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ESOPHAGUS Mary Hawn, M.D. Section of GI Surgery
<ul><li>Review Anatomy and Physiology </li></ul><ul><li>Diagnostic Evaluation of the Esophagus </li></ul><ul><li>Functiona...
<ul><li>Anatomically divided into three parts </li></ul><ul><ul><li>cervical </li></ul></ul><ul><ul><li>thoracic </li></ul...
 
Anatomy – Muscular tube <ul><li>25 – 30 cm long, C6 (level of cricoid cartilage) - T11 </li></ul><ul><li>Joins pharynx to ...
Esophagogastric Junction Definitions <ul><li>Junction of esophageal squamous and gastric columnar epithelium (ora serrata ...
Anatomy – Blood Supply <ul><li>Cervical – inferior thyroid arteries </li></ul><ul><li>Thoracic – 4-6 aortic esophageal art...
Venous Drainage <ul><li>Subepithelial channels </li></ul><ul><li>Periesophageal plexus </li></ul><ul><li>Cervical drainage...
Anatomy – Lymphatic Drainage <ul><li>Vessels run longitudinally, then penetrate wall to enter regional nodes </li></ul><ul...
Neck Esophagus Innervation <ul><li>Vagus – Superior Laryngeal – external and internal branches </li></ul><ul><li>External ...
Neck Esophagus Innervation <ul><li>Vagus – Recurrent Laryngeal Nerve (RLN) </li></ul><ul><li>RLN – (parasympathetic) cervi...
Thorax Esophagus Innervation <ul><li>Vagus – fibers to striated muscle and parasympathetic preganglionic fibers to smooth ...
Thoracic to Abdominal Innervation Transition <ul><li>Two major branches of the vagus lie on either side in the thorax and ...
Layers of Esophageal Wall  <ul><li>1. Mucosa </li></ul><ul><li>2. Submucosa </li></ul><ul><li>3. Muscularis </li></ul><ul>...
Muscle <ul><li>Outer longitudinal layer </li></ul><ul><li>Inner circular layer </li></ul><ul><li>Striated in upper portion...
Mucosa <ul><li>Squamous throughout </li></ul><ul><li>Distal 1-2 cm of lumen lined by columnar epithelium – squamocolumnar ...
Physiology <ul><li>Upper esophageal sphincter (UES) </li></ul><ul><li>Lower esophageal sphincter (LES) </li></ul><ul><li>H...
UES <ul><li>3 cm long zone of increased pressure at upper end of esophagus </li></ul><ul><li>Relaxes with swallowing – nor...
LES (functional sphincter) <ul><li>3-5 cm zone of increased pressure at lower end of esophagus </li></ul><ul><li>Relaxes w...
HPZ <ul><li>Classically believed to be equivalent to LES </li></ul><ul><li>Now felt to be determined by  esophageal enviro...
Swallowing <ul><li>Primary peristalsis – progressive, triggered by voluntary swallowing </li></ul><ul><li>Secondary perist...
Diagnostic Evaluation <ul><li>24 – hour pH monitoring </li></ul><ul><li>Manometry </li></ul><ul><li>Barium Swallow </li></...
24 – hour pH monitoring <ul><li>Objective Gold Standard test for GERD </li></ul><ul><li>Performed off acid suppression med...
24 – hour pH monitoring <ul><li>pH probe is placed 5 cm proximal to the LES </li></ul><ul><li>Number and duration of pH < ...
Manometry:  Indications <ul><li>Dysphagia after exclusion of a structural lesion </li></ul><ul><li>Chest pain of suspected...
Manometry <ul><li>Transnasal multichannel catheter measures pressure within esophagus </li></ul><ul><li>Characterized qual...
Normal Esophageal Motility
Barium Swallow <ul><li>Test of choice for initial evaluation of dysphagia </li></ul><ul><li>Differentiates between mechani...
Endoscopy <ul><li>Can be diagnostic and therapeutic </li></ul><ul><li>Grade esophagitis </li></ul><ul><li>Define anatomy <...
Esophageal Pathology <ul><li>GERD </li></ul><ul><li>Hiatal hernias </li></ul><ul><li>Diverticula </li></ul><ul><li>Motilit...
What is “reflux”? <ul><li>Symptoms of heartburn  </li></ul><ul><ul><li>episodic substernal chest pain </li></ul></ul><ul><...
Diagnosis <ul><li>24 hr pH monitoring  </li></ul><ul><ul><li>Solid state monitoring </li></ul></ul><ul><ul><li>BRAVO </li>...
Complications of reflux <ul><ul><li>Esophageal </li></ul></ul><ul><ul><ul><li>Esophagitis </li></ul></ul></ul><ul><ul><ul>...
Medical therapy <ul><li>Dietary changes </li></ul><ul><ul><li>Avoid caffeine, peppermint, chocolate </li></ul></ul><ul><li...
Surgery:  Indications <ul><ul><li>Refractory to Medical Theray </li></ul></ul><ul><ul><li>Co-existing hiatal hernia </li><...
Surgery:  Fundoplication <ul><ul><li>360 degree wrap of fundus around esophagus </li></ul></ul><ul><ul><li>Able to repair ...
 
Diverticula of the Esophagus <ul><li>Traction – True  </li></ul><ul><ul><li>Midesophageal / Parabronchial </li></ul></ul><...
