General Surgery (Board Review Series) 1st Edition

11

The Esophagus

James W. Thiele

  1. Anatomy and Physiology
  2. General anatomy (Figure 11-1)
  3. The esophagus
  • is a midline structure divided into 3 anatomical segments.
  1. The cervical esophagus
  • extends approximately 5 cm from the cricoid cartilage, where it is firmly attached, to the second thoracic vertebrae.
  1. The thoracic esophagus
  • is approximately 20 cm long.
  • extends from the thoracic inlet to the diaphragmatic esophageal hiatus.
  1. The abdominal esophagus
  • is 2 cm long.
  • is anchored by an extension of the transversalis fascia called the phrenoesophageal membrane.
  • extends from the diaphragm to the gastroesophageal junction.
  1. Esophageal sphincter function

 .    The upper esophageal sphincter (UES) is a distinct anatomic structure defined by the cricopharyngeus muscle.

  1. Relaxation of the UES
  • allows swallowed material propelled by a pharyngeal swallow to enter the upper esophagus.
  1. Contraction of the UES
  • 0.5 seconds later prevents regurgitation.
  1. The lower esophageal sphincter (LES)is a physiologic sphincter.
  • Its competence is determined by a variety of factors (see IV A 1).
  1. Areas of normal anatomic narrowing

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 .    Cervical constriction

  • occurs at the cricopharyngeus muscle.
  1. Broncho-aortic constriction
  • is located at the fourth thoracic vertebrae.
  • is secondary to the aortic arch and left mainstem bronchus crossing over the esophagus at this level.
  1. Diaphragmatic constriction
  • occurs as the esophagus passes through the diaphragm.
 

Figure 11-1. General anatomy of the esophagus. Measurements indicate clinically important endoscopic landmarks as measured from the incisors in adults. (Reprinted with permission from Shields TW: General Thoracic Surgery, 4th ed. Baltimore, Williams & Wilkins, 1994, p 1361.)

  1. The surgical approach to the esophagus

 .    The cervical esophagus

  • is best approached via a left thoracotomyowing to left-sided deviation of the esophagus in this region.
  1. The mid-thoracic esophagus
  • is best approached via a right thoracotomy, because this approach avoids the aortic arch.
  1. The lower thoracic esophagus

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  • is best approached via a left thoraco-abdominal incision.
  1. Histologic characteristics
  2. The entire esophagus is linedby stratified squamous epithelium.
  3. Muscular layers consist ofinner circular and outer longitudinal muscle.
  4. The upper 2–6 cm of the esophagus containsonly striated muscle fibers.
  • Smooth muscle fibers become more predominant more distally in the esophagus.
  1. There is no serosal coveringin the esophagus.
  2. Arterial supply and venous drainage (Figure 11-2)
 

Figure 11-2. Arterial supply and venous drainage of the esophagus. (Reprinted with permission from Shields TW: General Thoracic Surgery, 4th ed. Baltimore, Williams & Wilkins, 1994, p 1368.)

  1. The cervical esophagus
  • receives arterial blood from the inferior thyroid artery.
  • Venous blood from this region drains into the inferior thyroid veins.
  1. The thoracic esophagus
  • receives arterial blood from bronchial arteriesand directly from the aorta.
  • Venous blood in this region drains into either bronchial veins or into the azygos or hemiazygos systems.

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  1. The abdominal esophagus
  • receives arterial blood from the left gastricand inferior phrenic arteries.
  • Venous blood from this region drains into the coronary vein.
  1. Nervous supply and lymphatic drainage
  2. Innervation
  3. Parasympathetic innervation is
  • via the left and right vagus nerves.
  1. Sympathetic innervation is
  • from branches of the sympathetic chain
  • via the celiac ganglion.
  1. Lymphatic drainage
  • As a general rule, the lymphatic drainage of the upper two thirds of the esophagusis cephalad.
  • That of the lower thirdis caudad.
  1. Physiologic function of the esophagus
  2. Peristaltic waves in the esophagusare grouped into three types.
  3. Primary peristalsis
  • is progressive.
  • follows a voluntary swallow.
  1. Secondary peristalsis
  • is progressive.
  • is generated by esophageal distention or irritation.
  1. Tertiary contractions
  • are nonprogressive.
  • may occur either after voluntary swallows or between swallows.
  1. Relaxation of the LES
  • occurs 1.5–2.5 seconds after a voluntary swallow.
  • allows food propelled by primary and secondary peristalsis to enter the stomach.
  1. Factors that induce LES relaxationinclude
  • secretin, cholecystokinin, vasoactive intestinal peptide, progesterone, α-adrenergic antagonists, anticholinergic agents, nicotine, ethanol, chocolate, fatty meals, and gastric acidification.
  1. Factors that inhibit LES relaxationinclude
  • gastrin, motilin, vasopressin, glucagon, α-adrenergic agonists, cholinergic agents, and gastric alkalinization.
  1. Tests of Esophageal Anatomy and Function
  2. Barium esophagram with static radiographs
  3. This is the test of choice

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  • to evaluate structural changesin the esophagus.
  • to define surgical anatomy.
  1. Generally, it is the initial test of choicefor evaluating patients with
  • dysphagia.
  • suspected esophageal mass lesions.
  1. Endoscopic ultrasound

is useful for evaluation and staging of patients with mass lesions of the esophagus.

