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Esophageal Diseases
ABSITE Lecture Series
Faiz Bhora, MD
Attending Thoracic Surgeon
St. Luke’s Roosevelt Medical Center, NY,
Esophagus Lecture Part 1
Essential Esophageal Anatomy
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The esophagus is 25 cm in length. The lower 5-7 cm are below
the diaphragm
Average distance from incisors to GE junction is 38-40 cm in
men. The distance from the incisors to the cricopharyngeus is
15 cm
Topographically, the esophagus begins at the lower border of
C6. The diaphragmatic hiatus is at T10
The upper 1/3 esophagus is slightly to the left of midline, the
middle 1/3 slightly to the right, and the lower 1/3 slightly to the
left
Essential Esophageal Anatomy
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The upper 1/3 is composed of striated muscle and is innervated
by the vagus and its recurrent branch. The lower 2/3 is
composed of smooth muscle and is supplied by the vagus and
the intrinsic autonomic nerve plexus
The arterial blood supply is segmental. These include the
inferior thyroid artery, bronchial arteries and aortic branches, and
branches of the left gastric and inferior phrenic
The venous drainage likewise is via the azygous, hemiazygous,
intercostals, and left gastric veins
The lymphatic drainage of the upper 1/3 is to the internal
jugular, deep cervical and para tracheal nodes. The middle 1/3
drains into the subcarinal and inf pulmonary ligament nodes.
The lower 1/3 drains into the paraesophageal and celiac nodes.
Esophageal Spasm Syndromes
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Inadequate LES relaxation
Achalasia, epiphrenic diverticulum
Uncoordinated esophageal contraction
Diffuse esophageal spasm (DES)
Hypercontraction
High-amplitude peristaltic contraction (HAPC, “nutcracker
esophagus”), Hypertensive lower esophageal sphincter (HLES)
Hypocontarction
Ineffective esophageal motility (IEM)
Esophageal Spasm Syndromes
Pain is the Predominant Symptom
1.
DES
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Patients present with cardiac like chest pain
Dysphagia to both solids and liquids
Corkscrew esophagus on contrast esophagogram
Manometry shows > 10% of a series of wet swallows associated with
simultaneous contractions and with mean amplitudes of > 30 mmHg. LES
is normal
Treatment includes medications, pneumatic dilatation, botulinum toxin
injections. Operative intervention when conservative measures have failed
Esophageal Spasm Syndromes
Pain is the Predominant Symptom
2.
HAPC (Nutcracker esophagus)
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Patient’s present with sharp, episodic chest pain
Dysphagia uncommon
Contrast esophagogram of low yield
Manometry shows high amplitude, coordinated, peristaltic contractions
Treatment with diltiazem has been shown to be helpful. Long esophageal
myotomy and partial fundoplication if medical therapy fails
Esophageal Spasm Syndromes
Pain is the Not the Predominant Symptom
1.
Achalasia
Achalasia
Achalasia is best confirmed by:
1.
A birds beak appearance on barium esophagogram
2.
Aperistalsis of the cervical esophagus
3.
Failure of the LES to relax on swallowing
4.
LES pressure < 5 mmHg
5.
Biopsy proven esophagitis on flexible endososcopy
Achalasia
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Achalasia is a primary motor disorder of the esophagus
characterized by failure of relaxation of the LES and loss of
peristaltic waveform in the body
The cause is believed to be neuronal degeneration in the
myenteric plexus (Auerbach’s plexus)
Symptoms include dysphagia, regurgitation, weight loss, chest
pain, pneumonia
Achalasia is a premalignant condition, with carcinoma
developing in 1-10% of patients over 15-25 years
Characteristics of Achalasia
Manometry
1.
2.
3.
4.
Incomplete LES relaxation on swallowing
Aperistalsis of the body
Elevated LES pressure (>35 mmHg)
Increased resting esophageal pressure
Esophagogram
1.
2.
3.
Esophageal dilation
Air/Fluid level
Bird’s beak or Sigmoid esophagus
Endoscopy
1.
