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Motor Disorders of Esophagus

Esophageal Peristalsis

Flow of Material

  • Unidirectional flow of material

Sphincters

  • UES (Upper Esophageal Sphincter): 60 mm Hg
  • LES (Lower Esophageal Sphincter): 24 mm Hg

Phases of Peristalsis

  • Oral Phase
    • Elevation of tongue
    • Posterior movement of tongue
  • Pharyngeal Phase
    • Elevation of soft palate
    • Elevation of hyoid and larynx
    • Tilting of epiglottis
  • Esophageal Phase
    • Relaxation of UES
    • Closure within 0.5 seconds
    • Post-relaxation pressure returns to 90 mm Hg, then normalizes at 60 mm Hg

Peristalsis

Primary Peristalsis

  • Speed: 2-4 cm/sec
  • Reaches the LES in 9 seconds after swallowing
  • Intraluminal pressure: 40-80 mm Hg

Secondary Peristalsis

  • Progressive
  • Generated from distension or irritation of the esophagus
  • Local reflex to clear leftover food

Tertiary Peristalsis

  • Non-progressive, non-peristaltic
  • Monophasic
  • Responsible for esophageal spasm

LES (Lower Esophageal Sphincter)

Characteristics

  • High-pressure zone, not a true sphincter
  • Length: 2-5 cm
  • Resting pressure: 6-26 mm Hg
  • Located in both the chest and abdomen

Key Measurements

  • Minimum total length of 2 cm, with at least 1 cm of intra-abdominal length

RIP (Respiratory Inversion Point)

  • Transition from intrathoracic to intra-abdominal

Vagal Mediated Relaxation

  • Vagal-mediated relaxation opens the LES
  • Duration: 4-6 seconds

Esophageal Dysphagia

1. Symptoms with Solids Only

  • Probably Structural
    • Progressive
      • Slow progression, chronic heartburn → ? Stricturing Esophagitis
      • Rapid Progression, Weight loss → Cancer
    • Intermittent
      • Esophageal ring or web
      • Eosinophilic esophagitis

2. Symptoms with Solids and Liquids

  • Probably Dysmotility
    • Progressive
      • Chronic heartburn → Scleroderma, absent contractility
      • Regurgitation → Achalasia
    • Intermittent
      • Chest pain → Distal esophageal spasm

Clinical Features

  • Dysphagia to both liquids and solids (more to liquids), with postural variation
  • Associated chest pain
  • Long duration of complaints
  • Affects younger age group
  • Associated with medical conditions

High Resolution Manometry (HRM)

Key Features

  • Investigation of choice for motor disorders of the esophagus
  • Performed in both supine and standing positions
  • Graph Representation:
    • EPT (Esophageal Pressure Tomography) also known as Clouse plot
    • X-axis: Time
    • Y-axis: Length of esophagus
  • Sensors:
    • 36 solid-state sensors placed at 1 cm intervals
  • Requires 10 test swallows of 5 ml water

Clinical Applications

  • Preoperative evaluation of Achalasia and GERD
  • Partial wrap procedure with motor disorder in GERD
  • Diagnoses motor disorders such as Achalasia, Distal Esophageal Spasm (DES)

Technical Aspects

  • Intraluminal pressure sensor:
    • Water-perfused or solid-state
  • Conventional manometry:
    • 3-8 sensors placed 3-5 cm apart
  • HRM:
    • 36 solid-state sensors at 1 cm intervals
  • Conducted in supine or standing positions
  • Includes 30-second basal period followed by 10 test swallows (5 ml water each)

EPT (Esophageal Pressure Tomography)

  • Y-axis: Length of esophagus
  • X-axis: Time
  • Hot colors (Red/Orange) and Cool colors (Green/Blue) represent pressure levels

Contractile Deceleration Point

  • Represents the inflexion point
  • Transection point between the initial and terminal portions of the 30 mm Hg isobaric contour

Assessment Parameters

Evaluation of Esophagogastric Junction (EGJ)