  1. A cinematographic esophagram
  • differs from a standard esophagram in that the entire examination is videotaped.
  1. Videotape review
  • allows for a closer inspection of esophageal function.
  1. It is useful for detecting motility disordersand is the only test that can adequately evaluate the oropharyngeal phase of swallowing.
  2. Its sensitivityin detecting small mucosal abnormalities is low.
  3. Esophageal manometry
  • is used to evaluate the effectiveness of esophageal body and sphincter contractile function.
  1. Pressure sensitive transducersmeasure both the amplitude of contraction and the progression of the peristaltic wave.
  2. It can also evaluatethe major components of LES function (resting pressure, intra-abdominal length, and total length).
  3. Esophageal pH probe monitoring
  • can be used to evaluate the degree of acid reflux into the distal esophagus.
  1. A pH probeis placed 5 cm above the manometrically determined LES. Data is collected via a portable recorder.
  2. The distal esophageal pHis recorded every second over a 24-hour period. The data are analyzed to determine if pathologic reflux is present.
  3. Esophagoscopy
  • is used to directly visualize the esophageal mucosa.
  1. Flexible endoscopy
  • can be performed with sedation.
  • can be used to evaluate esophageal mucosal abnormalities and obtain biopsies.
  1. Rigid endoscopy
  • requires general anesthesia.
  • is better than flexible endoscopy for retrieving swallowed foreign objects.

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III. Disorders of Esophageal Motility

  1. Achalasia
  • is the most common esophageal motility disorder.
  1. This disorder is characterized by
  • esophageal aperistalsis and dilation
  • failure of the LES to relax.
  1. Patients typically presentin the third to fifth decades of life.
  2. Men and women are equally affected.
  3. Symptomsinclude
  • dysphagiainvolving both solids and liquids.
  • regurgitation of undigested food.
  1. Underlying pathologyinvolves
  • degenerative changes in the ganglia of Auerbach's plexus (myenteric)in the esophagus.
  1. These degenerative changesresult in a hypertensive LES.
  2. Failure of the LES to relax with swallowing results in
  • increased intraluminal pressure.
  • dilation.
  • eventual loss of peristalsis.
  1. In Third World countries
  • infection with Trypanosoma cruzi(Chagas' disease) produces similar degenerative changes.
  1. Patient evaluationshould include esophageal manometry to evaluate esophageal function and document LES hypertension.
  • Contrast radiographs may reveal a characteristic “bird's beak” appearance with proximal esophageal dilation.
  1. Treatment
  • can be medical, mechanical, or surgical.
  1. Medical treatment
  • with nitratesand calcium channel blockers can be used in very mild cases of achalasia, but has poor long-term success (less than 20%).
  1. Dilation of the LES
  • with rigid or pneumatic devices has had better (60%–80%) long-term success than medical therapy.
  1. Dilation may be repeated multiple times if symptoms recur.
  2. The main risk is ruptureof the esophagus.
  3. Surgical therapy
  • involves muscle division (myotomy) to mechanically disrupt the hypertensive LES and lower esophageal smooth muscle.
  1. Esophageal spasm syndromes
  • involve disturbances in the coordination of normal esophageal peristalsis.

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  1. Named disorders includenutcracker esophagus, diffuse esophageal spasm, and hypertensive LES. Each syndrome differs in the type of peristaltic disturbance seen (Table 11-1).
  2. The hallmark symptoms of these disorders are
  • chest painthat may radiate to the back, neck, ears, jaw, or arms.
  • dysphagiafor both liquids and solids.
  1. Diagnosis
  • Esophageal manometryis the gold standard for diagnosis.
  • Video esophagogram and endoscopy may also be useful.
  1. Treatment
  2. Medical therapy includes
  • nitratesand calcium channel blockers.
  • sedatives and muscle relaxants.
  1. Surgical therapy
  • consists of a long esophageal myotomy.
  • is generally reserved for those patients with incapacitating dysphagia.
  1. Surgical myotomy
  • results in a loss of high amplitude contractions.
  1. Palliation of dysphagia
  • is reported in 80%–90% of patients.

Table 11-1. Manometric Findings Noted in Esophageal Spasm Disorders

Disorder

Manometric Findings

Nutcracker esophagus

Normal LES; mean amplitude of esophageal contractions in the lower esophagus is > 180 mm Hg, but peristalsis is normal.

Diffuse esophageal spasm

LES relaxation is often inadequate; simultaneous contractions greater than 20% of the time, often with high amplitude contractions. Intermittent normal peristalsis is present.

Hypertensive LES

Elevated LES pressure (> 26 mm Hg) with complete relaxation. Peristalsis in the esophageal body is normal.

LES = lower esophageal sphincter.