2.
3.
Esophageal dilation
Retained food
Frequently normal
Treatment of Achalasia
Traditionally, the primary therapeutic approach for achalasia
involves pharmacological agents, endoscopic botulinum toxin
into the LES and pneumatic dilatation of the LES
Pharmacologic Agents
1.
Calcium channel blockers and long acting Nitrates both
decrease LES resting pressure. Usually poor, short-lived
response, side effects limit their effectiveness
2.
Endoscopic botulinum toxin is successful in 80% of patients
in relieving dysphagia. However, symptoms return in 50% in 6
months. Retreatment is successful in 50% of original
responders
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Treatment of Achalasia
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Pneumatic Dilatation
Disrupts LES muscle fibers and produces relief of symptoms in
50-85% of patients. However, most patients require multiple
dilatations, increasing the risk of perforation (up to 8%). Long
term relief of symptoms in 40-65%
Pneumatic Dilation vs Surgical Myotomy
Only one randomized controlled trial with long term follow-up
(dysphagia relieved in 91% vs 65%)
Treatment of Achalasia
Surgical Myotomy
 Transabdominal vs Transthoracic
 Dysphagia relief about 90% at 2 years
 Recurrent dysphagia within 2 months likely due to incomplete
myotomy, torsion of the repair or scarring of the mucosa from
cautery
 Late-onset dysphagia due to mucosal stricture from reflux, or the
latent effects of delayed gastric emptying. These patients
ultimately need gastric or esophageal resection
Treatment of Achalasia
Surgical Myotomy
 A resting LES > 36 mm Hg is associated with a good surgical
outcome
 Patients with esophageal dilation > 6 cm or with loss of the
esophageal axis (i.e. sigmoid, tortuous or convoluted esophagus)
will need an esophagectomy
Addition of an Anti-Reflux Procedure?
 Reflux symptoms occur in up to 30% of patients. A partial
fundoplication should be added.
 No difference between a 180-degree Dor or a 270-degree Toupet
partial fundoplication
Esophageal Spasm Syndromes
Pain is the Not the Predominant Symptom
2.
Hypertensive Lower Esophageal Sphincter (HLES)
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Most patients present with dysphagia
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Manometry shows elevated basal LES pressure, normal
peristalsis and normal LES relaxation
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Treatment options include medication, pneumatic dilatation or
myotomy
Esophageal Spasm Syndromes
Pain is the Not the Predominant Symptom
3.
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Ineffective Esophageal Motility (IEM)
Most often seen in scleroderma, rheumatoid arthritis, SLE, DM,
alcoholism
Most patients present with dysphagia and reflux
Contrast esophagogram shows a “lead-pipe” esophagus
Manometry shows low amplitude contractions, ineffective peristalsis and
decreased LES resting pressure
Therapy involves medical anti-reflux therapy. Esophageal shortening may
occur in these patients
Esophageal Spasm Syndromes
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2.
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5.
A 55 yr old woman has a 6 month history of intermittent
heartburn and dysphagia. Endoscopy shows severe
esophagitis. The barium swallow shows a lead-pipe esophagus.
The LES resting pressure is < 5 mmHg with markedly
diminished peristaltic activity. The most likely diagnosis is:
Achalasia
GERD
Cohn's disease
Scleroderma
Sjogren’s syndrome
Esophageal Diverticulum
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2.
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4.
5.
