Esophageal Motility Disorders. Disclosures

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Esophageal Motility Disorders V. Raman Muthusamy, MD FACG Director of Endoscopy Clinical i l Professor of Medicine i David Geffen School of Medicine at UCLA UCLA Health System Disclosures I am an interventional endoscopist! I do have a special interest in esophageal diseases/disorders I work with an amazing esophageal physiologist/motility expert: Dr. Jeff Conklin Copyright 15 American College of Gastroenterology 1

Overview Definitions/Types of Esophageal Motility Disorders Introduction to High Resolution Manometry Cases Esophageal Anatomy & Innervation Pandolfino & Gowron, JAMA, 15 Copyright 15 American College of Gastroenterology 2

Key Information Obtained from Manometry LES pressure and length Assessment of LES relaxation with swallowing Assessment of esophageal body function/contractility Assessment of the adequacy of peristalsis Chicago Classification (v. 3.0): Esophageal Motility Disorders Pandolfino & Gowron, JAMA, 15 Copyright 15 American College of Gastroenterology 3

Overview Definitions/Types of Esophageal Motility Disorders Introduction to High Resolution Manometry Cases Standard manometry WS 28 32 Di Pressure () 50 LES 36 44 istance from Nares (cm m) Gastric 3 sec 51 Copyright 15 American College of Gastroenterology 4

Conversion to 3D space X-axis: Time Y-axis: Esophageal location Z axis : Amplitude of pressure LES Gastric 3D recording from 36 sensors at 1 cm intervals UES LES Gastric Copyright 15 American College of Gastroenterology 5

3D recording with color assigned to pressure Pressure/ amplitude 175-1 170-175 165-170 1-165 155-1 150-155 145-150 1-145 135-1 Distance/ location time 130-135 125-130 1-125 115-1 110-115 105-110 -105 95-90-95 85-90 -85 75-70-75 65-70 -65 55-50-55 45-50 -45 35-30-35 25-30 -25 15-10-15 5-10 0-5 Copyright 15 American College of Gastroenterology 6

Putting It All Together. Pandolfino & Gowron, JAMA, 15 Key Manometric Terms Integrated Residual Pressure (Relaxation at GEJ) Distal Latency Distal Contractile Integral Copyright 15 American College of Gastroenterology 7

Representative HRM Studies 15 High-resolution Pressure Topography at Rest UES 1 Cm from Nares 25 30 35 45 * 5 sec I E EGJ 1 0 50 Copyright 15 American College of Gastroenterology 8

High-resolution Pressure Topography of Normal Esophageal Function 24 UES Cm from Nares 29 34 39 44 49 LES 3 sec 0 54 Transient LES relaxation (tlesr) 14 19 UES 1 Cm from Nares 24 29 34 1 39 44 EGJ * 49 10 sec 0 Copyright 15 American College of Gastroenterology 9

Integrated Residual (Relaxation) EGJ pressure EGJ * 1 1 Dista ance from Nares (cm) 52 39 EGJ ** IRP = 4.2 0 51 IRP = 27.6 Distal Latency (DL) 19 24 UES 1 Cm from Nares 29 34 39 1 44 49 54 5 sec DL = 7.2 sec EGJ Contraction Deceleration Point (CDP) 30 0 Copyright 15 American College of Gastroenterology 10

Distal Contractile Integral (DCI) 19 24 UES 1 Cm from Nares 29 34 39 DCI = 1539 -s-cm 1 44 49 54 5 sec EGJ 0 Achalasia Type I 81% response to treatment Pandolfino & Gowron, JAMA, 15 Copyright 15 American College of Gastroenterology 11

15 Achalasia Type I Cm from Nares 25 30 35 45 UES EGJ 1 1 50 10 sec 10 sec 0 Achalasia Type II 96% response to treatment Pandolfino & Gowron, JAMA, 15 Copyright 15 American College of Gastroenterology 12

Cm from Nares 14 UES 19 24 29 34 39 EGJ 44 49 Achalasia Type II 10 sec [Panesophageal Pressurization (> 30 )] 1 1 0 Achalasia Type III 66% response to treatment Pandolfino & Gowron, JAMA, 15 Copyright 15 American College of Gastroenterology 13

Cm from Nares 16 21 26 31 36 41 46 51 UES EGJ 10 sec Achalasia Type III 1 1 0 Type I LES Readings for Hiatal Hernia ** * (cm) Distance from Nares ( 54 37 51 30 1 Type II I E 1 ** * Type III 48 ** * 5 sec 0 Copyright 15 American College of Gastroenterology 14

