Achalasia cardia

includes detail about the achalasia cardia including recent advances

1. Achalasia cardia Target audience – Residents Date- 10.9.2021 Moderator – Dr A. K.Srivastava Professor Speaker – Dr Pooja Pandey PGJR2 Department of general surgery MIMS, Barabanki 10/9/2021 1

2. Achalasia cardia Learning objectives Surgical anatomy of esophagus Physiology of esophagus –Swallowing Physiologic reflux Test for the esophageal motility Classification of esophageal motility disorder Introduction on Achalasia cardia Clinical presentation How to diagnose Treatment 10/9/2021 2

3. Achalasia cardia Surgical anatomy 25-30 cm long . Posterior mediastinum. Pharynx to the cardia of the stomach . Musculature :- striated  transitional zone  smooth muscle 10/9/2021 3

4. Achalasia cardia Course of esophagus 10/9/2021 4

5. Achalasia cardia Constrictions Importance – Foreign body Endoscopy 10/9/2021 5

6. Achalasia cardia Blood supply Importance – Mobilisation of esophagus 10/9/2021 6

7. Achalasia cardia Venous drainage Importance- Collateral pathway 10/9/2021 7

8. Achalasia cardia Nervous supply Importance Vocal cord Aspiration 10/9/2021 8

9. Achalasia cardia Lymphatic Importance Regional spread Wide spread 10/9/2021 9

10. Achalasia cardia Function Primary function – transport material from pharynx to the stomach . Secondary function – constrain the amount of air that is swallowed and the amount of material that is refluxed . N.B- Transport of food bolus from mouth through esophagus into the stomach begins with swallowing -  postrelaxation contraction of LES (In transit- co-ordinated peristaltic contraction ) 10/9/2021 10

11. Achalasia cardia Physiology Swallowing 3 phases Oral Pharyngeal Esophageal 10/9/2021 11

12. Achalasia cardia Physiology Swallowing 6 Events of oropharyngeal swallowing 10/9/2021 12

13. Achalasia cardia Physiology Swallowing Esophageal phase Upper esophageal sphincter – Normal manometric values Total length – 4-5cm Resting pressure -60 mmHg Relaxation time -0.58 sec Residual pressure- 0.7-3.7mm Hg 10/9/2021 13

14. Achalasia cardia Physiology Swallowing Esophageal phase Peristalsis 3 types Primary Secondary Tertiary 10/9/2021 14

15. Achalasia cardia Physiology Swallowing Esophageal phase Peristalsis 3 types 10/9/2021 15

16. Achalasia cardia Physiology Swallowing Esophageal phase Primary Secondary Tertiary *Progressive *2-4cm/sec *Generate intraluminal pressure-40-80mmHg *Reach LES-9sec **Progressive **Abdominal distension or Irritation of the esophagus rather than voluntary swallowing . ***Non progressive ***Non peristaltic ***Monophasic or multiphasic ***Occur after voluntary swallowing or spontaneous between swallows throughout the esophagus . *** Uncordinated contraction of the smooth muscle – esophageal spasm 10/9/2021 16

17. Achalasia cardia Physiology Swallowing Esophageal phase Lower esophageal sphincter – Normal manometric values Total length – 3-5cm Abdominal length- 2-4cm Resting pressure- 6-26 mmHg Relaxation time- 8.4sec Residual pressure- 3mmHg 10/9/2021 17

18. Achalasia cardia Physiology Physiologic reflux More common when awake and in the upright position than during sleep in the supine position. The LES has intrinsic myogenic tone, which is modulated by neural and hormonal mechanisms. Α-adrenergic neurotransmitters or β-blockers stimulate the les, and α-blockers and β-stimulants decrease its pressure 10/9/2021 18

19. Achalasia cardia Physiology Physiologic reflux The hormones gastrin and motilin have been shown to increase LES pressure; and cholecystokinin, estrogen , glucagon, progesterone, somatostatin , and secretin decrease LES pressure . The peptides bombesin, l- enkephalin , and substance P increase LES pressure; and calcitonin generated peptide , gastric inhibitory peptide, neuropeptide Y, and vasoactive intestinal polypeptide decrease LES pressure 10/9/2021 19