A 65 yr old man has worsening dysphagia and
regurgitation. Barium swallow shows a 5 cm epiphrenic
diverticulum. Treatment should be:
Distal esophageal resection
Esophageal dilation and fundoplication
Resection of the diverticulum and long myotomy
Resection of the diverticulum only
Diverticulopexy
Esophageal Diverticulum
Epiphrenic Diverticulum
 Usually pulsion diverticulum located within the distal 10 cm of the thoracic
esophagus
 Usually right sided
 Most are found incidentally, however, the most common symptoms are
dysphagia, regurgitation
 Barium esophagogram remains the best test for diagnosis
 Endoscopy, 24 Hr PH and manometry should be performed
 Symptomatic, anatomically dependent and enlarging diverticulum should be
surgically repaired
 Surgical therapy includes diverticulectomy, myotomy and a partial
fundoplication as indicated (Transthoracic or Transabdominal)
Esophageal Diverticulum
Zenker’s Diverticulum
 Most common esophageal diverticulum
 Killian’s triangle is usually the site of weakness
 Symptoms include regurgitation, halitosis, chocking, aspiration,
nocturnal coughing, laryngitis. Motility determines symptoms
and not the pouch size
 Diagnosis made on barium swallow
 Endoscopy to rule out malignancy
 Surgical treatment recommended if symptomatic
 Treatment consists of cervical esophagomyotomy and pouch
resection
Esophageal Diverticulum
Midesophageal Diverticulum
 Usually traction diverticulum
 These are due to TB and histoplasmosis. Most asymptomatic
and need no intervention
 Midesophageal pulsion diverticulum are due to an underlying
motility disorder and are due to pulsion.
 Manometry is helpful to define the extent of myotomy
 Surgical intervention for large (> 5 cm) and symptomatic
diverticulum
 Buttress repair with pleura, pericardial fat or omentum
Esophagus Lecture Part 2
Esophageal Perforation
65 yr old female with achalasia complains of back pain after
pneumatic dilation. The CXR is normal post procedure.
The next best course of action is:
1.
Repeat endoscopy to identify any mucosal injury
2.
VATS/or thoracotomy and operative repair as you suspect an
esophageal perforation
3.
Infectious disease consult for the prompt administration of
antibiotic therapy
4.
Contrast study of the esophagus
5.
Admit to ICU, IV hydration. If stable and CXR normal,
upper GI endoscopy or gastrografin swallow the next morning
Esophageal Perforation
Common Causes of Esophageal Perforation
 Endoscopy, esophageal dilation, NG tube insertion, trauma
 Operative procedures associated with perforation: anterior spine
surgery (cervical), gastric fundoplication, thyroidectomy,
pneumonectomy
Common Sites of Esophageal Perforation
 Cricopharyngeus, aortic knob, gastro esophageal junction
Esophageal Perforation
Presentation and Diagnosis
Differential diagnosis includes myocardial infarction, pancreatitis, perforated peptic ulcer disease,
aortic dissection, acute gastric volvulus
 Symptoms vary depending on site, mechanism and interval to presentation.
However, pain is the most common complaint
 Fever, tachycardia, leucocytosis, subcutaneous emphysema and crepitus,
dysphagia, pleural effusion, peritonitis, sepsis
 Rapid diagnosis is the key: mortality of untreated esophageal perforation
increases from 10-20% to 40-60% after the first 24 hours
 CXR: mediastinal emphysema, pleural effusion, hydropneumothorax
 Gastrografin swallow (10% missed injuries), followed by thin barium
 CT may be helpful in equivocal cases and to help guide non-operative
treatment
Esophageal Perforation
When to Manage an Esophageal Perforation Non-Operatively?
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Minimal Symptoms
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Contrast study shows small, contained leak
1.
About 25% of esophageal perforations meet this criterion
2.
Lower tolerance of conservative management with thoracic
and abdominal vs cervical perforations
3.
All patients must be admitted to a monitored setting with
initiation of hydration, antibiotics and monitoring of urine
output
Esophageal Perforation
Operative Management of Cervical Perforation
 Drainage usually is sufficient
 Incision made anterior to the sternocleidomastoid muscle
 Mediastinoscopy can be used to drain the middle mediastinum
 Neck drained with closed suction or penrose drains
 Direct operative repair if perforation easily localized
 May be combined with thoracic drainage if extensive mediastinal
and pleural soilage present
Esophageal Perforation
Operative Management of Thoracic Perforation
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Most cases can be primarily repaired. Upper 2/3 approached via right 5th
intercostal space and lower 1/3 via left 7th intercostal space
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Must debride all necrotic muscle and identify the mucosa. Mucosa closed
as a separate layer (4-0 vicryl). Close muscle over the repair if possible.