  • Pressure
  • Relaxation or Non-relaxation of the EGJ

Peristalsis

  • Significant break in peristalsis can be normal
  • Large break (>5 cm): Indicates fragmented peristalsis
  • Contractile vigor (DCI - Distal Contractile Integral) measured using a 20 mm isobaric contour

Contraction Vigor (DCI)

  • Failed: DCI < 100 mm Hg-s-cm
  • Weak: DCI > 100 mm Hg-s-cm but < 450 mm Hg-s-cm
  • Ineffective: Either Failed or Weak
  • Normal: DCI > 450 mm Hg-s-cm but < 8000 mm Hg-s-cm
  • Hypercontractile: DCI > 8000 mm Hg-s-cm

Contraction Pattern

  • Distal Latency (DL): Time between the onset of UES relaxation and Contractile Deceleration Point (CDP) – represents the period of quiescence.
    • Premature Contraction:
      • DL < 4.5 seconds
    • Fragmented Contraction:
      • Large break (>5 cm) in the 20 mm Hg isobaric contour
      • DCI > 450 mm Hg-s-cm
    • Normal Contraction:
      • Not achieving any of the above diagnostic criteria

Intra-bolus Pressure Pattern (30-mm Hg Isobaric Contour)

  • Pan-esophageal Pressurization:
    • Uniform pressurization extending from the UES to the EGJ
  • Compartmentalized Esophageal Pressurization:
    • Pressurization extending from the contractile front to the EGJ
  • EGJ Pressurization:
    • Pressurization restricted to the zone between the LES and CD (with LES-CD separation, i.e., presence of a hiatal hernia)
  • Normal Pressurization:
    • No bolus pressurization >30 mm Hg

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Hierarchy Diagnostic Algorithm of Esophageal Motility Disorders (Chicago Classification 4.0)

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Step 1: Perform 10 Wet Swallows (Primary Position)

  1. Abnormal Median IRP (Integrated Relaxation Pressure)
    • Yes → Continue with diagnostic steps for Disorders of OGJ Outflow
    • No → Proceed to Step 2 for secondary position + MRS/RDC

Disorders of OGJ Outflow

  1. Achalasia I
    • 100% failed peristalsis without pan-esophageal pressurization (POP)
  2. Achalasia II
    • 100% failed peristalsis with POP in ≥20% of swallows
  3. Achalasia III
    • 20% of swallows with premature contractions
    • Failed peristalsis and POP may be present
  4. OGJOO (Esophagogastric Outflow Obstruction)
    • Elevated LOS IRP persists in varying positions + elevated IBP/POP
    • Abnormal TBO or FLIP

If No Abnormal Median IRP

  • Continue to Step 2: Wet swallows in secondary position + MRS/RDC

Step 2: Wet Swallows in Secondary Position + MRS/RDC

  1. 100% Absent Peristalsis
    • All swallows are either failed or premature
    • Diagnose disorders based on peristalsis pattern:
      • Absent Contractility
      • Distal Esophageal Spasm
      • Hypercontractile Esophagus
      • Ineffective Esophageal Motility

Key Diagnostic Outcomes

  • Absent Contractility: 100% failed peristalsis
  • Distal Esophageal Spasm: ≥20% swallows with premature contractions
  • Hypercontractile Esophagus: ≥20% swallows with hypercontractility
  • Ineffective Esophageal Motility: >70% ineffective or ≥50% failed swallows

Consider Meal Challenges Based on Symptoms

This algorithm helps in differentiating Disorders of OGJ Outflow and Disorders of Peristalsis based on IRP, peristalsis patterns, and further tests in secondary positions. You can now integrate this into your revision notes.

