THE GASTROINTESTINAL SYSTEM

Achalasia

A 42-year-old woman is admitted to the hospital because of difficulty in swallowing solid foods; liquids are
less difficult to swallow. Chest pain follows eating, and the patient frequently regurgitates swallowed food
after a meal. When the recumbent patient is observed by fluoroscopy after a barium swallow, her lower
esophagus is seen to be somewhat dilated, but her upper esophagus is of normal caliber. Subsequent
swallows initiated by the patient do not cause the barium to be cleared from the esophagus as rapidly as
in a normal individual. Rather, it appears that the lower esophageal sphincter (LES) relaxes very
transiently, allowing small amounts of barium to empty into the stomach. Administration of amyl nitrate
accelerates clearance of barium from the esophagus. The transient openings of the LES do not bear any
simple temporal relationship to the swallows initiated by the patient. Esophageal manometric studies of
the patient are then undertaken. The coordinated peristaltic wave that is observed in the esophagus of a
normal individual after a swallow does not occur in this patient. Rather, each swallow elicits a low pressure
increase in the esophagus; the pressure increase occurs almost simultaneously at various places along the
length of the esophagus. The resting pressure in the LES is about 60 mm Hg, and the LES pressure
decreases to about 45 mm Hg after a swallow. The patient is treated by dilating the LES by brief, forceful
inflations of a pneumatic device. After this procedure, the patient's ability to swallow solid food is
dramatically improved. Fifteen months after the dilation, the patient returns with the complaint that
swallowing has again become difficult.

l. What appears abnormal on fluoroscopic examination after a barium swallow?

2. What esophageal malfunctions might account for these observations?

3. What is learned from the patient's response to amyl nitrate administration?

4. What abnormalities are revealed by manometric studies?

5. What physiologic malfunctions are suggested by the manometric studies?

6. Why does the increase in pressure after a swallow occur at the same time at different locations along
the esophagus?

7. Why did the forceful dilation of the LES enhance the ability of the patient to swallow solid food?

8. Why did the patient's symptoms recur?

9. What therapeutic options remain when the patient's symptoms reappear?

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1. The lower part of the patient's esophagus is abnormally dilated. After subsequent swallows, the
barium is mostly retained in the esophagus. In a normal individual, subsequent swallows would
readily clear the barium into the stomach.

2. The most likely explanation for the fluoroscopic observations is that the patient's LES fails to relax
appropriately after a swallow.

3. The increased rate of emptying barium after amyl nitrate administration suggests that the patient's
LES is capable of relaxing and that no structural abnormality of the LES prevents emptying of the
esophagus.

4. Esophageal manometry revealed the following abnormalities in this patient: (a) a smaller than usual
increase in esophageal pressure after a swallow, (b) the failure of this pressure increase to propagate
in a peristaltic fashion, (c) a higher than normal resting pressure in the LES, and (d) the failure of the
LES pressure to fall as much as normal after a swallow.

5. The small increases in pressure that are observed after each swallow and the absence of detectable
esophageal peristalsis suggest deficits of esophageal contractility and deficits in the propagation of
the esophageal wave of contraction. This finding is consistent with dysfunction of the intramural
plexuses of the esophagus or of the vagal motor innervation. The resting pressure in the LES is about
double the normal resting LES pressure. More important, the LES fails to relax very much after a
normal swallow; this might result from hypertrophy of the LES or from a deficit or malfunction in the
neuronal circuitry that mediates LES relaxation. In the normal individual, the LES relaxes essentially
completely after a swallow to allow esophageal propulsion to proceed. This dysfunction of the LES is
the major problem in the patient's disorder, which is known as achalasia.

6. Because the esophageal sphincter remains closed, when the upper part of the esophagus (which
appears to be close to normal in this disorder) contracts, the pressure is transmitted down the
esophagus with essentially no time delay, because water is incompressible.

7. Forceful dilation of the LES tears some of the esophageal muscle fibers. This makes it possible for the
LES to relax sufficiently after a swallow to allow swallowing to be almost normal.

8. With time the damage done to the LES is repaired, and the original problems with swallowing, caused
by failure of the LES to relax sufficiently, recur.

9. The therapeutic options at this point are the same as those available when the disorder was first
diagnosed: (a) to treat the patient with drugs (calcium channel blockers such as nifedipine taken by
mouth just before eating), (b) to repeat the abrupt mechanical dilation of the LES, (c) to make a
longitudinal incision in the LES through the entire thickness of the musculature, which markedly
weakens the LES.