Swallowing Difficulty & Pain Tim Farrell, MD Tom Egan, MD

Assumptions Students understand the anatomy, physiology, and pathophysiology of the swallowing mechanism and the esophagogastric junction.

Objectives Students will understand: Differential diagnosis for a patient with dysphagia. Symptoms and treatment of GERD. Pathophysiology and treatment of achalasia and diffuse esophageal spasm. Etiology and treatment of esophageal diverticula. Common symptoms and management of hiatal hernias. Management of adenocarcinoma of the E-G junction. Presenting symptoms, etiology and treatment of esophageal rupture.

Case 1 An 80-year-old man presents with a trouble swallowing for a year. He regurgitates after meals, has heartburn, but no other pain and is in good health otherwise. He is thin, without neck mass. His chest is clear and his abdomen is soft and without masses.

Case 1 What is the differential diagnosis? Anatomic Tumor, Stricture, Compression, Foreign Body Functional GERD Motility Disorder (achalasia, scleroderma) Neurologic (Parkinson’s, bulbar paralysis) Psychological Globus Hystericus

Case 1 What test should be done, in what order, and why? Anatomic Assessment Functional Assessment Upper GI Series 24-hr pH EGD Esophageal Manometry Biopsy GES

GERD - Definition Protracted exposure of the esophageal lining to stomach juice

GERD - Causes Lower esophageal sphincter Hiatal Hernia Incompetent valve Inappropriate relaxations Hiatal Hernia Abnormal motility Impaired esophageal clearing Delayed gastric emptying Defective cytoprotection People will experience heartburn symptoms when excessive amounts of acid reflux into the esophagus.

GERD - Symptoms Typical Symptoms Heartburn Regurgitation Trouble Swallowing Atypical Symptoms Asthma Cough Hoarseness Chest Pain A Throat pharynx and larynx B Windpipe trachea C Esophagus D Lungs E Stomach GERD can Masquerade as Other Diseases Chest Pain: Patients with GERD may have chest pain similar to angina or heart pain. Usually, they also have other symptoms like heartburn and acid regurgitation. Asthma: Acid reflux may aggravate asthma. Clues that GERD may be worsening your asthma include 1) asthma that appears for the first time during adulthood, 2) asthma that gets worse after meals, lying down or exercise, and 3) asthma that is mainly at night. Treatment of acid reflux may cure asthma in some patients and decrease the need for asthmatic medications in others. Ear Nose and Throat Problems: Acid reflux may be a cause of chronic cough, sore throat, laryngitis with hoarseness, frequent throat clearing, or growths on the vocal cords.

GERD - Complications Peptic Stricture Esophagitis / Ulcers Barrett's Esophagus Patients with Longstanding GERD Can Experience Severe Complications Esophagitis Redness to Ulceration Peptic Stricture: A narrowing of the lower esophagus from an abundance of scar tissue. Patients complain of food sticking in the lower esophagus. Stretching of the esophagus and proton pump inhibitor medication are needed to control and prevent peptic strictures. Barrett's esophagus: The most serious complication of chronic GERD. The lining of the esophagus changes to resemble that found in the intestine. A pre-cancerous condition: 40-fold increased risk of developing cancer. Routine surveillance endoscopy by a trained gastroenterologist.

Indications for further Dx-Rx Persistent or frequent symptoms Dysphagia Frequent vomiting Early satiety Weight loss Significant respiratory complaints Age < 45

GERD - Diagnosis Barium Swallow Upper Endoscopy Esophageal Manometry 24-Hour Ambulatory Esophageal pH Gastric Emptying Study

GERD - Diagnosis Barium Swallow During a barium swallow, a patient drinks a liquid barium mixture, and a radiologist watches it travels down the esophagus and into the stomach.

GERD - Diagnosis Upper Endoscopy This test involves passing a small lighted flexible tube through the mouth into the esophagus and stomach to examine for abnormalities. The test is usually performed with the aid of sedatives. It is the best test to identify esophagitis and Barrett's esophagus.

GERD - Diagnosis Manometry 24 Hour Ambulatory pH Test If the diagnosis is still in question, the degree of acid refluxed into the esophagus may be measured by a similar tube left in place for 24 hours. Esophageal Manometry This test involves passing a small flexible tube through the nose into the esophagus and stomach in order to measure pressures and function of the esophagus.

