Izumi Harukuni

Basics

Description: Cardiospasm (=achalasia, achalasia cardiae, esophageal achalasia and esophageal aperistalsis) - is esophageal motility disorder involving the smooth muscle of the esophagus and the lower esophageal sphincter (LES) and characterized by aperistalsis and failure to relax the smooth muscle fibers LES, thus causing functional obstruction of the esophagus.

Epidemiology: 1 to 2 per 200,000

Prevalence: Cardiospasm affects young adults in their 20s and 30s

Pathophysiology: degeneration and disruption of esophageal vagal branches and loss of inhibitory ganglia of the intramural esophageal nerve plexus resulting in increased basal pressure and poor relaxation. Permanent aperistalsis and dilatation in proximal esophagus are also seen.

Etiology: is unknown. However viral cause appears to have limited contribution. Antibodies to myenteric neurons are present in 50% of patients, which suggest an autoimmune process. Genetic predisposition also has been described, with certain HLA loci.

Diagnosis

Signs and Symptoms: dysphagia for solid foods and liquids, regurgitation, chest discomfort/pain, weight loss. May present recurrent pneumonia secondary to aspiration.

Lab-Tests: barium swallow study with fluoroscopy results in barium retention due to poor esophageal emptying. Dilated distal esophagus and tapered end at LES (bird's beak) can be also revealed at barium swallowing.

Endoscopy: is carried out to exclude other diseases. Findings include dilatation and atony of esophagus, closed LES without stricture or mass.

Manometry: aperistalsis and incomplete LES relaxation after swallowing with or without an increased resting sphincter pressure.

Pathological Findings: a patchy inflammatory response consisting of T-lymphocytes, eosinophils, and mast cells. Loss of ganglion cells, and some degree of neural fibrosis in esophageal myenteric plexus

Differential Diagnosis:

• Malignancy (cancer, lymphoma, lymphangioma, mesothelioma)
• Chronic idiopathic intestinal pseudo-obstruction
• Amyloidosis
• Sarcoidosis
• Chagas disease
• Postvagotomy disturbances
• Von Recklinghausen neurofibromatosis
• Anderson-Fabry disease
• Familial glucocorticoid deficiency syndrome
• Multiple endocrine neoplasia, type IIb
• Juvenile Sjogren's syndrome with achalasia and gastric hypersecretion

Treatment

Palliation of symptoms and prevention of complication by elimination of outflow resistance. Effective peristalsis is rarely restored.

Medication (Drugs)

Not for long-term management due to poor effect on esophageal emptying. Drugs are unlikely to prevent esophageal dilatation and complications.

Nitrites (sublingual isosorbide dinitrate, 5 to 10 mg, before meals): improves symptoms in 50% to 70%, initially. Side effects include headache, hypotension, tachyphylaxis

Calcium channel blockers (diltiazem, nifedipine, verapamil) - their benefits are limited to patients with minimal esophageal dilatation (<5 cm)

Phosphodiesterase type 5 inhibitor (sildenafil 50 mg PO, 1 hour before meals): lowers LES pressure and some patients may benefit when other medications fail to control symptoms. However side effects limit its use (headache, dizziness, chest tightness, sleep disturbances).

Tricyclic antidepressant (trazodone 100 to 150 mg/d, imipramine 50 mg/d): appear to be effective in relieving chest pain. Probably due to visceral analgesic effect, unrelated to changes in esophageal motility or antidepressant effect.

Interventional: pneumatic dilatation has success rate of 70% to 80%, complication includes GE reflux (25% to 35%) and perforation (5%).

Intrasphincteric injection of botulinum toxin possess initial relief in 60%, but this method is only transitory - recurrence occurs within 1 year. Besides intrasphincteric injection of botulinum toxin may cause an inflammatory reaction at GE junction resulting in higher incidence of mucosal perforation in surgical myotomy.

Surgery: laparoscopic Heller myotomy and partial fundoplication are preferred as the primary treatment modality. Relief of dysphagia is achieved in 90% of cases. Benefit is sustained for 5 to 10 years. Major postoperative consideration is GE reflux and obstruction from too-tight fundoplication. The surgical mortality rate is 0.3%.

Follow-Up

Complications: esophagitis from chronic irritation or candidiasis. Increased risk for development of squamous cell carcinoma and adenocarcinoma. Aspiration pneumonia may also develop.

Patient Monitoring: patients should be followed by surveillance endoscopy.

References:

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2. Cannon RO, et al. Imipramine in patients with chest pain despite normal coronary angiograms. N Engl J Med. 1994;330(20):1411-1417.
3. Clouse RE, et al. Low-dose trazodone for symptomatic patients with esophageal contraction abnormalities. A double-blind, placebo-controlled trial. Gastroenterology. 1987;92(4):1027-1036.
4. Eherer AJ, et al. Effect of sildenafil on oesophageal motor function in healthy subjects and patients with oesophageal motor disorders. Gut. 2002;50:758-764.
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