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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. The esophagus is 25 cm in length. The lower 5-7 cm are below the diaphragm
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Esophageal DiseasesABSITE Lecture Series Faiz Bhora, MD Attending Thoracic Surgeon St. Luke’s Roosevelt Medical Center, NY,
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 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.
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 • 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 • 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
Esophageal Spasm Syndromes Pain is the Not the Predominant Symptom • Achalasia
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
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 15-25 years
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 • 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 • 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
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 270-degree Toupet partial fundoplication
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
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
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
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
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
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
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? • 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
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 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
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
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 • 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: • 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
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
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
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
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
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 : 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 • 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