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Information about Achalasia

Published on May 11, 2014

Author: rilaransi



Achalasia presentation, oesophageal motility disorder


 Introduction  Epidemiology  Aetiology  Pathogenesis  Clinical Features  Investigation  Treatment  Complications  Differential diagnosis

A-chalasia (Gr.Word) meaning failure of relaxation. It is a primary oesophageal motility disorder that occurs due to the failure of the normally tonically contracted lower oesophageal sphincter to relax.

 It may spread upwards to involve portions of or the whole oesophagus.  It is characterised by;  1. inability if the cardiac sphincter to relax fully in response to swallowing.  2.hypertrophy and dilatation of the rest of the oesophagus.  3. Absence or diminution of peristalsis in the oesophagus.

Achalasia may occur at any age, however, incidence peaks in individuals in the 3rd and 5th decade of life.  No sex predilection.

 Idiopahic  Proposed causes include;  1.Neuronal degeneration  2.viral infection  3.Chagas disease  4.gastroesophageal junction obstruction  5.genetic inheritance  6.Autoimune disease.

Insults to the oesophagus perhaps a viral infection or some other external factors results in myenteric plexus inflammation. Inflammation leads to autoimmune response in a susceptible population who may be genetically predisposed.

 Subsequent chronic inflammation leads to destruction of the inhibitory nitrigenic myenteric neurons resulting in inability of the L.O .S to relax in response to swallowing.  The muscle, especially the circular muscle, of the rest of the oesophagus overworks to propel food through the L. O. S and thus undergoes hypertrophy.

 The disease process spreads upwards, propulsive peristalsis ceases  Food and saliva now accumulate in the distal oesophagus and only trickle through the L.O.S when the hydrostatic pressure is high enough to overcome the intracardiac pressure.

Intermittent dysphagia. Retrostemal pain which may radiate to the neck and interscapular and subcostal regions. Halithosis

 Regurgitation of food.  Some may present with pulmonary symptoms such as dyspnoea, pneumonitis and chronic cough and purulent expectoration indicative of lung abscess due to aspiration of accumulated food.  Weight loss.

Aim: Confirm diagnosis Complications Optimize for treatment.

 Gold standard for diagnosis is oesophageal manometry, however, these investigations can be done in suspected cases;  Chest radiograph: retrocardiac dilation of the esophagus retrocardiac air-fluid level minimal or abscent gastric bubble signs of aspiration.

 Barium Swallow o Dilated, tortuous, oesophagus that smoothly tapers down at the OGJ giving the "Bird's beak" appearance. o absence of gas in the fundus of the stomach. o weak, irregular, uncoordinated or absent peristalsis on fluoroscopy.

Oesophagoscopy: o The oesophagus contains food debris and is dilated. o Possibility of associated tumour (seen in 5-10%)

 Oesophageal manometry may show a high resting pressure in the cardiac sphincter.  Normal Manometric Findings  LOS pressure 15-25mmHg with normal relaxation on swallowing.  Mean amplitude distal oesophageal peristaltic w.ave is 30-100mmHg

 Resting/ excercise ECG: to rule out cardiac cause of pain.  FBC.  Serum electrolyte, urea and creatinine.  Urinalysis.

 Aim is to reduce the pressure of the L.O.S. so as to allow food to pass into the stomach unimpeded.  Achieved by  i) pharmacological manipulation  ii) dilatation or stretching and disruption of the circu1ar muscle of the L.O.S to render it incompetent and  iii) surgical division of the circu1ar muscle of the L.O.S (cardiomyotomy).

 Calcium channel blockers (e.g. nifedipine) and nitrates (e.g. glyceryl trinitrate), which relax the smooth muscle of the L.O.S have been used.  Taken 10-30minutes before meals .  These are reserved primarily for patients who refuse or are not good candidates for more effective and invasive forms of therapy.

 Botulinum toxin injection:  Botulinum toxin (Botox) is a potent inhibitor of the release of acetylcholine from nerve endings.  It is injected endoscopically into the L.O.S and poisons the excitatory (acetylcholline releasing) neurons that increase the L.O.S tone.

 Cardiomyotomy Surgical intervention is indicated after failure of repeated dilatation, in mega-oesophagus, when associated carcinoma is suspected or as first line treatment.

 1. Shock.  2. Perforation of the oesophagus  3. Bleeding.  4. Mediastinitis.  5. Pneumonia.  6. Septicaemia.  7. Oesophageal Stricture.  8. Gastric outlet obstruction.  9. Malignant change may occur in a strictured oesophagus of more than 16 years duration.

Carcinoma of lower end of oesophagus Stricture of lower end of oesophagus Hiatus hernia Scleroderma

Principles and Practice of Surgical Practice( Badoe) The American Journal of Gastroenterology(2005)

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