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Achalasia

Learn about achalasia, a rare disorder affecting the esophagus, its clinical features, pathophysiology, stages, and differential diagnosis. Discover the history, symptoms, investigations, and treatment options available. Medical professionals and patients can find valuable insights on this condition.

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Achalasia

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

  2. 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).]

  3. 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.

  4. Who is this

  5. Who described this?

  6. Who is this?

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

  8. History • Term Achalasiacioned by Hurt and Rake 1929 • First laparoscopic Hellersperforme by Shimi in UK 1991 • Botox introduced 1994

  9. Clinical Features • 1/100000 • Equal sex distribution • Occurs at all ages especially after seventh decade

  10. Clinical Symptoms • Dysphagia • Regurgitation (80-90%) • Chest Pain (17-63%) • Heartburn/Cough/Recurrent Chest Infection/Weight loss

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

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

  13. Chest pain • Mechanism unclear • Oesophageal Distention • Oesophageal irritation • Tertiary contraction • No correlation with manometry • 84% resolved with Manometry • Heterogenous cause

  14. 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

  15. Huh?!? • What does that all mean • Oesophageal Motility • Sphincter dysmotility

  16. Aetiology • Viral • Autoimmune • Allergy • But truly no one knows

  17. Investigations • Barium Swallow • Endoscopy and Biopsy • Manometry

  18. Barium Swallow • Characteristic Findings • Aperistalsis of Distal Oesophagus • Bird Beaking • Dilatation or tortuousity

  19. Figure 1 Esophagrams of a patient with early achalasia pre- and posttreatment. GI Motility online (May 2006) | doi:10.1038/gimo53

  20. 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

  21. 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.

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

  23. Manometry • Characteristic findings • Absence of peristalsis • Pressure maybe hypertonic (VigourousAchalasia) • Pressure maybe hypotonic • May have distal barrier function (Non relaxing sphincter)

  24. Figure 2 Esophageal manometric findings in achalasia. GI Motility online (May 2006) | doi:10.1038/gimo22

  25. Figure 3 Contour plot topographic analysis of esophageal motility in achalasia. GI Motility online (May 2006) | doi:10.1038/gimo22

  26. Figure 4 Esophageal manometric findings in vigorous achalasia. GI Motility online (May 2006) | doi:10.1038/gimo22

  27. Figure 5 Esophageal manometric findings in achalasia variant with preserved LES relaxation. GI Motility online (May 2006) | doi:10.1038/gimo22

  28. Differential Diagnosis • Secondary Achalasia • Cancer • Infection • Allergy • Other Oesophageal Dysmotilities • Diffuse Oesophageal Spasm • Presbyoesophagus • Scleroderma

  29. 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

  30. Treatment • Conservative • Medical • Drugs • Botox • Dilatation • Surgical • Hellers • Oesophagectomy

  31. Conservative • Dietetic Support • Enteral forms of feeding • Stent

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

  33. 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

  34. 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

  35. 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%

  36. Botox • Increases operative complications • Recommended only for • Elderly • Low pressure sphincter

  37. Dilatation vs Botox

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

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

  40. Medical vs Surgical

  41. 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

  42. Questions

  43. Other Dysmotilities

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