Traction Diverticula <ul><li>Midesophageal / Parabronchial – conjunction with mediastinal granulomatous disease.  </li></u...
Pulsion Diverticula – Zenker’s <ul><li>Most common symptomatic diverticula </li></ul><ul><li>Almost all have GER which is ...
Epiphrenic <ul><li>Result of functional or mechanical obstruction </li></ul><ul><li>Location – distal 10 cm of thoracic es...
Functional Esophageal Disorders <ul><li>Motor disorders of skeletal muscle </li></ul><ul><li>Motor disorders of smooth mus...
Motor disorders of Skeletal Muscle <ul><li>Result in defective swallowing and aspiration </li></ul><ul><li>Causes  </li></...
Motor Disorders of Smooth Muscle <ul><li>Primary – Achalasia, Vigorous Achalasia, Diffuse Esophageal Spasm, Nutcracker Eso...
Achalasia <ul><li>Greek ‘absence of relaxation’ </li></ul><ul><li>Pathology </li></ul><ul><ul><li>degeneration of esophage...
Achalasia <ul><li>5/100,000; most common primary: ages 35-45 </li></ul><ul><li>Chagas’ disease:  Trypanosoma cruzi , mimic...
Achalasia:  Symptoms <ul><li>Dysphagia </li></ul><ul><li>Regurgitation </li></ul><ul><li>Weight Loss </li></ul><ul><li>Ass...
Achalasia:  Diagnosis <ul><li>Barium swallow </li></ul><ul><ul><li>dilated esophagus </li></ul></ul><ul><ul><li>classic bi...
Achalasia <ul><li>Diagnosis: </li></ul><ul><ul><li>chest x-ray  show fluid filled, dilated esophagus and absence of gastri...
Achalasia
Achalasia <ul><li>Treatment – decrease LES tone </li></ul><ul><ul><li>Medical: nitrates, CCB, Botox Injection (blocks acet...
Achalasia:  Medical Treatment <ul><li>Mechanism - smooth muscle relaxation </li></ul><ul><ul><li>Ca++ Channel blockers, ni...
Achalasia:  Endoscopic Therapy <ul><li>Pneumatic dilation </li></ul><ul><ul><li>Initial response 80-90% </li></ul></ul><ul...
Achalasia:  Endoscopic Therapy <ul><li>Botulinum Toxin Injection </li></ul><ul><ul><li>Binds presynaptic receptors and inh...
Achalasia:  Surgical Therapy <ul><li>Heller myotomy </li></ul><ul><ul><li>first performed in 1913 </li></ul></ul><ul><ul><...
Achalasia:  Surgical Therapy <ul><li>Laparoscopic Heller myotomy and Dor anterior fundoplication  </li></ul><ul><ul><li>my...
Achalasia:  Surgical Therapy Indications <ul><li>primary treatment for achalasia </li></ul><ul><li>secondary treatment fol...
Achalasia:  Surgical Therapy Results <ul><li>> 90% can be completed laparoscopically </li></ul><ul><li>90-95% successfully...
Achalasia:  Surgical Therapy Complications <ul><li>Esophageal perforation (1-5%) </li></ul><ul><ul><li>most recognized and...
Diffuse Esophageal Spasm <ul><li>Symptoms </li></ul><ul><ul><li>chest  pain  and dysphagia </li></ul></ul><ul><ul><li>aggr...
Diffuse Esophageal Spasm Motility Spectrum <ul><li>DES </li></ul><ul><ul><li>simultaneous repetitive contractions of incre...
Diffuse Esophageal Spasm Evaluation <ul><li>Diagnosis of exclusion </li></ul><ul><ul><li>r/o cardiac or other GI etiology ...
Diffuse Esophageal Spasm <ul><li>Loss of normal peristaltic coordination – simultaneous contractions of segments of the es...
Diffuse Esophageal Spasm Treatment <ul><li>Eliminate triggering factors </li></ul><ul><ul><li>avoid ingestion of cold liqu...
Diffuse Esophageal Spasm Medical Treatment <ul><li>Mechanism of Action: </li></ul><ul><ul><li>smooth muscle relaxation </l...
Nutcracker Esophagus <ul><li>Characterized manometrically by prolonged, high-amplitude peristaltic waves associated with c...
Scleroderma <ul><li>Esophageal manifestations in 60-80%, often earliest site </li></ul><ul><li>Atrophy of smooth muscle of...
Esophageal Stricture <ul><li>Benign – congenital or acquired </li></ul><ul><ul><li>Congenital webs </li></ul></ul><ul><ul>...
Congenital Webs <ul><li>Only true congenital esophageal strictures </li></ul><ul><li>Represent failure of canalization dur...
Acquired Strictures <ul><li>Rings or Webs – occur at any level </li></ul><ul><li>Schatzki’s ring – occur in lower esophagu...
Cricopharyngeal Dysfunction Symptoms <ul><ul><li>cervical dysphagia </li></ul></ul><ul><ul><li>globus </li></ul></ul><ul><...
Cricopharyngeal Dysfunction Etiology <ul><li>Neurogenic </li></ul><ul><ul><li>ALS, MS, Parkinsons, brain tumors </li></ul>...
