Esophageal Motility Disorders and Diverticula

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                            E S O P H A G E A L

                            M O T I L I T Y

                            D I S O R D E R S                                   A N D

                            D I V E R T I C U L A


                           D     erangement of the propulsive activity or of the
                           sphincteric mechanisms guarding the upper and lower
                           ends of the esophagus leads to profound changes in
                           individuals’ ability to swallow and therefore nourish
                           themselves. Dysphagia, or difficulty in swallowing, is an
                           ominous complaint, very rarely exaggerated or mis-
                           represented by the patient, and should alert the physi-
                           cian that there is a serious, if not life-threatening disorder
                           involving the esophagus. A history of difficulty in swal-

lowing solids and/or liquids demands prompt and thor-       ated nausea. He also noted that increasingly he would be
ough investigation to find the cause so that prompt treat-   awakened at night because of coughing. He found this
ment may be started and adequate swallowing and food        particularly disturbing. Occasionally, food particles were
intake can be restored.                                     present in the sputum. He had lost no weight and had not
                                                            changed his eating habits. He did note that he was more
                                                            careful to chew his food thoroughly before swallowing.
CASE 1                                                           His physician found no abnormalities of the mouth or
ZENKER’S DIVERTICULUM                                       neck on physical examination. He was referred for an
                                                            esophagoscopy, which was reported as normal. Next, a
A 75-year-old male complained of mild dysphagia. He         swallow of a thickened barium preparation under cine-
noted from time to time that the “food didn’t go down       fluoroscopy showed a cricopharyngeal diverticulum
normally.” He would occasionally choke or sputter while     (Zenker’s diverticulum). Operation was advised to prevent
eating. The dysphagia was not accompanied by pain. Near     further bouts of aspiration. The large diverticulum in the
the end of his meal he would occasionally regurgitate       left neck was excised. Following discharge he experienced
some food particles recently eaten. There was no associ-    no further dysphagia or nocturnal coughing episodes.

1 1 6     U P P E R     G A S T R O I N T E S T I N A L         T R A C T

CASE 2                                                             The muscular coats of the esophagus give it distinct
ACHALASIA                                                     anatomic and physiologic features. Muscle fibers from the
                                                              cricoid cartilage and pharynx covering the upper third of
A 55-year-old female was seen for progressive difficulty in    the esophagus are voluntarily controlled striated muscle.
swallowing solid foods over the past 4 months. She first       The distal two-thirds of the esophagus is smooth muscle
noted that on eating the evening meal she would experi-       under involuntary control. Thus, while we can initiate swal-
ence some lower substernal discomfort. Initially this         lowing to a point (try swallowing more than 20 times!) the
would be relieved by drinking a glass of water. As her        action is primarily involuntary (you can swallow as much
symptoms progressed she took to cutting her meat into         liquid or food as your stomach can hold; your esophagus
smaller pieces, which initially helped. Gradually, however,   does not tire). This smooth, coordinated activity between
they produced the same subxyphoid and substernal dis-         striated and smooth muscle fibers to propel contents down
tress that was not adequately relieved by a drink of water.   the lumen into the stomach is only minimally understood.
She was now convinced that her food could get down to         The inner circular muscle and outer longitudinal muscle
here (pointing to her xyphoid) where it tended to get         layer, whether striated or smooth, act in an intricately coor-
stuck. Her diet had changed imperceptibly to softer foods     dinated fashion as a single physiologic unit.
eaten in smaller meals. She had lost 25 lb of weight, for          Normally, when swallowing solid food, the tongue
which she was in some ways grateful. Her energy and vi-       presses against the palate, pushing the food into the phar-
tality were not appreciably altered.                          ynx where a coordinated, voluntary swallowing action be-
     Her physician could find no masses or lymphadenopa-       gins. There is closure of the glottis so as to protect the air-
thy on examination. He scheduled an esophagoscopy. The        way. Respiration is suspended and the normal high resting
latter showed a thickened, dilated esophagus throughout       pressure at the pharyngoesophageal sphincter rapidly re-
with a reddened, slightly edematous mucosa at the distal      laxes and the food bolus enters the esophagus. The food
end of the esophagus. The endoscope was advanced into         bolus initiates a complex peristaltic wave, relaxing the
the stomach, which was normal. Biopsies of the reddened       esophagus immediately in front of the bolus, while con-
distal esophagus showed only normal tissue with mild          tracting the circular muscle fibers immediately behind the
inflammation.                                                  bolus. This propels the bolus of food toward the stomach
     It was recommended that she have a Heller procedure      at approximately 4–6 cm/sec. When we are upright, grav-
done. At operation, esophagoscopy was done while the          ity causes liquids and many foods to fall more rapidly than
surgeon exposed the lower 8 cm of the esophagus and the       peristalsis can propel them. However, peristalsis is suffi-
gastric cardia through the laparoscope. A long esophageal     ciently powerful to permit swallowing of solids and liquids
myotomy was done, cutting across the cardioesophageal         even when we are standing on our heads, with gravity act-
sphincter. Both the endoscopist and the surgeon noted         ing against the swallowing mechanism.
the esophageal mucosa, which was not cut, to herniate or           The wave of contraction—beginning with food enter-
“pout” through the esophageal incision. The esophagus         ing the upper esophagus and sweeping down the entire
now appeared capacious. She was discharged the follow-        length of the esophagus to the stomach—is called primary
ing day and 2 months later had regained 20 lb and experi-     peristalsis. When food becomes lodged at some point in
enced no further dysphagia.                                   the esophagus, the local distention will stimulate a series
                                                              of peristaltic waves beginning at that point, called sec-
                                                              ondary peristalsis. The latter is designed to aid in clearing