  1. Scleroderma
  • is a disease that affects esophageal motility in 80% of patients.
  1. The effects in the gastrointestinal tract are characterized by
  • smooth muscle atrophy.
  • collagen deposition.
  1. Diagnosis
  • is made manometrically.
  1. Normal peristalsis

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  • is seen in the proximal esophagus owing to the predominance of striated muscle.
  1. Peristaltic function
  • diminishes more distally in the esophagus as the smooth muscle content increases.
  1. Symptoms are related to
  • gastroesophageal reflux secondary to a weak LES and poor clearance of refluxed acid because of diminished lower esophageal peristalsis.
  1. Treatment(either medical or surgical)
  • The goal of management is to prevent the associated gastroesophageal reflux (see IV).
  1. Diverticuli of the esophagus
  • may be either pulsion or traction in origin.
  1. Pulsion divertuculi or pseudodiverticuli
  • develop as a result of increased intraluminal esophageal pressure.
  • do not involve all layers of the esophageal wall (hence they are considered pseudodiverticula).
  1. A pharyngeo-esophageal or Zenker's diverticulum
  • is the most common diverticulum in the esophagus.
  1. This type of diverticulum
  • occurs just proximal to the cricopharyngeus muscle.
  • is most common in the fifth to eighth decades of life.
  1. Symptomsinclude
  • dysphagia.
  • regurgitation of undigested food.
  • choking.
  • halitosis.
  1. Diagnosis
  • is made by barium esophagram.
  • endoscopy (careful to not rupture diverticulum).
  1. Treatmentinvolves
  • cricopharyngeal myotomyto relieve intraluminal pressure in addition to resection of large (> 2 cm) diverticuli.
  1. An epiphrenic diverticulum
  • is rare and is usually associated with an esophageal motility disorder.
  1. These can occuranywhere, but are most prevalent in the distal 10 cm of the esophagus.
  2. Many patients are asymptomatic
  • but common presenting symptoms include dysphagia and regurgitation.
  1. Diagnosis
  • Both esophagram and esophageal manometry are necessary to define any underlying motility disorder.

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  1. Treatmentinvolves
  • diverticulectomy and long esophageal myotomy on the side opposite the diverticulectomy.
  1. Traction diverticuli
  • result from inflammation in surrounding tissues.
  1. These diverticuli
  • are generally midesophagealand are considered true diverticuli because they involve all layers of the esophageal wall.
  1. Most are asymptomatic
  • but rare complications include fistula formation between the trachea or great vessels.
  1. Treatment
  • consists of excision of the inflamed tissue and primary closure of the esophagus.
  1. Gastroesophageal Reflux Disease (GERD) and Esophageal Hernia
  2. GERD is characterized by
  • the loss of the normal gastroesophageal barrier to reflux of stomach contents.
  • decreased clearance of refluxed materials from the distal esophagus.
  1. Pathologic reflux
  • is caused by the loss of mechanical competency of the LES. This is defined by three parameters, all of which can be measured manometrically:
  1. A resting pressure greater than 6 mm Hgis associated with a competent LES.
  2. Overall length of more than 2 cmis also associated with a competent LES.
  3. Intra-abdominal length of more than 1 cmresults in transmission of the positive abdominal pressure to the LES and facilitates competence.
  4. Symptoms of GERD

 .    Heartburn and epigastric pain

  1. Recurrent pneumonia and chronic cough from recurrent aspiration
  2. Sour taste in the mouth upon waking (“water brash”) and choking sensations
  3. Symptoms are worse when the patient is lying or bending down, and after meals.
  4. Evaluation of patients with severe GERD

 .    Esophageal pH probe monitoring

  • documents the presence of prolonged or repeated exposure of the distal esophagus to acid reflux.

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  1. Manometryboth evaluates the competency of the LES and documents the peristaltic ability of the esophagus.
  • Distal esophageal dysfunctioncan accompany GERD.
  • The type of surgical proceduremay be altered in the face of severe esophageal dysfunction (see IV A 4 b).
  1. Endoscopydocuments and evaluates the complications of GERD, such as
  • stricture.
  • ulcerations.
  • Barrett's esophagus (see VI A).
  1. Therapy
  • for these patients may be medical or surgical depending on the severity of symptoms and the presence of complications.
  1. Medical therapy
  2. Medical therapy is generally used in patients with new onset disease or those with mild symptoms.
  3. Therapyincludes
  • H2-blockers, such as famotidine.
  • proton pump inhibitors, such as omeprazole.
  • prokinetic agents, such as metoclopramide or erythromycin.
  • antibiotic therapy, for patients with H. pylori.
  1. Indications for surgeryin patients with severe GERD include:
  2. Failure of medical therapyto control symptoms.
  3. Recurrence of symptomsafter medical therapy is stopped.
  4. The presence of complicationsof the disease, such as
  • esophageal stricture.
  • pulmonary insufficiency secondary to aspiration.
  1. The surgical procedure
  • of choice for GERD is a fundoplication.
  1. This procedureis done by wrapping the gastric fundus around the distal esophagus to increase LES pressure (Figure 11-3). Fundoplication serves to re-establish the reflux barrier.
  2. Operations
  3. A full 360° (Nissen) operation
  • is used in patients with normal esophageal function.
  • can be performed via an open procedure laparoscopically.
  1. A partial 270° (Belsey Mark IV operation) or loose wrap
  • is used for patients with dysmotilityto avoid dysphagia.
  1. It is imperativethat the fundus and not the body of the stomach be used for the wrap. Otherwise, it will relax after swallowing, as does the LES.
  2. Any defect in the esophageal hiatusshould also be repaired.
  3. Esophageal hernias (Figure 11-4)
  4. A type I or sliding esophageal hernia

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  • occurs when the gastroesophageal junction moves cephalad from the stomach into the chest.
  • is the most common type of esophageal hernia.
  1. This type of herniais often associated with GERD.
  2. Indications for surgical repairinclude
  • severe refractory symptoms.
  • severe esophageal mucosal dysplasia(e.g., Barrett's esophagus) resulting from GERD.
 