20. Achalasia cardia Physiology Physiologic reflux Pharmacologic agents such as antacids, cholinergics , agonists, domperidone , metoclopramide, and prostaglandin F2 are known to increase LES pressure; and anticholinergics , barbiturates, calcium channel blockers, caffeine, diazepam, dopamine, meperidine , prostaglandin E1 and E2, and theophylline decrease LES pressure. Peppermint, chocolate, coffee, ethanol, and fat are all associated with decreased les pressure and may be responsible for esophageal symptoms after a sumptuous meal. 10/9/2021 20

21. Achalasia cardia Assessment of esophageal function (a) Tests to detect structural abnormalities of the esophagus; (b) Tests to detect functional abnormalities of the esophagus; (c) Tests to detect increased esophageal exposure to gastric juice; and (d) Tests of duodenogastric function as they relate to esophageal disease. 10/9/2021 21

22. Achalasia cardia Esophageal motility study (EMS) Indication Motor abnormality of the esophagus –on the basis of complaints of dysphagia, odynophagia, or noncardiac chest pain. Barium swallow or endoscopy unclear about structural abnormality. To confirm the diagnosis of specific primary esophageal motility disorders (i.e. Achalasia, diffuse esophageal spasm [DES], nutcracker esophagus, and hypertensive LES). 10/9/2021 22

23. Achalasia cardia Esophageal motility study (EMS) Indication Identifies nonspecific esophageal motility abnormalities and motility disorders secondary to systemic disease such as scleroderma, dermatomyositis , polymyositis , or mixed connective tissue disease. Symptomatic GERD , manometry of the esophageal body can identify a mechanically defective les and evaluate the adequacy of esophageal peristalsis and contraction amplitude. Preoperative evaluation of patients before antireflux surgery, 10/9/2021 23

24. Achalasia cardia Esophageal motility A. Grade I flap valve appearance. Note the ridge of tissue that is closely approximated to the shaft of the retroflexed endoscope. It extends 3 to 4 cm along the lesser curve. 10/9/2021 24

25. Achalasia cardia Esophageal motility B. Grade II flap valve appearance. The ridge is slightly less well defined than in grade I and it opens rarely with respiration and closes promptly. 10/9/2021 25

26. Achalasia cardia Esophageal motility C. Grade III flap valve appearance. The ridge is barely present, and there is often failure to close around the endoscope. It is nearly always accompanied by a hiatal hernia 10/9/2021 26

27. Achalasia cardia Esophageal motility D. Grade IV flap valve appearance. There is no muscular ridge at all. The gastroesophageal valve stays open all the time, and squamous epithelium can often be seen from the retroflexed position. A hiatal hernia is always present. 10/9/2021 27

28. Achalasia cardia EMS 10/9/2021 28

29. Achalasia cardia EMS The pressure profile is repeated with Each of the five radially oriented Transducers- The average values for sphincter pressure Above gastric baseline, Overall sphincter length, And abdominal length of the sphincter are calculated. 10/9/2021 29

30. Achalasia cardia A mechanically defective sphincter is identified by having one or more of the following characteristics: An average LES pressure of <6 mmHg An average length exposed to the positive-pressure environment in the abdomen of 1 cm or less, and/or An average overall sphincter length of 2 cm or less. 10/9/2021 30

31. Achalasia cardia High-Resolution Manometry - normal study 10/9/2021 31

32. Achalasia cardia High-Resolution Manometry - defective LES 10/9/2021 32

33. Achalasia cardia High-Resolution Manometry - Achalasia cardia 10/9/2021 33

34. Achalasia cardia High-Resolution Manometry - DES 10/9/2021 34

35. Achalasia cardia Esophageal Impedance 10/9/2021 35

36. Achalasia cardia Esophageal Transit Scintigraphy . The esophageal transit of a 10-mL water bolus containing technetium-99m (99mTc) sulfur colloid can be recorded with a gamma camera . Using this technique, delayed bolus transit has been shown in patients with a variety of esophageal motor disorders, including achalasia, scleroderma , DES, and nutcracker esophagus. 10/9/2021 36

37. Achalasia cardia Video- and Cineradiography Computerized capture of videofluoroscopic images and manometric tracings is now available and is referred to as manofluorography . Manofluorographic studies allow precise correlation of the anatomic events , such as opening of the upper esophageal sphincter, with manometric observations, such as sphincter relaxation 10/9/2021 37

38. Achalasia cardia Motility disorder 10/9/2021 38

39. Achalasia cardia Chicago classification of esophageal motility 10/9/2021 39 Type I (classic) achalasia: Impaired LES relaxation, absent peristalsis, and normal esophageal pressure. • Type II achalasia: Impaired LES relaxation, absent peristalsis, and increased panesophageal pressure. • Type III (spastic) achalasia: Impaired LES relaxation, absent peristalsis, and distal esophageal spastic contractions.

