Essay

8 April 2023

Georgia

Essay

Introduction

Achalasia is an important but relatively uncommon disorder, with an estimated incidence of 1 in 100 000
and prevalence of 1 in 10 000.1 Long-term effective treatment aims to lower lower esophageal sphincter
(LES) pressure, principally via pneumatic dilation or Heller myotomy. Recent studies have demonstrated
that these treatmentsarebotheffectiveanddurable.2–5 Nonetheless, irrespective of treatment,
esophageal cancer remains a long-term potential complication of achalasia.

Discussion

One of the most feared long-term complications of achalasia remains esophageal carcinoma. However,
while the link between these two conditions has biological and observational validity, the role of
surveillance in achalasia remains unclear. Our findings reinforce this uncertainty, with a nearly even
division among experts regarding the need for cancer screening in achalasia. This debate would seem to
center around the perceived risk of cancer in these patients, with significant differences of opinion seen
in this study. Nearly half of experts estimated the lifetime risk of esophageal cancer in achalasia to be
between 0.1% and 0.5%. However, among the remaining experts, there was an even distribution among
those who believed that the risk was no different than the general population and those who believed
the risk was 1–5% over their lifetime. Interestingly though, these differences in perceived risk did not
correlate with the practice of screening and surveillance in this study. This finding is somewhat
surprising, given screening practices for other premalignant conditions, most notably Barrett’s
esophagus. The estimated risk of cancer in non-dysplastic and low-grade Barrett’s esophagus has
recently been estimated at 0.1% and 0.3% per patient year, respectively.30,31 This risk is similar to that
reported in achalasia, with several studies reporting a risk of 0.3–0.4% per patient year.14,20,29
However, although many of our experts endorsed a similar risk of cancer in achalasia and Barrett’s, the
recommendation for screening was highly variable and inconsistent. The reasons for these differences in
perception are unclear and likely based on the paucity of data dictating clear recommendations and
factors that increase cancer risk in achalasia. Identification of these factors may help frame a consensus
regarding screening practices in achalasia.

Conclusion

Survey responses were received from 17 of 28 (61%) contacted experts. Participants came from
throughout the world, with seven (41%) from the United States, eight (47%) from Europe and Australia,
and two (12%) from Asia. All participants reported relatively high achalasia patient volumes, with 15
(88%) seeing more than 50 patients annually with achalasia in their practice and the remaining two with
20–50 achalasia patients (Table 1). Screening for esophageal cancer in achalasia was highly variable,
with nine (53%) experts advocating routine screening for cancer while eight experts (47%) did not. The
location of the experts did not influence thedecisiontoperformsurveillancewith3/7(43%)US experts, 4/8
(50%) European or Australian experts, andbothAsianexpertsemployingroutineesophageal cancer
screening in achalasia (P = 0.64). Practice was also not dependent on patient volume.

Reference

1 Birgisson S, Richter J E. Achalasia in Iceland, 1952–2002: an epidemiologic study. Dig Dis Sci 2007; 52:
1855–60. 2 Vela M F, Richter J E, Khandwala F et al. The long-term efficacy of pneumatic dilation and
Heller myotomy for the treatment of achalasia. Clin Gastroenterol Hepatol 2006; 4: 580–7. 3 Katzka D
A, Castell D O. Review article: an analysis of the efficacy, perforation rates and methods used in
pneumatic dilation for achalasia. Aliment Pharmacol Ther 2011; 34: 832–9. 4 Zaninotto G, Costantini
M, Rizzetto C et al. Four hundred laparoscopic myotomies for esophageal achalasia: a single centre
experience. Ann Surg 2008; 248: 986–93.