Cricopharyngeal Dysfunction Evaluation <ul><li>Barium swallow </li></ul><ul><ul><li>may show hypertonic UES, and/or associ...
Cricopharyngeal Dysfunction Treatment <ul><li>Treat underlying associated medical conditions  i.e. GERD, neurologic </li><...
GERD-related Dysmotility <ul><li>Hypotensive distal peristaltic contractions </li></ul><ul><ul><li>amplitide <40mmHg </li>...
Transient LES relaxation <ul><li>Most common cause of GERD </li></ul><ul><li>Vago-vagal reflex initiated by gastric disten...
Transient LES relaxation:   Experimental  Treatment <ul><li>CCK atagonist - Loxiglumide </li></ul><ul><ul><li>decrease res...
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  1. 1. ESOPHAGUS Mary Hawn, M.D. Section of GI Surgery
  2. 2. <ul><li>Review Anatomy and Physiology </li></ul><ul><li>Diagnostic Evaluation of the Esophagus </li></ul><ul><li>Functional disorders of the Esophagus </li></ul><ul><ul><li>Motility </li></ul></ul><ul><ul><li>GERD </li></ul></ul>
  3. 3. <ul><li>Anatomically divided into three parts </li></ul><ul><ul><li>cervical </li></ul></ul><ul><ul><li>thoracic </li></ul></ul><ul><ul><li>abdominal </li></ul></ul><ul><li>Functionally divided into </li></ul><ul><ul><li>upper esophageal sphincter </li></ul></ul><ul><ul><li>esophageal body </li></ul></ul><ul><ul><li>lower esophageal sphincter </li></ul></ul>
  4. 5. Anatomy – Muscular tube <ul><li>25 – 30 cm long, C6 (level of cricoid cartilage) - T11 </li></ul><ul><li>Joins pharynx to stomach </li></ul><ul><li>Anterior to verterbral column & longus colli muscles; posterior to trachea; adjacent to descending aorta </li></ul><ul><li>Cervical, thoracic, and abdominal segments </li></ul><ul><li>Esophageal diaphragmatic hiatus – usually formed soley from right crus </li></ul><ul><li>Phrenoesophageal ligament </li></ul>
  5. 6. Esophagogastric Junction Definitions <ul><li>Junction of esophageal squamous and gastric columnar epithelium (ora serrata or Z line) – most clinically practical </li></ul><ul><li>Point at which tubular esophagus joins gastric pouch </li></ul><ul><li>Junction of esophageal circular muscle layer with oblique sling fibers of stomach (loop of Willis or collar of Helvetius) </li></ul>
  6. 7. Anatomy – Blood Supply <ul><li>Cervical – inferior thyroid arteries </li></ul><ul><li>Thoracic – 4-6 aortic esophageal arteries and branches of left bronchial arteries </li></ul><ul><li>Abdominal – left gastric artery and inferior phrenic artery </li></ul><ul><li>Rich interconnecting submucosal arterial plexus – runs longitudinally </li></ul>
  7. 8. Venous Drainage <ul><li>Subepithelial channels </li></ul><ul><li>Periesophageal plexus </li></ul><ul><li>Cervical drainage – inferior thyroid veins </li></ul><ul><li>Thoracic drainage – azygos/hemiazygos veins </li></ul><ul><li>Abdominal drainage – left gastric vein </li></ul>
  8. 9. Anatomy – Lymphatic Drainage <ul><li>Vessels run longitudinally, then penetrate wall to enter regional nodes </li></ul><ul><li>Cervical – supraclavicular </li></ul><ul><li>Thoracic – tracheal, tracheobronchial, posterior mediastinal, diaphragmatic </li></ul><ul><li>Abdominal – celiac axis </li></ul>
  9. 10. Neck Esophagus Innervation <ul><li>Vagus – Superior Laryngeal – external and internal branches </li></ul><ul><li>External – (motor) cricothyroid muscle and part of inferior pharyngeal constrictor </li></ul><ul><li>Internal – (sensory) pharyngeal surface of larynx and base of tongue </li></ul>
  10. 11. Neck Esophagus Innervation <ul><li>Vagus – Recurrent Laryngeal Nerve (RLN) </li></ul><ul><li>RLN – (parasympathetic) cervical esophagus and upper esophageal sphincter (UES) </li></ul><ul><li>RLN injury – hoarseness, UES dysfunction (secondary aspiration upon swallowing) </li></ul>
  11. 12. Thorax Esophagus Innervation <ul><li>Vagus – fibers to striated muscle and parasympathetic preganglionic fibers to smooth muscle </li></ul><ul><li>Sympathetic </li></ul><ul><ul><li>Cervical: from superior and inferior cervical sympathetic ganglia </li></ul></ul><ul><ul><li>Thorax: from upper thoracic and splanchnic nerves </li></ul></ul><ul><ul><li>Abdominal: from celiac ganglion </li></ul></ul>
  12. 13. Thoracic to Abdominal Innervation Transition <ul><li>Two major branches of the vagus lie on either side in the thorax and form two nerve plexuses supplying the esophagus and lungs </li></ul><ul><li>Esophageal vagus plexuses coalesce 2-6 cm above hiatus as single trunks (left and right vagus) </li></ul><ul><li>Left Vagus – anterior to esophagus at hiatus </li></ul><ul><li>Right Vagus – posterior to esophagus at hiatus </li></ul>