Figure 11-3. Nissen fundoplication procedure for gastroesophageal reflux disease. (A) Approximation of diaphragmatic crura. (B) Sites of stitches for fundoplication. (C)Complete fundoplication. (D) Cross-section of completed fundoplication. (Adapted with permission from Lippincott Williams & Wilkins. Zuidema GD: Surgical Treatment: abdominal approach. In Gastroesophageal Reflux and Hiatal Hernia. Edited by Skinner DB, Belsey RH, Hendrix TR. Boston: Little, Brown, 1972, p 154.)

  1. A type II or rolling (paraesophageal) esophageal hernia
  • The gastroesophageal junction is in its normal position (intra-abdominal).
  • A hernia sac containing gastric fundus and body develops along the side of the esophagus.
  1. Reflux is rarely present because of
  • pressure from the herniated stomach.
  • the normal position of the gastroesophageal junction.
  1. Symptoms include
  • postprandial chest pain.
  • dysphagia.
  • early satiety.

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  1. Surgical repair
  • is indicated for all patients with type II herniasbecause gastric obstruction as well as strangulation and necrosis of the stomach can occur in as many as 30% of patients.
 

Figure 11-4. Hiatal hernias. (Reprinted with permission from Cameron J: Current Surgical Therapy, 6th ed. St. Louis, Mosby, 1998, p 14.)

  1. Type III esophageal hernias
  • are combinations of type Iand type II.
  • should be treated as a type II.
  1. Evaluationshould include
  • endoscopy to evaluate the position of the gastroesophageal junction.
  • a barium esophagram to define hernia type.
  1. Surgical therapy

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  • differs for each type of hernia.
  1. Type I hernias are repaired with
  • fundoplication.
  • closure of the hiatal defect.
  1. Type II and III hernias are repaired by
  • returning the stomach to the abdomen.
  • closing the hiatal defect.
  • fundoplication, if there was evidence of reflux disease pre-operatively.
  1. Benign Lesions of the Esophagus
  2. Schatzki's ring
  • is a thin submucosal circumferential ring in the distal esophagus at the gastroesophageal junction.
  1. Almost all patients with a Schatzki's ring
  • will have an associated sliding hiatal hernia.
  1. The upper surface of the ring is covered by squamous epithelium, while the lower surface is covered by columnar epithelium.
  2. Patients usually present clinically
  • with short episodes of dysphagia usually after a hurried swallow.
  1. Treatment
  • Symptomatic patientsusually respond to dilation of the ring.
  • Coexisting reflux symptoms, however, may require antireflux therapy or surgery.
  1. Esophageal webs
  • are similar lesions occurring in the upper esophagus.
  1. Plummer-Vinson syndrome is characterized by
  • the presence of esophageal webs.
  • associated iron deficiency anemia.
  1. Treatment
  • involves administration of iron supplements.
  1. Leiomyomas
  • are the most common benign tumors of the esophagus.
  • can occur throughout the esophagus, but are rare in the neck.
  1. These tumors
  • are submucosal.
  • generally occur between the ages of 20 and 50.
  • are solitary in approximately 90% of cases.
  1. The most common presenting symptomis dysphagia, however, most are asymptomatic.

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  1. Diagnosis
  • is generally made by esophagogram.
  1. These masses appearas smooth, concave defects with sharp borders.
  2. Endoscopy
  • is indicated to rule out carcinoma.
  1. Endoscopic ultrasound
  • is also useful for characterizing the lesion.
  1. Biopsy is not recommended
  • if leiomyoma is suspectedbecause scarring from the biopsy site will make excision more difficult.
  1. Asymptomatic lesions
  • can be followed with serial esophagrams; whereas, symptomatic lesions warrant resection.
  1. Esophageal leiomyomas
  • can generally be carefully dissected away from the underlying tissues without esophageal resection.
  1. Recurrenceof these lesions is rare.
  2. Esophageal polyps
  • are the second most common benign esophageal tumors.
  1. These lesionsare most common in older men.
  • Eighty percent are found in the cervical esophagus.
  1. Large lesionsmay be regurgitated into the mouth.
  2. Additional symptomsinclude
  • dysphagia.
  • hematemesis or melena.
  1. Resection
  • Smallpolyps can be resected endoscopically.
  • Largerlesions require resection through a cervical incision.
  1. Premalignant and Malignant Lesions of the Esophagus
  2. Barrett's esophagus
  • is a premalignant lesion of the distal esophagus caused by chronic gastroesophageal reflux.
  1. This condition involves replacing the normal squamous epithelium with gastric epitheliumin the distal esophagus.
  2. Injury to the distal esophagus
  • results in metaplasia of the distal esophageal mucosa.
  1. The diagnosis of Barrett's esophagus
  • is made when the gastric epithelium extends more than 2 cminto the tubular esophagus.

