Esophageal motor disorders Achalasia
Prof.Dr.Khalid A. Jasim Al-Khazraji M.B.CH.B , MD, C.A.B.M , FRCP , FACPI- 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: 1:100 000 , 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.Investigations
1- Chest X-ray: dilated esophagus, fluid level seen behind heart, fundal gas shadow is absent.
2- Barium swallow: lack of peristalsis, synchronous contraction in thebody of esophagus, sometime with dilatation. Lower end shows a “swanneck 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).BEFORE
AFTERIntrasphincteric injection of Botulinum toxin A Producesatisfactory initial results but the effects wear off withinMonths and further injections needed but it’s safer andsimpler than dilation.
3- Surgery:If the Last measures fail;Surgical division of LES is performed (cardiomyotomy orHeller’s operation). -But reflux esophagitis complicates all procedures & theaperistalsis 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.