ARANSI RILWAN A.

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)