T        GENERAL CONSIDERATIONS                               the esophagus either of ingested food or of gastric con-
                                                              tents, which may reflux back into the esophagus when
      he esophagus connects the mouth and the stomach         there is relaxation or failure of the cardioesophageal
beginning at about the sixth cervical vertebrae and ending    sphincter. Such secondary peristaltic waves are generally
just below the diaphragm, where it blends into the stom-      perceived by the individual, while primary peristalsis does
ach. Most of the esophagus is intrathoracic, where it         not occur unless the food bolus is unduly hot or cold.
passes behind the bronchus just to the left of the carina.    Nonpropulsive contractions, occurring locally or through-
The esophagus is a muscular tube, a flattened H in shape       out the esophagus, are called tertiary and are considered
in the resting state, lined by stratified squamous epithe-     to be abnormal. They may, however, be present in elderly
lium. Scattered throughout the epithelium are mucus           patients who are asymptomatic.
glands serving as a lubricating mechanism. The surround-           The neural integration of swallowing and esophageal
ing muscle layer does not have a serosal covering like the    propulsion are only partially understood. The swallowing
remainder of the gastrointestinal tract. This lack of a       reflux originates in the medulla oblongata, while the pha-
serosal layer, among other things, is a major reason why      ryngeal and esophageal propulsion are innervated by motor
operations on the esophagus involving an anastomosis are      branches from the 5th, 7th, 9th, 10th, 11th, and 12th cranial
prone to leak or disruption.                                  nerves. The relative importance of these nerves and the
   E S O P H A G E A L            M O T I L I T Y          D I S O R D E R S    A N D     D I V E R T I C U L A          1 1 7