Reinforce with intercostal, pleural or pericardial flap
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Widely open the mediastinal pleura and drain the pleural cavity
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NG drainage for 7 days, followed by a contrast study
1.
Esophageal exclusion should rarely be used primarily
2.
Esophagectomy reserved if there is an underlying malignancy or with endstage achalasia
3.
The use of esophageal stent is controversial, but very promising
Paraesophageal Hernias
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Organoaxial stomach rotates about its longitudinal
Mesoaxial stomach rotates about a line perpendicular to the cardiopyloric
line
Classification
Type I: Sliding, fundus only, 1-5 cm, no rotation
Type II: True Paraesophageal, fundus/body, 1-5 cm, organoaxial rotation
Type III: Mixed, fundus and body, ½ or more of stomach, organoaxial and
mesoaxial rotation
Type IV: Mixed with other organs, fundus and body plus other organs, ½ or
more of stomach and colon, organoaxial and mesoaxial rotation
Paraesophageal Hernias
Symptoms and Signs
 50% are asymptomatic, although minor symptoms are usually
overlooked
 Typical symptoms include epigastric pain, post-prandial
discomfort in the chest, heartburn, regurgitation, vomiting,
weight loss, dyspnea
 Important signs include anemia, pneumonia
 An incarcerated intrathoracic stomach is a surgical emergency.
These patients present with acute chest or epigastric pain and
retching, but the inability to vomit
Paraesophageal Hernias
Diagnosis
 CXR shows a retro cardiac air bubble, with or without an air
fluid level
 Barium swallow confirms the diagnosis and usually shows a
large, intrathoracic upside down stomach
 Endoscopy helpful to evaluate for ulcers, Barrett’s and
neoplasms
 Esophageal motility studies helpful in an elective setting to help
guide decisions regarding a wrap
Paraesophageal Hernias
Treatment
All patients with symptoms or signs should undergo elective
repair in the absence of prohibitive surgical risk
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Approaches include transthoracic, abdominal or laparoscopic
Transthoracic approach provides the ability to mobilize the esophagus,
relative ease of dissection of the hernia sac, and optimal exposure for secure
crural closure. A Collis gastroplasty can also easily be performed
The main advantage of the abdominal approach is the ability to place the
stomach in the appropriate anatomic orientation
Laparoscopy is associated with 5-8% incidence of esophageal perforation,
and higher recurrence rate than the open procedure
Paraesophageal Hernias
Treatment
All patients with symptoms or signs should undergo elective
repair in the absence of prohibitive surgical risk
 Need for esophageal mobilization and lengthening
 The importance of sac excision
 The role of gastrostomy and gastropexy
 The need for mesh in crural repair
Caustic Ingestion
A 7 year old boy swallowed some ammonia (glass cleaner).
He is crying, complaining of pain and is drooling.
Which of the following is true:
1.
The patient should immediately be intubated to secure his
airway
2.
Endoscopy is contraindicated, as it could exacerbate the injury
3.
Sodium hypochlorite (bleach) ingestion has the highest
likelihood of perforation
4.
Gastrografin swallow is performed at 3 weeks and helps guide
dilation of strictures
5.
Steroids help decrease the incidence of strictures
Gastroesophageal Reflux Disease
True statements regarding an abdominal approach versus a
thoracic approach to anti-reflux surgery include:
1.
A Collis gastroplasty for a shortened esophagus is easier
performed through the abdomen
2.
Large hernias are easier repaired laparoscopically with better
results
3.
Redo operations are better performed through the abdomen
4.
There is a lower incidence of esophageal perforation
5.
The Nissen repair is easier performed transabdominally
Gastroesophageal Reflux Disease
Preoperative Work-up
1) esophagogastroduodenoscopy
2) esophageal manometric evaluation.