40. Achalasia cardia Chicago classification of esophageal motility 10/9/2021 40

41. Achalasia cardia Introduction Failure to relax. Primary motility disorder of the esophagus is achalasia. *Incidence of 1 per 100,000 population per year worldwide. *Prevalence – 9-10 per 100,000 people . 10/9/2021 41 * Maingot’s abdominal operation 13 th edition pg no 972

42. Achalasia cardia Aetiopathogenesis Idiopathic- it occurs due to absence/degeneration of auerbach’s plexus throughout the body of oesophagus , causing improper integration of parasympathetic impulse . Acquired variety- in america , caused by trypanosoma cruzi which destroys ganglion cells of auerbach’s plexus.( Chagas disease). Stress Emotional factors vitamin B1 deficiencies 10/9/2021 42

43. Achalasia cardia Pathogenesis Neurogenic degeneration , which is either idiopathic or due to infection. This degeneration results in hypertension of the LES  a failure of the sphincter to relax on swallowing  elevation of intraluminal esophageal pressure  esophageal dilatation, and a subsequent loss of progressive peristalsis in the body of the esophagus. The esophageal dilatation results from the combination of a nonrelaxing sphincter, which causes a functional retention of ingested material in the esophagus, and elevation of intraluminal pressure from repetitive pharyngeal air swallowing . 10/9/2021 43

44. Achalasia cardia Clinical features Women around 20-40 yrs. of age are commonly affected Female: male:: 3:2 Progressive Dysphagia-which is more for liquids than solid food. Regurgitation and recurrent pneumonia are common Malnutrition and ill health Retrosternal discomfort - pain also radiates to interscapular region Odynophagia and weight loss 10/9/2021 44

45. Achalasia cardia Clinical features 10/9/2021 45

46. Achalasia cardia Triad of Achalasia Staging I - Proximal dilatation <4cm Staging II - Dilatation b/w 4-7 cm Staging III - Dilatation >7cm Dysphagia Weight loss Regurgitation 10/9/2021 46

47. Achalasia cardia Investigations Oesophagoscopy dilated sac containing stagnant food and fluid due to stasis LES is closed with air insufflation, rosette apperance Oesophageal manometry - Aperistalsis in body of oesophagus Ultrasound- detects subepithelial tumor infiltration in 2ndy achalasia due to distal carcinoma 10/9/2021 47

48. Achalasia cardia Oesophagoscopy FIGURE 22-1 Example of retained food and saliva at the time of upper endoscopy in a patient with an esophageal motility disorder. 10/9/2021 48

49. Achalasia cardia Investigations Barium swallow- • bird beak appearance of lower oesophagus , • Dilatation of proximal oesophagus • Absence of fundic gas bubble • Sigmoid oesophagus X-ray chest- retrocardiac air fluid level lateral view Plain X-ray abdomen erect- fundic air bubble is absent due to stasis of fluid in oesophagus 10/9/2021 49

50. Achalasia cardia Bird’s beak appearance 10/9/2021 50

51. Achalasia cardia High Resolution Manometry 10/9/2021 51

52. Achalasia cardia Conservative Treatment Forceful dilatation- using pneumatic balloon under fluoroscopic control within LOS(300mmHg pressure applied for 15 sec) .(S/E- eso.perf ) Injection treatment- inj botulinum toxin is injected in LES endoscopically ,blocks Ach release (Recurr-6months) Drugs- sublingual nifedipine gives short term relief 10/9/2021 52

53. Achalasia cardia Conservative Treatment Nifedipine (10-30 mg administered 30-45 minutes before meals ). I sosorbide dinitrate (5-10 mg administered 10-15 minutes before meals ). P hosphodiesterase-5 inhibitors, such as sildenafil used to treat patients with achalasia. 10/9/2021 53