Chicago Classification of Esophageal Motility (Table 8.2) SKF

Diagnostic Criteria

Achalasia and EGJ Outflow Obstruction

  1. Type I Achalasia (Classic Achalasia)
    • Elevated median IRP > 15 mm Hg
    • 100% failed peristalsis (DCI < 100 mm Hg-s-cm)
  2. Type II Achalasia (with Esophageal Compression)
    • Elevated median IRP > 15 mm Hg
    • 100% failed peristalsis with pan-esophageal pressurization in ≥ 20% of swallows
  3. Type III Achalasia (Spastic Achalasia)
    • Elevated median IRP > 15 mm Hg
    • No normal peristalsis, premature contractions (spastic) with DCI > 450 mm Hg-s-cm in ≥ 20% of swallows
  4. EGJ Outflow Obstruction
    • Elevated median IRP > 15 mm Hg
    • Evidence of obstruction, but criteria for achalasia types I-III are not met

Other Major Motility Disorders

  1. Absent Contractility
    • Normal IRP, 100% of swallows with failed peristalsis
    • Consider achalasia if IRP is borderline and there is esophageal pressurization
  2. Distal Esophageal Spasm
    • Normal IRP, ≥ 20% premature contractions with DCI > 450 mm Hg-s-cm
    • Some normal peristalsis may be present
  3. Hypercontractile Esophagus (Jackhammer)
    • At least two swallows with DCI > 8000 mm Hg-s-cm
    • Hypercontractility may involve or be localized to the LES

Minor Disorders of Peristalsis

  1. Ineffective Esophageal Motility (IEM)
    • ≥ 50% ineffective swallows
    • Ineffective swallows may be failed or weak (DCI < 450 mm Hg-s-cm)
    • Multiple repetitive swallow assessments may help determine peristaltic reserve
  2. Fragmented Peristalsis
    • ≥ 50% fragmented contractions with DCI > 450 mm Hg-s-cm
  3. Normal Esophageal Motility
    • Not fulfilling any of the above classifications

This format should fit well into your revision notes.

Achalasia Cardia

Overview

  • Disorder of both the body and LES
  • Most common motor disorder, idiopathic in nature
  • Equal male-to-female prevalence, age range 30-60
  • Associated conditions:
    • Trypanosoma cruzi (Chagas disease)
    • Allgrove syndrome: Achalasia, ACTH adrenal insufficiency, neurological disturbances
    • Inhibitory neurons of Auerbach's plexus to the LES are affected

Clinical Features

  • Dysphagia, regurgitation, and weight loss
  • Megaesophagus, esophagitis, pulmonary infections
  • Considered a premalignant condition: 8% risk after 20 years; most common malignancies:
    • Squamous Cell Carcinoma (SCC)
    • Adenocarcinoma (ACC)

Diagnosis

  • Barium Swallow:
    • Bird beak or Rat tail appearance
    • Type III Achalasia: Corkscrew esophagus (also seen in DES)
    • Sigmoid esophagus
  • Timed Barium Swallow: Radiographs taken at 1 and 5 minutes
  • X-ray: Absence of gastric air bubble
  • UGI Series: Resistance at LES, normal endoscopy
  • Pseudoachalasia: Mimics achalasia, caused by gastric cardia cancer

Manometry (Gold Standard)

  • Absence of relaxation of LES (IRP > 15 mm Hg)
  • Absent peristalsis
    • Esophageal body pressurization (PEP)
    • Simultaneous mirrored contractions (Premature contractions)
    • Low amplitude waveforms indicating muscle tone loss
  • Types of Achalasia:
    1. Type I: IRP > 15 mm Hg, absent peristalsis
    2. Type II: IRP > 15 mm Hg, absent peristalsis, pan-esophageal pressurization
    3. Type III: IRP > 15 mm Hg, premature high-amplitude contractions
  • FLIP (Functional Lumen Imaging Probe): Assesses the esophageal lumen

Non-Operative Treatment

  • NTG and Calcium Channel Blockers
  • Botox: Presynaptic inhibitor of acetylcholine
    • Success rate: 40% in a year, multiple injections needed
    • Especially for elderly patients who are poor surgical candidates
    • Success rate: 54% after 1 year

