Gastro esophageal reflux disease - pathophysiology, symptoms, management and Achalasia cardia

1. GASTROESOPHAGEAL
REFLUX DISEASE AND
ACHALASIA CARDIA
- Dr. RISHABH HANDA

2. DEFINITION
• Gastroesophageal reflux disease (GERD) is a
• chronic disorder related to the retrograde flow of gastric contents into the
esophagus,
• results in a spectrum of symptoms with or without tissue injury.
• Classic GERD symptoms in the absence of esophageal mucosal
complications are the hallmarks of Nonerosive reflux disease (NERD).
• Patients with NERD account for up to 70% of those with GERD in the
community.

3. SYMPTOMS
Typical GERD symptoms-
• Heartburn (ascending retrosternal burning)
• Regurgitation
• Dysphagia – It can be divided into
• an oropharyngeal etiology, which is characterized by difficulty
transferring food out of the mouth into the esophagus, and

4. • esophageal etiology, which is characterized by the sensation of
food sticking in the lower chest.
• Dysphagia can be a sign of underlying malignancy and should be
aggressively investigated with upper endoscopy.
Atypical symptoms
• cough,
• globus sensation,
• hoarseness,
• throat clearing,

5. • Chest pain
• precipitated by meals,
• occurring at night while supine,
• Non-radiating,
• responsive to antacid medication,
• or accompanied by other symptoms such as dysphagia and/or
regurgitation - should trigger an evaluation for an esophageal
cause.
• very important to exclude a cardiac etiology.

6. PATHOPHYSIOLOGY OF GERD
The antireflux mechanism includes four important components:
1) lower esophageal sphincter (LES)
2) esophageal peristalsis;
3) crural diaphragm; and
4) stomach (the reservoir).

7. 1. Lower esophageal sphincter (LES)
• Gastroesophageal junction (GEJ) - composed of both intrinsic (LES)
and extrinsic (crural diaphragm) contractile elements.
• LES is a high-pressure zone located at the GEJ
• Creates the barrier between the esophagus and stomach that
normally prevents reflux.

8. • LES relaxation occurs in two situations:
1. immediately following a swallow, when it momentarily relaxes
to allow passage of food into the stomach, and
2. when the fundus is distended with gas, it is eliminated to allow
venting of the gas (a belch)—transient LES relaxation (TLESR).

9. • For an LES to be effective, it must possess three characteristics: an adequate
1. total length
2. intra-abdominal length
3. resting pressure
• A defective LES is identified by one or more of the following characteristics:
1. an average pressure of less than 6 mm Hg,
2. an average overall length of 2 cm or less, and
3. an average length exposed to the positive pressure environment of the
abdomen (intra-abdominal length) of 1 cm or less.

10. • The most common cause of a permanently defective LES is an
inadequate abdominal length, secondary to the high prevalence
of a hiatal hernia in patients with GERD.
• Frequent and prolonged TLESR can be associated with the
development of GERD, and this may explain the etiology of
disease observed in the 40% of patients with a manometrically
normal sphincter.

11. • “Transient sphincter” shortening occurs in the initial stages of GERD
and is the mechanism for the early complaint of excessive postprandial
reflux.
• This process is associated with the common complaints of belching and
bloating in patients with GERD.

12. • There is an increased frequency of swallowing (air and saliva)
observed in GERD patients because the ingestion of saliva (pH 7)
serves to neutralize the acidic fluid (pH 1) in the esophagus.
• Therefore, GERD may begin in the stomach, secondary to gastric
distention due to overeating and a high-fat diet, which delays gastric
emptying.

14. 2. Esophageal peristalsis
• Clears physiologic reflux and thus reduces contact time
between the esophageal epithelium and gastric fluid.
• Ineffective esophageal motility can result in an abnormal
esophageal exposure to gastric juice even in individuals with
a mechanically effective LES and normal gastric function.

15. • However, ineffective motility is more often seen in patients with a
mechanically defective LES, where distal esophageal body function
deteriorates as a direct result of repetitive inflammation.
• This effect further prolongs the esophageal exposure to gastric juice,
which creates a vicious cycle leading to more severe disease.