EG Outflow Obstruction Pandolfino & Gowron, JAMA, 15 Outlet obstruction/ Bolus pressurization (> 30 ) Cm from Nares 17 UES 1 22 1 27 32 37 42 47 EGJ 52 5 sec 0 Copyright 15 American College of Gastroenterology 15

Cm from Nares 17 22 27 32 37 Spasm (DL < 4.5 sec) UES 1 1 42 DL = 2.6 sec 47 EGJ 52 5 sec 0 Hypercontractile/ Jackhammer Esophagus (DCI > 8,000) Cm from Nares 19 24 29 34 39 44 49 54 UES DCI = 52,975 -s-cm DL = 5.6 sec EGJ 1 1 1 1 10 sec 0 Copyright 15 American College of Gastroenterology 16

Cm from Nares 21 26 31 36 41 46 51 56 Failed Peristalsis (DCI < ) 10 sec UES EGJ 1 1 0 Cm from Nares 25 30 35 Ineffective Esophageal Motility (DCI < 450) UES 1 1 45 50 55 10 sec EGJ 0 Copyright 15 American College of Gastroenterology 17

Cm from Nares 17 22 27 32 37 42 47 Fragmented Peristalsis (Gap > 5cm, DCI > 450) UES 1 1 LES 52 5 sec 0 SUMMARY Copyright 15 American College of Gastroenterology 18

Overview Definitions/Types of Esophageal Motility Disorders Introduction to High Resolution Manometry Cases CASE Copyright 15 American College of Gastroenterology 19

History 32 yo man with 1-year history of dysphagia described as sensation of food and water sticking in the substernal region. Substernal chest pain when eating. WU for cardiac disease negative. Night time regurgitation and cough 25 lb weight loss in 4 months Endoscopy negative 17 High-resolution Impedance Manometry Cm from Nares 22 27 32 37 42 47 52 UES LES 10 sec 1 1 0 Copyright 15 American College of Gastroenterology

Case continued Heller myotomy 3 months later continues to complain of dysphagia and regurgitation Why and what to do? High-resolution Impedance Manometry Failed Heller Myotomy Cm from Nares 15 25 30 35 45 50 UES mm balloon dilation No Improvement IU botulinum toxin - Resolution Plan Redo myotomy when symptoms recur (POEM preferred if available) 1 1 30 10 sec 0 Copyright 15 American College of Gastroenterology 21

CASE yo woman with dysphagia and regurgitation Scleroderma since 15 years old GERD for years controlled with PPI and life style change Morbid obesity y( (BMI =48) Pulmonary hypertension Copyright 15 American College of Gastroenterology 22

Laproscopic sleeve gastrectomy Sleeve Gastrectomy yo woman with dysphagia and regurgitation Scleroderma since 15 years old GERD for years controlled with PPI and life style change Morbid obesity y( (BMI =48) Pulmonary hypertension yo woman with dysphagia and regurgitation Scleroderma since 15 years old GERD for years controlled with PPI and life style change Morbid obesity y( (BMI =48) Pulmonary hypertension 6 months post operation 64 kg weight loss Dysphagia, regurgitation, chest discomfort Endoscopy No esophageal or EGJ abnormality Bx 50 eos/hpf Fluticasone and PPI no response Esophageal manometry - Achalasia Copyright 15 American College of Gastroenterology 23

yo woman with dysphagia and regurgitation Scleroderma since 15 years old GERD for years controlled with PPI and life style change Morbid obesity y( (BMI =48) Pulmonary hypertension 6 months post operation 64 kg weight loss Dysphagia, regurgitation, chest discomfort Endoscopy No esophageal or EGJ abnormality Bx 50 eos/hpf Fluticasone and PPI no response Esophageal manometry - Achalasia yo woman with dysphagia and regurgitation Scleroderma since 15 years old GERD for years controlled with PPI and life style change Morbid obesity y( (BMI =48) Pulmonary hypertension 6 months post operation 64 kg weight loss Dysphagia, regurgitation, chest discomfort Endoscopy No esophageal or EGJ abnormality Bx 50 eos/hpf Fluticasone and PPI no response Esophageal manometry - Achalasia Now solid food dysphagia and regurgitation Copyright 15 American College of Gastroenterology 24

