Esophageal motor disorders Achalasia Prof.Dr.Khalid A. Jasim Al-Khazraji M.B.CH.B, MD, C.A.B.M, FRCP, FACP

I- Oropharyngeal dysphagia: due to neuromuscular disorders of the oropharynx and the skeletal portion of esophagus, including: stroke, Parkinson's disease, amyotrophic lateral sclerosis, MS, myasthenia gravis, polymyositis, and myotonic dystrophy II- Esophageal dysphagia:- include 1- Achalasia. 2- Diffuse esophageal spasm. 3- Scleroderma. 4- Nutcracker esophagus. 5- Hypertensive LES. 6- Ineffective peristalsis.

Achalasia : term means “failure to relax”, define as aperistalsis and impaired relaxation of LES. -Incidence: 1: , Male = female. -Usually develops in middle life but can occur in all ages event it’s rare in childhood. Clinical features:- 1- Dysphagia: predominant symptom in nearly all patients, progressive, often first to solids and then to liquids. Worsened by emotional stress and hurried eating. 2- Regurgitation of undigested food happen especially at night. 3- Chest pain: due to esophageal spasm. 4- Weight loss. Heartburn does not occur. Dilated, fluid-filled esophagus

Pathogenesis -The aetiology is unknown. -Abnormalities detected in both muscle and nerve components, but neural lesions the primary one. - Degeneration of nerves in Auerbach’s plexus. -Defective release of nitric oxide by inhibitory neurons in LES. -Reduction in number of ganglion cells. -Loss of the dorsal vagal nuclei within brain stem in later stages. -Infection with Trypansoma cruzi in chagas’ disease cause syndrome indistinguishable from achalasia. - Some patients have autoantibodies to a dopamine – carrying protein on the surface of the cells in the myenteric plexus.

1- Chest X-ray: dilated esophagus, fluid level seen behind heart, fundal gas shadow is absent. Investigations

2- Barium swallow: lack of peristalsis, synchronous contraction in the body of esophagus, sometime with dilatation. Lower end shows a “swan neck deformity” due to failure of the sphincter to relax.

3- Esophageoscopy: should always be done to exclude a carcinoma of cardia that mimic the presentation and radiological and manometric features of achalasia ( pseudo-achalasia). In true achalasia the endoscope passes through LES with little resistance. 4- CT scan:- exclude distal esophageal cancer. A coronal CT image showing marked dilatation of the esophagus.

5- Manometry:- shows aperistalsis and failure of relaxation of LES. Confirms the high pressure, non-relaxing LES, with poor contractility of the esophageal body. The tracing illustrates the findings in classic achalasia with esophageal body aperistalsis with low-amplitude simultaneous esophageal body contractions and failed relaxation of the LES.

Differential Diagnosis 1- Malignancy ( e.g. gastric adenocarcinoma, esophageal squamous cell carcinoma, lymphoma, lung carcinoma, pancreatic carcinoma, ….etc.). 2- Amyloidosis. 3- Sarcoidosis. 4- Chagas’ disease. 5- Postvagotomy disturbance.

Treatment -All current forms of treatment are palliative. 1- Drug therapy: Nitrites, Calcium channel blockers (nifedipine, diltiazem), sildenafil. But rarely produce satisfactory relief. 2- Endoscopy: Dilatation of LES using hydrostatic balloon under x-ray control is treatment of choice (successful initially in 80% of cases, about 50% required a 2nd or 3rd dilatation in the first 5 years with low but significant risk of perforation).

Intrasphincteric injection of Botulinum toxin A Produce satisfactory initial results but the effects wear off within Months and further injections needed but it’s safer and simpler than dilation. BEFORE AFTER

3- Surgery: If the Last measures fail; Surgical division of LES is performed (cardiomyotomy or Heller’s operation). -But reflux esophagitis complicates all procedures & the aperistalsis remains.

Complications 1- There is slight increase in the incidence of squamous carcinoma of the esophagus in both treated and untreated cases, 7% in after 25 years. 2- Esophagitis. 3- Aspiration of esophageal contents.

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