Botulinum Toxin

  • Blocks acetylcholine release from motor neurons
  • Requires multiple sessions
  • Not long-lasting; used primarily in high-risk elderly patients

Pneumatic Dilatation

  • Dilatation of LES to at least 30 mm
  • Uses plastic balloons (e.g., Rigiflex dilator, Witzel dilator)
  • Serial dilatations: 30-35-40 mm
  • Success rate: 70-90%
  • Best response in patients:
    • Age > 45, female, undilated esophagus, Type II
  • Risk of perforation: 0.4-5%

Heller's Myotomy

  • Laparoscopic Heller's Myotomy is the treatment of choice
    • 6 cm on esophagus, 2-3 cm on gastric side
    • Divides longitudinal and circular muscles
    • Performed with Dor or Toupet fundoplication
    • Success rate: 90% at 2 years
  • Most common cause of failure: Inadequate myotomy on the gastric side
  • GORD occurs in 40% of cases

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Complications of Heller's Myotomy

  • Most common complication: Mucosal perforation
    • Occurrence rate: 2.9-6%
    • Can be repaired laparoscopically with similar outcomes

Results of Heller's Myotomy

  • 90% symptom improvement
  • Dysphagia rates increase over time
  • GERD occurs in 6% to 41.7%
  • Without fundoplication, 100% of patients develop GERD

POEM (Per Oral Endoscopic Myotomy)

Overview

  • Introduced by Hary Inoue in Japan
  • Involves division of the circular muscle, with or without the longitudinal muscle, after creating a mucosal tunnel
  • Efficacy is similar to Heller's Myotomy
  • Treatment of choice for Type III Achalasia (requires longer myotomy)
  • Can be performed for:
    • Redo surgery (posterior POEM)
    • Sigmoid esophagus

Complications

  • Reflux is a more frequent long-term complication compared to Heller's (occurs in 57% of patients)

Procedure

  • Performed under general anesthesia (GA) with endotracheal intubation

  • Steps:

    • Saline lift and mucosotomy
    • Submucosal tunneling
    • Circular muscle myotomy (mediastinal exposure, splitting of longitudinal muscle)
    • Myotomy closure
    • Extended proximal myotomy for Type III Achalasia

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Contraindications

  • No absolute contraindications except inability to tolerate general anesthesia
  • Large hiatus hernia is a relative contraindication
  • Can be performed for failed Heller's Myotomy and sigmoid esophagus

Results

  • Greater than 90% symptom relief, with complete relief from dysphagia
  • Eckardt Clinical Score evaluates:
    • Dysphagia, chest pain, regurgitation, weight loss
  • Normal post-procedure findings:
    • Pneumomediastinum and pneumoperitoneum (due to CO2 leak)
  • 10-40% of patients experience reflux symptoms similar to Heller's Myotomy

This detailed breakdown should fit well into your revision notes.

Diffuse Esophageal Spasm

Overview

  • Disorder of the esophageal body with normal LES
  • Characterized as distal esophageal spasm
  • Commonly seen in females with multiple medical conditions (e.g., IBS, pylorospasm)
  • Abnormality of the esophageal body: repetitive simultaneous, high-amplitude contractions

Clinical Features

  • Chest pain and dysphagia
  • Triggers:
    • Acid, cold water, stress, gallstones, peptic ulcer disease

Diagnosis

  • Barium Swallow:
    • Corkscrew esophagus or pseudodiverticulosis
    • Presence of tertiary contractions
  • Manometry:
    • IRP is normal
    • Distal Latency (DL) < 4.5 seconds (≥20% premature contractions)
    • DCI > 450 mm Hg-s-cm
    • Can have normal peristalsis

Management

  • Conservative
  • Treat underlying medical conditions
  • Psychiatric evaluation
  • Medications:
    • Nitrates
    • Calcium Channel Blockers (CCB)
    • Peppermint oil
  • Bougie dilation or Botulinum toxin
  • Long myotomy or POEM

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Nutcracker Esophagus

Overview

  • Disorder of the esophageal body
  • Equal prevalence in males and females, affects all ages