local myogenic mechanisms involved in peristaltic activity                     DIAGNOSIS
are unknown.
     The major tool in understanding esophageal physiology                    ysphagia from cricopharyngeal sphincter dysfunc-
is manometry. This is done by passing a bundle of three fine         tion occurs mainly in individuals over 60 years of age. The
polyethylene catheters with open ports 5 cm apart. The              dysphagia develops immediately on swallowing, particu-
catheters are constantly and gently perfused with a saline          larly solids. Coughing and sputtering are common, as the
solution to ensure their patency and are connected to               dysmotility causes minor degrees of aspiration of both
manometers and recording devices. By passing the bundle             food and saliva. The pathophysiology is related to either
the length of the esophagus, normal high pressure zones at          an incomplete relaxation of the cricopharyngeal sphincter,
both the upper and lower ends can be seen, with an inter-           or incoordination between the swallowing mechanism and
vening lower pressure region in the intrathoracic portion of        the sphincter. A cinefluoroscopic study of a barium swal-
the esophagus. When the bundle is positioned in the mid-            low shows a hesitant passage of the contrast, often due to
esophagus, and swallowing is initiated, a normal pressure           what appears to be a mechanical obstruction from the
tracing and transit can be ascertained (Fig. 15.1). Manomet-        contracting pharyngeal musculature at the lower end of
ric studies help determine the location and type of peri-           the pharynx. Aspiration may be seen. Manometry, which is
staltic abnormality encountered. These studies are occasion-        more difficult at the proximal sphincter, confirms the lack
ally used in conjunction with cinefluorography. Endoscopy            of coordination between the swallow and the relaxation of
is almost always done to determine the nature of the patho-         the sphincter.
logic process by obtaining visualization and by biopsies.                At the other end of the esophagus, achalasia, a pri-
                                                                    mary failure of the cardioesophageal sphincter to relax in
 K E Y       P O I N T S                                            response to swallowing, can occur. This neuromuscular
                                                                    disorder’s etiology is unknown. The dysmotility produces
 • Major tool in understanding esophageal physiology is
                                                                    dilatation of the proximal esophagus and hypertrophy of
 manometry, done by passing bundle of three fine polyethylene
 catheters with open ports 5 cm apart; catheters constantly and
                                                                    the sphincter, particularly of the circular muscle.
 gently perfused with saline solution to ensure patency and con-         Because of the dysmotility, which results in a func-
 nected to manometers and recording devices                         tional but not in an organic obstruction, the patients expe-
 • By passing the bundle the length of esophagus, normal high
                                                                    rience dysphagia (Case 2). In particular, the history is one
 pressure zones at both upper and lower ends can be seen, with      of a progressive difficulty, first in swallowing solid materi-
 an intervening lower pressure region in intrathoracic portion of   als such as a bolus of meat or bread, and progressively in
 esophagus                                                          difficulty in handling large quantities of liquids. Patients
                                                                    can generally localize the pain or discomfort to the distal

        FIGURE 15.1 Deglutition. Normal esophageal peristaltic waves and pressures during consecutive swal-
        lows. Note the orderly downward progression of the waves. U, upper; M, mid; L, lower.
1 1 8      U P P E R     G A S T R O I N T E S T I N A L          T R A C T