A. ESOPHAGUS, DISTAL, BIOPSY: - Esophagitis with extensive parakeratosis, mild intraepithelial eosinophilia, edema and basal cell hyperplasia, consistent with reflux esophagitis - No fungal forms identified - No intestinal metaplasia B. ESOPHAGUS, MID, BIOPSY: - Acute Candida esophagitis - Mild intraepithelial eosinophilia C. ESOPHAGUS, PROXIMAL, BIOPSY: - Acute Candida esophagitis Copyright 15 American College of Gastroenterology 25

MW Copyright 15 American College of Gastroenterology 26

1. High-grade obstruction ti at the level l of the junction of the gastric cardia and gastric sleeve with marked dilatation (7cm) of a patulous, largely aperistaltic esophagus. 2. Widely patent gastroesophageal junction. Copyright 15 American College of Gastroenterology 27

11/7/11 EGD/Stent t 1.8 x 12cm Bona stent t 2/10/12 Stent removal 2/13/12 Sleeve revision to LRYGB Copyright 15 American College of Gastroenterology 28

CASE Copyright 15 American College of Gastroenterology 29

RS is a 46-yo female with chest pain. Sharp stabbing pain in the epigastrium improved by drinking water or eructation. Dysphagia to solids sensed at the sternal notch Endoscopy was normal No improvement with dilation or PPI Copyright 15 American College of Gastroenterology 30

1. Sliding hiatal hernia and elicited gastroesophageal reflux Seen to the level of the thoracic aortic arch 2. Weakened esophageal motility. Decreased number and strength of primary and secondary peristaltic stripping waves in the esophagus Copyright 15 American College of Gastroenterology 31

Cm from Nares 15 25 30 35 0 45 UES LES 1 1 0 50 Antinuclear antibodies < (<) SCL-70 antibodies 5 (<) Rheumatoid Factor < (<) Neuronal cell antibodies 7 (0-54) Thyroid stimulating hormone 1.55 (0.39-4.) Hemoglobin A1C 5.3 (4.5-5.8) Returns after 6 mo with a complaint of regurgitation of bubbly fluid (saliva) and worsening dysphagia. Copyright 15 American College of Gastroenterology 32

1. Marked decrease in peristalsis, i worse with solids and stasis with solid material in esophagus until cleared by liquid 2.No bird beak 3.No hiatal hernia or reflux Copyright 15 American College of Gastroenterology 33

15 * UES 1 25 1 Cm from Nares 30 35 0 45 LES 0 50 CASE Copyright 15 American College of Gastroenterology 34

DO is a 52-yo female with chest pain. Solid food dysphagia since s a food impaction in 30s Dysphagia at base of neck, must wash down with liquid Bread, meat, potato No heartburn, nausea, vomiting or melena Dec 09 in Libya dysphagia with chopped salad, drinking fluid led to explosion in the chest and epigastrium, chest pain. Initial diagnosis cholecystitis Flown to Malta 30-hr delay. Dx esophageal perforation Rx with chest tube, TPN and antibiotics for 5-6 weeks. Transferred to USA 1/13/10 CT/Barium swallow Rx Drainage, Zosyn and vancomycin Jejunal feeding 1/14/10 Copyright 15 American College of Gastroenterology 35

1/14/10 1. There is a very small perforation originating i from the rightside id of the esophagus about 3-1/2 cm proximal to the cardioesophageal junction. Contrast extends both superiorly into the right posterior mediastinum and inferiorly into a subdiaphragmatic position. I would not consider this to be a contained perforation. 1/28/10 Copyright 15 American College of Gastroenterology 36

1/28/10 No evidence of perforation. The extravasation of contrast on the right side of the esophagogastric junction noted on 01/14/10 is no longer present. Continued to have dysphagia intermittantly 24-hr ambulatory intraesophageal ph study Degree of Acid Exposure: TIME PH<4 LOWER ESOPHAGUS UPPER ESOPHAGUS Total 0.0% (normal <5%) 0.0%(normal <1%) Upright Upright 0.0% 0.0% Supine 0.0% 0.0% Post- prandial 0.0% 0.0% Esophageal manometry, March 10 Copyright 15 American College of Gastroenterology 37

Esophageal manometry, March 10 Endoscopy, May 10 Copyright 15 American College of Gastroenterology 38

Treatment with Fluticasone 4 mg BID What happened? Copyright 15 American College of Gastroenterology 39