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  1. Metaplasia may progressto dysplasia or adenocarcinoma of the esophagus if the chronic acid reflux is allowed to continue.
  2. Initial managementincludes
  • medical therapy with acid suppression.
  • prokinetic agents.
  • life-long endoscopic surveillance of the distal esophageal mucosa.
  1. Fundoplication(see IV A 4 c)
  • may be necessary in those patients who continue to have reflux symptoms while on medical therapy.
  1. Although fundoplication will halt further progressionof the Barrett's, those changes that have already taken place will not regress.
  2. Endoscopic surveillance
  • is necessary to monitor the Barrett's mucosa.
  1. Mild or moderate dysplastic changes
  • may be monitored with frequent endoscopic surveillance and biopsy.
  1. Severe dysplastic or cancerous changes
  • require esophageal resection.
  1. Malignant lesions of the esophagus
  2. Squamous cell carcinoma and adenocarcinoma
  • are the two most common types of esophageal cancer.
  1. Squamous cell carcinoma
  • of the esophagus accounts for 60% of esophageal cancers.
  1. Risk factors are diverseand include
  • smoking.
  • alcohol consumption.
  • long-standing achalasia.
  • previous caustic injuries.
  • human papilloma virus infection.
  • nitrosamines.
  1. These lesions are more commonin the proximal two thirds of the esophagus.
  2. Adenocarcinoma
  • was once an unusual diagnosis.
  • now accounts for 40% of all esophageal cancers in Western countries.
  1. The most important risk factor
  • is Barrett's esophagus.
  1. Patients with Barrett's esophagus
  • are 40 times more likely than those without the condition to develop adenocarcinoma of the esophagus.
  1. Adenocarcinoma is more commonin the distal third of the esophagus.

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  1. Common symptomsinclude
  • dysphagia.
  • weight loss.
  • anemia.
  1. Staging is based on
  • the depth of invasion of the primary tumor.
  • the presence of lymph node and distant metastasis (Table 11-2).
  1. Endoscopic ultrasoundis useful for staging.
  2. Patients with upper or middle third lesions
  • should undergo bronchoscopyto determine if there is tracheal or bronchial invasion, which is a contraindication to esophagectomy.
  1. Distant metastasis
  • documented by computed tomography (CT) scan also precludes curative resection.

Table 11-2. The TNM* Staging Criteria for Esophageal Cancer

Criteria

Primary tumor (T)

T1

Tumor < 5 cm long, noncircumferential, without obstruction, and without extraesophageal spread

T2

Tumor without spread beyond the muscularis propria, but with the presence of obstruction, a circumferential lesion, or a lesion > 5 cm

T3

Evidence of extraesophageal spread of the tumor

Nodal status (N)

N0

No involvement of regional lymph nodes

NX

Regional lymph nodes cannot be assessed

N1

Palpable, mobile, unilateral nodes

N2

Palpable, mobile, bilateral nodes

N3

Fixed lymph nodes

Distant Metastases (M)

M0

No distant metastases

M1

Distant metastases

Staging

Stage I

T1

N0

M0

Stage IIA

T2
T3

N0
N0

M0
M0

Stage IIB

T1
T2

N1
N1

M0
M0

Stage III

T3
T4

N1
Any N

M0
M0

Stage IV

Any T

Any N

M1

*TNM = tumor-node-metastasis.

  1. Treatment

 .    Resection of the esophagus

  • may be performed through an abdominal incision combined with either

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right thoracotomy (Ivor-Lewis approach) or a cervical/transhiatal approach.

  1. Ivor-Lewis approach
  • is preferred if the tumor is adherent to structures in the chest because much of the thoracic dissection is blind in the transhiatal approach.
  1. A transhiatal approach
  • prevents the need for thoracotomy. An Ivor-Lewis approach, however, allows for more thorough lymph node sampling.
  1. Re-anastomosis
  • is generally performed by pulling the stomach into the chest or neck and attaching it to the proximal esophagus.
  1. Alternative approaches to re-anastomosis include
  • the use of either a colon or small bowel interposition graft.
  1. Chemotherapy or radiation therapy alone
  • does not appear to affect survivalof these patients.
  1. Combined modality
  • (combination chemotherapy and radiation therapy) appears to have some promise; its effect on survival remains to be determined.
  1. Five-year survival
  • for stage Ilesions is only 50%–70%.
  • for stage IVlesions is generally less than 5%.
  1. Palliation therapy
  • for nonresectable patients generally centers on relief of dysphagiaand nutritional support.
  1. Transient relief of dysphagia can be provided by
  • endoscopic laser fulguration of the tumor mass.
  • placement of esophageal stents.
  • palliative resection or bypass.
  1. Nutritional support
  • can be provided by gastrostomy or jejunostomy tubes.

VII. Other Considerations in Esophageal Disease

  1. Esophageal perforation
  2. The most common causesare
  • iatrogenic rupture.
  • spontaneous rupture.
  • trauma.
  1. Iatrogenic rupture
  • related to interventions such as endoscopy and dilation of the esophagus is the most common cause of rupture.
  • accounts for 50% to 75% of all cases.
  1. Spontaneous rupture (Boerhaave's syndrome)

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  • typically accounts for 15% of all cases.
  • is caused by straining.
  1. Rupture after forceful vomiting
  • is the most common cause.
  1. Perforation can also be induced by
  • childbirth.
  • straining with defecation (especially in young children).
  1. Trauma
  2. accounts for 20% of spontaneous ruptures.
  3. Traumasinclude
  • blunt or penetrating trauma.
  • caustic injuries.
  • ingested foreign bodies.
  1. Diagnosis

 .    Signs and symptoms may include

  • chest pain.
  • fever.
  • tachycardia.
  • respiratory distress.
  • hypotension.
  • odynophagia.
  • mediastinal emphysema.
  • pneumothorax.
  • Hamman's sign (mediastinal “crunch” associated with mediastinal emphysema).
  1. water-solubleor barium contrast study can help to characterize the location and severity of the perforation.
  2. Treatment
  • largely depends on the duration of symptoms prior to presentation and treatment.
  1. Perforation is frequently fatal if not diagnosed and treated early.
  2. Immediate treatment for all patientsincludes
  • stopping all oral intake.
  • instituting intravenous hydration.
  • starting broad spectrum antibiotics.
  • placing a nasogastric tubefor stomach decompression.
  1. For a perforation less than 24 hours old, patients are generally treated with
  • débridement.
  • primary repair of the defect.
  • reinforcement of the area with autologous tissue (e.g., intercostal muscle).
  1. Patients presenting more than 24 hours after rupture may be treated with

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  • débridement.
  • primary repair.
  • Some patients, however, will require esophageal exclusion and diversion to allow the rupture site to heal (Figure 11-5).
 

