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The best known and best understood primary motility disorder of the esophagus is achalasia,
with an incidence of six per 100,000 population per year. Although complete absence of
peristalsis in the esophageal body has been proposed as the major abnormality, present
evidence indicates achalasia is a primary disorder of the LES. This is based on 24-hour
outpatient esophageal motility monitoring, which shows that, even in advanced disease, up to
5% of contractions can be peristaltic. Simultaneous esophageal waves develop as a result of
the increased resistance to esophageal emptying caused by the nonrelaxing LES. This
conclusion is supported by experimental studies in which a band placed loosely around the
GEJ in experimental models did not change sphincter pressures, but resulted in impaired
relaxation of the LES and outflow resistance. This led to a markedly increased frequency of
simultaneous waveforms and a decrease in contraction amplitude. The changes were
associated with radiographic dilation of the esophagus and were reversible after removal of
the band. Observations in patients with pseudoachalasia due to tumor infiltration, a tight
stricture in the distal esophagus, or an antireflux procedure that is too tight also provide
evidence that dysfunction of the esophageal body can be caused by the increased outflow
obstruction of a nonrelaxing LES. The observation that esophageal peristalsis can return in
patients with classic achalasia following dilation or myotomy provides further support that
achalasia is a primary disease of the LES.

The pathogenesis of achalasia is presumed to be a neurogenic degeneration, which is either

idiopathic or due to infection. In experimental animals, the disease has been reproduced by
destruction of the nucleus ambiguus and the dorsal motor nucleus of the vagus nerve. In
patients with the disease, degenerative changes have been shown in the vagus nerve and in
the ganglia in the myenteric plexus of the esophagus itself. This degeneration results in
hypertension of the LES, a failure of the sphincter to relax on swallowing, elevation of
intraluminal esophageal pressure, esophageal dilatation, and a subsequent loss of progressive
peristalsis in the body of the esophagus. The esophageal dilatation results from the
combination of a nonrelaxing sphincter, which causes a functional retention of ingested
material in the esophagus, and elevation of intraluminal pressure from repetitive pharyngeal
air swallowing (Fig. 25-54). With time, the functional disorder results in anatomic alterations
seen on radiographic studies, such as a dilated esophagus with a tapering, "bird's beak"–like
narrowing of the distal end (Fig. 25-55). There is usually an air-fluid level in the esophagus
from the retained food and saliva, the height of which reflects the degree of resistance
imposed by the nonrelaxing sphincter. As the disease progresses, the esophagus becomes
massively dilated and tortuous.

Fig. 25-54.

Pressurization of esophagus: Ambulatory motility tracing of a patient with achalasia. A.

Before esophageal myotomy. B. After esophageal myotomy. The tracings have been
compressed to exaggerate the motility spikes and baseline elevations. Note the rise in
esophageal baseline pressure during a meal represented by the rise off the baseline to the left
of panel A. No such rise occurs postmyotomy (panel B).

Fig. 25-55.

Barium esophagogram showing a markedly dilated esophagus and characteristic "bird's

beak" in achalasia.

(Reproduced with permission from Waters PF, DeMeester TR: Foregut motor disorders and
their surgical management. Med Clin North Am 65:1244, 1981. Copyright Elsevier.)

A subgroup of patients with otherwise typical features of classic achalasia has simultaneous
contractions of their esophageal body that can be of high amplitude. This manometric pattern
has been termed vigorous achalasia, and chest pain episodes are a common finding in these
patients. Differentiation of vigorous achalasia from DES can be difficult. In both diseases,
videoradiographic examination may show a corkscrew deformity of the esophagus and
diverticulum formation.

Diffuse and Segmental Esophageal Spasm

DES is characterized by substernal chest pain and/or dysphagia. DES differs from classic
achalasia in that it is primarily a disease of the esophageal body, produces a lesser degree of
dysphagia, causes more chest pain, and has less effect on the patient's general condition.
Nonetheless, it is impossible to differentiate achalasia from DES on the basis of symptoms
alone. Esophagogram and esophageal manometry are required to distinguish these two
entities. True symptomatic DES is a rare condition, occurring about five times less frequently
than achalasia.

The causation and neuromuscular pathophysiology of DES are unclear. The basic motor
abnormality is rapid wave progression down the esophagus secondary to an abnormality in
the latency gradient. Hypertrophy of the muscular layer of the esophageal wall and
degeneration of the esophageal branches of the vagus nerve have been observed in this
disease, although these are not constant findings. Manometric abnormalities in DES may be
present over the total length of the esophageal body, but usually are confined to the distal two
thirds. In segmental esophageal spasm, the manometric abnormalities are confined to a short
segment of the esophagus.

The classic manometric findings in these patients are characterized by the frequent
occurrence of simultaneous waveforms and multipeaked esophageal contractions, which may
be of abnormally high amplitude or long duration. Key to the diagnosis of DES is that there
remain some peristaltic waveforms in excess of those seen in achalasia. A criterion of 30% or
more peristaltic waveforms out of 10 wet swallows has been used to differentiate DES from
vigorous achalasia. However, this figure is arbitrary and often debated.

The LES in patients with DES usually shows a normal resting pressure and relaxation on
swallowing. A hypertensive sphincter with poor relaxation may also be present. In patients
with advanced disease, the radiographic appearance of tertiary contractions appears helical,
and has been termed corkscrew esophagus or pseudodiverticulosis (Fig. 25-56). Patients with
segmental or diffuse esophageal spasm can compartmentalize the esophagus and develop an
epiphrenic or midesophageal diverticulum between two areas of high pressure occurring
simultaneously (Fig. 25-57).

Fig. 25-56.

Barium esophagogram of patient with diffuse spasm showing the corkscrew deformity.

Fig. 25-57.

Barium esophagogram showing a high epiphrenic diverticulum in a patient with diffuse

esophageal spasm.

[Reproduced with permission from DeMeester TR, Stein HJ: Surgery for esophageal motor
disorders, in Castell DO (ed): The Esophagus. Boston: Little, Brown, 1992, p 415.]