Plummer-Vinson

Syndrome

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style="text-align: justify">This uncommon

clinical syndrome is characterized by dysphagia as­sociated with atrophic oral mucosa, spoon-shaped fingers with

brit­tle nails, and chronic anemia. It characteristically occurs in middle-aged edentulous women. Because iron-

deficiency anemia is a common finding, another name for this condition is sideropenic dys­phagia. The syndrome is

more common in the Scandinavian coun­tries than in the United

States, and its presentation is variable. Not all patients exhibit the classic

syndrome; some lack iron-deficiency anemia and others have the typical clinical stigmata, but lack dys­phagia or

the presence of an esophageal web.

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style="text-align: justify">Clinical

observation with the use of medical microscopes suggests that the esophageal web once thought to be a component of

the syndrome in some patients may actually be a drug-induced lesion, caused by ingestion of ferrous sulfate, a drug

commonly prescribed in cases of iron-deficiency anemia. Ferrous sulfate is known to cause esophageal injury, and a

number of patients may have had a drug-induced esophageal in­jury develop at the site where the web is commonly

observed. Not knowing the cause of the esophageal abnormality, early observers reported the web as part of the

syndrome. Malignant lesions of the oral mucosa, hypopharynx, and esophagus that were studied with the use of

medical microscopes have been noted to occur in up to 100% of patients when followed long-

term.

Videoradiographic studies, as

well as endoscopic findings, have demonstrated a fibrous web just below the cricopharyngeus mus­cle as the cause

of dysphagia in these patients. Treatment consists of dilation of the web and iron therapy to correct the

nutritional deficiency.

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class="MsoNormal" style="text-align: justify">Schatzki’s ring

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style="text-align: justify">Schatzki’s

ring is a thin submucosal circumferential ring in the lower esophagus at the squamocolumnar junction, often

associated with a hiatal hernia. Its significance and pathogenesis are unclear. Templeton first noted the ring, but

Schatzki and Gary defined it as a distinct entity in 1953. Its prevalence varies from 0.2 to 14% in the general

population, depending on the tech­nique of diagnosis and the criteria used. Stiennon believed the ring to be a

pleat of mucosa formed by infolding of redundant esophageal mucosa due to shortening of the esophagus. Others

believe the ring to be congenital, and still others suggest it is an early stricture result­ing from inflammation

of the esophageal mucosa caused by chronic reflux.

class="MsoNormal" style="text-align: justify">Schatzki’s ring is a distinct clinical entity having different symptoms, upper gastrointestinal function

studies, and response to treatment when compared with

patients with a hiatal hernia, but without a ring. Twenty-four-hour esophageal pH monitoring has shown that

patients with a Schatzki’s ring have a lower incidence of reflux than hiatal hernia controls. They also have

better LES func­tion. This, together with the presence of a ring, as observed using medical microscopes, could

represent a protective mechanism to prevent gastroesophageal reflux.

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class="MsoNormal" style="text-align: justify">Symptoms associated

with Schatzki’s ring are brief episodes of dysphagia during hurried ingestion of solid foods. Its treatment has

varied from dilation alone to dilation with antireflux measures, antireflux procedure alone, incision, and even

excision of the ring. Little is known about the natural progression of Schatzki’s rings. Using radiologic

techniques, Chen and colleagues showed progres­sive stenosis of rings in 59% of patients, whereas Schatzki found

that the rings decreased in diameter in 29% of patients and remained unchanged in the

rest.

Symptoms in patients with a ring are caused more by the pres­ence of the ring than by

gastroesophageal reflux. Most patients with a ring but without proven reflux respond to a single dilation, while

most patients with proven reflux require repeated dilations. It is through these findings that the majority of

Schatzki’s ring patients without proven reflux have a history of ingestion of drugs known to be damaging to the

esophageal mucosa. Bonavina and associates have suggested drug-induced injury as the cause of stenosis in patients

with a ring, but without a history of reflux. Since rings also occur in patients with proven reflux, it is likely

that gastroesophageal reflux also plays a part. This is supported by the fact that there is less drug ingestion in

the history of these patients. Schatzki’s ring is probably an acquired lesion that can lead to stenosis from

chemical-induced injury by pill lodgment in the distal esophagus, or from reflux-induced injury to the lower

esophageal mucosa.

style="font-size: 10pt; font-family: Arial">The best form of treatment of a symptomatic Schatzki’s ring in

patients who do not have reflux consists of esophageal dilation for relief of the obstructive symptoms. In patients

with a ring who have proven reflux and a mechanically defective sphincter, an antireflux procedure is necessary to

obtain relief and avoid repeated dilation.

class="MsoNormal" style="text-align: justify">Mallory-

weiss syndrome

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In 1929, Mallory and Weiss described four patients with

acute upper gastrointestinal bleeding who were found at autopsy using medical microscopes to have mucosal tears at

the gastroesophageal junction. This syndrome, characterized by acute upper gastrointestinal bleeding following

repeated vomiting is considered to be the cause of up to 15% of all severe upper gastrointestinal

bleeds. The mechanism is similar to spontaneous esophageal perforation: an acute increase in intra-abdominal

pressure against a closed glottis in a patient with a hiatal hernia.