  13. 14. Layers of Esophageal Wall <ul><li>1. Mucosa </li></ul><ul><li>2. Submucosa </li></ul><ul><li>3. Muscularis </li></ul><ul><li>4. Adventitia </li></ul><ul><li>5. Striated muscle </li></ul><ul><li>6. Striated and smooth </li></ul><ul><li>7. Smooth muscle </li></ul><ul><li>8. Lamina muscularis mucosae </li></ul><ul><li>9. Esophageal glands </li></ul>
  14. 15. Muscle <ul><li>Outer longitudinal layer </li></ul><ul><li>Inner circular layer </li></ul><ul><li>Striated in upper portion </li></ul><ul><li>Smooth in lower portion </li></ul><ul><li>Auerbach’s plexus between inner and outer muscle layers </li></ul>
  15. 16. Mucosa <ul><li>Squamous throughout </li></ul><ul><li>Distal 1-2 cm of lumen lined by columnar epithelium – squamocolumnar junction lies within lower esophagus </li></ul>
  16. 17. Physiology <ul><li>Upper esophageal sphincter (UES) </li></ul><ul><li>Lower esophageal sphincter (LES) </li></ul><ul><li>High-pressure zone (HPZ) </li></ul>
  17. 18. UES <ul><li>3 cm long zone of increased pressure at upper end of esophagus </li></ul><ul><li>Relaxes with swallowing – normally remains closed (prevents swallowing of air with inspiration) </li></ul><ul><li>Contracts thereafter </li></ul><ul><li>Contraction of UES in sequence with pharynx above and esophagus below </li></ul><ul><li>Essentially same as criopharyngeus muscle </li></ul>
  18. 19. LES (functional sphincter) <ul><li>3-5 cm zone of increased pressure at lower end of esophagus </li></ul><ul><li>Relaxes with swallowing </li></ul><ul><li>Contracts thereafter in sequence with transmitted pressure increases – prevents reflux </li></ul><ul><li>Sphincter tone provided by intrinsic myogenic activity </li></ul><ul><li>Sphincter relaxation due to neural activity </li></ul>
  19. 20. HPZ <ul><li>Classically believed to be equivalent to LES </li></ul><ul><li>Now felt to be determined by esophageal environment ( Not entirely a result of active motor tone of LES; HPZ results primarily from exposure of segment of distal esophagus to intraabdominal positive pressure ) </li></ul>
  20. 21. Swallowing <ul><li>Primary peristalsis – progressive, triggered by voluntary swallowing </li></ul><ul><li>Secondary peristalsis – progressive, generated by distention or irritation usually from bolus not traversing through the esophagus </li></ul><ul><li>Tertiary peristalsis – nonprogressive (simultaneous) and uncoordinated, after voluntary or spontaneously between swallows – responsible for “corkscrew” appearance of spasm of Barium Swallow </li></ul>
  21. 22. Diagnostic Evaluation <ul><li>24 – hour pH monitoring </li></ul><ul><li>Manometry </li></ul><ul><li>Barium Swallow </li></ul><ul><li>Endoscopy </li></ul>
  22. 23. 24 – hour pH monitoring <ul><li>Objective Gold Standard test for GERD </li></ul><ul><li>Performed off acid suppression medication </li></ul><ul><li>Response to PPIs almost as sensitive as pH probe </li></ul><ul><li>May not be reflective of actual amount of reflux </li></ul>
  23. 24. 24 – hour pH monitoring <ul><li>pH probe is placed 5 cm proximal to the LES </li></ul><ul><li>Number and duration of pH < 4 events recorded for a 24 hour period </li></ul><ul><li>Patient keeps diary of meals and when recumbant </li></ul><ul><li>Composite score and overall % time pH less than 4 used to quantify reflux </li></ul>
  24. 25. Manometry: Indications <ul><li>Dysphagia after exclusion of a structural lesion </li></ul><ul><li>Chest pain of suspected esophageal origin </li></ul><ul><li>Localization of the LES in preparation for pH monitoring </li></ul><ul><li>Further characterization of motor dysfunction detected on barium radiography </li></ul><ul><li>Preoperative evaluation for antireflux surgery </li></ul><ul><li>Confirmation of effect of treatment on LES pressure (e.g., for achalasia) </li></ul>
  25. 26. Manometry <ul><li>Transnasal multichannel catheter measures pressure within esophagus </li></ul><ul><li>Characterized quality and strength of esophageal contractions </li></ul><ul><li>Patient performs ten wet swallows </li></ul><ul><li>Gold standard test for evaluation of motility disorders </li></ul>
  26. 27. Normal Esophageal Motility
