1. Difficulty Swallowing Have you considered an Esophageal Motility Disorder ? by Janet R. King BSN.RN.CGRN

3. Objectives :  List the 3 phases of swallowing  Identify landmarks of a normal esophageal manometry study using high resolution technology  Review primary motility disorders focusing on Achalasia

4. I still have difficulty Swallowing How can you say my EGD was normal ? Now What ?

5. Esophageal Motility Procedure Indications :  Dysphagia  Evaluate motility disorders  Odynophagia  Noncardiac chest pain  Preoperative to anti-reflux surgery  Obtain LES location pre-pH study

6. Review : Phases of Swallowing

7. Three phases of Swallowing  1. Oral Phase – Voluntary  2. Pharangeal Phase – Involuntary  3. Esophageal Phase - Involuntary

8. Oral Phase  Food bolus moves from mouth to pharynx  Impulses sent to the swallowing center in the brain  Control switches to involuntary

9. Pharyngeal Phase  Food is moved from the pharynx to the esophagus  Involves a cycle of involuntary contractions and relaxations lasts 1-2 seconds  The UES relaxes and Primary Peristalsis begins

10. Esophageal Phase  Food is moved through the body of the esophagus to the stomach by Peristaltic Activity  The LES relaxes

11. Esophageal Phase Two Methods of Peristalsis  Primary Peristalsis  Initiated in the pharynx (voluntary)  Secondary Peristalsis  Initiated in the esophagus (involuntary)

12. Esophageal Motility  Efficient transport by the esophagus requires a coordinated, sequential motility pattern peristalsis

13. Esophageal Manometry Procedure  Evaluates the 4 Region of the Esophagus  Pharynx  Upper Esophageal Sphincter (UES)  Esophageal Body  Lower Esophageal Sphincter (LES)

14. High-Resolution Manometry Catheter Development started in 2004 36 solid state circumferential sensors 1cm distance between each sensor Simultaneous monitoring of the entire pressure profile from pharynx to the stomach Software for Topographic Pressure Plotting

15. Conventional Manometry

16. UES LES

17. Review of Anatomy

18. Pharynx

19. Upper Esophageal Sphincter

21. Lower Esophageal Sphincter

22. CORTICAL SWALLOWING CENTER OF THE BRAIN NEUROMUSCULAR CONNECTION Dorsal Motor Nucleus Nucleus Ambiguous

24. T

25. (Gastroenterology 2008) High Resolution Contour

26. Normal High Resolution Contour

27. Chicago Classification of Esophageal Motility Disorders  Developed by : Dr. John Pandolfino, Dr. Peter Kahrilas, and International High Resolution Manometry Working Group in Switzerland.  Purpose: Use the enhanced capacity of HR technology to devise a new classification of esophageal dysmotility that would describe functional abnormalities in precise mechanical terms

28. Neurogastroenterology & Motility 2012;24  suppl 1),57-65

29. Primary Motility Disorder Achalasia

30. Achalasia  Definition of Achalasia “Failure to Relax”  Achalasia is a disorder of motor activity Esophageal peristalsis LES functioning

31. Epidemiology - Achalasia  Rare disorder  Annual Incidence: New incidence approximately 1.6 cases per 100,000 individuals Preexisting prevalence of 10 cases per 100,000 individuals

32. Epidemiology- Achalasia  Occurs at any age  Effects all races  Onset before adolescence is rare  Usually diagnosed in patients between 25-60 years old  Equal frequency in men and women

33. Pathophysiology- Achalasia

34. Degeneration of Neurons Loss of Inhibitory Neurons  Esophageal Body – smooth muscles has Aperistalsis  LES sphincter – pressure rises and LES can no longer relax normally

35. Etiology - Achalasia  Cause of the degeneration of neurons is still unknown  Suggestion of an autoimmune disorder  Some investigators ? Chronic infections with herpes zoster or measles virus-(data inconclusive)

36. Patient Symptoms- Achalasia  Slow progressive problems with swallowing  Dysphagia for both solids/Liquids  Regurgitation –undigested food/saliva  Respiratory complications (nocturnal cough and aspiration)  Chest pain  Heartburn  Weight Loss

37. Diagnosis - Achalasia  Esophageal Imaging Barium Swallow  Esophageal Manometry Shows validation (had the highest sensitivity for DX)

