1. ACHALASIA
BY: BILAL HUSSEIN

2. Objectives
Introduction Pathophysiology Clinical features Diagnosis Manometric types Differential diagnosis Treatment Complications

3. introduction
Achalasia is an esophageal motility disorder characterized by the absence of esophageal peristalsis and impaired relaxation of the lower esophageal sphincter (LES) in response to swallowing. 
During achalasia the LES fails to open up during swallowing, thus cause a backup of food within the esophagus .

4. Pathophysiology

5. *generally the causes are unknown but many theories suggest (infectious, hereditary and autoimmune ) causes. the pathophysiology as follows: -defective release of nitric oxide by inhibitory neurons in LES. -degeneration of ganglion cells within the LES and the body of esophagus. -failure of propagated esophageal contraction leading to progressive dilatation of the gullet . -loss of dorsal vagal nuclei within the brain stem can be demonstrated in the later stages .

6. Clinical features

7. Usually occurs in middle age (can occur in any age).
Usually present with :
Dysphagia
Pain
Rgurgitation
Overspill into the trachea especially at night .
Weight loss.

8. Diagnosis
Manometry
endoscopy
Barium radiology
Plain x-ray

9.  X-ray of the chest may be helpful in diagnosing achalasia as it may show enlarged mediastenum .
 endoscopy will show closed LES with dilated esophagus and accumulation of food.
 barium swallow: during this procedure the patient will swallow barium prepared in liquid form and vissulaize the esophagus under the x-ray while the barium moves down the esophagus.
 esophageal manometry :This involves placing a tube in the esophagus while the patient swallows and the tube records the muscle activity (amplitude, speed of contraction and pressure inside the esophagus).

10. Manometric types of achalasia

11. Differential diagnosis

12. Carcinoma of lower end of esophagus .
Stricture of lower end of esophagus .
Scleroderma .
Pancreas or bronchial cancer .
Hiatal hernia .
Chagas disease .

13. Treatment
medications

14. Most pharmacological treatments aim to help to relax the LES .
these include:
Calcium channel blockers and nitrates are used to decrease LES pressure. Approximately 10% of patients benefit from this treatment. This treatment is used primarily in elderly patients who have contraindications to either pneumatic dilatation or surgery..
Anticholinergic drugs .
Sedatives

15. PROCEDURES

16. Endoscopic treatment includes an intrasphincteric injection of botulinum toxin to block the release of acetylcholine at the level of the LES, thereby restoring the balance between excitatory and inhibitory neurotransmitters. Only 30% of patients treated endoscopically still have relief of dysphagia 1 year after treatment. Most patients need repeated botulinum toxin injections, with short-lasting clinical benefits,it can cause an inflammatory reaction at the level of the gastroesophageal junction, making a subsequent myotomy very difficult. 
Pneumatic dilatation is the recommended treatment in those sporadic cases in which surgery is not appropriate.  A balloon is inflated at the level of the gastroesophageal junction to blindly rupture the muscle fibers while leaving the mucosa intact. The success rate is 70-80%, and the perforation rate is approximately 5%. If a perforation occurs, emergency surgery is needed to close the perforation and to perform a myotomy. As many as 50% of patients may require more than 1 dilatation. The incidence of pathologic gastroesophageal reflux after the procedure is approximately 30%.

17. Hellers myotomy : this treatment provides a fine balance in relieving symptoms of dysphagia by performing the myotomy and in preventing gastroesophageal reflux by adding a partial wrap. A prospective, randomized study from Vanderbilt University indicated that there is significantly less risk of postoperative reflux following a Heller myotomy plus a partial fundoplication than there is after a Heller myotomy alone.

18. POEM

19. POEM : per oral endoscopic myotomy .
Less invasive , efficacy more than 90% and low risk copmlication.

20. Complications
Esophageal cancer
Aspiration pneumonia
esophagitis

21. Esophagus
Respiratory
Malnutrition
Progressive dilatation
Retention esophagitis
Esophageal cancer :
squamous (due to retention esophagitis), adenocarcinoma (due to post treatment reflux-barrett’s epithelium )
Aspiration pneumonia
Dyspnea due to extrinsic tracheal compression .

22. Reference
Davidson’s principles and practice of medicine , 22nd edition , part 2 ,chapter 22 ,p 868 .
Bailey and love’s short practice of surgery ,26th edition ,part 11 ,chapter 62 , p

23. THANK YOU