  27. 28. Barium Swallow <ul><li>Test of choice for initial evaluation of dysphagia </li></ul><ul><li>Differentiates between mechanical and functional causes </li></ul><ul><li>Defines anatomy </li></ul>
  28. 29. Endoscopy <ul><li>Can be diagnostic and therapeutic </li></ul><ul><li>Grade esophagitis </li></ul><ul><li>Define anatomy </li></ul><ul><li>Tissue diagnosis </li></ul>
  29. 30. Esophageal Pathology <ul><li>GERD </li></ul><ul><li>Hiatal hernias </li></ul><ul><li>Diverticula </li></ul><ul><li>Motility disorders </li></ul>
  30. 31. What is “reflux”? <ul><li>Symptoms of heartburn </li></ul><ul><ul><li>episodic substernal chest pain </li></ul></ul><ul><ul><li>Worse after meals </li></ul></ul><ul><ul><li>Burning pain that radiates to the mouth </li></ul></ul><ul><ul><li>Associated with bitter taste in mouth </li></ul></ul><ul><ul><li>Effortless Regurgitation </li></ul></ul>
  31. 32. Diagnosis <ul><li>24 hr pH monitoring </li></ul><ul><ul><li>Solid state monitoring </li></ul></ul><ul><ul><li>BRAVO </li></ul></ul><ul><li>Barium study </li></ul><ul><li>Manometry </li></ul><ul><li>EGD </li></ul>
  32. 33. Complications of reflux <ul><ul><li>Esophageal </li></ul></ul><ul><ul><ul><li>Esophagitis </li></ul></ul></ul><ul><ul><ul><li>Stricture </li></ul></ul></ul><ul><ul><ul><li>Barrett’s esophagus </li></ul></ul></ul><ul><ul><ul><li>Adenocarcinoma of the esophagus </li></ul></ul></ul><ul><ul><li>Extra-esophageal </li></ul></ul><ul><ul><ul><li>Pulmonary fibrosis </li></ul></ul></ul><ul><ul><ul><li>Asthma </li></ul></ul></ul><ul><ul><ul><li>Cough </li></ul></ul></ul><ul><ul><ul><li>Hoarseness </li></ul></ul></ul><ul><ul><ul><li>Globus </li></ul></ul></ul>
  33. 34. Medical therapy <ul><li>Dietary changes </li></ul><ul><ul><li>Avoid caffeine, peppermint, chocolate </li></ul></ul><ul><li>Behavior modification </li></ul><ul><ul><li>Elevate HOB, don’t eat close to bedtime </li></ul></ul><ul><ul><li>***WEIGHT LOSS***** </li></ul></ul><ul><li>H2 Blocker </li></ul><ul><li>PPI </li></ul>
  34. 35. Surgery: Indications <ul><ul><li>Refractory to Medical Theray </li></ul></ul><ul><ul><li>Co-existing hiatal hernia </li></ul></ul><ul><ul><li>Complications related to reflux </li></ul></ul><ul><ul><ul><li>Stricture </li></ul></ul></ul><ul><ul><ul><li>Asthma </li></ul></ul></ul><ul><ul><ul><li>Cough </li></ul></ul></ul><ul><ul><ul><li>Laryngeal symptoms </li></ul></ul></ul>
  35. 36. Surgery: Fundoplication <ul><ul><li>360 degree wrap of fundus around esophagus </li></ul></ul><ul><ul><li>Able to repair co-existing hiatal hernia </li></ul></ul><ul><ul><li>Prevents reflux – doesn’t just neutralize the acid </li></ul></ul><ul><ul><li>> 90% effective in controlling reflux </li></ul></ul><ul><ul><li>Side effects: dysphagia and bloating </li></ul></ul>
  36. 38. Diverticula of the Esophagus <ul><li>Traction – True </li></ul><ul><ul><li>Midesophageal / Parabronchial </li></ul></ul><ul><li>Pulsion – False </li></ul><ul><ul><li>1) Pharyngoesophageal (Zenker’s) </li></ul></ul><ul><ul><li>2) Epiphrenic </li></ul></ul>
  37. 39. Traction Diverticula <ul><li>Midesophageal / Parabronchial – conjunction with mediastinal granulomatous disease. </li></ul><ul><li>Symptoms – (rare) chronic cough, usually due to chronic fistula </li></ul><ul><li>Treatment – operative excision of diverticula with adjacent inflammatory mass </li></ul>
  38. 40. Pulsion Diverticula – Zenker’s <ul><li>Most common symptomatic diverticula </li></ul><ul><li>Almost all have GER which is thought to produce the cricopharyngeal dysfunction </li></ul><ul><li>Symptoms – </li></ul><ul><ul><li>progressive cervical dysphagia </li></ul></ul><ul><ul><li>cough with recumbent positioning </li></ul></ul><ul><ul><li>choking and aspiration </li></ul></ul><ul><li>Diagnosis – Barium Swallow </li></ul><ul><li>Treatment – Cricopharyngeal myotomy and diverticulectomy or suspension </li></ul>
  39. 41. Epiphrenic <ul><li>Result of functional or mechanical obstruction </li></ul><ul><li>Location – distal 10 cm of thoracic esophagus </li></ul><ul><li>Symptoms – many asymptomatic at diagnosis; due to diverticula or underlying disorder </li></ul><ul><li>Diagnosis – Contrast Esophagogram (endoscopy and function studies to identify underlying pathophysiology) </li></ul><ul><li>Treatment </li></ul><ul><ul><li>Diverticulectomy or diverticulopexy </li></ul></ul><ul><ul><li>any mechanical obstruction must be corrected </li></ul></ul>
  40. 42. Functional Esophageal Disorders <ul><li>Motor disorders of skeletal muscle </li></ul><ul><li>Motor disorders of smooth muscle </li></ul>
