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Pathophysiology of Esophageal Motility Disorders Thomas W. Rice MD Traditional manometry 20 cm 15 cm 10 cm 5 cm LES MANOMETRY CATHETER EVOLUTION 1960-2010 Conventional manometry High Resolution Manometry Free selection mode Topographic display mmHg HRM - Advances Intraluminal pressure measurement sensors every 1 cm computer interpolation between sensors Display pressure topogram y-axis position x-axis time color pressure High Resolution Manometry: Events and effect on pressure Esophageal Body Hypotensive Peristalsis: Assess Bolus Transit ? Mild Severe Normal Terminology - Peristalsis Normal < 3cm defect in 20 mmHg isobar CFV < 9 cm s-1 IBP < 15 mmHg DCI < 8000 mmHg cm s-1 Bredenoord AJ, et al. Neurogast & Mot 2012;24:57 Lower Esophageal Sphincter (LES) LES Function Integrated Relaxation Pressure (IRP) (Normal <15 mmHg) 5 sec Four noncontinguous intervals totaling 4 sec in 10 sec window after UES relaxation. Rice T, Shay S. Sem Thor and Cardiovas Surg 2011; 181-90. LES Morphology Subtypes of LES-crural diaphragm (CD) pressure morphology Pandolfino, Fox, Bredenoord, Kahrilas. Neurogastroenterol Motil 2009;21:796-806. Hierarchical Analysis of Esophageal Motility based on the Chicago Classification Esophageal motility disorders IRP = LES relaxation; normal <15 mmHg; Normal distal latency >4.5 sec; Breaks large >5-cm, small 3-5 cm; Nutcracker is DCI 5-8,000; Rapid contraction <9 cm/sec 1. Primary motility disorders should be considered as cause of dysphagia and/or chest pain only after first evaluating for structural disorders, EoE, and cardiac disease. 2. Branch 4 abnormalities can be seen in a normal population and clinical significance remains to be established. Bredenoord A, Fox M, Kahrilas P, Pandolfino J, Schwizer W, Smout A, et al. Chicago Classification criteria of esophageal motility disorders defined in HRM esophageal pressure topography. Neurogastroenterol Motil 2012;24:57-65 (supp). Achalasia Hierarchical Analysis of Esophageal Motility Note: IRP = LES relaxation; normal <15 mmHg 1. Primary motility disorders should be considered as cause of dysphagia and/or chest pain only after first evaluating for structural disorders, EoE, and cardiac disease. 2. Branch 4 abnormalities can be seen in a normal population and clinical significance remains to be established. Bredenoord A, Fox M, Kahrilas P, Pandolfino J, Schwizer W, Smout A, et al. Chicago Classification criteria of esophageal motility disorders defined in HRM esophageal pressure topography. Neurogastroenterol Motil 2012;24:57-65 (supp). Achalasia - Simultaneous contraction, - LES high, poor relaxation Normal THREE TYPE OF ACHALASIA Type I 1 Type 75 50 25 Pressure Scale Pharynx Pharynx Pressure Scale Type Type 3 3 Type 22 Type 50 25 Pressure Scale 75 50 25 100 UES UES 75 100 75 50 25 25 100 13-cm 50 75 25 50 25 25 50 25 75 50 100 Gastric 25 75 25 75 50 25 100 LES 50 50 100 3-cm 3-cm 25 50 75 LES 50 100 75 Gastric 75 75 8-cm 8-cm 13-cm 75 Note: Type 2 has the best results with treatment 75 50 25 CCF © 2009 EGJ Outflow Obstruction Hierarchical Analysis of Esophageal Motility Note: IRP = LES relaxation; normal <15 mmHg 1. Primary motility disorders should be considered as cause of dysphagia and/or chest pain only after first evaluating for structural disorders, EoE, and cardiac disease. 