16. 3. Crural diaphragm
• Provides an extrinsic component to the gastro-esophageal barrier.
• The pinchcock action of the diaphragm is particularly important as a
protection mechanism against reflux induced by sudden increases in intra-
abdominal pressure.
• This mechanism is disrupted by the presence of a hiatal hernia where the
intrinsic LES has “migrated” proximal to the diaphragmatic pinch.

17. 4. Stomach
 Impaired function of the stomach such as abnormal gastric
emptying may contribute to GERD by increasing intragastric pressure,
distension, and LES unrolling.
This may occur in patients with a
• large hiatal hernia, in which the herniated stomach in the chest does not
empty appropriately,
• gastric outlet obstruction either from malignancy or peptic ulcer disease
• diabetic gastroparesis.

18. ASSESSMENT OF PATIENTS WITH GERD
• Purpose of esophageal objective testing
• to determine if the symptoms are due to gastroesophageal reflux
events, and
• to define the severity of GERD and esophageal motility that will
impact on the selection of the type of surgical therapy.

19. • Esophageal objective testing includes
• barium esophagram,
• upper gi endoscopy,
• esophageal manometry,
• esophageal pH monitoring, and
• MII-pH (MULTICHANNEL INTRALUMINAL IMPEDANCE-pH MONITORING)
• Gastric emptying studies may be considered in patients with suspicious
symptoms such as bloating and nausea.

20. 1. Barium Esophagram
It is used to
• evaluate the entire anatomy of esophagus, including the
esophageal body and both sphincters.
• document the presence and size of a hiatal hernia,
• stricture severity and location,
• diverticula,

21. • esophageal emptying, and
• the presence of gastroesophageal reflux, both spontaneously and
induced by provocative maneuvers - reliable indicator but the
absence of roentgenographic evidence of reflux does not exclude
disease.
• Esophageal motility can be assessed - to some extent

25. 2. Upper GI endoscopy
• More than 50% of patients who have typical GERD symptoms have
normal endoscopic examinations.
• Mild changes of GERD that may be visible to the endoscopist include
mucosal erythema, edema, hyper- vascularity, friability, and blurring
of the SCJ. Identification of those changes is a subjective skill

26. • More severe GERD can result in esophageal erosions and ulcerations.
Histologically,
• erosions - are defined as superficial necrotic defects that do not
penetrate the muscularis mucosae, whereas
• ulcerations are deeper defects that extend through the muscularis
mucosae into the submucosa
• the distinction between an esophageal ulceration and an erosion
usually is based on a subjective assessment of the depth of the
necrotic lesion

28. • The best validated and most widely used classification system for reflux
esophagitis now is the Los Angeles classification.
• In this system, a mucosal break is defined as, “an area of slough or
erythema with a discrete line of demarcation from the adjacent, more
normal-looking mucosa”.
• Erosions and ulcerations both are referred as “mucosal breaks”.

30. • Abnormalities of the gastroesophageal flap valve (gastric portion of
the LES) can be visualized by retroflexion of the endoscope.
• Hill’s classification - appearance of the gastroesophageal valve is
graded from I to IV according to the degree of unfolding or
deterioration of the normal valve architecture.
• The appearance of the valve correlates with the presence of
increased esophageal acid exposure, occurring predominantly in
patients with grades III and IV valves.

32. 3. Esophageal Manometry
• Most accurate method to assess the coordination and pressure of the
lower esophageal sphincter (LES) and the esophageal body.
• Findings in GERD-
• a defective LES or
• impaired esophageal motility.
• Important component in the preoperative workup of antireflux
surgery.

33. • Excludes achalasia that may be occasionally misdiagnosed as GERD.
• Characterizes the esophageal motility, and this information will be
used to determine the surgical approach (Nissen or partial
fundoplication).
• Enables measurement of the precise location of the LES for accurate
pH probe placement.

34. 4. Esophageal pH monitoring
• 24-hour esophageal pH monitoring has been a gold standard for the
diagnosis of GERD.
• It is particularly important that preoperative pH testing be performed
off medication in patients being considered for antireflux surgery.
• Antisecretory medications should be discontinued 10–14 days prior to
the study.