Figure 11-5. Esophageal diversion procedure for an esophageal perforation. (Reprinted with permission from Cameron J: Current Surgical Therapy, 6th ed. St. Louis, Mosby, 1998, p 14.)

  1. Caustic injuries of the esophagus
  2. These injuries are most commonly a result of
  • accidental ingestion in children younger than 5 years old.
  • suicide attempts in adults.
  1. Acid and alkali burns
  • have different characteristics and clinical courses.
  1. Acidscause a coagulative type necrosis.
  • This necrosis is more superficial and less likely to penetrate beyond the submucosa.
  1. Alkaliscause liquefactive type necrosis.
  • This necrosis results in the destruction of surface lipoproteins and penetration deep into muscle and surrounding tissues.
  1. Management

 .    Induction of vomiting is contraindicated

  • because it may re-expose the esophagus to the inciting agent.
  1. Nasogastric tube placement is contraindicated
  • because of the possibility of perforation of the friable, injured mucosa.

P.256

 

  1. Early endoscopy is indicated
  • to evaluate and grade the injury, which helps to determine treatment (Table 11–3).
  1. Surgical indications include
  • continued bleeding.
  • free mediastinal or peritoneal air.
  • evidence of peritonitis or mediastinitis.
  1. Delayed complications include
  • stricture.
  • 500-fold increase in the risk of esophageal cancer.
  • development of an aortoesophageal fistula.

Table 11-3. Classification and Treatment of Esophageal Burns

Grade

Findings on Endoscopy

Treatment

I

Mucosal edema and hyperemia

Observation for 24–48 hours, then discharge if stable

II

Mucosal ulcerations, pseudo-membrane formation, sloughing

Patient is kept NPO for several days and parenteral nutrition is started. Esophagram is obtained in 2–4 weeks to look for stricture formation.

III

Deep ulcerations with eschar formation; severe edema with obliteration of the lumen

Treatment is similar to grade II injuries in the absence of complications such as bleeding or perforation.

NPO = nothing by mouth.

P.257

 

Review Test

Directions: Each of the numbered items or incomplete statements in this section is followed by answers or by completions of the statement. Select the ONE lettered answer or completion that is BEST in each case.

  1. A 52-year-old man is referred for evaluation of dysphagia. Endoscopy reveals an ulcerated mass near the gastroesophageal junction and biopsies of this lesion are positive for adenocarcinoma. Which of the following statements is true?

(A) Most esophageal cancers are diagnosed early

(B) Neither chemotherapy nor radiation therapy alone have been shown to improve survival in these patients

(C) Smoking and alcoholism are important risk factors for development of this lesion

(D) With surgical resection, the 5-year survival for patients with esophageal cancer approaches 75%

(E) Cancers in the gastroesophageal junction tend to metastasize to cervical lymph nodes

1–B. Most esophageal cancers grow to a significant size before producing symptoms of dysphagia because the esophagus lacks a serosa, thus permitting the smooth muscle in the esophagus to stretch and accommodate the enlarging mass. Because of this, most esophageal cancers present at an advanced stage that often precludes cure. The overall 5-year survival rate for patients with esophageal cancer after attempted curative resection is only 40%–50%. While smoking and alcohol abuse are important risk factors for squamous cell cancers, Barrett's esophagus is the most important risk factor for the development of adenocarcinoma. Tumors in the distal third of the esophagus tend to metastasize to caudad lymph nodes.

  1. A 47-year-old man presents with complaints of dysphagia when eating solid food. He denies any history of weight loss and is not anemic on laboratory evaluation. A barium swallow reveals a smooth, nonulcerated filling defect in the middle third of the esophagus that is highly suspicious for a leiomyoma. Which of the following statements is true regarding this patient?

(A) Endoscopy and biopsy is indicated before proceeding with surgical resection

(B) These tumors can generally be enucleated without the need for esophageal resection

(C) Multiple lesions are noted in at least 50% of cases

(D) Resection is indicated even in small, asymptomatic lesions due to malignant potential

(E) Dysphagia is an uncommon presenting symptom

2–B. Esophageal leiomyomas are generally asymptomatic until they grow large enough to produce dysphagia, which is the most common presenting symptom. They are solitary in approximately 90% of cases and small, asymptomatic lesions can generally be followed with serial examinations, because these lesions have almost no malignant potential. Endoscopy is indicated to rule out malignancy, but if leiomyoma is suspected biopsy is contraindicated because it may make subsequent resection much more difficult due to scarring at the biopsy site.

  1. A 51-year-old woman presents for evaluation of postprandial epigastric pain associated with intermittent episodes of gastroesophageal reflux. Upper gastrointestinal contrast study reveals a type III esophageal hernia and Barrett's changes are noted on endoscopic examination of the distal esophagus. Of the following statements, which most appropriately pertains to this patient?