  41. 43. Motor disorders of Skeletal Muscle <ul><li>Result in defective swallowing and aspiration </li></ul><ul><li>Causes </li></ul><ul><ul><li>Neurogenic </li></ul></ul><ul><ul><li>Myogenic </li></ul></ul><ul><ul><li>Structural </li></ul></ul><ul><ul><li>Iatrogenic </li></ul></ul><ul><ul><li>Mechanical </li></ul></ul><ul><ul><li>Most are not surgically correctable </li></ul></ul>
  42. 44. Motor Disorders of Smooth Muscle <ul><li>Primary – Achalasia, Vigorous Achalasia, Diffuse Esophageal Spasm, Nutcracker Esophagus </li></ul><ul><li>Secondary – response to inflammation or systemic disorders that produce fibrosis which impairs peristalsis (Progressive systemic sclerosis, Complications) </li></ul>
  43. 45. Achalasia <ul><li>Greek ‘absence of relaxation’ </li></ul><ul><li>Pathology </li></ul><ul><ul><li>degeneration of esophageal myenteric plexus </li></ul></ul><ul><li>Characterized by </li></ul><ul><ul><li>aperistalsis of esophagus </li></ul></ul><ul><ul><li>hypertensive LES with impaired relaxation </li></ul></ul><ul><li>Etiology unknown </li></ul><ul><ul><li>viral </li></ul></ul><ul><ul><li>autoimmune </li></ul></ul>
  44. 46. Achalasia <ul><li>5/100,000; most common primary: ages 35-45 </li></ul><ul><li>Chagas’ disease: Trypanosoma cruzi , mimics, primarily in South America </li></ul><ul><li>Etiology unknown (deficiency/changes in ganglia of Auerbach’s plexus); loss of effective esophageal body peristalsis and failure of LES relaxation (hence elevated LES pressure) </li></ul><ul><li>Symptoms – progressive dysphagia, regurgitation immediately after meals, odynophagia, aspiration with bronchitis/pneumonia, chest pain (due to spasm) </li></ul>
  45. 47. Achalasia: Symptoms <ul><li>Dysphagia </li></ul><ul><li>Regurgitation </li></ul><ul><li>Weight Loss </li></ul><ul><li>Associated respiratory symptoms secondary to recurrent aspiration and mechanical compression </li></ul>
  46. 48. Achalasia: Diagnosis <ul><li>Barium swallow </li></ul><ul><ul><li>dilated esophagus </li></ul></ul><ul><ul><li>classic bird-beak appearance of distal esophagus </li></ul></ul><ul><li>Esophageal motility and manometry </li></ul><ul><ul><li>absent peristalsis and hypertensive LES which fails to relax with deglutation </li></ul></ul><ul><li>EGD </li></ul><ul><ul><li>Must r/o cancer, stricture </li></ul></ul>
  47. 49. Achalasia <ul><li>Diagnosis: </li></ul><ul><ul><li>chest x-ray show fluid filled, dilated esophagus and absence of gastric air bubble. </li></ul></ul><ul><ul><li>Barium esophagogram shows “birds beak” and proximal dilatation </li></ul></ul><ul><ul><li>Manometry is definitive diagnosis (absence of peristalsis, dysfunction in LES relaxation </li></ul></ul><ul><ul><li>Endoscopy to rule out pseudoachalasia (malignancy or benign stricture) </li></ul></ul>
  48. 50. Achalasia
  49. 51. Achalasia <ul><li>Treatment – decrease LES tone </li></ul><ul><ul><li>Medical: nitrates, CCB, Botox Injection (blocks acetylcholine release from nerve terminals) </li></ul></ul><ul><ul><li>Surgery: </li></ul></ul><ul><ul><ul><li>Heller Esophagomyotomy </li></ul></ul></ul><ul><ul><ul><ul><li>95% v. 65% using pneumatic bougienage – Gut 30:299, 1989 </li></ul></ul></ul></ul><ul><ul><ul><li>concomitant antireflux procedure to reduce postoperative reflux </li></ul></ul></ul>
  50. 52. Achalasia: Medical Treatment <ul><li>Mechanism - smooth muscle relaxation </li></ul><ul><ul><li>Ca++ Channel blockers, nitrates </li></ul></ul><ul><li>Effectiveness </li></ul><ul><ul><li>nitrates produce transient responses in ~50% of patients, but results are not sustained </li></ul></ul><ul><ul><li>Ca++ channel blockers no more effective than placebo </li></ul></ul>
  51. 53. Achalasia: Endoscopic Therapy <ul><li>Pneumatic dilation </li></ul><ul><ul><li>Initial response 80-90% </li></ul></ul><ul><ul><li>Recurrence rate 40-80% depending on length of follow-up </li></ul></ul><ul><ul><li>Complications include perforation (4%), prolonged chest pain (15%), GERD (15%) </li></ul></ul><ul><ul><li>? Increase subsequent surgical risk </li></ul></ul>
  52. 54. Achalasia: Endoscopic Therapy <ul><li>Botulinum Toxin Injection </li></ul><ul><ul><li>Binds presynaptic receptors and inhibits Ach release </li></ul></ul><ul><ul><li>90% immediate response </li></ul></ul><ul><ul><li><50% sustained response at 6 months </li></ul></ul><ul><ul><li>? Increase surgical complication rate </li></ul></ul>
