Achalasia

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Achalasia

• Mr Yuen Soon
• Laparoscopic Tutor
• Consultant Oesophagogastric and Laparoscopic
  Surgeon

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Definition

• achalasia   (ak'?-la'zh?)    n.  The failure
  of a ring of muscle fibers, such as a sphincter
  of the esophagus, to relax.New Latin a-1
  Greek khalasis, relaxation (from khalan, to
  loosen).

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Definition

• Achalasia is primary a disorder of motility of
  the lower oesophageal or cardiac sphincter. The
  smooth muscle layer of the oesophagus has
  impaired peristalsis and failure of the sphincter
  to relax causes a functional stenosis or
  functional oesophageal stricture.

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Who is this
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Who described this?
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Who is this?
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History

• First described by Sir Thomas Willis 1672
• Described as Cardiospasm by Von Mikulicz 1881
• Ernest Heller performed the first operation 1913

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History

• Term Achalasia cioned by Hurt and Rake 1929
• First laparoscopic Hellers performe by Shimi in
  UK 1991
• Botox introduced 1994

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Clinical Features

• 1/100000
• Equal sex distribution
• Occurs at all ages especially after seventh decade

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Clinical Symptoms

• Dysphagia
• Regurgitation (80-90)
• Chest Pain (17-63)
• Heartburn/Cough/Recurrent Chest Infection/Weight
  loss

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Dysphagia

• Inability to swallow
• Non prgressive
• Constant
• Due to
• motility dysfunction
• Cardiac spasm

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Regurgitation

• Food refluxing from distal to proximal oesophagus
• Usually stale food
• Predisposes to
• Halitosis
• Chest infections
• Sometimes mistaken for heartburn

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Chest pain

• Mechanism unclear
• Oesophageal Distention
• Oesophageal irritation
• Tertiary contraction
• No correlation with manometry
• 84 resolved with Manometry
• Heterogenous cause

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Pathophysiology

• Loss of nerve cells in the oesophagus
• Fibrosis and inflammation
• Hypertrophy and degeneration of oesophageal
  muscle
• Loss of Nitric Oxide deficiency
• Preservation of Acetyl Choline Nerves and other
  promoters of muscle tone
• Eosinophils

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Huh?!?

• What does that all mean
• Oesophageal Motility
• Sphincter dysmotility

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Aetiology

• Viral
• Autoimmune
• Allergy
• But truly no one knows

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Investigations

• Barium Swallow
• Endoscopy and Biopsy
• Manometry

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Barium Swallow

• Characteristic Findings
• Aperistalsis of Distal Oesophagus
• Bird Beaking
• Dilatation or tortuousity

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Figure 1 Esophagrams of a patient with early
achalasia pre- and posttreatment.
GI Motility online (May 2006) doi10.1038/gimo53
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Figure 1 a Barium esophagram showing a dilated,
tortuous esophagus and a bird's beak appearance
of the lower esophageal sphincter (LES).
GI Motility online (May 2006) doi10.1038/gimo29
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Stages of Achalasia

• 2-3 cm is normal
• 4-5 cm is stage two and bird beak looking
• 5-7 cm is stage three
• 8 cm is sigmoid or stage 4.

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Endoscopy

• To ensure no other causes of symptoms
• Usual findings
• Excess stale food in oesophagus
• Candidiasis

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Manometry

• Characteristic findings
• Absence of peristalsis
• Pressure maybe hypertonic (Vigourous Achalasia)
• Pressure maybe hypotonic
• May have distal barrier function
• (Non relaxing sphincter)

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Figure 2 Esophageal manometric findings in
achalasia.
GI Motility online (May 2006) doi10.1038/gimo22
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Figure 3 Contour plot topographic analysis of
esophageal motility in achalasia.
GI Motility online (May 2006) doi10.1038/gimo22
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Figure 4 Esophageal manometric findings in
vigorous achalasia.
GI Motility online (May 2006) doi10.1038/gimo22
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Figure 5 Esophageal manometric findings in
achalasia variant with preserved LES relaxation.
GI Motility online (May 2006) doi10.1038/gimo22
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Differential Diagnosis

• Secondary Achalasia
• Cancer
• Infection
• Allergy
• Other Oesophageal Dysmotilities
• Diffuse Oesophageal Spasm
• Presbyoesophagus
• Scleroderma

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• Achalasia
•   Allgrove's syndrome (AAA syndrome)10, 36
•   Hereditary cerebellar ataxia37
•   Familial achalasia38
•   Sjögren's syndrome39
•   Sarcoidosis40
•   Postvagotomy41
•   Autoimmune polyglandular syndrome type II11
• Achalasia with generalized motility disorder
•   Multiple endocrine neoplasia (MEN) IIb
  (Sipple's syndrome)12, 42
•   Neurofibromatosis (von Recklinghausen's
  Disease)13
•   Chagas' disease (Trypanosoma cruzi)
•   Paraneoplastic syndrome (Anti-Hu antibody)17,
  18
•   Parkinson's disease8
•   Amyloidosis43, 44
•   Eosinophilic gastroenteritis45, 46
•   Fabry's disease47
•   Down syndrome
•   Hereditary cerebellar ataxia37

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Treatment

• Conservative
• Medical
• Drugs
• Botox
• Dilatation
• Surgical
• Hellers
• Oesophagectomy

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Conservative

• Dietetic Support
• Enteral forms of feeding
• Stent

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Medical

• Drugs
• Seldom long lasting
• Seldom effective
• Nitrates (GTN)
• Calcium Channel Antagonist (Nifedipine)
• Sildenafil (Viagra)

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Medical

• Dilatation
• 60 success at a year and 24 at 5 years
  following single dilatation
• Symptoms reoccur in 50 within 5 years
• In general 60 have good results at 5 years with
  one or more dilatation

