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Achalasia Of The Cardia

  1. The classic triad of presenting symptoms consists of dysphagia, regurgitation, and weight loss. However, heartburn, postprandial choking, and nocturnal coughing are seen commonly.
  2. The dysphagia that patients experience begins with liquids and progresses to solids.
  3. Regurgitation of undigested, foul-smelling foods is common, and with progressive disease, aspiration can become life-threatening.
  4. It is disorder of esophageal motility characterized by decreased or absent peristalsis of the esophageal body, increased pressure in the esophagus, and impaired relaxation of the lower esophageal sphincter.
  5. Achalasia cardia is a motor disorder of the esophageal smooth muscle in which the LES does not  relax normally with swallowing & the esophageal body undergoes nonperistaltic contractions. (Primary peristalsis is absent or reduced)
  6. The condition can be divided into primary and secondary forms.
    1. Primary achalasia is due to loss of ganglionic cells in the myenteric plexus of Auerbach and is more common than secondary achalasia.
    2. Secondary achalasia which may be due to malignancy, diabetes, or Chagas disease (Trypanosoma cruzi).
  7. Patients with this condition have an increased incidence of malignancy; approximately 5% developing squamous cell carcinoma, usually in the mid-esophagus.
  8. Progressive Dysphagia is the cardinal most common symptom. Regurgitation, weight loss, and chest pain    or discomfort are other symptoms.(Patients with achalasia may have chronic aspiration pneumonia involving Mycobacterium fortuitum-chelonei.)
  9. The controversy: In the surgical treatment of achlasia, a balance must be found – There must be sufficient decrease in esophageal obstruction to provide symptomatic relief but on excessive decrease in LES pressur result in GERD. An antireflu procedure is sometimes added to avoid this, known as laparoscopic Heller – D or fundoplication. Randomized controlled trial of myotomy with or without antireflux are going on to provide a sufficient answer.
  10. The pathogenesis of Achlasia is poorly understood. It involves:-
    1. Neurogenic degeneration either idiopathic or due to infection. The degenerative changes are either intrinsic (degeneration of ganglion cells of aurebach’s myentric plexus) or extrinsic (extraesophageal vagus nerve or the dorsal motor nucleus of vagus)
    2. Pharmacologic studies suggest dysfunction of inhibitory neurons containing nitric oxide and vasoactive intestinal polypeptide in the distal esophagus (LES). The clolingergic innervation of the LES is intact or affected only in advanced disease.
    3. As a result of the abnormally, the LES fails to relax, primary peristalis is absent in esophagus which dilates. As the disease progresses the esophagus becomes massively dilated and tortuous.
Clinical findings
  1. Both sexes are equally affected
  2. May develop at any age but peak years are from 30 to 60.
  3. Classical clinical symptom is slowly progressive dysphagia more for liquids then solid.
  4. Dysphagia is worsened by emotional stress and hurried eating.
  5. Regurgitation and pulmonary aspiration occur because of retention of large volume of saliva and ingested food in the esopgagus.
  6. Esophagitis with ulceration occur with chronic retention of food.
  7. Pain is infrequent in classical achlasia but a variant called vigorous achlasia is characterized by chest pain and esophageal spasms that generate non propuloine high pressure waves in the body of the esophgus.
Investigation :
  1. There is usually an air-fluid level in the esophagus from the retained food and saliva, the height of which reflects the degree of resistance imposed by the nonrelaxing sphincter. As the disease progresses, the esophagus becomes massively dilated and tortuous.
  2. This manometric pattern has been termed "vigorous achalasia," and chest pain episodes are a common finding in these patients.
    1. It is the most confirmatory investigationQ
    2. It is able to distinguish between various forms of motor disorders of esophagus.
    3. Manometric characteristics of Achlasia.
      1. Incomplete lower esophageal sphincter relaxation (<75% relaxation)
      2. Elevated LES pressure
      3. Loss of primary peristaltic waves in the esophageal body but disorganized muscular activity may be present.
  1. Chest radiography:- which often demonstrates a homogeneous, usually right-sided, paramediastinal soft-tissue opacity. Mediastinal widening, air-fluid levels, absence of a gastric air bubble (due to a water-seal effect), Complications such aspiration pneumonia or lung abscess.
  2. Fluoroscopic barium swallow demonstrates failure of the contrast agent to enter the stomach when the patient is in the recumbent position, nonpropulsive tertiary esophageal contractions, various degrees of dilatation, and the bird-beak sign (ie, abrupt, smooth tapering of the distal esophagus).
  3. CT findings are nonspecific and insensitive, with esophageal dilatation usually present. Symmetric wall thickening helps to distinguish achalasia from pseudoachalasia of malignancy, in which mucosal irregularity or mass effect at the cardia is usually present.
  4. Endoscopy can yield biopsy samples to exclude malignancy and permit direct visualization of esophagitis or ulcers.
  5. Pseudoachalasia may be present
  6. Cause of Pseudoachalasia include esophageal and gastric malignancies and other tumors involving distal esophagus or LES.
  7. Normal findings on barium swallow study do not completely exclude especially in its early stage, because physiological derangements associated with achloric precede the development of anatomic find out.
Therapeutic interventions,
  1. Heller myotomy (Treatment of choice) done with a laparoscopic technique usually results in progressive improvement and compares favorably with open surgery with regard to relief of dysphagia and GERD.  It is most (90%) effective treatment for achalasia cardia. The myotomy extends 1 cm on to the stomach & to several centimeters above palpable region of LES.
  2. Pneumatic balloon dilation of LES to a diametric of approximately 3 cm is necessary to fear circular smooth muscle. Q
  3. Botulinum toxin injection, may be performed during the procedure (High rate of recurrents) (AIPG 02).
  4. Medical therapies :- Calcium channel blockers and nitrates, are effective in a few patients and may be tried in patients with contraindications to pneumatic dilation or surgery and waiting for surgery.
Extra Edge: 
Radiological Features Seen in
  Apple core lesion on barium enema Carcinoma colon
  Claw appearance on barium enema Intussusception
  Saw tooth appearance Colonic diverticula
Bird beak appearance Achalasia
Cork screw appearance
Rosary bead appearance
Pseudodiverticular appearance
Diffuse esophageal spasm
String sign of Kantor Crohn's disease
Thumb print sign Ischemic colitis
Squeeze sign, Cushion sign, Tenting sign, naked fat sign Colonic lipoma
Rat tail appearance Achalasia

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