Peds: Atypical Variants of Classic Achalasia Are Common and Currently Under-Recognized

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http://highwire.stanford.edu/cgi/medline/pmid;21435920

Medline Abstract

Atypical Variants of Classic Achalasia Are Common and Currently

Under-Recognized: A Study of Prevalence and Clinical Features.

KM Galey, CL Wilshire, S Niebisch, CE , DP , VR Litle, TJ ,

and JH s

J Am Coll Surg,

March 22, 2011;

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 Division of Thoracic and Foregut Surgery, Department of

Surgery, University of Rochester Medical Center, Rochester, NY.

BACKGROUND: Advances in esophageal manometry have facilitated

identification of variants of achalasia, suggesting they are more common

than previously thought. This study assesses the frequency and clinical

characteristics of patients with motility abnormalities similar to, but

not meeting criteria for, classic achalasia. STUDY DESIGN: Records of

patients undergoing high-resolution esophageal manometry between January

2008 and January 2010 were screened for diagnosis of achalasia,

impaired lower esophageal sphincter (LES) relaxation, or severe

peristaltic dysfunction of the esophageal body. Forty-four patients with

classic achalasia and 31 with variant characteristics were identified.

Clinical and manometric characteristics were recorded and compared.

RESULTS: Variant achalasia was almost as common as the classic type (31

versus 44 patients). Eighty-two percent (36 of 44) of those with classic

and 48% (15 of 31) of those with variant characteristics had dysphagia.

Classic achalasia patients' mean age was 62 years (SD 19 years) versus

53 years (SD 14 years) in the variant group. The classic achalasia group

had 26 male patients and 18 female patients and the variant group had 9

male patients and 22 female patients. Two thirds (21 of 31) of the

variant group had impaired LES relaxation. Three variant patterns

emerged: impaired LES relaxation with normal/hypertensive peristalsis (n

= 10), impaired/borderline LES relaxation with mixed

peristalsis/simultaneous contractions (n = 14), and impaired/normal LES

and aperistalsis with occasional short segment peristalsis (n = 7). Mean

intrabolus pressure was 16.3 mmHg in variant patients with normal LES

relaxation and 23.1 mmHg in those with impaired relaxation. CONCLUSIONS:

Variants of achalasia are more common than previously recognized. LES

dysfunction defined by high relaxation pressure occurs in two-thirds of

variant achalasia patients and might be a hallmark that could direct

therapy. The notion that esophageal body dysfunction/aperistalsis in

achalasia is all or none should be reconsidered.

PMID: 21435920

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