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class="MsoNormal" style="text-align: justify">Mallory-Weiss tears

are characterized by arterial bleeding, which may be massive. Vomiting is not an obligatory

factor, as there may be other causes of an acute increase in infra-abdominal pressure, such as paroxysmal coughing,

seizures, and retching. The diagnosis requires a high index of suspicion, particularly in the patient who develops

upper gastrointestinal bleeding following prolonged vomiting or retching. The use of upper endoscopy and medical

microscopes confirms the suspicion by identifying one or more longitudinal fissures in the mucosa

of the herniated stomach as the source of bleeding.

style="text-align: justify">For the majority of patients, the

bleeding will stop spontaneously with no operative management. In addition to blood replacement, the stomach should

be decompressed and antiemetics administered. A distended stomach and continued vomiting aggravate further

bleeding. A Sengstaken-Blakemore tube will not stop the bleeding, as the pressure in the balloon

is not sufficient to overcome arterial pressure.

style="text-align: justify">Surgery is required occasionally to

stop blood loss. The procedure consists of laparotomy and high gastrotomy with over sewing of the linear tear.

Mortality is uncommon and recurrence is rare.

class="MsoNormal" style="text-align: justify">Scleroderma

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Scleroderma is a systemic disease accompanied by

esophageal ab­normalities in approximately 80% of patients. In most cases, the disease follows a prolonged course.

Renal involvement occurs in a small percentage of patients and signals a poor prognosis. The onset of the disease

is usually in the third or fourth decade of life, occurring twice as frequently in women as in

men.

Small vessel inflammation, as observed using medical microscopes, appears to be an

initiating event, with subsequent perivascular deposition of normal collagen, which may lead to vascular

compromise. In the gastrointestinal tract, the pre­dominant feature is smooth muscle atrophy. Whether the atrophy

in the esophageal musculature is a primary effect or occurs secondary to a neurogenic disorder is unknown. The

results of pharmacologic and hormonal manipulation, with agents that act either indirectly via neural mechanisms or

directly on the muscle, suggest that sclero­derma is a primary neurogenic disorder. Methacholine, which acts

directly on smooth muscle receptors, causes a similar increase in LES pressure in normal controls and in patients

with scleroderma. Edrophonium, a cholinesterase inhibitor that enhances the effect of acetylcholine when given to

patients with scleroderma, causes an increase in LES pressure that is less marked in these patients than in normal

controls, suggesting a neurogenic rather than myogenic etiology. Muscle ischemia due to perivascular compression

has been suggested as a possible mechanism for the motility abnormality in scleroderma. Others have observed that

in the early stage of the dis­ease, the manometric abnormalities may be reversed by reserpine, an agent that

depletes catecholamines from the adrenergic system. This suggests that in early scleroderma an adrenergic

overactivity may be present that causes a parasympathetic inhibition, supporting a neurogenic mechanism for the

disease. In advanced disease mani­fested by smooth muscle atrophy and collagen deposition, reserpine no longer

produces this reversal. Consequently, from a clinical per­spective, the patient can be described as having a poor

esophageal pump and a poor valve.

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The diagnosis of scleroderma can be made manometrically

by the observation of normal peristalsis in the proximal striated esoph­agus, with absent peristalsis in the

distal smooth muscle portion. The LES pressure is progressively weakened as the disease advances. Because many of

the systemic sequelae of the dis­ease may be nondiagnostic, the motility pattern is frequently used as a specific

diagnostic indicator. Gastroesophageal reflux commonly occurs in patients with scleroderma, since they have both

hypoten­sive sphincters and poor esophageal clearance. This combined defect can lead to severe esophagitis and

stricture formation. The typical barium swallow shows a dilated, barium-filled esophagus, stomach, and duodenum, or

a hiatal hernia with distal esophageal stricture and proximal dilatation.

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Traditionally, esophageal symptoms have been treated with medications such as H2 blockers, antacids,

elevation of the head of the bed, and multiple di­lations for strictures, with generally unsatisfactory results.

The de­gree of esophagitis is usually severe and leads to marked esophageal shortening. Consequently a Collis

gastroplasty in combination with a Belsey antireflux repair is the usual procedure for the surgical management of

this problem. Surgery reduces esophageal acid ex­posure, but does not return it to normal because of the poor

clearance function of the body of the esophagus. Only 50% of the patients have a good-to-excellent result. If the

esophagitis is severe, or there has been a previous failed antireflux procedure and the disease is associated with

delayed gastric emptying, a gastric resection with Roux-en-Y esophagojejunostomy and a Hunt-Lawrence pouch has

proved the best option.