of stay (days) Complications
1 7.6 Ivor-Lewis 16 Nil
2 21.0 Ivor-Lewis 10 Nil
3 35.1 Ivor-Lewis 12 Nil
4 4.8 Ivor-Lewis 7 Nil
5 8.3 Ivor-Lewis 6 Nil
6 31.5 Transhiatal (open) 19 Respiratory failure (ICU re-admission), poor conduit emptying
7 6.5 Ivor-Lewis 13 Pleural effusion
8 7.9 Ivor-Lewis 14 Pneumonia

ICU, intensive care unit.

Patients proceeded to esophagectomy after a median of 8.1 years (IQR, 7.3–23.6 years). Esophagectomy was not associated with mortality (Table 2). Complications occurred in 3 patients (37.5%). These included one pleural effusion, a pneumonia and one re-admission to the intensive care unit for respiratory failure and pain crisis. There were no anastomotic leaks, either clinically or demonstrated on postoperative contrast swallows. The median postoperative hospital length of stay was 12.5 days (IQR, 6–19 days).

Questionnaire responses

All eight participants stated that they considered the decision to operate to be correct. Seven were satisfied with the outcome of surgery, with a mean satisfaction score of 7.6 out of 10. Of the 6 patients who were working prior to surgery, five returned to their normal employment and one made the decision to retire.

Reflux was reported by all patients with a variable frequency of one episode per month to ‘4 times/day’. Post-prandial nausea was recorded in 6 patients (75%) at a frequency of one episode per month to ‘all the time’. Half of the cohort reported vomiting at least once a month. Loss of appetite was recorded up to 4 times a month. Five patients (62.5%) denied dysphagia to liquids whilst the remaining three reported dysphagia to liquids three times daily to ‘all the time’. Dysphagia to solids occurred in half of the cohort, with two patients stating that it occurred ‘all the time’. Five participants (62.5%) recorded bloating after eating at least once a day. Post-prandial pain was reported at a variable rate of one episode per month to ‘all the time’. The least cited long-term symptoms included dizziness and sweating after eating.

Discussion

Esophagectomy for achalasia has been noted in the literature for more than 40 years. In 1991, Pinotti et al. published their results after performing transhiatal esophagectomy on 108 achalasia patients with a mortality rate of 3.4% (19). In 1989, Orringer et al. reported favorable results in 26 patients who underwent esophagectomy for the treatment of end-stage disease (17). Similar outcomes were also published by other authors (14-24).

A key finding from our study is a high level of satisfaction reported by patients who underwent esophagectomy to treat end-stage achalasia. The majority of patients returned to their normal vocation, despite ongoing reflux symptoms, postprandial nausea and bloating. This is consistent with data published by Tassi et al. who compared quality of life indicators in end-stage achalasia patients who underwent a pull-down Heller-Dor myotomy versus esophagectomy (27).

Treatment of achalasia is challenging as current options are unable to restore peristalsis of the esophagus. In the absence of a curative approach, the aim is to palliate symptoms. Patients will often undergo a variety of treatments, including pneumatic dilatations, laparoscopic cardiomyotomy, POEM, or temporary paralysis of the lower esophageal sphincter with Botulinum toxin. Re-intervention is common with up to a 25% failure rate and a re-intervention rate of 20% (28). Failure of initial treatment can occur due to several reasons including the presence of a hiatus hernia, severe reflux and/or esophagitis, an incomplete myotomy, fibrosis or malignancy (29). The 2018 ISDE guidelines summarize recommendations for re-intervention. Pneumatic dilatation is recommended after a failed Heller’s myotomy or POEM with success rates of 60% to 82%. Refractory symptoms after a POEM can be treated with a Heller’s myotomy, or vice versa (12). In end-stage patients where less invasive methods have failed, guidelines suggest progression to esophagectomy.

In the current study, each participant had undergone at least two or more interventions prior to esophagectomy. In these cases, there are a few technical points to be wary of when operating on end-stage achalasia patients. Prior intervention may result in transmural inflammation causing an adherent esophagus to surrounding structures. The gastric conduit may be shortened due to preceding fundoplication and subsequent abdominal adhesions. Finally, the hypertrophied esophageal mucosa with associated neovascularization may increase the risk of bleeding and subsequent re-operation (21,30).

The literature supporting esophagectomy for end-stage achalasia shows a mortality rate of 0–2.69% (9,30,31) and a postoperative complication rate of 19–59% (9,32). In our study, the mortality and post operative complication rates were 0% and 37.5%, respectively. The incidence of post-operative pneumonia was 12.5%, a rate consistent with the literature (9,32). Anastomotic leakage was not seen in our study although the case numbers were small. In a systematic review and meta-analysis of 8 studies by Aiolfi et al., an incidence of leakage of 7% was identified, a comparable rate to the leak rate following esophagectomy for cancer (9). Finally, there was no reported incidence of an esophageal cancer in our study cohort. Chronic stasis in end-stage achalasia may lead to squamous hyperplasia with papillomatosis and basal cell hyperplasia, with a mean of 20 to 25 years since achalasia diagnosis (33). As a result, several authors have demonstrated a 3–10% increase in risk of developing squamous cell cancer (30,34).

Our study had several limitations. Even though we managed approximately 20 new patients each year with achalasia across the study period, the study cohort was small, reflecting the low rate of progression to esophagectomy and the relative rarity of achalasia. Also, of the potentially eligible patients, 3 (27.3%) did not respond to the initial mailout or subsequent follow-up. One of these patients was lost to follow-up with contact details unknown. The variability of long-term symptom follow-up meant an inability to standardize the frequency of symptoms. Finally, our study did not have baseline quality of life nor functional outcome data.

Conclusions

Esophagectomy can be considered as a treatment option for patients with end-stage achalasia. Esophagectomy in our patient cohort had a zero risk of perioperative mortality and an acceptable rate of post-operative complication. All patients were positive about their decision to undergo major surgery at long-term follow-up. Despite recognition as an intervention of ‘last resort’, the decision to proceed to esophagectomy for end-stage achalasia should not be discounted.

Acknowledgments

Our abstract has been accepted for presentation at the 21st ISDE World Congress, Diseases of the Esophagus, Volume 38, Issue Supplement 1, August 2025 (doaf061.138). DOI: https://doi.org/10.1093/dote/doaf061.138.

Footnote

Reporting Checklist: The authors have completed the SURGE reporting checklist. Available at https://aoe.amegroups.com/article/view/10.21037/aoe-25-15/rc

Data Sharing Statement: Available at https://aoe.amegroups.com/article/view/10.21037/aoe-25-15/dss

Peer Review File: Available at https://aoe.amegroups.com/article/view/10.21037/aoe-25-15/prf

Funding: None.

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://aoe.amegroups.com/article/view/10.21037/aoe-25-15/coif). S.K.T. serves as an unpaid editorial board member of Annals of Esophagus from September 2025 to August 2027. The other authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. The study was conducted in accordance with the Declaration of Helsinki and its subsequent amendments. The study was approved by the Southern Adelaide Clinical Human Research Ethics Committee (No. LNR/21/SAC/188). Participation in this survey was voluntary and anonymous, and informed consent was obtained from all participants.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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doi: 10.21037/aoe-25-15
Cite this article as: Kundu NR, Irvine T, Watson DI, Myers JC, Devitt PG, Thompson SK. Esophagectomy for end-stage achalasia: functional and quality of life outcomes. Ann Esophagus 2025;8:19.

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