(A) The presence of a type III esophageal hernia is an indication for surgery

(B) Type III esophageal hernias are the least common type

(C) Fundoplication is not indicated in this patient

(D) Complications occur in less than 5% of patients with this type of hernia

(E) Manometry is not necessary before proceeding with fundoplication in this patient

3–A. Because of the risk of complications such as gastric volvulus and ischemia associated with type II and type III esophageal hernias, it is generally recommended that these defects be surgically repaired once they are identified. Complications can occur in as many as 30% of patients with type I and type II esophageal hernias. Fundoplication is not always indicated in these patients, because many will have no signs or symptoms of gastroesophageal reflux disease. However, if the patient complains of reflux symptoms, has signs of reflux on esophagogastroduodenoscopy (e.g., Barrett's changes), or if there is evidence of an incompetent lower esophageal sphincter on manometry, then a fundoplication is indicated.

  1. A 40-year-old man presents with a 6-month history of dysphagia for both solids and liquids, as well as regurgitation of undigested food after meals. Esophagram shows a dilated esophagus with a significant “bird's beak” narrowing at the lower esophageal sphincter (LES). Manometry reveals poor esophageal peristalsis and a very hypertensive LES. Which of the following statements is true regarding this patient's condition?

(A) Medical treatment with nitrates and calcium channel blockers is successful in most cases

(B) The surgical procedure of choice for this disorder is a long esophageal myotomy

(C) Although dysphagia is common, regurgitation is unusual in these patients

(D) Rigid or pneumatic LES dilation can offer a long-term success rate as high as 80%

(E) Manometry is not indicated if the patient has the classic presenting symptoms and esophagram findings

4–D. This patient presents with the classic symptoms and manometric findings associated with achalasia. Regurgitation is common in patients with long-standing disease as they often retain undigested food in the dilated proximal esophagus. Medical therapy has a long-term success rate of less than 20% and is reserved for those patients with mild disease. The surgical treatment of choice in these patients is a lower esophageal myotomy designed to disrupt the lower esophageal sphincter, not a long esophageal myotomy. Manometry is always indicated in the work-up of any patient who presents with symptoms of esophageal dysmotility.

  1. A 33-year-old man presents to the emergency room 30 minutes after having ingested alkaline drain cleaner. He is currently stable and exhibits no signs of respiratory distress. Examination of the oropharynx reveals only mild erythema of the posterior pharynx. Which of the following statements is true regarding the treatment of this patient?

(A) Induction of vomiting is indicated if the ingestion occurred less that 1 hour ago

(B) Alkali ingestion tends to cause a less serious injury because the stomach acid largely neutralizes it

(C) Early endoscopy is indicated to evaluate the extent of injury

(D) The lack of severity of the oropharyngeal injury indicates that the esophageal injury is not likely to be severe and endoscopy is not indicated

(E) There is a 2- to 4-fold increase in the risk of esophageal cancer in these patients

5–C. Early endoscopy is indicated to evaluate and grade the injury, which helps to determine treatment. The induction of vomiting after ingesting either acid or alkaline substances is contraindicated because further injury to the esophagus may occur as the caustic substance is expelled. Alkaline substances result in liquefactive necrosis in the wall of the esophagus and stomach, which causes deeper burns and thus a more significant injury. The severity of the oropharyngeal injury can be a poor indicator of the severity of the more distal gastrointestinal tract. The risk of esophageal cancer in these patients is increased 500-fold.

  1. A 70-year-old man presents with dysphagia, occasional regurgitation of undigested food, and chronic halitosis. Manometric studies are normal but a barium esophagram reveals a 4-cm Zenker's diverticulum. Which of the following statements is true?

(A) This is the least common diverticulum of the esophagus

(B) A Zenker's diverticulum is a true diverticulum

(C) This type of diverticulum is most common in the third and fourth decades of life

(D) A Zenker's diverticulum occurs just distal to the cricopharyngeus muscle

(E) The procedure of choice in this patient would be a cricopharyngeal myotomy and diverticulectomy

6–E. Zenker's diverticula are the most common diverticula of the esophagus and are generally seen in the fifth to eighth decades of life. This is a pulsion type of diverticulum and is considered a pseudodiverticulum because it does not involve all layers of the esophageal wall. Zenker's diverticula occur just proximal to the cricopharyngeus muscle and the surgical procedure of choice to repair these defects is a cricopharyngeal myotomy. A diverticulectomy should also be performed if the diverticulum is greater than 2 cm.

  1. A 42-year-old woman with scleroderma presents with symptoms of gastroesophageal reflux disease (GERD) that have persisted despite 6 months of adequate medical therapy. Which of the following statements best describes her condition?

(A) Further medical therapy is warranted before surgical intervention is considered

(B) Therapy is generally aimed at controlling gastroesophageal reflux

(C) Because this disease affects primarily the distal esophagus, resection of this segment is curative

(D) The risk of esophageal cancer is higher in these patients

(E) No further work-up is needed in this patient before proceeding with surgery

7–B. Scleroderma is a disease that affects primarily smooth muscle resulting in atrophy and collagen deposition. The distal esophagus is composed entirely of smooth muscle and is thus affected to a greater degree than the proximal esophagus, which is primarily striated muscle. The presence of scleroderma does not change the evaluation and treatment of symptomatic gastroesophageal reflux disease (GERD). Treatment in these patients is aimed at preventing gastroesophageal reflux and the basic treatment and evaluation is the same as for patients without scleroderma. When symptoms are refractory to medical therapy, fundoplication is usually the next recommended step in treatment. Resection of the distal esophagus is not indicated unless there is evidence of severe dysplasia or adenocarcinoma of the distal esophagus secondary to the effects of chronic GER. The risk of esophageal cancer in these patients is the same as in any patient with chronic GER.