3) 24-hour intraesophageal pH monitoring
4) barium cineradiography
Gastroesophageal Reflux Disease
Indications for Surgery
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Surgical therapy should be considered in those individuals with documented GERD
who:
1) have failed medical management
2) opt for surgery despite successful medical management (due to life style
considerations including age, time or expense of medications, etc.)
3) have complications of GERD (e.g. Barrett's esophagus; grade III or IV
esophagitis)
4) have medical complications attributable to a large hiatal hernia. (e.g. bleeding,
dysphagia)
5) have "atypical" symptoms (asthma, hoarseness, cough, chest pain, aspiration)
and reflux documented on 24 hour pH monitoring
Gastroesophageal Reflux Disease
Types of Repairs:
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Nissen
Hill
Toupet
Dor
Belsey Mark IV
Collis Gastroplasty
Barrett’s Esophagus
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1.
2.
3.
4.
5.
False statements regarding Barrett’s esophagus include:
20-40% of patients with severe dysplasia will have invasive
carcinoma in the resected specimen
The likelihood of developing cancer in the first 3-5 years after
severe dysplasia has been identified is 25-50%
The ideal therapy for Barrett's with severe dysplasia is
endoscopic laser ablation of the mucosa and an anti-reflux
procedure
The ideal therapy for uncomplicated Barrett's esophagus is
controversial
It is a premalignant condition and occurs in 10% of all
patients with reflux
Esophageal Cancer
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14,000 new cases each year, more common in men
Adenocarcinoma now represents about 60-70% of these
tumors
Presentation
1.
90% present with dysphagia
2.
70% present with weight loss
3.
50% present with substernal or epigastric pain
4.
Hoarseness is rare
Esophageal Cancer
Role of EUS in T Stage Assessment
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Esophageal carcinoma presents as a hypo echoic mass that
disrupts the normal anatomy of the esophagus
Accuracy for assessing depth of tumor penetration is 85-90%
Role of EUS in N Stage Assessment
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Hypo echoic nodes, sharply demarcated and larger than 5 mm
are likely to be malignant
Accuracy for overall N stage accuracy is 77%. FNA added to
EUS increases the accuracy to 91%
Esophageal Cancer
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PET/CT now widely used for pre operative assessment
Bronchoscopy is done if tracheal or bronchial
involvement is suggested
Esophageal Cancer
Staging
T1: invades lamina propria or sub mucosa
T2: invades muscularis propria
T3: invades adventitia
T4: invades adjacent structures
N0: no lymph nodes
N1: regional lymph nodes
M1: distant metastasis, including celiac or cervical nodes
Esophageal Cancer
Staging
T1: invades lamina propria or sub mucosa
T2: invades muscularis propria
T3: invades adventitia
T4: invades adjacent structures
N0: no lymph nodes
N1: regional lymph nodes
M1: distant metastasis, including celiac or cervical nodes
Stage I : T1 N0
Stage 2A : T2 N0 and T3 N0
Stage 2B : T1 N1 and T2 N1
Stage 3 : T3 N1 and T4 any N
Stage 4 : M1
Esophageal Cancer
Neoadjuvant Therapy
 5 yr survival with surgery alone is 25%
 Although 7 randomized trials have been done, only one 1996
study (Walsh) shows a survival benefit at 3 years
 We employ neoadjuvant therapy for Stage 2B or higher
 Chemotherapy is 5FU and Cisplatin
 Radiation is about 40G
Esophageal Cancer
Surgical Approaches
 Transhiatal esophagectomy
 Ivor-Lewis esophagectomy
 Esophagectomy with cervical anastomosis
 Thoracoabdominal with left chest anastomosis
Esophageal Cancer
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1.
2.
3.
4.
5.
Regarding esophageal cancer and its management, which
statement is false:
Its incidence is rising in the US
Preoperative workup should include EUS, PET/CT and PFT’s
Neoadjuvant treatment is the standard of care for T2, N0
esophageal cancer
There is no significant difference in survival between the
transhiatal or transthoracic approach
The leak rate is higher with the transhiatal approach