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Medical

• Predictors of good outcome
• Low residual pressure
• Older patients
• Complications
• Perforation 3-7 (0-21 Range)
• Reflux 2
• Higher rate of complication if followed by myotomy

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Medical

• Botox
• High quality symptom relief
• 1 month 75-100
• 6 month 44-100
• Duration of response upto 15 months
• 50 will need other forms of treatment within 2
  years
• Reduces Sphincteric pressure by 40

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Botox

• Increases operative complications
• Recommended only for
• Elderly
• Low pressure sphincter

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Dilatation vs Botox
Author Annese89 Annese89 Vaezi86 Vaezi86 Mikaeli36 Ghoshal87 Ghoshal87 Muehldorfer88 Muehldorfer88
  BoT PD BoT PD BoT PD BoT PD BoT PD
These authors used 200 U of Dysport. These authors used 200 U of Dysport. These authors used 200 U of Dysport. These authors used 200 U of Dysport. These authors used 200 U of Dysport. These authors used 200 U of Dysport. These authors used 200 U of Dysport. These authors used 200 U of Dysport. These authors used 200 U of Dysport. These authors used 200 U of Dysport. These authors used 200 U of Dysport.
NR not reported LESP lower esophageal sphincter pressure. NR not reported LESP lower esophageal sphincter pressure. NR not reported LESP lower esophageal sphincter pressure. NR not reported LESP lower esophageal sphincter pressure. NR not reported LESP lower esophageal sphincter pressure. NR not reported LESP lower esophageal sphincter pressure. NR not reported LESP lower esophageal sphincter pressure. NR not reported LESP lower esophageal sphincter pressure. NR not reported LESP lower esophageal sphincter pressure. NR not reported LESP lower esophageal sphincter pressure. NR not reported LESP lower esophageal sphincter pressure.
Modified from Zhao and Pasricha.67 Modified from Zhao and Pasricha.67 Modified from Zhao and Pasricha.67 Modified from Zhao and Pasricha.67 Modified from Zhao and Pasricha.67 Modified from Zhao and Pasricha.67 Modified from Zhao and Pasricha.67 Modified from Zhao and Pasricha.67 Modified from Zhao and Pasricha.67 Modified from Zhao and Pasricha.67 Modified from Zhao and Pasricha.67
Response rate Response rate Response rate Response rate Response rate Response rate Response rate Response rate Response rate Response rate Response rate
1 month 8/8 8/8 14/20 15/20 13/20 18/20 6/8 8/10 9/12 10/12
12 months 6/8 NR 7/20 14/20 3/20 10/19 3/8 6/8 NR NR
Reduction in LESP Reduction in LESP Reduction in LESP Reduction in LESP Reduction in LESP Reduction in LESP Reduction in LESP Reduction in LESP Reduction in LESP Reduction in LESP Reduction in LESP
1 month -49 -72 -6 -66 -24 -26 -53 -62 -44 -51
12 months NR NR NR NR NR NR NR NR NR NR
Reduction in retention Reduction in retention Reduction in retention Reduction in retention Reduction in retention Reduction in retention Reduction in retention Reduction in retention Reduction in retention Reduction in retention Reduction in retention
1 month 47 59 33.6 50.2 NR NR NR NR NR NR
12 months NR NR 13.4 54.6 NR   NR NR NR NR
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Surgery

• Laparoscopic
• Long myotomy 6-8cm above and 3 below
• Good long term results for dysphagia 90-95

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Surgery

• Reflux 17-28 to 6 if antireflux procedure added
• Oesophageal perforations 1-5
• Pneumothorax 3

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Medical vs Surgical
  Dilation (n 32) Surgery (n 42)
LESP, lower esophageal sphincter pressure. LESP, lower esophageal sphincter pressure. LESP, lower esophageal sphincter pressure.
Source Modified from Csendes et al.34 Source Modified from Csendes et al.34 Source Modified from Csendes et al.34
Type of procedure Mosher dilation (1215 psi for 1020 seconds) Myotomy antireflux (Dor)
Clinical response 65 95
LESP at 5 years ( baseline) 16 mm Hg (50) 10 mm Hg (25)
GE junction at late follow-up ( baseline) 7.2 mm (270) 9 mm (321)
Esophageal diameter at late follow-up ( base line) 29 mm (70) 26 mm (50)
Reflux 8 28
Perforation 5.60
Deaths 0 0
Need for surgery 22
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Surveillance

• Do we need it
• Rise in Squamous cancers of oesophagus
• 33-100x
• ie 3.4/1000 patient years vs 0.1/1000 patient
  years
• If done then needs chromoendoscopy from 10 years
  after symptoms starts

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Questions
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Other Dysmotilities
Motor disorder Manometric abnormality
Achalasia Absent, incomplete, or abnormally timed LES relaxation Absent peristalsis May have elevated resting LES and intraesophageal pressures
Diffuse esophageal spasm gt20 of swallows result in simultaneous contractions May have multipeaked and/or prolonged contractions Amplitude of the contractions may be increased, normal, or decreased
Hypertensive peristalsis (nutcracker esophagus) Peristaltic contractions of increased amplitude (gt180 mmHg) and/or increased duration (gt8 sec)
Hypertensive LES Increased resting LES pressure (gt40 mm Hg above intragastric pressure)
Hypotensive peristaltic contractions (ineffective peristalsis) Decreased amplitude (lt30 mmHg) peristaltic or nonperistaltic contractions in distal esophagus with 30 of wet swallows With or without hypotensive LES
Hypotensive LES and increased frequency of TLESR LES pressure lt10 mmHg TLESRs cannot be evaluated by usual manometric studies