Anatomy and Motility Disorders
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1 Anatomy and Motility Disorders Normal anatomy and physiology Abnormal anatomy: Congenital Acquired Manometry and motility disorders Miscellaneous Eosinophilic esophagitis Pill esophagitis Steven Shay MD CLASSIC DIVISIONS AND ADJACENT STRUCTURES Distance from incisors Normal narrowings Lipham et al. In: Surgery of the Chest. Sabiston, 2010, p517. 1
2 Hypopharynx-UES- Proximal Esophagus (Posterior view) EG JUNCTION ESOPHAGEAL MUSCULATURE AND RELEVANCE TO DISORDERS From Mashimo H et al. GI Motility on Line
3 Lymphatics in Submucosa: Importance in Esophageal Cancer 2009 Note: 1) Small lymphatics extend to just below epithelium 2) No serosa SMOOTH MUSCLE IN NORMAL PERISTALSIS A B Stimulus Stimulus A. Latency to contraction increases down esophagus after initial panesophageal relaxation B. Cholinergic excitatory vs noncholinteric inhibitory Goyal et al. In: The Esophagus, Ed 4. Castell, 2004, p17. 3
4 Barium Bolus Progression: Simultaneous Manometry and Flouroscopy Note: 1) Leading edge of barium bolus thrust to mid/distal esophagus by oropharynx 2) Barium clears from each site before clearing contraction begins Kahrilas et al. Gastroenterology 1988; 94:. Deglutative Inhibition Note: Latency persists with multiple swallows until they end; then, the contraction can propagate thru the esophagus Modified from Goyal et al. In: The Esophagus, Ed 4. Castell, 2004, p13. 4
5 DYSPHAGIA LUSORIA Normal great vessel anatomy Abnormal right subclavian take off Levitt B, Richter JE. Dis Esoph 2007; 20: Note: Oblique ascending extrinsic compression of esophagus trapped between trachea and right subclavian DUPLICATION CYST Note: Extrinsic compression of the esophagus. EUS shows this to be a cyst with hyperechoic proteinaceous material (white arrow) and fluid (black area) adjacent to esophagus. 5
6 Sliding Type 1 Hiatal hernia types Paraesophageal Type 2 Hiatal hernia types TYPE 3: MIXED TYPE 4: Hiatal hernia + Other Viscera 6
7 EPIPHRENIC DIVERTICULUM EPIPHRENIC DIVERTICULUM Endoscopic view of EG junction LES opening 7
8 STEP 1: RESECT DIVERTICULUM STEP 3: PARTIAL FUNDOPLICATION STEP 2: HELLER MYOTOMY 8
9 What is true regarding Zenkers diverticulum? a. It protrudes thru weakness in the muscle layer at Killians triangle. b. Measuring UES pressure at manometry is very valuable in management? c. Cricopharyngeal myotomy and diverticulectomy is standard surgical therapy? d. Aspiration pneumonia is not a complication. 1. a only 2. a, b, c only 3. a, c only 4. All are true Hypopharynx-UES- Proximal Esophagus (Posterior view) 9
10 CRICOPHARYNGEAL BAR ( UES SPASM) Early Zenker s BAR ZENKER S DIVERTICULUM Body 10
11 Zenker s diverticulum Large, mixed hiatal hernia Note: Same patient with large mixed hiatal hernia below the Zenker s diverticulum SURGERY FOR ZENKERS DIVERTICULUM: Myotomy and Diverticulectomy Note: Surgery done via neck incision 11
12 ENDOSCOPIC CRICOPHARYNGEAL MYOTOMY Modified from GI Motilility on line; May 2006 What is true regarding achalasia? a. Poor LES relaxation and aperistalsis are characteristic findings at esophageal manometry? b. Endoscopy is not necessary once the diagnosis is made by barium swallow and esophageal manometry. c. Pneumatic dilation gives much poorer results in intial therapy compared to Heller myotomy / partial fundoplication. d. Aspiration pneumonia is not a complication. 1. a only 2. a, b, c only 3. b, d only 4. d only 5. All are true 12
13 20 cm 15 cm 10 cm 5 cm LES Traditional manometry Time (seconds) High Resolution manometry Time (seconds) Rice T, Shay S. A primer of high-resolution esophageal manometry. Sem Thor and Cardiovas Surg 2011; Pressure scale (mmhg) cm Pressure Distance (mmhg)(cm) Time (seconds) Anatomic Segment Hypopharynx UESp falls to 0 with swallow Striated muscle contraction Rice T, Shay S. Sem Thor and Cardiovas Surg 2011; Smooth muscle Contraction Peristalsis parameters Antegrade Amplitude 65 mmhg 65 mmhg 3 cm/s 70 mmhg Basal Relax Overshoot 15 mmhg 0 mmhg 35 mmhg Bolus pressure 5 mmhg LES pressure Intragastric 13