35. Types : Catheter based and Catheter free
Catheter based
• The conventional catheter-based pH monitoring system principally
consists of a flexible catheter with one or more pH sensors and a
data logger.
Catheter- free : wireless pH capsule(Bravo)
• Improved patient comfort
• less effect on reflux-provoking activities
• fixed placement of the pH electrode (6 cm above the SCJ),

36. Results of 24hr pH monitoring are expressed in form of DeMeester score.
6 variables are measured and factored in to this composite score –
• Total number of reflux events
• Percentage of total time spent in an acid environment with a pH less
than 4
• Percentage of upright time spent in an acid environment with a pH less
than 4
• Percentage of supine time spent in an acid environment with a pH less
than 4
• Duration of the longest reflux episode
• Number of reflux episodes lasting more than 5 minutes

37. • First four of these factors - evaluate the frequency and severity of
reflux, and the
• Last two assess the ability of the esophagus to clear acid.

38. 5. MII-pH (MULTICHANNEL INTRALUMINAL
IMPEDANCE-pH MONITORING
• Detects the intraesophageal bolus movement on the basis of a
change in the resistance to electric current across adjacent electrode
pairs positioned in a serial manner along a catheter.

39. • Able to detect, localize, and classify reflux events as
• acid,
• weakly acid, or
• nonacid.
• MII-pH has been posited as the future standard for reflux detection and
monitoring, especially in patients with persistent typical and/or atypical
GERD symptoms despite PPI therapy.
• However, the clinical utility of MII-pH is still being investigated.

40. TREATMENT OF GERD
• MEDICAL – includes both lifestyle modifications and pharmacologic
interventions.
• SURGICAL

41. MEDICAL
LIFESTYLE CHANGES
• Foods –
• Acidic liquid such as colas and teas, and citrus products such as orange,
grapefruit, and tomato juice are direct esophageal irritants and will
exacerbate symptoms in the GERD patient.
• Coffee and chocolate can decrease LES pressure and may exacerbate
reflux.
• A high-fat meal will increase reflux frequency in both normals and
patients with GERD.

42. • Fat may increase the risk of reflux by delaying gastric emptying.
• Eating slowly will reduce reflux as will meals with smaller volume.
• Carbonated beverages are not recommended
• Body weight:
• A BMI greater than 30 is associated with almost three times higher risk of
frequent reflux symp- toms.
• Weight gain has been associated with an increased risk of symptoms of GERD
and weight loss associated with a decrease in risk.

43. Sleep and body position
• Patients with nighttime GERD should begin the sleeping period with
the left side down. - Decrease in transient lower esophageal sphincter
relaxations (TLESRs) on the left side as compared to the right.
• Refrain from eating within 2 to 3 hours of going to sleep - As reflux is
more frequent in the first half of the sleeping period.
Alcohol
• Alcohol is a smooth muscle relaxant and reduces lower esophageal
sphincter pressure (LESP). Should be used judiciously.

44. Smoking
• May increase esophageal sensitivity to acid by impairing intracellular
regulation of pH.
• Smoking cessation in individuals with GERD has been shown to
reduce the number of upright reflux episodes.

45. RECOMMENDATIONS FOR LIFESTYLE CHANGES
•eat more slowly,
•have smaller meals,
•avoid late-night meals and sleeping pills, and
•make every effort to reduce body weight (or waist
circumference)

46. Pharmacotherapy
• Antisecretory therapy (with either H2RAs or PPIs) is still the choice
for medical therapy for GERD.
• Antacids are widely used as first-line treatment for the heartburn
symptoms of GERD
• Sucralfate
• mucosal protective agent
• Little or no place in normal modern therapy of GERD

47. • Motility agents
• act by increasing LES pressure, stimulating esophageal peristalsis,
and accelerating gastric emptying.
• only effective in healing milder degrees of erosive esophagitis
• HISTAMINE H2 RECEPTOR ANTAGONISTS
• ranitidine, famotidine, and nizatidine
• inhibit histamine-stimulated gastric acid secretion
• antisecretory capabilities are best at night, with duration of acid
inhibition longer when the drug is taken in the evening or before
bedtime.
• patients, particularly those with infrequent or mild symptoms can
be managed long-term on as- needed or daily H2RAs.

48. PROTON PUMP INHIBITORS
• PPIs provide prolonged inhibition of acid secretion, irrespective of the
stimulus.
• Mechanism - Inhibit the hydrogen potassium ATPase, the final common
pathway of acid secretion,
• Suppress daytime, nighttime, and meal-stimulated acid secretion to a
significantly greater degree than H2RAs.
• PPIs are generally approved for once-daily dose and prescribed in the
morning
• Some patients need an increase in PPI dose because of incomplete
symptom relief, extraesophageal symptoms, or Barrett esophagus.

