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Esophageal

Diseases

ABSITE Lecture Series


Faiz Bhora, MD
Attending Thoracic Surgeon
St. Lukes Roosevelt Medical
Center, NY,

Esophagus Lecture Part 1

Essential Esophageal
Anatomy

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
The upper 1/3 is composed
of striated muscle and is
Anatomy
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

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

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)

Patients 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

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 (Auerbachs 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
Birds 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

Treatment of Achalasia

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 270degree Toupet partial fundoplication

Esophageal Spasm
Syndromes
Pain is the Not the Predominant Symptom
2.
Hypertensive Lower Esophageal Sphincter
(HLES)

Most patients present with dysphagia

Manometry shows elevated basal LES pressure,


normal peristalsis and normal LES relaxation

Treatment options include medication,


pneumatic dilatation or myotomy

Esophageal Spasm
Syndromes
Pain is the Not the Predominant Symptom
3.

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

1.
2.
3.
4.
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
Sjogrens syndrome

Esophageal Diverticulum

1.
2.
3.
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
Zenkers Diverticulum
Most common esophageal diverticulum
Killians 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 NonOperatively?

Minimal Symptoms

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

Most cases can be primarily repaired. Upper 2/3 approached


via right 5th intercostal space and lower 1/3 via left 7 th
intercostal space

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

Widely open the mediastinal pleura and drain the pleural cavity

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 end-stage achalasia
3.
The use of esophageal stent is controversial, but very promising

Paraesophageal Hernias

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, or more of stomach,
organoaxial and mesoaxial rotation
Type IV: Mixed with other organs, fundus and body plus other
organs, 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, postprandial 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,
Barretts 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

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

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:

Nissen
Hill
Toupet
Dor
Belsey Mark IV
Collis Gastroplasty

Barretts Esophagus

1.
2.

3.

4.
5.

False statements regarding Barretts 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 2550%
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

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

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

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

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
Stage 2A
Stage 2B
Stage 3
Stage 4

: T1 N0
: T2 N0 and T3 N0
: T1 N1 and T2 N1
: T3 N1 and T4 any N
: 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

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 PFTs
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