14 Achalasia ESOPHAGEAL MOTILITY DISORDERS Diffuse esophageal spasm Hypotensive peristalsis (e.g. scleroderma) Hypertensive peristalsis ( nutcracker ) Modified from Table Kahrilas, P et al. GI and Liver Disease. Schlesinger and Fordtran, 2010; 42: 699. ESOPHAGEAL MOTILITY DISORDERS Impaired LES (EGJ) relaxation (>15 mmhg) Achalasia, 3 types: Absent peristalsis every swallow Functional EGJ obstruction (eg stricture): Some normal peristalsis Normal LES (EGJ) relaxation Esophageal spasm: >20% simultaneous contraction >30 mmhg No contraction every swallow (eg, scleroderma) Hypotensive peristalsis every swallow (eg, GERD) Hypertensive peristalsis Modified from Table Kahrilas, P et al. GI and Liver Disease. Schlesinger and Fordtran, 2010; 42:
15 Achalasia - Simultaneous contraction, - LES high, poor relaxation Pressure scale (mmhg) Normal Rice T, Shay S. A primer of high-resolution esophageal manometry. Sem Thor and Cardiovas Surg 2011; Type I Three Types of Achalasia Type 2 Type 3 Type 1 Type 2 Type 3 Pressure Scale Pharynx Pressure Scale Pharynx 100 Pressure Scale 100 UES UES 13-cm 13-cm cm 8-cm cm 3-cm 100 LES LES 100 Gastric Gastric LES >30 mmhg thruout Esophageal pressurization < mmhg thruout esophagus LES > mmhg thruout CCF 2009 Esophageal pressurization >40 mmhg thruout esophagus Vigorous achalasia Type 2: Best response to surgery 15
16 ADVANCED ACHALASIA Fig Kahrilas P et al. GI and Liver Disease. Schlesinger and Fordtran; 42: 694, Achalasia Primary Secondary Bird beak appearance From Levine MS et al. Clin Gastroenterol Hepatol 2008;6:11-. Mass effect on EGJ and cardia from cancer 16
17 Achalasia Treatment Pneumatic dilation Initial symptom relief in ~ 90% 2-4% perforation rate Open thoracotomy Laparoscopic Heller-Dor Initial symptom relief in ~ 90% Risks General anesthesia Surgery GERD ± stricture Note: Target of treatment is palliation by reducing LES pressure since there is no therapy to reverse underlying neuropathology. Which of the following are true? a. The diagnosis of DES by manometry requires both normal peristalsis after some swallows and simultaneous contractions after > 20%. b. Possible treatments for DES include smooth muscle relaxants and botox injection of the spastic segment of the esophagus. c. Scleroderma affects distal esophageal muscle but proximal esophageal muscle is preserved. d. Nutcracker esophagus causes a severe delay in bolus transit. 1. a only 2. a, b, c only 3. b, d only 4. d only 5. All are true 17
18 ESOPHAGEAL MOTILITY DISORDERS Impaired LES (EGJ) relaxation (>15 mmhg) Achalasia, 3 types: Absent peristalsis every swallow Functional EGJ obstruction (eg stricture, tight fundoplication): Some normal peristalsis Normal LES (EGJ) relaxation Esophageal spasm: >20% simultaneous contraction >30 mmhg No contraction every swallow (eg, scleroderma) Hypotensive peristalsis every swallow (eg, GERD) Hypertensive peristalsis Modified from Table Kahrilas, P et al. GI and Liver Disease. Schlesinger and Fordtran 2010; 42: 699. Functional Obstruction EG Junction A A B B A Very high Intrabolus pressure: mean 55 mmhg (nl < 15) B Increased contraction pressure: 0 mmhg; nl <180 Rice T, Shay S. A primer of high-resolution esophageal manometry. Sem Thor and Cardiovas Surg 2011;
19 ESOPHAGEAL MOTILITY DISORDERS Impaired LES (EGJ) relaxation (>15 mmhg) Achalasia, 3 types: Absent peristalsis every swallow Functional EGJ obstruction (eg stricture): Some normal peristalsis Normal LES (EGJ) relaxation Esophageal spasm: >20% simultaneous contraction >30 mmhg No contraction every swallow (eg, scleroderma) Hypotensive peristalsis every swallow (eg, GERD) Hypertensive peristalsis Modified from Table Kahrilas, P et al. GI and Liver Disease. Schlesinger and Fordtran, 2010; 42: 699. DIFFUSE ESOPHAGEAL SPASM Normal peristalsis > 30 mmhg Simultaneous >30 mmhg Rice T, Shay S. A primer of high-resolution esophageal manometry. Sem Thor and Cardiovas Surg 2011;
20 Diffuse Esophageal Spasm From Levine MS et al. Clin Gastroenterol Hepatol 2008;6: DIFFUSE ESOPHAGEAL SPASM: Therapy Smooth muscle relaxants Calcium channel blockers Sublingual nitroglycerin Sildenafil Botox injection of spastic segment Esophageal dilation 20