49. Delayed- release PPIs
• omeprazole, lansoprazole, rabeprazole, pantoprazole, and
esomeprazole.
Two newer formulations
• omeprazole immediate-release sodium bicarbonate (a combination of
non–enteric-coated omeprazole granules with sodium bicarbonate)
• dexlansoprazole, the R-enantiomer of lansoprazole.

50. ANTI REFLUX SURGERY
Primary Indications for Antireflux Surgery
• Patients with esophageal and/or extraesophageal GERD symptoms that are
responsive but not completely eliminated by PPIs
• Patients with heartburn eliminated by PPIs but continued nonacid reflux
• Patients with well-documented reflux events preceding symptoms such as chest
pain, cough, or wheezing
• Patients with GERD complications such as peptic stricture, Barrett esophagus, or
vocal cord injury while taking PPIs twice a day
• Patients with well-documented GERD who desire to stop chronic PPI use despite
excellent symptom control for any reason (e.g., side effects, lifestyle, expense)

51. Procedure Selection
• A laparoscopic approach has been widely accepted, and the
laparoscopic Nissen fundoplication is the procedure of choice for a
primary antireflux surgery in the majority of patients with good
esophageal motility and normal esophageal length.

52. • Laparoscopic approach is associated with
• shorter hospital stay,
• less post- operative pain,
• fewer wound-related complications, and
• earlier return to work.
• Despite these advantages, selection of the open versus the
laparoscopic approach should depend on surgeon experience and the
patient’s previous surgical history.
• The intraoperative steps of surgical repair are relatively similar in both
approaches.

53. Laparoscopic Nissen fundoplication
Primary Principles of Nissen Fundoplication
• Circumferential crural dissection with preservation of the vagus
nerves
• Circumferential dissection of the esophagus at the gastroesophageal
junction
• Adequate mobilization of the esophagus (or Collis gastroplasty) to
attain 2 to 3 cm of intraabdominal esophagus without inferior
traction

54. • Crural closure with interrupted sutures
• Gastric fundus mobilization and adequate short gastric vessel
division
• Creation of a short (<2 cm), floppy (loose around an 18- to 20-mm
dilator) fundoplication anchored to the esophagus in several places

62. Post op care and follow up
• Liberal use of antiemetic medications occurs to prevent retching that
could disrupt the wrap in the immediate postoperative period.
• Clear liquids started once patients are awake and alert on the evening
of surgery.
• The diet can be advanced to soft foods the following day.
• Patients can be dismissed home in 1 to 2 days.
• 1-month followup; no studies are routine, but a barium swallow
serves as an excellent screening test to evaluate postoperative
dysphagia or reflux-like symptoms.

63. Laparoscopic Partial Fundoplication
• Indicated in patients with impaired esophageal motility.
• Dor antireflux procedure - partial anterior fundoplication
• Toupet antireflux procedure – partial posterior fundoplication,
• The difference between a complete and a partial fundoplication is the
structure of a newly created valve;
• a “nipple” valve in a complete fundoplication versus
• a “flap” valve in a partial fundoplication.

64. DOR REPAIR
• Described by Jacques Dor in 1962.
• After mobilization of the distal esophagus and GEJ and hiatal closure,
a 60F Bougie is placed.
• The initial suture of the Dor fundoplication incorporates
• the greater curvature side of the gastric fundus 2 cm distal to the GEJ,
• lateral wall of the esophagus 2 cm proximal to the GEJ, and
• base of the left crus to re-create the angle of His.
• Re-creation of the angle of His is an essential element of the Dor
fundoplication.

65. • The greater curvature is then sutured to the arch of the diaphragm
toward the apex of the crura, bringing the anterior fundus over the
anterior wall of the distal esophagus with seven interrupted 0
nonabsorbable sutures.
• This repair is carried over to the right crus to the 9 o’clock position. At
completion, the fundoplication should appear smooth without
creases indicating the absence of tension.

66. TOUPET FUNDOPLICATION
• The gastric fundus is passed behind the esophagus through the
retroesophageal space after hiatal closure and placement of an
esophageal dilator.
• The right side of the fundus is sutured to the right margin of the
esophagus with four nonabsorbable sutures,
• the most distal suture is placed to the lesser curvature of the stomach
immediately distal to the GEJ.