  53. 55. Achalasia: Surgical Therapy <ul><li>Heller myotomy </li></ul><ul><ul><li>first performed in 1913 </li></ul></ul><ul><ul><li>performed both anterior and posterior myotomies </li></ul></ul><ul><li>Approach has evolved over time </li></ul><ul><ul><li>transabdominal and transthoracic approaches </li></ul></ul><ul><ul><li>only single myotomy made </li></ul></ul><ul><ul><li>+/- the addition of an antireflux procedure </li></ul></ul>
  54. 56. Achalasia: Surgical Therapy <ul><li>Laparoscopic Heller myotomy and Dor anterior fundoplication </li></ul><ul><ul><li>mytomy extends 7cm onto anterior esophagus and 1.5 cm onto stomach </li></ul></ul><ul><ul><li>performed over 40-44 Fr bougie </li></ul></ul><ul><ul><li>anterior (Dor) fundoplication performed without division of short gastric vessels </li></ul></ul>
  55. 57. Achalasia: Surgical Therapy Indications <ul><li>primary treatment for achalasia </li></ul><ul><li>secondary treatment following failed pneumatic dilatation or botox treatment </li></ul>
  56. 58. Achalasia: Surgical Therapy Results <ul><li>> 90% can be completed laparoscopically </li></ul><ul><li>90-95% successfully treated with myotomy </li></ul><ul><li>Early failures(5-10%) </li></ul><ul><ul><li>Technical </li></ul></ul><ul><ul><ul><li>inadequate myotomy </li></ul></ul></ul><ul><ul><ul><li>improper formation of fundoplication </li></ul></ul></ul><ul><ul><li>Disease related </li></ul></ul><ul><ul><ul><li>transmural scarring </li></ul></ul></ul><ul><li>Late failures (1%) </li></ul><ul><ul><li>peptic stricture, reapproximation of myotomy </li></ul></ul>
  57. 59. Achalasia: Surgical Therapy Complications <ul><li>Esophageal perforation (1-5%) </li></ul><ul><ul><li>most recognized and repaired intra-operatively </li></ul></ul><ul><li>Reflux (3-50%) </li></ul><ul><ul><li>most patients asymptomatic </li></ul></ul><ul><ul><li>partial fundoplication prevents reflux </li></ul></ul>
  58. 60. Diffuse Esophageal Spasm <ul><li>Symptoms </li></ul><ul><ul><li>chest pain and dysphagia </li></ul></ul><ul><ul><li>aggravated by stress, ingestion of cold food/liquid, GERD </li></ul></ul><ul><ul><li>often associated with history of other spastic GI conditions </li></ul></ul><ul><li>Etiology </li></ul><ul><ul><li>unknown, no underlying pathologic abnormality </li></ul></ul>
  59. 61. Diffuse Esophageal Spasm Motility Spectrum <ul><li>DES </li></ul><ul><ul><li>simultaneous repetitive contractions of increased duration </li></ul></ul><ul><li>Nutcracker Esophagus </li></ul><ul><ul><li>hypertensive contractions (amplitude >180 mmHg) and increased duration </li></ul></ul><ul><li>Vigorous Achalasia </li></ul><ul><ul><li>simultaneous repetitive contractions with abnormal LES relaxation </li></ul></ul>
  60. 62. Diffuse Esophageal Spasm Evaluation <ul><li>Diagnosis of exclusion </li></ul><ul><ul><li>r/o cardiac or other GI etiology </li></ul></ul><ul><li>Barium swallow </li></ul><ul><ul><li>corkscrew appearance in extreme cases </li></ul></ul><ul><ul><li>r/o distal obstructing lesions as etiology </li></ul></ul><ul><li>Motility </li></ul><ul><ul><li>simultaneous hypertensive deglutive contractions </li></ul></ul>
  61. 63. Diffuse Esophageal Spasm <ul><li>Loss of normal peristaltic coordination – simultaneous contractions of segments of the esophageal body </li></ul><ul><li>Symptoms – severe spastic pain, occurs spontaneously at night; dysphagia; regurgitation; weight loss </li></ul><ul><li>Diagnosis – esophageal manometry (Spontaneous activity, repetitive waves, prolonged, high-amplitude contractions. Broad, multipeaked contrations); IV Bethanechol (parasympathomimetic) can provoke pain and contractions </li></ul><ul><li>Treatment – CCB, nitrates to reduce amplitude but ultimately surgery (long esophagomytomy from stomach to aortic arch with an antireflux procedure) </li></ul>
  62. 64. Diffuse Esophageal Spasm Treatment <ul><li>Eliminate triggering factors </li></ul><ul><ul><li>avoid ingestion of cold liquids </li></ul></ul><ul><ul><li>treat underlying GERD </li></ul></ul><ul><li>Psychosocial counseling </li></ul><ul><ul><li>address underlying stress </li></ul></ul>
  63. 65. Diffuse Esophageal Spasm Medical Treatment <ul><li>Mechanism of Action: </li></ul><ul><ul><li>smooth muscle relaxation </li></ul></ul><ul><li>Effectiveness: </li></ul><ul><ul><li>nifedipine and nitates have been used with minimal success </li></ul></ul><ul><li>Experimental: </li></ul><ul><ul><li>nitric oxid donors (L-arginine) control non-cardiac chest pain </li></ul></ul>
