European Surgery

, Volume 50, Issue 2, pp 58–64 | Cite as

Esophagectomy for stage IV achalasia

Case series and literature review
  • Alberto Aiolfi
  • Emanuele Asti
  • Carlo Galdino Riva
  • Luigi Bonavina
original article



Achalasia is a rare esophageal motility disorder characterized by defective lower esophageal sphincter relaxation and esophageal body aperistalsis. Endoscopic balloon dilation and laparoscopic myotomy are effective initial treatments in patients with early-stage disease. Indications for upfront esophagectomy in stage IV disease are still debated.


Retrospective, observational, single center study including consecutive patients who underwent esophagectomy for end-stage achalasia. A literature search was performed additionally.


Out of 385 patients referred to our department for achalasia, 6 (1.6%) had previous unsuccessful surgical myotomy and underwent esophagectomy for stage IV disease. There were 4 females and the median age was 54.4 years (range 33–69). Most commonly reported symptoms were regurgitation (100%), dysphagia (83%), chest pain (67%), and recurrent episodes of pneumonia and/or chronic cough (50%). Two patients were incidentally diagnosed with squamous-cell carcinoma in the surgical specimen. A hybrid Ivor-Lewis esophagectomy was the most commonly performed operation. The median operative time was 320 min (range 290–365). The median in-hospital stay was 13 days. No anastomotic leaks occurred. The overall postoperative morbidity rate was 33% and there was no mortality. A total of 1422 esophagectomies for end-stage achalasia were reported in the literature. The stomach was the most commonly used esophageal substitute (94%). The anastomotic leak rate varied from 0 to 17%, and the postoperative mortality from 0 to 5.4%.


Selected stage IV achalasia patients with recurrent and disabling symptoms after previously failed Heller myotomy and/or endoscopic treatments should be offered minimally invasive esophagectomy.


Achalasia End-stage achalasia Megaesophagus Sigmoid esophagus Esophageal resection Esophageal carcinoma 


Compliance with ethical guidelines

Conflict of interest

A. Aiolfi, E. Asti, C.G. Riva, and L. Bonavina declare that they have no competing interests.

Ethical standards

All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2008. Informed consent was obtained from all patients for being included in the study.


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Copyright information

© Springer-Verlag GmbH Austria, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Biomedical Sciences for Health, Division of General Surgery, IRCCS Policlinico San DonatoUniversity of MilanMilanItaly

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