Diagnosis

  • DCI > 8000 mm Hg-s-cm, normal LES pressure
  • May have a normal barium esophagogram

Question 1: 24-year-old female with epigastric pain and dysphagia

  • Question: A 24-year-old female presents with epigastric pain and dysphagia. She gives a history of food regurgitation. Upper GI endoscopy is normal. Further work-up includes all except:
    • A. pH study
    • B. Barium swallow
    • C. CECT chest and abdomen
    • D. X-ray abdomen
  • Answer: C. CECT chest and Abdomen

Workup of Achalasia

  • Upper GI endoscopy
  • Gold standard: High-Resolution Manometry (HRM)
  • pH study: To rule out GERD as a cause of dysfunction
  • CECT chest and abdomen: Not uniformly required, used to rule out pseudoachalasia

Question 2: Esophageal body dysfunction

  • Question: Which of the following disorders is a dysfunction of the esophageal body?
    • A. Achalasia
    • B. Distal esophageal spasm
    • C. Scleroderma
    • D. All of the above
  • Answer: D. All of the above

Classification of LES and Body disorders

Disorders of LES and Body

  • Achalasia
  • EGJOO (Esophagogastric Junction Outflow Obstruction)

Disorders of the Esophageal Body

  • DES (Distal Esophageal Spasm)
  • Nutcracker Esophagus
  • Hypertensive LES

Primary Esophageal Disorders

  • Achalasia
  • DES (Distal Esophageal Spasm)
  • IEM (Ineffective Esophageal Motility)

Secondary Esophageal Disorders

  • Scleroderma
  • Diabetes Mellitus (DM)
  • Polymyositis (PM)
  • Lupus

Question 1: Characteristics of Diffuse Esophageal Spasm (DES)

  • Question: Which of the following is not true about Diffuse Esophageal Spasm (DES)?
    • A. 100% absent peristalsis
    • B. Disorder of esophageal body
    • C. Degeneration of vagus nerve is seen
    • D. High amplitude, premature contraction is seen
  • Answer: A. 100% absent peristalsis

Key Points about DES

  • Distal esophageal spasm, more common in the lower third of the esophagus
  • Muscular hypertrophy and degeneration of the vagus nerve are seen
  • High amplitude premature contractions occur
  • Peristalsis is not completely absent (hence, option A is incorrect)

Question 2: Management of Corkscrew Esophagus

  • Question: A 35-year-old female complains of chest pain and dysphagia. She is a known case of GERD with irregular bowel habits. Upper GI endoscopy is normal. Barium shows the following finding (Corkscrew esophagus). Which of the following is true?
    • A. Barium appearance is specific for this disorder
    • B. POEM is the treatment of choice
    • C. Bougie dilatation provides 70-80% relief of dysphagia
    • D. CT scan is mandatory to rule out secondary disorder
  • Answer: C. Bougie dilatation provides 70-80% relief of dysphagia

Evaluation of DES

  • Barium Study:
    • Corkscrew esophagus or pseudodiverticulosis
    • Tertiary contractions seen in advanced disease
    • Distal bird beaking or normal peristalsis
  • HRM (High-Resolution Manometry):
    • Normal IRP
    • ≥ 20% premature contractions
    • DCI > 450 mm Hg-s-cm

Treatment of DES

  • Identification of trigger factors: Gallstones, peptic ulcer disease, acid reflux, specific foods
  • Non-surgical management is the mainstay:
    • Psychiatric evaluation for stress-related symptoms
    • Medications:
      • Calcium Channel Blockers (CCB)
      • Nitrates
      • Proton Pump Inhibitors (PPI)
      • Peppermint oil
    • Bougie dilatation: Provides 70-80% relief from dysphagia
  • Surgical Management:
    • Performed in cases of failed medical or endoscopic treatment
    • Surgery for complications like pulsion diverticulum
    • Long esophagomyotomy with dor fundoplication
    • POEM