  64. 66. Nutcracker Esophagus <ul><li>Characterized manometrically by prolonged, high-amplitude peristaltic waves associated with chest pain </li></ul><ul><li>Treatment – CCB and long acting nitrates; Esophagomyotomy is of uncertain benefit </li></ul>
  65. 67. Scleroderma <ul><li>Esophageal manifestations in 60-80%, often earliest site </li></ul><ul><li>Atrophy of smooth muscle of distal esophagus, deposition of collagen in connective tissue, and subintimal arteriolar fibrosis. Normal contractions are present in the striated muscle of the proximal esophagus </li></ul>
  66. 68. Esophageal Stricture <ul><li>Benign – congenital or acquired </li></ul><ul><ul><li>Congenital webs </li></ul></ul><ul><ul><li>Acquired Strictures </li></ul></ul><ul><li>Malignant </li></ul>
  67. 69. Congenital Webs <ul><li>Only true congenital esophageal strictures </li></ul><ul><li>Represent failure of canalization during development – occur at any level </li></ul><ul><li>Imperforate web must be distinguished from a TEF </li></ul><ul><li>Perforate web may not be symptomatic until feedings become solid </li></ul>
  68. 70. Acquired Strictures <ul><li>Rings or Webs – occur at any level </li></ul><ul><li>Schatzki’s ring – occur in lower esophagus at Z line, hiatal hernia always present, etiology is presumed GER, esophagitis rarely present </li></ul><ul><li>Symptoms – progressive dysphagia to solid food, begin when lumen <12 mm </li></ul><ul><li>Diagnosis – Barium Swallow, Esophagoscopy, biopsy to exclude malignancy </li></ul><ul><li>Treatment – Medical management of GER with periodic dilatation for dysphagia. </li></ul>
  69. 71. Cricopharyngeal Dysfunction Symptoms <ul><ul><li>cervical dysphagia </li></ul></ul><ul><ul><li>globus </li></ul></ul><ul><ul><li>horseness </li></ul></ul><ul><ul><li>weight loss </li></ul></ul><ul><ul><li>halitosis (associated Zenkers) </li></ul></ul>
  70. 72. Cricopharyngeal Dysfunction Etiology <ul><li>Neurogenic </li></ul><ul><ul><li>ALS, MS, Parkinsons, brain tumors </li></ul></ul><ul><li>Myogenic </li></ul><ul><ul><li>Myasthenia gravis, muscular dystrophy </li></ul></ul><ul><li>Mechanical </li></ul><ul><ul><li>neoplasia, webs, thyromegaly, osteophytes </li></ul></ul><ul><li>Iatrogenic </li></ul><ul><li>GERD </li></ul>
  71. 73. Cricopharyngeal Dysfunction Evaluation <ul><li>Barium swallow </li></ul><ul><ul><li>may show hypertonic UES, and/or associated Zenkers (pulsion) diverticulum </li></ul></ul><ul><ul><li>R/O cancer </li></ul></ul><ul><li>Motility studies </li></ul><ul><ul><li>R/O diffuse esophageal dysmotility </li></ul></ul><ul><li>pH probe </li></ul>
  72. 74. Cricopharyngeal Dysfunction Treatment <ul><li>Treat underlying associated medical conditions i.e. GERD, neurologic </li></ul><ul><li>Dilatation of UES via bougianage </li></ul><ul><li>Hypertonic UES with/out associated Zenkers diverticulum </li></ul><ul><ul><li>Cervical myotomy +/-diverticulectomy or pex </li></ul></ul><ul><ul><li>endoscopic diverticulostomy </li></ul></ul>
  73. 75. GERD-related Dysmotility <ul><li>Hypotensive distal peristaltic contractions </li></ul><ul><ul><li>amplitide <40mmHg </li></ul></ul><ul><ul><li>present in 25% patients with mild esophagitis, 50% of patients with severe esophagitis </li></ul></ul><ul><li>Treatment </li></ul><ul><ul><li>r/o distal esophageal stricture </li></ul></ul><ul><ul><li>medical: treat esophagitis </li></ul></ul><ul><ul><li>surgical: no increased dysphagia from 360 degree wrap compared to partial wrap </li></ul></ul>
  74. 76. Transient LES relaxation <ul><li>Most common cause of GERD </li></ul><ul><li>Vago-vagal reflex initiated by gastric distension </li></ul>Gastric distension Vagal afferents Vagal efferents Brainstem nuclei Relax LES
  75. 77. Transient LES relaxation: Experimental Treatment <ul><li>CCK atagonist - Loxiglumide </li></ul><ul><ul><li>decrease response to gastric distension </li></ul></ul><ul><li>NO synthase inhibitors </li></ul><ul><ul><li>decrease response to gastric distension </li></ul></ul><ul><ul><li>also may increase distal esophageal peristaltic amplitude </li></ul></ul><ul><li>GABA-receptor agonist - Baclofen </li></ul><ul><ul><li>decrease tLESr mediated via GABA receptors </li></ul></ul>

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