Esophagectomy for stage IV achalasia

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.