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Achalasia. Mr Yuen Soon Laparoscopic Tutor Consultant Oesophagogastric and Laparoscopic Surgeon. Definition.

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Achalasia

Achalasia

Mr Yuen Soon

Laparoscopic Tutor

Consultant Oesophagogastric and Laparoscopic Surgeon


Definition
Definition

ach·a·la·sia   (āk'ə-lā'zhə)    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 khalān, to loosen).]


Definition1
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.





History
History

  • First described by Sir Thomas Willis 1672

  • Described as Cardiospasm by Von Mikulicz 1881

  • Ernest Heller performed the first operation 1913


History1
History

  • Term Achalasiacioned by Hurt and Rake 1929

  • First laparoscopic Hellersperforme by Shimi in UK 1991

  • Botox introduced 1994


Clinical features
Clinical Features

  • 1/100000

  • Equal sex distribution

  • Occurs at all ages especially after seventh decade


Clinical symptoms
Clinical Symptoms

  • Dysphagia

  • Regurgitation (80-90%)

  • Chest Pain (17-63%)

  • Heartburn/Cough/Recurrent Chest Infection/Weight loss


Dysphagia
Dysphagia

  • Inability to swallow

    • Non prgressive

    • Constant

  • Due to

    • motility dysfunction

    • Cardiac spasm


Regurgitation
Regurgitation

  • Food refluxing from distal to proximal oesophagus

    • Usually stale food

    • Predisposes to

      • Halitosis

      • Chest infections

  • Sometimes mistaken for heartburn


Chest pain
Chest pain

  • Mechanism unclear

    • Oesophageal Distention

    • Oesophageal irritation

    • Tertiary contraction

  • No correlation with manometry

  • 84% resolved with Manometry

  • Heterogenous cause


Pathophysiology
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


Achalasia
Huh?!?

  • What does that all mean

    • Oesophageal Motility

    • Sphincter dysmotility


Aetiology
Aetiology

  • Viral

  • Autoimmune

  • Allergy

  • But truly no one knows


Investigations
Investigations

  • Barium Swallow

  • Endoscopy and Biopsy

  • Manometry


Barium swallow
Barium Swallow

  • Characteristic Findings

    • Aperistalsis of Distal Oesophagus

    • Bird Beaking

    • Dilatation or tortuousity


Achalasia

Figure 1 Esophagrams of a patient with early achalasia pre- and posttreatment.

GI Motility online (May 2006) | doi:10.1038/gimo53


Achalasia

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) | doi:10.1038/gimo29


Stages of achalasia
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.


Endoscopy
Endoscopy

  • To ensure no other causes of symptoms

  • Usual findings

    • Excess stale food in oesophagus

    • Candidiasis


Manometry
Manometry

  • Characteristic findings

    • Absence of peristalsis

    • Pressure maybe hypertonic (VigourousAchalasia)

    • Pressure maybe hypotonic

    • May have distal barrier function

      (Non relaxing sphincter)


Achalasia

Figure 2 Esophageal manometric findings in achalasia.

GI Motility online (May 2006) | doi:10.1038/gimo22


Achalasia

Figure 3 Contour plot topographic analysis of esophageal motility in achalasia.

GI Motility online (May 2006) | doi:10.1038/gimo22


Achalasia

Figure 4 Esophageal manometric findings in vigorous achalasia.

GI Motility online (May 2006) | doi:10.1038/gimo22


Achalasia

Figure 5 Esophageal manometric findings in achalasia variant with preserved LES relaxation.

GI Motility online (May 2006) | doi:10.1038/gimo22


Differential diagnosis
Differential Diagnosis

  • Secondary Achalasia

    • Cancer

    • Infection

    • Allergy

  • Other Oesophageal Dysmotilities

    • Diffuse Oesophageal Spasm

    • Presbyoesophagus

    • Scleroderma


Achalasia

  • 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 (Trypanosomacruzi)

  • Paraneoplastic syndrome (Anti-Hu antibody)17, 18

  •   Parkinson's disease8

  •   Amyloidosis43, 44

  • Eosinophilic gastroenteritis45, 46

  • Fabry's disease47

  •   Down syndrome

  •   Hereditary cerebellar ataxia37

  • Achalasia with associated Hirschsprung's disease15

  •   Hereditary hollow visceral myopathy16

  • Achalasia associated with cancer. (Cancer-associated achalasia may be due to local invasion of the esophagealmyenteric plexus or as a part of a paraneoplastic syndrome.)

  • Squamous cell carcinoma of the esophagus

  • Adenocarcinoma of the esophagus

  •   Gastric adenocarcinoma

  •   Lung carcinoma

  • Leiomyoma

  •   Lymphoma

  •   Breast adenocarcinoma

  • Hepatocellular carcinoma

  •   Reticulum cell sarcoma

  • Lymphangioma

  •   Metastatic renal cell carcinoma

  • Mesothelioma

  •   Metastatic prostate carcinoma

  •   Pancreatic adenocarcinoma


Treatment
Treatment

  • Conservative

  • Medical

    • Drugs

    • Botox

    • Dilatation

  • Surgical

    • Hellers

    • Oesophagectomy


Conservative
Conservative

  • Dietetic Support

  • Enteral forms of feeding

  • Stent


Medical
Medical

  • Drugs

    • Seldom long lasting

    • Seldom effective

    • Nitrates (GTN)

    • Calcium Channel Antagonist (Nifedipine)

    • Sildenafil (Viagra)


Medical1
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


Medical2
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


Medical3
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%


Botox
Botox

  • Increases operative complications

  • Recommended only for

    • Elderly

    • Low pressure sphincter



Surgery
Surgery

  • Laparoscopic

  • Long myotomy 6-8cm above and 3 below

  • Good long term results for dysphagia 90-95%


Surgery1
Surgery

  • Reflux 17-28% to 6% if antireflux procedure added

  • Oesophageal perforations 1-5%

  • Pneumothorax 3%



Surveillance
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