From the Department of Medicine, Division of Gastroenterology & Hepatology, Northwestern University Medical School, Chicago, Ill.
Objective.— To review the pathophysiology and management of achalasia.
Data Sources.— Peer-reviewed publications located via MEDLINE using the search term esophageal achalasia (subheadings: complications, drug therapy, epidemiology, etiology, physiopathology, surgery, and therapy)
published in English from 1966 to December 1997.
Study Selection.— Of 2632 citations identified, 4.5% were selected for inclusion by authors'
blinded review of the abstracts. New developments in the understanding of
achalasia or reports of therapeutic efficacy in either controlled trials or
uncontrolled consecutive series involving 10 patients or more observed for
a year or longer were reviewed in detail.
Data Extraction.— All 6 controlled therapeutic trials were included, and therapeutic efficacy
in uncontrolled series was assessed by the authors extracting the patients
with a good-to-excellent response from each study and calculating a pooled
estimate of response rate with individual studies weighted proportionally
to sample size.
Data Synthesis.— Achalasia results from irreversible destruction of esophageal myenteric
plexus neurons causing aperistalsis and failed lower sphincter relaxation.
The only therapies that adequately compensate for this dysfunction for a sustained
time are pneumatic dilation and Heller myotomy. The single controlled trial
comparing these treatments found surgery superior to dilation (95% vs 51%
nearly complete symptom resolution, P<.01). In
uncontrolled trials pneumatic dilation (weighted mean [SD]) is 72% (26%) effective
vs 84% (20%) for Heller myotomy. The limitation of dilation is a 3% risk of
perforation; thoracotomy morbidity has been the major limitation of myotomy.
Surgical morbidity has been sharply reduced by laparoscopic techniques.
Conclusions.— Both pneumatic dilation and surgical myotomy are effective therapies
for achalasia; laparoscopic Heller myotomy is emerging as the optimal surgical
Spiess AE, Kahrilas PJ. Treating Achalasia: From Whalebone to Laparoscope. JAMA. 1998;280(7):638–642. doi:10.1001/jama.280.7.638
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