54. Achalasia cardia Surgical Treatment Open myotomy Four important principles: (a) Complete division of all circular and collar-sling muscle fibers, (B) A dequate distal myotomy to reduce outflow resistance, (c) “Undermining” of the muscularis to allow wide separation of the esophageal muscle, and (d) Prevention of postoperative reflux. 10/9/2021 54

55. Achalasia cardia Treatment Heller’s cardiomyotomy (laparoscopic cardiomyotomy ) surgical 7-10cm long incision made through lower oesophageal end and carried over to stomach ,muscles are cut till mucosa bulges out . Myotomy should be extended upto aortic arch and distally up to stomach to 1-2cm below the junction. 10/9/2021 55

56. Achalasia cardia Heller’s cardiomyotomy A myotomy through all muscle layers is performed, extending distally over the stomach to 1 to 2 cm below the junction, and proximally on the esophagus for 4 to 5 cm. The cardia is reconstructed by suturing the tongue of gastric fundus to the margins of the myotomy to prevent rehealing of the myotomy site and to provide reflux protection in the area of the divided sphincter . If an extensive dissection of the cardia has been done, a more formal Belsey repair is performed. 10/9/2021 56

57. Achalasia cardia Heller’s cardiomyotomy The tongue of gastric fundus is allowed to retract into the abdomen. Traditionally , nasogastric drainage is maintained for 6 days to prevent distention of the stomach during healing. An oral diet is resumed on the seventh day , after a barium swallow study shows unobstructed passage of the bolus into the stomach without extravasation. 10/9/2021 57

58. Achalasia cardia Modified Heller’s cardiomyotomy L eft thoracotomy incision in the sixth intercostal space along the upper border of the seventh rib. 10/9/2021 58

59. Achalasia cardia Fundoplication- Dor and Toupet 10/9/2021 59

60. Achalasia cardia Recent Advances Peroral endoscopic myotomy (POEM) First described in 2010 by Inoue et al A llows a long myotomy to be performed from the lumen of the esophagus with an endoscope. T ype 3 achalasia (vigorous achalasia ) 10/9/2021 60

61. Achalasia cardia Peroral endoscopic myotomy (POEM) Opening the esophageal mucosa 10 cm above the lower esophageal sphincter with a needle–knife electrosurgery device passed through an endoscope. A long submucosal plane is developed with the endoscope, down to and below the les. The circular muscle of the LES, above and below the gastroesophageal junction, is divided with endoscopic electrosurgery . The submucosal entry site in the esophagus is then closed with endoscopic clips. 10/9/2021 61

62. Achalasia cardia Peroral endoscopic myotomy (POEM) 10/9/2021 62 After performing a submucosal injection, a mucosotomy is performed to gain access to the submucosal space . B. The submucosal tunnel is continued down the length of the esophagus and onto the stomach using intermittent injections of methylene blue solution and electrocautery .

63. Achalasia cardia Peroral endoscopic myotomy (POEM) 10/9/2021 63 C. Following creation of the submucosal tunnel, the myotomy is performed. There are numerous variations on this approach; however, the ultimate goal is to divide at minimum the circular fibers on the esophagus and onto the stomach . D . Following completed myotomy , the mucosotomy created at the beginning is then closed with either clips or endoscopic sutures

64. Achalasia cardia Complications The rate of esophageal squamous cell carcinoma is increased in patients with achalasia compared to the general population. There is also some concern for increased risk of adenocarcinoma ; however, this risk is significantly lower than that for squamous cell carcinoma. 10/9/2021 64

65. References Schwartz’s principle of surgery vol-2 11 th edition pg no 1080-1086 Sabiston text book of surgery vol-2 1 st south asia edition pg no 1010-1020 Bailey & Love’s short practice of surgery 27 th edition pg no 1095-1099 Maingot’s abdominal operation 13 th edition (980-987) 10/9/2021 65

66. Questions Q1)What is achalasia ? Explain it’s pathophysiology? Q2) What is the prognosis of achalasia?When should a diagnosis of achalasia be considered? Q3)What are the findings on the barium swallow that indicates achalasia ? Q4)What are the findings on the esophageal manometry that indicates achalasia ? Q5)What is the goal of therapy for achalasia? Q6)What are the treatment options for achalasia? Q7)Expand the POEM and how it helps in treatment of achalasia ? 10/9/2021 66

67. Thank you 10/9/2021 67