38. Barium Swallow : Findings show the “BIRD’s BEAK” IMAGE

39. Esophageal Motility Procedure Chicago Classification: Achalasia Subtypes All 3 Subtypes are Characterized by : 1. Absent Contractile Activity 2. Impaired EGJ Relaxation - IRP (INTEGRATED RELAXATION PRESSURE>15) Pandolfino and Roman Thoracic Surg Clinical 2011

40. Type I achalasia

41. Type II achalasia

42. Type III Achalasia

43. Current Treatment - Achalasia Etiology is Still Unknown:  Focus on relieving the functional obstruction at the level of the LES

45. European Achalasia Trial Treatment Analysis by Subtype  Background : Patient’s with Achalasia were treated with either Pneumatic Dilation (PD) or Laporscopic Heller Mytotomy (LHM )  176 Patients pretreatment Manometry Procedure  Classified using 3 Chicago Subtypes  Achalasia type I (25%)  Achalasia type II (65%)  Achalasia type III (10%)

46. Success Rates of Achalasia Treatments based on subtypes Gastroenterology 2013;144:718-725

47. European Achalasia Trial: Choice of Treatment by Manometric Subtype  Type II: success rate for PD was higher than for LHM (100% vs 93%).  Type III: success rate the LHM was higher than for PD (86% vs 40%)  Type I: success rates for LHM and PD were similar (81% vs 85%) Gastroenterology 2013;144:718-725

48. Patient Study Mr. O 76 year old man  Retired courthouse clerk since 2000  2014 Diagnosed Diffuse large cell B-cell Lymphoma on right axillary node bx. Received 2 cycles chemotherapy refused further chemo.  CAD, Hypertension, 2009 Endovascular repair of infrarenal AAA, pos+Hep B

49. Patient Study Mr. O  February 1 st ED with difficulty swallowing, ? Food impaction EGD –food found in lower 1/3 of esophagus and pushed through Mild stenosis at GE junction dilated with scope and bxs taken +Hpylori-Rxed triple antibiotics Omeprazole 20 mg qam started

50. Patient Study Mr. O  Feb 20 Returned to ED with difficulty swallowing ? Food Impaction  EGD Mild inflammation at GE junction. Bxs taken. No food in the distal esophagus noted. GEJ was easily traversed with scope.  Recommendations :  EUS to evaluate GEJ area.  Barium Swallow outpatient (possible hypertensive LES or Achalasia)  Esophageal Manometry for further work up of patients dysphagia

51. Patient Study  2/25 Barium Swallow Impression : Some features of achalasia with numerous abnormal peristaltic waves throughout. Narrowing at distal esophagus was not characteristic beak-like appearance. No evidence of tumor at GE junction  2/27 Upper Esophageal Ultrasound No malignancy noted on exam No compression of the GEJ

53. Primary Motility Disorder Jackhammer

54. Jackhammer –  Hypercontractile Esophagus  Rare Disorder  Patient symptoms : Dysphagia Chest pain

55. Jackhammer

56. Jackhammer-Treatment (further studies needed)  Calcium Channel Blockers  Botox Injection

57. Patient Study: 76 year old woman c/o dysphagia and chest pain Am J Gastro 2012;107:37-45

58. The Chicago Classification is an evolutionary Process

59. COME JOIN US !!!

60. References  Bredenoord A.J., Fox M., Kahrilas S., Chicago classification criteria of esophageal motility disorders defined in high resolution esophageal pressure topography. Neurogastroenterologoy&Motility 24 Supplement 1,57-65. 2012  Holloway RH, Dodds WJ, Helm JF, et al. Integrity of cholinergic innervation to the lower esophageal sphincter in achalasia. Gastroenterology 1986; 90:924.  Pandolfino J., Roman S., High Resolution Manometry:An Atlas of Esophageal Motility Disorders and Findings of GERD using Esopphageal Pressure Topography. Thorac.Surg Clin.November;21(4)465-475, 2011  Roman,S Pandolfino J Chen,J Peyotypes and Clinical Context of Hypercontractility in High Resoluton Esophageal Pressure Topography. American Journal Gastroenterologoy V 107, 37-45 Jan 2012.  Salvatore T., Paolo L., Gianmattia D., Recent trends in endoscopic management of achalasia. World Journal of Gastrointestinal Endoscopy September 16;6(9) 407-414. 2014  Stavropoulos, S., Friedel D., Modayil R., Endoscopic approaches to treatment of achalasia. Therepeutic Advances in Gastroenterology 6(2) 115-135,2013