Medical symptom checker - swallowing problem ( dysphagia symptoms ) - Achalasia treatment

Swallowing problems ( dysphagia) - Achalasia

Introduction

Generally achalasia is a neuromuscular disorder. Achalasia affects the esophagus with the absent of peristalsis ( aperistalsis) as well as the failure of the relaxation of the lower esophagus sphincter to relax while swallowing or dysphagia. Retention of the ingested food will be detected in the esophagus. Radiologically may reveals a dilated esophagus with aperistalsis ( absent of primary peristalsis) and conically narrowed junction of the cardio esophageal. Manometry and cineradiography may also reveals aperistalsis .

Pathology

Pathologically, it is a neuromuscular disorder causes by hypertrophy of the circular muscular layers of the esophagus without organic stenosis. In response to swallowing , the cardio esophageal junction fails to relax and primary peristalsis is absent. There is an evidence of destruction, atrophy , disintegration and absent of ganglionic cells of Auerbach myenteric plexus of the esophagus which disrupts the coordinated peristalsis. It is also associated with reduction in nerve fibers within the wall of esophagus, Degeneration of dorsal vagal nucleus in the brainstem medulla may be seen.

Etiology

The causes of achalasia is unknown but there are two theory exists. The first theory is achalasia is associated with degenerative disease of the neuron. The second theory is. it is associated with Trypanosoma cruzi infection which is more common in South America. Other cause include infection of the virus ( herpes zoster ).

Epidemiology

Common in the ages of 30 - 60 years old. May develop at any age. It affect male > female. Rare in children . In UK the annual incidence is 1 in 100 000.

( Achalasia may also be associated with Addison disease and alacrimation ( Triple A syndrome ).

Complaints

Patient typically complains of weight loss, intermittent dysphagia ( swallowing problem) involving liquid and solid, retrosternal cramping chest pain and regurgitation of food particularly at night.

Investigation

Investigation requires chest x ray, contrast radiography and endoscopy, manometry and blood.

Chest x ray may reveals fluid level behind the heart and dilated esophagus. ( double right heart border ).

Contrast radiography and endoscopy such as barium swallow and oesophagoscopy . Barium swallow may reveals dilated body of esophagus which blends into a smooth conically ending shaped of 3 - 6 cm narrow long. Barium swallow may also reveals narrowing of the cardio - esophageal junction and lack of peristalsis. The esophagus will become tortuous as the disease progress. Oesophagoscopy is useful to rule out malignancy.

Manometry is useful to identify and measures the pressure of the gastroesophageal sphincter. In achalasia, after swallowing the relaxation of the lower esophageal sphincter is absent or incomplete and the pressure of the gastroesophageal sphincter increase. In achalasia, the abnormal resting pressure is < 30 mmHg.

Blood test is useful to detect the present Changas disease. The detection of the serology for antibodies against T. cruzi is performed. The blood film might also detect any parasites present.

The differential diagnosis for difficulty swallowing besides achalasia include diffuse esophageal spasm, carcinoma at or near the cardio - esophageal junction and benign stricture of the lower esophagus. Therefore, to confirm the present of achalasia, if the patient complains of difficulty swallowing , the patient should requires contrast radiography and endoscopy. Endoscopy is important to establish the diagnosis and manometry to confirm the diagnosis. pH studies is also useful if the patient suffer with symptoms of reflux and fundoplication is planned.

Management

The management of achalasia includes medical and surgery. The medical approach includes isosorbide mononitrate for short term relief and calcium channel blocker/ antagonists such as nifedipine and verapamil. Endoscopic botulinum toxin injection is also useful.

The surgical goal of surgery is to relieve the functional obstruction either by ballon dilation or Heller cardiomyotomy longitudinal division of all the esophagus muscular layer. The endoscopic ballon dilation of the lower esophagus carries 80% success rates and small risk of perforation.

Heller cardiomyotomy involves thoracic and abdominal approaches. Fundoplication is performed in some cases to prevent reflux as reflux esophagitis may present and complicated the Heller cardiomyotomy.

Treatment monitoring may include measurement of rates of passages of Technetium Tc - 99m labelled solid meal . Esopahgoscopy performed periodically as achalasia will increase the risk of squamous cell carcinoma,

Complication

The complication of achalasia includes weight loss, aspiration pneumonia which leads to pneumonitis and malnutrition as well as small mucosal ulceration and 3- 5 % risk of esophageal malignancy mostly squamous cell carcinoma , regardless of treatment . Mostly 25 years after diagnosis of achalasia.

Prognosis

The prognosis is good if treated. The dilation procedure may produce only 50% good long term result while surgical approach or Heller cardiomyotomy ranges from good to excellent in 90% patient. If left untreated , the dilation of the esophagus will worsen and compress the mediastinal structure.

Arrows indicate areas  of dilated esophagus
Arrows indicate areas of dilated esophagus
X ray shows dilated esophagus with constricted ending
X ray shows dilated esophagus with constricted ending

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