GERD - Treatment Environmental Medical - OTC Medical -Prescription Antacids H2-Blockers Medical -Prescription Proton-Pump Inhibitors Endoscopic Surgical Fundoplication

Dietary Modifications Avoid large meals Limit foods which decrease LES pressure Fatty foods, chocolate, mints, and alcohol Avoid irritating foods and beverages Citrus, tomatoes, pepper, etc. Limit caffeine and carbonated beverages Increases acid production Increased gastric distension Candy or gum to increase saliva Alkaline saliva neutralizes acid Increases motility and clearance Enhances drug effect May allow control of symptoms with less aggressive management

Lifestyle Modifications Weight Loss Avoid smoking Decreases LES pressure Avoid lying down for 2-3 hours after meals Limits supine reflux Sleep with elevated head of bed Improves esophageal clearance Candy or gum to increase saliva c

Medications Worsening Reflux Calcium channel blockers Anticholinergics Theophylline Progesterone β2-antagonists, α-antagonists Nitrates Meperidine Diazepam

GERD - Medical Treatment Medications may be used to: Neutralize acid Increase LES tone Improve gastric emptying Over-the-Counter Medications (for occassional symptoms) Large # of Americans use antacids (Maalox, Mylanta). Recently, the FDA approved non-prescription strength medicines called H-2 blockers (Zantac, Pepcid). Prescription Medicines (for more frequent symptoms - >2x/wk) H-2 Blockers: Symptoms are eliminated in up to 50% of patients with twice a day prescription dosage of the H2 blockers. Healing of esophagitis may require higher dosing. These agents maintain remission in about 25% of patients. Good firstline treatment and maintenance agent for some patients. Proton Pump lnhibitors (Prilosec, Prevacid): Provide symptom relief with elimination of symptoms in most cases, even in those with esophageal ulcers. The best long-term maintenance therapy of esophagitis, particularly for those patients with moderate to severe esophagitis. Promotility Agents (Reglan, Propulsid): These drugs improvethe movement of food from the stomach.

OTC H2 Blockers Lower-dose formulations Acute treatment or prophylaxis Slower onset than antacids Longer duration of acid inhibition

Proton Pump Inhibitors

Endoscopic Treatment Modalities Thermal (Stretta®) RFA since 1921…. 2000 Stretta used in GERD Exclusion: Active esophagitis, Long segment barretts nad HH >3 cm 400 Khz to 1 MHz Temp set to 85 C, at 65 C collagen contraction 90 sec, muscularis, two deployments/ring, 6 rings Mechanical + Neural--> blocks intramural abberant vegal afferent fibres Thermal energy Mechanical / Neural

Endoscopic Treatment Modalities Endoscopic Suturing Suturing or plication EndoCinch ®

Endoscopic Treatment Modalities Biocompatible Material Enteryx ®

GERD - Surgical Treatment What are the reasons to consider surgical treatment of reflux disease? Non-response to medical treatment (persistent regurgitation). Prevalence of respiratory symptoms. Paraesophageal Hiatal Hernia. Excessive cost of medicines. Concern over the long-term effects of medicines. Desire not to take medicines for the rest of one’s life. Complicated GERD (Stricture, Barrett’s)

GERD - Surgical Treatment Antireflux Surgery is done laparoscopically Requires specialized optics and video systems.

Nissen Fundoplication Technique

Nissen Fundoplication Technique

Nissen Fundoplication Technique

Nissen Fundoplication Technique

Nissen Fundoplication Technique

Nissen Fundoplication Technique

Nissen Fundoplication Technique

GERD - Surgical Treatment Results Procedure - 2 Hours Hospital - 1-2 Days Full Activity - 2 weeks Full Diet - 3 weeks Need to Open <1% Need for Blood <1% Off Medications - 95% Off Steroids - 50% Need 2nd Procedure - 5%

Effects of Fundoplication augments LES resting pressure lessens frequency of transient LES relaxations reestablishes anatomy of the LES and crura may improve esophageal clearance may improve gastric emptying Fundoplication resists reflux by: 1. augmenting LES resting pressure 2. lessening the frequency of transient LES relaxations and 3. reestablishing normal anatomic relationships between the LES and crural diaphragm. In addition, fundoplication may improve esophageal clearance and gastric emptying.

Case 2 A 61-year-old man presents with progressive difficulty swallowing. He has history of indigestion and heartburn. Until 12 months ago, food would come up into his throat when he was supine, with a sour taste and sometimes a cough. About 12 months ago, these symptoms improved but he developed progressive dysphagia. He smokes 1 PPD and drinks two beers at dinner. Exam is unremarkable except for barrel chest.