end of the esophagus by pointing to the area of the             imflammatory changes and metaplasia of the luminal ep-
xyphoid or slightly above. Discomfort and pain from dis-        ithelium (Barrett’s epithelium).
tention are common, and are usually followed by regurgi-            Not infrequently the esophageal disease is mistaken for
tation. Obstruction may be accompanied by vigorous              angina, as diffuse esophageal spasm or pain mimics the sub-
esophageal contractions, producing a substernal pain.           sternal distress of angina. Sublingual nitroglycerin tablets
     Passage of a nasogastric tube down the esophagus and       cause the prompt relief of symptoms from angina but
into the stomach, if possible, should be the initial investi-   not esophageal disease. Most importantly, patients with
gation. The presence of large quantities of fluid in the         esophageal motility disorders will have had several attacks
mid-esophagus with undigested food particles passing            in the past. Myocardial infarction will seldom occur as
through the tube confirms the diagnosis of esophageal ob-        often, and the anginal attacks thereafter are generally
struction. The esophagus should be gently lavaged with          treated with sublingual nitroglycerin successfully. Esoph-
saline, and endoscopy should be done. Esophagoscopy             ageal motility studies are of help in the differentiation.
generally reveals a dilated esophagus with a smooth aper-           The most important differential is between achalasia
ture at the lower esophagus through which the instrument        and cancer at the cardioesophageal junction. Esophagos-
can be advanced (Case 2). This distinguishes it as a physi-     copy and biopsy is the most direct and expeditious route
ologic obstruction, rather than a mechanical obstruction        to diagnosis.
from a malignancy of either the distal esophagus or proxi-          Scleroderma can also present as achalasia. Biopsy of
mal stomach.                                                    the lower esophagus aids in the differential.
     Although a barium esophagram is commonly done, it
is less helpful than esophagoscopy and potentially danger-       K E Y        P O I N T S
ous due to the risk of aspiration. A very characteristic         • Dysphagia from cricopharyngeal sphincter dysfunction occurs
esophagram is that of a proximal dilated esophagus, which        mainly in individuals over 60 years of age; develops immediately
symmetrically tapers at its lower portion to a thin smooth       on swallowing, particularly solids
stream entering into the stomach.                                • Coughing and sputtering are common, as dysmotility causes
     Manometry shows a very characteristic dysmotility           minor degrees of aspiration of food and saliva
pattern. The peristaltic activity in the body of the esopha-
                                                                 • Pathophysiology related to either incomplete relaxation of
gus is disorganized if not absent. In particular, primary        cricopharyngeal sphincter, or incoordination between swallow-
peristaltic waves are absent. One of the theories of acha-       ing mechanism and sphincter
lasia formation is intrinsic autonomic denervation of the
                                                                 • Passage of nasogastric tube down esophagus and into stom-
distal esophagus. These patients are sensitive to stimula-       ach, if possible, should be initial investigation
tion with bethanechol, which produces a strong contrac-
                                                                 • Presence of large quantities of fluid in mid-esophagus with
tion of the esophagus, often accompanied by brief but se-
                                                                 undigested food particles passing through the tube confirms di-
vere pain.                                                       agnosis of esophageal obstruction
     The pain from diffuse esophageal spasm is substernal
                                                                 • Esophagus should be gently lavaged with saline, and en-
and sustained; hence ischemic cardiac disease must always
                                                                 doscopy done
be considered in the differential diagnosis. Fluoroscopic
and manometric studies are diagnostic for diffuse                • Esophagoscopy generally reveals a dilated esophagus with a
                                                                 smooth aperture at the lower esophagus through which the in-
esophageal spasm. Esophagoscopy is done to rule out an
                                                                 strument can be advanced
inflammatory process such as esophagitis or a tumor re-
sponsible for the pain and dysmotility. Treatment varies         • Manometry shows very characteristic dysmotility pattern;
                                                                 peristaltic activity in the body of esophagus is disorganized if
according to the severity of the symptoms. Many patients
                                                                 not absent
can be managed with long-acting nitrates or anticholiner-
gic drugs. Severe or refractory forms of the disease are         • Reflux esophagitis can produce chronic inflammatory changes
                                                                 at the lower esophagus; patients experience dysphagia because
treated by a long myotomy. Clinically esophageal motility
                                                                 of dysmotility induced by inflammation
disorders can be confused with reflux esophagitis and my-
ocardial infarction.
     Reflux esophagitis can produce chronic inflammatory
changes at the lower esophagus. Patients experience dys-

                                                                T          TREATMENT
phagia because of the dysmotility induced by the inflam-
mation. A good history aids in the differential, as patients          he operation of choice is a Heller procedure, in
with chronic reflux esophagitis usually report improve-          which a longitudinal myotomy is carried out, extending
ment in their symptoms by avoiding alcohol, smoking, and        from the inferior pulmonary vein down to the upper por-
certain foods and by taking antacid medications. Esoph-         tion of the stomach. The approach can be through the tho-
agoscopy shows the lower end of the esophagus to be se-         rax or the abdomen, but currently is being done with in-
verely inflamed and edematous. Biopsies will show severe         creasing frequency as a combined laparoscopic approach
   E S O P H A G E A L            M O T I L I T Y          D I S O R D E R S       A N D        D I V E R T I C U L A          1 1 9

