Background & Aims: In certain cases of achalasia, particularly those in ear
ly stages with minimal endoscopic or radiographic abnormalities, the diagno
sis may rely on manometry, which is the most sensitive test for the disease
. The aim of this study was to critically evaluate the manometric criteria
in a population of patient with idiopathic achalasia. Methods: Clinical his
tories and manometric recordings of 58 patients with idiopathic achalasia a
nd 43 control subjects were analyzed with regard to esophageal body contrac
tion amplitude, peristaltic effectiveness in terms of both completeness and
propagation velocity, lower esophageal sphincter (LES) resting pressure, L
ES relaxation pressure, and intraesophageal-intragastric pressure gradient,
Variants of achalasia were defined by finding manometric features that sig
nificantly differed from the remainder of achalasia patients, such that the
diagnosis might be questioned. Results: Four manometrically distinct varia
nts were identified. These variants were characterized by (1) the presence
of high amplitude esophageal body contractions, (2) a short segment of esop
hageal body aperistalsis, (3) retained complete deglutitive LES relaxation,
and (4) intact transient LES relaxation, In each instance, the most extrem
e variant is discussed and compared with the remainder of the achalasia pop
ulation and with controls. Conclusions: The significance in defining these
variants of achalasia lies in the recognition that these sometimes confusin
g manometric findings are consistent with achalasia when combined with addi
tional clinical data supportive of the diagnosis. Furthermore, such variant
s provide important clues into the pathophysiology of this rare disorder.