Case 2 What is the differential diagnosis? Anatomic Tumor, Stricture, Compression, Foreign Body Functional GERD, Motility Disorder (achalasia, scleroderma) Neurologic (Parkinson’s, bulbar paralysis) Psychological Globus Hystericus

Case 2 How would you evaluate this patient? Anatomic Assessment Functional Assessment Upper GI Series 24-hr pH EGD Esophageal Manometry Biopsy GES

Case 2 What are the treatment options for benign esophageal stricture? Medications Endoscopic Dilation Surgery

Case 2 What are the treatment options for carcinoma of the esophagus? Esophagogastectomy Ivor-Lewis Transhiatal

Barrett’s Esophagus Epidemiology Affects 10% of patients with severe GER 40-fold increased risk of cancer Patients require endoscopic surveillance Esophagectomy for severe dysplasia/cancer

Barrett’s Esophagus Endoscopic Appearance

Barrett’s Esophagus Pathologic Diagnosis Normal squamous epithelium transforms to intestinal-type (columnar) epithelium 40x increased cancer risk No increased cancer risk

PPI-Induced Regression? Peters FT, et al., Gut 1999;45:489-94.

Surgery-Induced Regression? 56 Barrett’s patients had antireflux surgery Annual flexible endoscopy 24 Barrett’s regressed 8 cm 4 cm 9 Barrett’s progressed 6 cm 10 cm 23 No change Sagar: Br J Surg 1995;82:806-10.

Barrett’s Esophagus Development of Cancer Based on Grade No dysplasia 3% Low-grade dysplasia 18% High-grade dysplasia 28% Morales and Sampliner, Arch Int Med 1999;159:1411-16.

Barrett’s Esophagus Following Patients Without Dysplasia Studies of cost-effectiveness are mixed Few cancers found during surveillance are node-positive, versus >50% otherwise Optimal surveillance interval debated, but data suggest q2-3 years

Barrett’s Esophagus Patients With Low-Grade Dysplasia Repeat endoscopy to avoid sampling error Surveillance q6 mo. x 1 year then q12 mo. May regress allowing increased interval

Barrett’s Esophagus Patients With High-Grade Dysplasia Must confirm the diagnosis Treatment is controversial Some advocate aggressive biopsy protocol Some advocate esophagectomy

Barrett’s Esophagus Patients With High-Grade Dysplasia Case for Aggressive Surveillance (q3-6 mos.): Regression may occur (25%) Most patients will not progress to cancer Cancers remain surgically curable Esophagectomy carries morbidity (up to 40%) and mortality (3-6%)

Barrett’s Esophagus Patients With High-Grade Dysplasia Case for Esophagectomy: 40% may already have cancer Surveillance delays definitive treatment Risk of esophagectomy low in high-volume centers

Barrett’s Esophagus Specific Treatment Ablative Techniques laser electrocautery photodynamic therapy (PDT) Resective Techniques Endoscopic mucosal resection (EMR)

Barrett’s Esophagus Take-Home Points Barrett’s esophagus is not a contraindication to antireflux operation Medical or surgical therapy does not eliminate need for Barrett’s surveillance Management of high-grade dysplasia is evolving away from esophagectomy

Case 3 A 53-year-old patient presents with a history of difficulty swallowing for years. More recently she is having increasing trouble swallowing, and has been regurgitating undigested food. Exam is unrevealing, but on chest film there is an air fluid level seen behind the heart in the mid chest.

Case 3 Describe a differential diagnosis and diagnostic evaluation.

Case 3 Discuss the management options for a patient with achalasia.

Achalasia Incidence 0.5 new cases / 100,000 population / year Dysphagia, regurgitation, cough, wheezing, aspiration, pulmonary infections 50% initially misdiagnosed

Achalasia Pathophysiology Involves degeneration of Auerbach’s plexus and elevated LES resting pressure Poor LES relaxation results in esophageal dilation with progressive loss of peristalsis

Achalasia Diagnosis Ba swallow: EGD: Esophageal manometry: esophageal dilation / narrowing at GE junction EGD: patulous esophagus, retained food, thickening Esophageal manometry: LES resting pressure LES relaxation on swallowing primary peristalsis