67. • The right posterior aspect of the fundus is sutured to the right side of
the proximal preaortic fascia.
• A similar line of sutures secures the left limb of the fundoplication to
the esophagus.
• The posterior aspect of the gastric fundus on left is sutured to the
base of the left crus.

68. GIANT DIAPHRAGMATIC (HIATAL) HERNIAS
1. Type I -Sliding hernia - characterized by an upward dislocation of
the cardia in the posterior mediastinum
2. Type II - Rolling or Paraesophageal - characterized by an upward
dislocation of the gastric fundus alongside a normally positioned
cardia
3. Type III - Combined sliding-rolling or mixed hernia - characterized
by an upward dislocation of both the cardia and the gastric fundus
4. Type IV - an additional organ, usually the colon, herniates along
with the stomach

70. Incidence
• Paraesophageal hernia - median age is 61 years
• Sliding hiatal hernias - 48 years .
• PEHs are more likely to occur in women by a ratio of 4:1.
Etiology
• The development of a hiatal hernia is an age-related phenomenon
secondary to repetitive upward stretching of the
phrenoesophageal membrane.

71. Paraesophageal Hernias: Preoperative Symptoms and
Findings
• Typical heartburn 47%
• Dysphagia 35%
• Epigastric pain 26%
• Vomiting 23%
• Anemia 21%
• Barrett epithelium 13%
• Aspiration 7%

72. Preoperative Evaluation
1. ESOPHOGRAM - delineates how much stomach is herniated
through the hiatus
2. ENDOSCOPY - enables examination of the mucosa to evaluate for
esophagitis, BE, stricture, or malignancy. Endoscopy may also
estimate the size of the hiatus.
3. MANOMETRY AND pH TESTING –
• Most patients who have a PEH have poor esophageal motility. However, up to
80% may demonstrate improvement following surgical repair of the hiatal
hernia.
• use of pH probe testing is not necessary

74. Operative Approach
Goals:
1. Restore the normal anatomy by returning the GEJ and stomach to
the abdomen and
2. Correct the condition that contributed to the development of the
anatomic problem, namely GERD
Approach:
• Laparoscopic
• Transthoracic - A left posterolateral thoracotomy is performed with a
planned entry at the seventh interspace
• Transabdominal

75. Laparoscopic
• The hernia sac is gently reduced
• The sac is incised providing access to the fibroareolar plane within the
posterior mediastinum.
• Circumferential mobilization facilitates reduction of the entire hernia sac
into the abdomen
• An antireflux procedure is routinely performed.
• The crura are reapproximated using nonabsorbable 0-suture.
• Bioprosthetic mesh is used selectively to buttress the hiatal closure.

79. Short Esophagus and PEH
• Giant PEH can be associated with a short esophagus in up to 5% to
20% of patients as a result of chronic cephalad displacement of the
GEJ
• Collis gastroplasty achieves esophageal lengthening by using the
gastric cardia to create a neoesophagus.
• Stapler is applied on the left side and parallel to the esophagus with a
16-mm dilator in place.

82. COMPLICATIONS OF GERD
(1) Mucosal complications
(2) Extraesophageal
3) Metaplastic and neoplastic

83. Mucosal complications
• Esophagitis
• Stricture
• Predisposing factors-
• a mechanically defective LES and
• an increased esophageal exposure to the gastric fluid with a pH less than 4
and greater than 7

84. Extraesophageal / Pulmonary
• Laryngeal or respiratory symptoms such as
• cough
• recurrent pneumonia
• asthma
• progressive pulmonary fibrosis
• Two mechanisms have been proposed as the pathogenesis of
reflux-induced respiratory symptoms:
• aspiration of gastric contents and
• vagally mediated bronchoconstriction.

85. Metaplastic and neoplastic
Metaplastic-
Barrett’s esophagus (BE) is defined as
• a columnar lined segment of esophagus of any length visible on
endoscopy with a biopsy showing intestinal metaplasia with the
presence of goblet cells.
• Classification :
1. According to length:
• short-segment BE (<3 cm)
• long-segment BE (≥3cm).