with esophagoscopy control. The esophageal incision is              Manometric studies show sustained forceful or high am-
carried through all the muscle layers until the mucosa is           plitude contractions that are not propulsive. Contrast fluo-
seen “pouting” between the severed muscular ends. The               roscopic studies show areas of esophageal narrowing from
object of the procedure is to render the cardioesophageal           spastic disordered contractions producing a “corkscrew
sphincter incompetent. Thus, esophageal reflux may occur             esophagus” appearance. Some patients with diffuse esoph-
postoperatively. For this reason, many surgeons incorpo-            ageal spasm have a concomitant motility disorder of the
rate a Nissen fundoplication in conjunction with the Heller         cardioesophageal junction (achalasia), suggesting that a
procedure, in order to avoid the postoperative complica-            common pathologic mechanism is responsible for both
tion of reflux. The other complication is inadvertent perfo-         disorders.
ration of the esophageal mucosa with a subsequent
esophageal leak. However done, the Heller myotomy is ef-             K E Y         P O I N T S
fective in relieving the obstruction and giving good to ex-          • The motility disorder affecting the mid-portion of the esopha-
cellent results in 85% of patients. When done through the            gus is diffuse esophageal spasm; patients note dysphagia shortly
laparoscopic approach, the postoperative morbidity is ex-            after initiating swallowing
tremely low so that patients experience a near normal pas-
sage of food through the esophagus almost immediately.
     Therapy is directed at relieving the physiologic obstruc-
tion by disrupting the abnormal sphincter. In the past, at-
                                                                    SUGGESTED READINGS
tempts at frequent dilatations with a variety of esophageal
dilatators have been done. Although these produce tran-             Chakkaphak S, Chakkaphak K, Ferguson MK: Disorders of
sient relief of symptoms they have been consistently ineffec-           esophageal motility. Surg Gynecol Obstet 172:325, 1991
tive in permanent alleviation of the physiologic obstruction.       Massey BT, Dodos WJ, Hogan WJ et al: Abnormal esophageal
More recently, forceful dilatation of the esophageal gastric            motility: an analysis of concurrent radiographic and mano-
junction with pneumatic balloon devices has provided                    metric findings. Gastroenterology 101:344, 1991
longer relief of symptoms, but has failed in most patients.              An excellent review of our current understanding of the eti-
All prospective comparative studies have demonstrated the                ology and interpretation of these diagnostic modalities.
efficacy of surgery over other treatment modalities.

 K E Y       P O I N T S                                            QUESTIONS
 • Operation of choice is a Heller procedure, in which longitudi-
 nal myotomy is carried out, extending from inferior pulmonary      1. An acute patient complaint of dysphagia is best man-
 vein down to upper portion of stomach
                                                                    aged by?
 • Approach can be through thorax or abdomen, but currently
                                                                        A. A careful head and neck examination followed by
 done with increasing frequency as a combined laparoscopic ap-
 proach with esophagoscopy control
                                                                        B. Indirect laryngoscopy.
 • The esophageal incision is carried through all the muscle lay-
                                                                        C. A barium swallow preferably with fluoroscopy.
 ers until the mucosa is seen “pouting” between the severed
                                                                        D. Esophagoscopy with biopsy of any lesions.
 muscular ends
                                                                    2. Complaints of pressure sensation and substernal pain
                                                                    can be found in?

T         DIFFUSE ESOPHAGEAL SPASM                                      A. Esophageal disease.
                                                                        B. Cardiac disease.
      he motility disorder affecting the mid-portion (if not            C. Peptic ulcer disease.
the entire esophagus) is diffuse esophageal spasm. The pa-              D. All of the above.
tients note dysphagia shortly after initiating swallowing.          (See p. 603 for answers.)

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