Achalasia Treatment Options Non-Surgical options: Nitrates and Ca++-channel blockers Endoscopic injection of Botox Pneumatic balloon dilatation Surgical options: Heller myotomy (laparoscopic, thoracoscopic) Meds: response is short-lived and associated with side effects. botulinum toxin provides two-thirds of patients short-term symptom relief (mean 1.3 years. Both Dilation and surgery have high initial success in overcoming dysphagia, although dilatation carries four times greater risk for esophageal perforation. Surgery provides more durable symptom relief according to retrospective (94 vs. 81 percent) and prospective (95 vs. 65 percent) studies of late outcomes. Most gastroenterologists attempt dilatation(s) initially, reserving surgical referral for treatment failures. Dissemination of minimally invasive methods may broaden the appeal of operative strategies for achalasia.

Heller Myotomy Technique

Heller Myotomy Technique

Heller Myotomy Technique

Heller Myotomy Outcomes 40 laparoscopic Heller myotomies No conversions, mean op time - 180 min Median hospital stay - 2 days One intraop mucosal injuriey repaired Dysphagia alleviated in > 95%

Case 3 Discuss the management of a patient with paraesophageal hernia.

Epidemiology Hiatal Hernias Herniation of the stomach through the esophageal hiatus Para-esophageal type - 5% Occurs in elderly patients (~ 65 years) Frequent co-morbid conditions

Classification Hiatal Hernias Classification depends on location of GEJ Type I- “sliding” hiatal hernia Type II- true paraesophageal hernia Type III- “mixed” hernia- sliding hernia and true paraesophageal hernia Type IV- intra-abdominal organ involvement

Sliding Hiatal Hernia Type I GE junction “slides” into the mediastinum Most HH May be associated with symptomatic GERD Surgery not indicated

True Paraesophageal Hernia Type II GEJ in the abdominal cavity, fundus in the mediastinum 5% of all HH Risk of incarceration and strangulation

Mixed Paraesophageal Hernia Type III GE junction and gastric fundus are located in mediastinum 5% of all HH Risk of incarceration and strangulation

Paraesophageal Hernia Volvulus Mesoaxial Volvulus Organoaxial Volvulus

Paraesophageal Hernia X-ray

Paraesophageal Hernia Upper GI series Type II Type III

Paraesophageal Hernia EGD

Paraesophageal Hernia Treatment Options Observation Medical Therapy Surgery

Paraesophageal Hernia Observation Assumes a low rate of gastric strangulation Allen et al. 23 of 147 patients followed for 12-268 mos (median 78 mos). Only 4 pts had progressive symptoms and 2 had elective repair Estimate prevalence of one gastric strangulation per 245 pts J Thorac Cardiovasc Surg 1993;105:253

Paraesophageal Hernia Medical Therapy One-third of patients have heartburn alone Acid inhibition Patient clearly informed of risk of gastric strangulation and consequences Excessive (10-50%) mortality for surgical repair of gastric strangulation

Paraesophageal Hernia Principles of Operative Repair Hernia Reduction Hernia sac excision Crural repair Gastric fixation Fundoplication controversial

Paraesophageal Hernia Hernia Reduction Entire stomach and at least 2 cm of esophagus must be intra-abdominal

Paraesophageal Hernia Sac Excision Entire sac must be excised to decrease risk of recurrence Remnants of sac along inferior border of left crus lead to recurrence

Paraesophageal Hernia Crural Repair Primary repair alone Primary repair with relaxing incision Mesh repair

Paraesophageal Hernia Fundoplication Recent series report high rate of GERD without fundoplication Wrap provides bulk to create “plug” at site of crural repair

Paraesophageal Hernia Surgical Outcomes Luketich et al.: 100 pts lap PH repair 12% intraop complications; technically demanding 3 conversions to open procedures 28% postop complication rate; 0% mortality 3% reoperation rate 91% satisfied, 2-day hospital stay Ann Surg 2000;232:608

Paraesophageal Hernia Take-Home Points Uncommon, rarely present with strangulation Repair advised for non-GER symptoms Repair is technically demanding Laparoscopic vs. open remains controversial Prospective study to determine recurrence

Case 4 A 47-year-old woman has chest pain after eating dinner at home 4 hours following upper GI endoscopy for dilatation of her achalasia. What is the presumed diagnosis?

Case 4 What is the best means of making the diagnosis?

Case 4 What is the appropriate management? Under what circumstances might you manage this non-operatively? What might be an appropriate management for a small perforation at the GE junction with minimal soiling?