86. 2. According to dysplasia:
• BE without dysplasia,
• indefinite for dysplasia,
• low-grade dysplasia (LGD),
• high-grade dysphagia (HGD).
Neoplastic – Adenocarcinoma of esophagus

87. Treatment options
• PPI therapy
• Antireflux surgery – Laparoscopic Nissen’s Fundoplication
• Management of dysplasia –
• Aggressive PPI therapy,
• Fundoplication
• Endoscopic resection of visible lesions Radiofrequency ablation
• Esophagectomy

88. ACHALASIA CARDIA

89. ACHALASIA (Greek word meaning failure to relax.)
• Primary motility disorder affecting the esophagus.
• Complete aperistalsis of the esophagus
• Most frequently encountered motility disorder seen by surgeons
• Rare condition, with an incidence of <10 per 100,000 population

90. PATHOGENESIS
• Histopathologic hallmark : Near complete or total loss of the
myenteric plexus (Auerbach’s) ganglion cells as a result of injury and
fibrosis of these cells and myenteric nerves (which normally secrete
vasoactive intestinal polypeptide and nitric oxide).

91. • 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.

92. PATIENT PRESENTATION
• Age group: 2nd to 5th decades of life.
• Sex: No predilection toward either sex.
• Symptoms:
1. dysphagia,
2. regurgitation of indigested food,
3. complaints of food “sticking” in the chest.
4. Often worsen after lying supine, with regurgitation occurring even the
next day of the previous day’s meal.

93. 5. Cold liquids frequently exacerbate symptoms, with inability to ingest cold
water being a common complaint.
6. Maneuvers employed in attempts to allow passage of food through
nonrelaxing LES
• raising arms over the head,
• swallowing liquids to try to “wash down” food, or
• remaining upright for extended periods of time.

94. 7. As the disease progresses  esophagus dilates, in effect acting like a
stomach reservoir,  regurgitation of the prior day’s food contents
becomes more common.
8. Avoidance of social situations by patients due to fear of regurgitating
food in front of others.
9. Pulmonary complications.
10. Weight loss - tends to correlate with disease severity.

95. • Refers to esophageal motility abnormalities that are similar to achalasia
• Caused by: other disease entities, most commonly malignancy and
occasionally intestinal pseudoobstruction, postvagotomy states,
amyloidosis, and sarcoidosis.
SECONDARY ACHALASIA

96. • Malignancy-induced achalasia, is often referred to as
pseudoachalasia. It is caused by adenocarcinoma of the gastric cardia
in 75% of cases.
• In older patients (>60 years), recent onset of symptoms (<6 months)
and significant weight loss (>10–20 lb) should stimulate concern for
pseudoachalasia.

97. DIAGNOSIS
• BARIUM ESOPHAGOGRAM
• MANOMETRY
• UPPER GI SCOPY

98. 1. BARIUM ESOPHAGOGRAM
• Esophageal dilation with distal tapering to the GE
junction, resulting in a “bird’s beak” appearance.
• Air fluid levels are often seen.
• Slow emptying of the barium.

100. 2. MANOMETRIC CHARACTERISTICS
• Incomplete lower esophageal sphincter (LES) relaxation(<75% relaxation)
• Aperistalsis in the esophageal body
• Elevated LES pressure ≤26 mmHg
• Increased intraesophageal baseline pressures relative to gastric baseline
• Vigorous achalasia, a variant in which high-amplitude waveforms are
present, can be encountered, and it is usually found in patients with earlier
stages of the disease before complete destruction of the myenteric
ganglion cells ensues.

101. Normal Achalasia

102. 3. ENDOSCOPY
• This offers the chance to directly inspect the mucosa and evaluate the
GE junction.
• Any abnormalities should be biopsied to rule out causes of
pseudoachalasia, as well as evaluated with CT and/or endoscopic
ultrasound.

103. TREATMENT
INCLUDES-
• Pharmacological,
• Endoscopic, and
• Surgical therapies

104. PHARMACOLOGICAL
• Smooth muscle relaxants such as nitrates (isosorbide dinitrate) or
calcium channel blockers (diltiazem, nifedipine, verapamil) have
been proposed as pharmacologic treatment of achalasia.
• Administered sublingually immediately before eating and they can
relieve dysphagia in achalasia by reducing the LES pressure.
• However, placebo-controlled crossover trials have found only minimal
benefit.

