1. Esophageal Diseases ABSITE Lecture Series
Faiz Bhora, MD
Attending Thoracic Surgeon
St. Luke’s Roosevelt Medical Center, NY,

2. Esophagus Lecture Part 1

3. 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 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

4. Essential Esophageal Anatomy
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.

5. 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)

6. Esophageal Spasm Syndromes
Pain is the Predominant Symptom
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

7. Esophageal Spasm Syndromes
Pain is the Predominant Symptom
HAPC (Nutcracker esophagus)
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

8. Esophageal Spasm Syndromes
Pain is the Not the Predominant Symptom
Achalasia

9. Achalasia Achalasia is best confirmed by:
A birds beak appearance on barium esophagogram
Aperistalsis of the cervical esophagus
Failure of the LES to relax on swallowing
LES pressure < 5 mmHg
Biopsy proven esophagitis on flexible endososcopy

10. 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 (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 years

11. Characteristics of Achalasia
Manometry
Incomplete LES relaxation on swallowing
Aperistalsis of the body
Elevated LES pressure (>35 mmHg)
Increased resting esophageal pressure
Esophagogram
Esophageal dilation
Air/Fluid level
Bird’s beak or Sigmoid esophagus
Endoscopy
Retained food
Frequently normal

12. 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
Calcium channel blockers and long acting Nitrates both decrease LES resting pressure. Usually poor, short-lived response, side effects limit their effectiveness
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

13. 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%)

14. 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

15. 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

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

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

18. Esophageal Spasm Syndromes
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

19. Esophageal Diverticulum
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

20. 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)

21. 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

22. 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

23. Esophagus Lecture Part 2

24. 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:
Repeat endoscopy to identify any mucosal injury
VATS/or thoracotomy and operative repair as you suspect an esophageal perforation
Infectious disease consult for the prompt administration of antibiotic therapy
Contrast study of the esophagus
Admit to ICU, IV hydration. If stable and CXR normal, upper GI endoscopy or gastrografin swallow the next morning

25. 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

26. 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

27. Esophageal Perforation
When to Manage an Esophageal Perforation Non-Operatively?
Minimal Symptoms
Contrast study shows small, contained leak
About 25% of esophageal perforations meet this criterion
Lower tolerance of conservative management with thoracic and abdominal vs cervical perforations
All patients must be admitted to a monitored setting with initiation of hydration, antibiotics and monitoring of urine output

28. 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

29. 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 7th 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
Esophageal exclusion should rarely be used primarily
Esophagectomy reserved if there is an underlying malignancy or with end-stage achalasia
The use of esophageal stent is controversial, but very promising

30. 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, 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

31. 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

32. 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

33. 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

34. 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

35. 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:
The patient should immediately be intubated to secure his airway
Endoscopy is contraindicated, as it could exacerbate the injury
Sodium hypochlorite (bleach) ingestion has the highest likelihood of perforation
Gastrografin swallow is performed at 3 weeks and helps guide dilation of strictures
Steroids help decrease the incidence of strictures

36. Gastroesophageal Reflux Disease
True statements regarding an abdominal approach versus a thoracic approach to anti-reflux surgery include:
A Collis gastroplasty for a shortened esophagus is easier performed through the abdomen
Large hernias are easier repaired laparoscopically with better results
Redo operations are better performed through the abdomen
There is a lower incidence of esophageal perforation
The Nissen repair is easier performed transabdominally

37. Gastroesophageal Reflux Disease
Preoperative Work-up
1) esophagogastroduodenoscopy
2) esophageal manometric evaluation.
3) 24-hour intraesophageal pH monitoring
4) barium cineradiography

38. 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

39. Gastroesophageal Reflux Disease
Types of Repairs:
Nissen
Hill
Toupet
Dor
Belsey Mark IV
Collis Gastroplasty

40. Barrett’s Esophagus
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

41. Esophageal Cancer 14,000 new cases each year, more common in men
Adenocarcinoma now represents about 60-70% of these tumors
Presentation
90% present with dysphagia
70% present with weight loss
50% present with substernal or epigastric pain
Hoarseness is rare

42. 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%

43. Esophageal Cancer PET/CT now widely used for pre operative assessment
Bronchoscopy is done if tracheal or bronchial involvement is suggested

44. 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

45. Esophageal Cancer Staging Stage I : T1 N0 Stage 2A : T2 N0 and T3 N0
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

46. 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

47. Esophageal Cancer Surgical Approaches Transhiatal esophagectomy
Ivor-Lewis esophagectomy
Esophagectomy with cervical anastomosis
Thoracoabdominal with left chest anastomosis

48. Esophageal Cancer
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