2. Branch 4 abnormalities can be seen in a normal population and clinical significance remains to be established. Bredenoord A, Fox M, Kahrilas P, Pandolfino J, Schwizer W, Smout A, et al. Chicago Classification criteria of esophageal motility disorders defined in HRM esophageal pressure topography. Neurogastroenterol Motil 2012;24:57-65 (supp). Functional obstruction EG Junction 5 mmHg A B 5 mmHg A – Very high Intrabolus pressure: mean 55 mmHg B – Increased contraction pressure: 250 mmHg Normal EG Junction 70 mmHg 3 cm/s Normal <25 mmHg Normal <180 mmHg Occurs in: Tight wrap; stricture; paraesophageal hernia; EoE; etc Spastic Disorders Hierarchical Analysis of Esophageal Motility Note: DCI = Measure of esophageal contraction strength; normal <5,000 1. Primary motility disorders should be considered as cause of dysphagia and/or chest pain only after first evaluating for structural disorders, EoE, and cardiac disease. 2. Branch 4 abnormalities can be seen in a normal population and clinical significance remains to be established. Bredenoord A, Fox M, Kahrilas P, Pandolfino J, Schwizer W, Smout A, et al. Chicago Classification criteria of esophageal motility disorders defined in HRM esophageal pressure topography. Neurogastroenterol Motil 2012;24:57-65 (supp). DIFFUSE ESOPHAGEAL SPASM DL Normal peristalsis > 30 mmHg Distal latency – 3.1 s (nl<4.5) Simultaneous >30 mmHg Jackhammer Esophagus (Hypercontractile esophagus) DCI >8,000 with repetitive contractions Roman S, Tutuian R. Esophageal hypertensive peristaltic disorders Neurogastroenterol Motil 2012;24:20-26. Peristaltic Abnormalities Hierarchical Analysis of Esophageal Motility based on the Chicago Classification Esophageal motility disorders IRP = LES relaxation; normal <15 mmHg; Normal distal latency >4.5 sec; Breaks large >5-cm, small 3-5 cm; Nutcracker is DCI 5-8,000; Rapid contraction <9 cm/sec 1. Primary motility disorders should be considered as cause of dysphagia and/or chest pain only after first evaluating for structural disorders, EoE, and cardiac disease. 2. Branch 4 abnormalities can be seen in a normal population and clinical significance remains to be established. Bredenoord A, Fox M, Kahrilas P, Pandolfino J, Schwizer W, Smout A, et al. Chicago Classification criteria of esophageal motility disorders defined in HRM esophageal pressure topography. Neurogastroenterol Motil 2012;24:57-65 (supp). Terminology - Peristalsis Weak Large breaks in 20 mmHg isobar (> 5cm) Small breaks in 20 mmHG isobar (2 – 5cm) Frequent Failed >30% but <100% of swallows with failed peristalsis Rapid Contraction 20% of swallow with rapid contraction, DL < 4.5 s Hypertensive peristalsis Mean DCI > 5,000 mmHg-s-cm, but not meeting spastic criteria (Nutcracker esophagus) Bredenoord AJ, et al. Neurogast & Mot 2012;24:57 Hypotensive Peristalsis: Assess Bolus Transit ? Mild Severe Normal DISTAL LATENCY (DL; Nl >4.5 SEC) CONTRACTILE FRONT VELOCITY (CFV; Nl <9 cm/s) CDP: Contractile deceleration point Pandolfino J, Sifrim D. Evaluation of esophageal contractile propagation using esophageal pressure topography. Neurogastroenterol Motil 2012;24 (suppl 1):20-26. Three Abnormal Contraction Types Based on Distal Latency (DL; Nl>4.5) and Contraction Front Velocity (CFV;Nl<9 cm/s) Premature + Rapid Premature only Rapid Only Pandolfino J, Sifrim D. Evaluation of esophageal contractile propagation using esophageal pressure topography. Neurogastroenterol Motil 2012;24 (suppl 1):20-26. Nutcracker Esophagus 300 Pressure Scale 200 100 B Pharynx Pressure Scale Pharynx (Hypertensive peristalsis (Mean DCI>5,000)) A 200 100 UES UES 300 200 100 13-cm 13-cm 300 200 100 8-cm 8-cm 300 200 100 3-cm 3-cm 300 200 100 LES LES 300 200 100 Gastric Gastric 300 CC Antegrade esophageal contraction (CFV <9) Increased contraction amplitude (DCI - 6,100) Roman S, Tutuian R. Esophageal hypertensive peristaltic disorders Neurogastroenterol Motil 2012;24:20-26. Scleroderma Esophagus: Hypotensive LES (<5 mmHg); no smooth (but normal striated) muscle contraction LES