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Achalasia

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

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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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