105. • Sildenafil is another smooth muscle relaxant that can decrease LES
pressure in patients with achalasia by blocking phosphodiesterase
type 5.
• Sildenafil enhances nitric oxide action, which has an inhibitory effect
on esophageal contractile activity and LES tone.
• The practicality of using sildenafil clinically is limited by its cost.

106. ENDOSCOPIC
• Includes
1. Pneumatic dilation
2. Botulinum toxin injection

107. Pneumatic Dilatation
• Requirement - Distention of the LES to a diameter of at least 30 mm
to effect a lasting reduction of LES pressure
• Mechanism - Partially disruption of the circular muscle of the
sphincter
• Basic element of an achalasia dilator - long, noncompliant, cylindrical
balloon that can be positioned fluoroscopically (Rigiflex dilator) or
endoscopically (Witzel dilator) across the LES and then inflated to a
characteristic diameter in a controlled fashion using a hand-held
manometer.

108. • Technique-
• Most practitioners begin with a 30-mm-diameter balloon inflated
to 12 psi or complete effacement as evident by fluoroscopy.
• The inflation periods range from several seconds to 5 minutes
• Most important aspect - positioning the balloon across the EGJ
and assessing obliteration of the indentation on the expanding
balloon (waist) related to the contracting LES.

109. • Failure of response with a 30-mm dilator can be retreated with
greater-diameter balloons, sequentially progressing to 35 to 40 mm
diameters if needed.
• Success rates - 70% to 90%.
• Need for further dilations is determined by the persistence of
symptoms approximately 4 weeks after treatment.

110. • In this case, a larger- diameter balloon (35-mm, sometimes 40-mm)
may be used.
• Major complication –
• esophageal perforation; however, mortality is very rare.
• Incidence of esophageal perforation from pneumatic dilation -
0.4% to 5%

112. Rigiflex® (Boston Scientific, USA) pneumatic dilation balloon,
which reaches 30 mm diameter when fully inflated.

113. Botulinum toxin injection
• Because LES tone is partially mediated via a cholinergic pathway, blockade
of acetylcholine release from excitatory motor neurons should partially
eliminate the neurogenic component of LES pressure thereby decreasing
LES pressure.
• Mechanism - irreversibly inhibits the release of acetylcholine from
presynaptic cholinergic terminals.
• However, because this effect is eventually reversed by the growth of new
axons, botulinum toxin is not long-lasting therapy.

114. • Technique- Divided doses of botulinum toxin (usually 100 units total)
are injected into four quadrants of the LES with a sclerotherapy
catheter during an upper endoscopy.
• Expense outweighs the potential economic benefits of added safety,
unless the patient’s life expectancy is minimal.
• Thus, this option is mainly reserved for elderly or frail individuals
who are poor risks for definitive treatments.

116. SURGERY
• Surgical myotomy was first described by Ernst Heller in 1913.
• His original description involved performing both an anterior and
posterior myotomy.
• This has evolved in most centers to performing an anterior myotomy
only.
• Earlier- Invasive - performed via laparotomy or thoracotomy.

117. • Eventually evolved to the minimally invasive approaches via thoracos-
copy or laparoscopy.
• Drawbacks to this thoracoscopic approach
• included the need for single-lung ventilation,
• postoperative chest tubes, and
• being unable to perform an antireflux procedure.
• The minimally invasive approach has moved predominantly to the
laparoscopic myotomy approach that has eliminated these drawbacks
of the thoracoscopic approach.

118. Laparoscopic Heller’s Myotomy
• Position : supine with legs abducted ; right arm is tucked against the
patient’s side, and the left arm remains on an arm board
• Surgeon stands between the abducted legs.
• Laparoscopic instrumentation required:
• two 12-mm trocars and three 5-mm trocars ;
• a 30-degree laparoscope
• a device for lifting the left liver lobe,
• atraumatic forceps for pulling down the stomach,
• a cautery hook and scissors
• plus a couple of standard forceps for tissue handling and a needle holder for
suturing.
• Small bipolar cauterizing forceps may be useful to control bleeding from the
edges of the myotomy.

120. • An assistant on the surgeon’s left-hand side lifts the left liver lobe
using an atraumatic retractor, thus exposing the cardia region.
• An assistant on the surgeon’s right-hand side grasps the gastric
fundus with atraumatic forceps, maintaining a caudal traction on the
esophago-gastric junction.
• The operation begins with a minimal dissection of the anterior part
of the esophagus.