  1. A 47-year-old man presents with a 6-month history of intermittent chest pain and dysphagia for both solids and liquids. Cardiac work-up is negative and standard barium esophagram was unremarkable. Esophageal manometry reveals high amplitude contractions and intermittent normal peristalsis with simultaneous contractions 40% of the time. Which of the following statements is true regarding this patient's condition?

(A) Surgery is the treatment of choice

(B) Medical therapy includes nitrates and H2-blockers

(C) In patients with incapacitating dysphagia, 80%–90% report palliation of their symptoms with surgical intervention

(D) Esophagram is the gold standard for identification of this disorder

(E) Surgical therapy consists of a distal esophageal myotomy

8–C. Patients with diffuse esophageal spasm usually present with chest pain and dysphagia for both solids and liquids. Although surgical intervention consisting of a long esophageal myotomy can be very successful in treating these patients, it is reserved only for those patients who have incapacitating dysphagia. Medical therapy consists of nitrates and calcium channel blockers as well as sedatives and muscle relaxants. H2-blockers have no role in the treatment of esophageal spasm. Although video esophagram may be included in the work-up of these patients, manometry is the gold standard in identifying all of the esophageal spasm disorders.

Directions: Each set of matching questions in this section consists of a list of four to twenty-six lettered options followed by several numbered items. For each numbered item, select the appropriate lettered option(s). Each lettered option may be selected once, more than once, or not at all.

Questions 9–12

  1. Gastroesophageal reflux disease
  2. Esophageal leiomyoma
  3. Zenker's diverticulum
  4. Type I esophageal hernia
  5. Type II esophageal hernia
  6. Type III esophageal hernia
  7. Esophageal cancer
  8. Caustic ingestion
  9. Nutcracker esophagus
  10. Boerhaave's syndrome
  11. Achalasia
  12. Diffuse esophageal spasm
  13. Barrett's esophagus

Match the following case scenarios with the most appropriate cause(s).

  1. A 40-year-old man presents with a 1-year history of progressive dysphagia. He first noted difficulty swallowing only solids but his symptoms recently progressed to liquids as well. Esophagram reveals a distal esophageal stricture that is benign by biopsy. (SELECT 2 CAUSES)

9–A, H. In addition to Barrett's changes in the distal esophagus, gastroesophageal reflux disease (GERD) can also result in stricture formation secondary to repeated injury to the esophageal mucosa. Stricture formation is also a late complication of ingesting a caustic substance and can occur years after the original injury. It is imperative that any newly diagnosed esophageal stricture be biopsied to rule out malignancy. Multiple biopsies should be obtained from the involved mucosa because foci of malignancy could easily be missed if only a single biopsy is obtained.

  1. A 56-year-old woman presents with progressive dysphagia for solids and liquids as well as intermittent chest pain. She noticed some relief in her symptoms after she was recently started on a calcium channel blocker for hypertension but her symptoms have recently returned. (SELECT 3 CAUSES)

10–I, K, L. Dysphagia for both solids and liquids accompanied by intermittent chest pain is the typical presentation of patients suffering from esophageal spasm disorders. The only way to effectively distinguish these syndromes from one another is to evaluate the patient with esophageal manometry. Achalasia can also be associated with dysphagia and occasional chest pain that may be relieved with nitrates or calcium channel blockers.

  1. A 58-year-old man presents with a history of dysphagia and intermittent regurgitation of undigested food. (SELECT 2 CAUSES)

11–C, K. Both achalasia and a Zenker's diverticulum should be suspected in a patient who presents with a history of dysphagia as well as the regurgitation of undigested food. The history of this type of regurgitation is important in differentiating these disorders from other causes of dysphagia. In achalasia, swallowed food is unable to pass into the stomach because of poor peristalsis and a hypertensive lower esophageal sphincter (LES). The proximal esophagus eventually dilates and serves as a reservoir for undigested food that is eventually regurgitated by the patient. Patients with a Zenker's diverticulum generally report regurgitation symptoms that are less severe as the diverticulum is a smaller reservoir. A Zenker's can be documented by esophagram, which may also show the esophageal changes of achalasia. Manometry is the gold standard in the diagnosis of achalasia.

  1. A 56-year-old man presents with a history of 4 episodes of right lower middle lobe pneumonia in the past year. He also reports the presence of a persistent, dry cough. Pulmonary work-up, including bronchoscopy, has been negative and he is a nonsmoker. A barium esophagram recently obtained was normal. (SELECT 1 CAUSE)

12–A. Some patients with symptomatic gastroesophageal reflux disease (GERD) have an absence of gastrointestinal complaints. Other symptoms such as recurrent pneumonia caused by occult aspiration and chronic cough from reflux irritation of the upper airway should prompt a full work-up for GERD. Significant GER can often be missed on barium esophagram and definitive tests—including upper endoscopy, manometry, and 24-hour pH probe—should be performed to evaluate the lower esophagus as well as the lower esophageal sphincter, and to document the presence of reflux.



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