21 ESOPHAGEAL MOTILITY DISORDERS Impaired LES (EGJ) relaxation (>15 mmhg) Achalasia, 3 types: Absent peristalsis every swallow Functional EGJ obstruction (eg stricture): Some normal peristalsis Normal LES (EGJ) relaxation Esophageal spasm: >20% simultaneous contraction >30 mmhg No contraction every swallow (eg, scleroderma) Hypotensive peristalsis every swallow (eg, GERD) Hypertensive peristalsis Modified from Table Kahrilas, P et al. GI and Liver Disease. Schlesinger and Fordtran, 2010; 42: 699. A NUTCRACKER B SCLERODERMA Pressure Scale Pharynx Pressure Scale Pharynx 300 UES UES cm cm cm cm cm cm 300 LES LES 300 Gastric Gastric Antegrade esophageal contraction Increased contraction amplitude CCF 2009 No LES pressure No contraction smooth muscle 21
22 ESOPHAGEAL MOTILITY DISORDERS Impaired LES (EGJ) relaxation (>15 mmhg) Achalasia, 3 types: Absent peristalsis every swallow Functional EGJ obstruction (eg stricture): Some normal peristalsis Normal LES (EGJ) relaxation Esophageal spasm: >20% simultaneous contraction >30 mmhg No contraction every swallow (eg, scleroderma) Hypotensive peristalsis every swallow (eg, GERD) Hypertensive peristalsis Modified from Table Kahrilas, P et al. GI and Liver Disease. Schlesinger and Fordtran, 2010; 42: 699. Hypotensive Failed Note: Large defects in 30-mmHg isobar (i.e., waveform outlined by 30 mmhg line) Note: No waveform with 30-mmHg isobar Rice T, Shay S. A primer of high-resolution esophageal manometry. Sem Thor and Cardiovas Surg 2011;
23 MISCELLANEOUS TOPICS Eosinophilic esophagitis (EoE) Pill esophagitis EOSINOPHILIC ESOPHAGITIS: DEFINITION EoE represents a chronic, immune / antigen mediated, esophageal disease characterized clinically by symptoms of esophageal dysfunction and histologically by eosinophilpredominant inflammation Liacouras C et al, J Allergy Clinc Immunol
24 EOSINOPHILIC ESOPHAGITIS: HISTOLOGY Note: at least one biopsy must have > 15 eos in hpf Furuta G, et al. Gastroenterology 2007;133: EOSINOPHILIC ESOPHAGITIS SYMPTOMS Chest pain Dysphagia Food impaction CLUES FH esophageal dilations or recalcitrant GERD Pretreatment may mask EoE (i.e., topical steroids for other atopic diseases) Straumann et al, Allergy
25 Eosinophilic Esophagitis: Endoscopy EGD normal in 10% Gonsalves N et al. Gastrointest Endosc 2006;64: EOSINOPHILIC ESOPHAGITIS: TREATMENT Traditional initial therapy PPI s Topical fluticasone / viscous budesonide Other therapy that may be necessary Esophageal dilation (slight increased risk of perforation, though rare) Diet therapy Adult: 6-food elimination diet; Child:elemental After food allergy testing? Systemic steroids
26 EOSINOPHILIC ESOPHAPGITIS 6-Food Elimination Diet Nuts Fish/shellfish Wheat Eggs Dairy Soy EOSINOPHILIC ESOPHAGITIS: TREATMENT Traditional initial therapy PPI s Topical fluticasone / viscous budesonide Other therapy that may be necessary Esophageal dilation (slight increased risk of perforation, though rare) Diet therapy Adult: 6-food elimination diet; Child:elemental After food allergy testing? Systemic steroids 26
27 Which of the following pills can occasionally cause deep esophageal ulcers and/or strictures? a. Potassium supplements b. Foxamax c. NSAID s d. Tetracycline 1. a only 2. a, b, c only 3. b, d only 4. d only 5. All are true Pill Esophagitis Clinical Presentation Odynophagia Chest pain Dysphagia 27
28 PILL ESOPHAGITIS (% of 1088 cases in largest publication to date) Severe damage ocasionally (deep ulcer/stricture) Potassium supplements (n=33) Quinidine (n=13) Fosomax (All biphosphonates; n=127) Non-steroidal anti-inflammatory (n=121) Ferrous sulfate (sustained release form; n=24) Mild (superficial ulcers) Tetracycline (n=437) Kikendall JW. Diseases of the Esophagus
Figure 3: Dysphagia. 14 meets. esophageal. esophageal manometry +/- +/- impedance measurement. structural lesion? no. 19 yes
Figure 3: Dysphagia 1 patient with dysphagia 2 history and physical exam. suggestive of nesophageal etiology? 3 evaluate and treat as as indicated 4 upper GI GI endoscopy with biopsies 15 achalasia, absent
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