121. • With the cautery hook, the peritoneum over the esophagogastric
junction is divided.
• Any adipose tissue (usually found at this level) is removed, paying
attention to the small vessels coming from the gastric wall, which
should be coagulated with the bipolar forceps; they usually mark the
inferior limit of the myotomy.
• The left vagus nerve (which becomes anterior at this level where it
crosses the anterior esophageal wall from left to right) is clearly
evident and must not be damaged

122. Myotomy:
• Started with the cautery hook on the dilated part of the distal
esophagus, above the lower esophageal sphincter that, in cases of
long-standing achalasia, is often marked by a whitish, sclerotic area.
• Started 2 cm above the esophagogastric junction to expose the
esophageal submucosal layer with the least risk of perforation.
• First the longitudinal muscle fibers, then the circular fibers are
hooked, lifted, and coagulated.

123. • Submucosal layer  forms a slight bulge between the two margins of
the myotomy  small scissors are used to bluntly dissect the muscle
layer from the submucosal layer.
• A 6 to 8-cm-long myotomy is performed, extending it on the gastric
side to 1.5 to 2 cm below the oblique muscle fibers, representing the
beginning of the gastric muscle, and thus exposing the gastric
submucosa.

124. • 30-mm Rigiflex balloon is placed inside the esophageal cavity at the
cardia level using an endoscopically positioned guidewire.
• During the myotomy, the balloon is gently inflated and deflated with
30 to 50 cc of air using a syringe: This exposes the circular fibers so
that they can be stretched and easily cut or torn apart.

127. Antireflux Fundoplication
• Although some surgeons perform only the myotomy, a fundoplication
is normally added to prevent postoperative gastroesophageal reflux
disease (GERD), which may be a severe complication.
• Given the esophageal body’s lack of propulsive peristaltic activity, a
partial fundoplication is usually performed, either anteriorly or
posteriorly;
• Anterior fundoplication (Dor) - advantages
• protects the exposed esophageal mucosa and
• can be performed without completely mobilizing the esophagus, thus
preserving the natural antireflux mechanisms.

128. • Posterior hemifundoplication (Toupet procedure) - abdominal
esophagus must be mobilized completely, and the gastric wrap is
passed behind the esophagus

129. POSTOPERATIVE CARE
• Esophagogram (with Gastrograffin) - on the first postoperative day to rule
out any mucosal perforation
• Diet:
• A liquid diet is started, and patients are discharged after another 24 to 48 hours, once
a soft diet has been started
• A soft diet is recommended for 8 to 10 days, after which a normal diet is allowed.
• Barium swallow after 1 month.
• Endoscopy and function tests - performed after 6 months to rule out any
postoperative GERD.
• Endoscopy is recommended every 2 years to rule out any neoplasia.

130. Per Oral Endoscopic Myotomy (POEM)
• Developed in Japan.
• Started by 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.

131. • The submucosal entry site in the esophagus is then closed with
endoscopic clips. While the results of POEM are still accumulating,
the procedure is attractive because it is extremely minimally invasive,
and can be done as an outpatient.
• The major downside of POEM is that an effective antireflux valve
cannot be created, exposing the patient to a 40%–50% risk of GERD
post procedure.

132. COMPLICATIONS
1. Esophagitis - by irritation from stasis and by infection.
2. Epiphrenic diverticula - represent pulsion diverticula secondary to
increased intraesophageal pressure.
3. Pulmonary disease: from microaspiration of retained food and
secretions can occur in up to 33% of patients.
4. Squamous cell esophageal cancer, presumably because of chronic
mucosal irritation.

133. 5. Surgical myotomy and endoscopic treatments of achalasia may
also increase the risk for esophageal adenocarcinoma-
• because of disruption of the LES with resulting gastroesophageal
reflux and
• the development of Barrett esophagus.

134. Newly diagnosed patients should be treated with
pneumatic dilation or a surgical myotomy?
• Long- term follow-up studies have shown that pneumatic dilation achieves
adequate relief of dysphagia and pharyngeal regurgitation in 50% to 60% of
patients.
• Close follow- up is required, and if dilation fails, myotomy is indicated.
• For those patients who have a dilated and tortuous esophagus or an
associated hiatal hernia, balloon dilation is dangerous and surgery is the
better option.

135. Thank you