1. ESOPHAGEAL MOTILITY DISORDERS

2. Esophageal neuromuscular dysfunction: dysphagia,chest pain, or heartburn
ACHALASIA
Diffuse Esophageal Spasm (DES)
GERD
Secondary Motility disorders : pseudoachalasia, Chagas’ disease, scleroderma

3. ACHALASIA
Rare disease , loss of ganglion cells within the esophageal myenteric plexus
Incidence 1:100,000 , age 25 and 60 , Aganglionosis in longstanding disease
Both excitatory (cholinergic) and inhibitory (nitric oxide) ganglionic neurons
Inhibitory neurons (deglutitive LES relaxation) and sequential propagation peristalsis
Absence Inhibitory neurons … impaired deglutitive LES relaxation and absent peristalsis
Causes of ganglion cell degeneration :autoimmune process (latent infection HSV1 combined with genetic susceptibility)

4. Long-standing achalasia : progressive dilatation sigmoid deformity of the esophagus with hypertrophy of the LES
Clinical manifestations : Dysphagia, regurgitation, chest pain (early course result from esophageal spasm), weight loss , solid and liquid food dysphagia
Regurgitation (retention of food, fluid, and secretions in the dilated esophagus)
In advanced achalasia : Bronchitis, pneumonia or lung abscess (chronic regurgitation and aspiration)

5. Patients describe a squeezing, pressure-like retrosternal pain, sometimes radiating to the neck, arms, jaw, and back.
Some patients complain of heartburn that may be a chest pain equivalent
Treatment of achalasia is less effective in relieving chest pain than it is in relieving dysphagia or regurgitation
DDx : DES, Chagas’disease, and pseudoachalasia
Chagas’ disease : endemic in central Brazil, Venezuela, and northern Argentina , spread by the bite of the reduviid bug that transmits the protozoan, Trypanosoma cruzi

6. The chronic phase of the disease develops years after infection and results from destruction of autonomic ganglion cells throughout the body, including the heart, gut, urinary tract, and respiratory tract.
Pseudoachalasia : Tumor infiltration, most commonly in gastric fundus or distal esophagus carcinoma, can mimic idiopathic achalasia
Up to 5% , advanced age, abrupt onset of symptoms (<1 year), weight loss
Endoscopy is a necessary part of the evaluation of achalasia

7. When endoscopy nondiagnostic, computed tomography (CT) scanning or EUS may be of value
Rarely, pseudoachalasia can result from a paraneoplastic syndrome with circulating antineuronal antibodies
Dx : barium swallow x-ray (dilated esophagus with poor emptying, an air- fluid level, and tapering at the LES giving it a beak-like appearance)
Occasionally, an epiphrenic diverticulum is observed
Endoscopy has a relatively minor role other than to exclude pseudoachalasia
Esophageal manometry :Impaired LES relaxation and absent peristalsis
High-resolution manometry (HRM) : The most sensitive diagnostic test , identifies early disease before esophageal dilatation and food retention , three subtypes differentiated based on the pattern of pressurization in the nonperistaltic esophagus

12. Tx : There is no known way of preventing or reversing achalasia
Reducing LES pressure so that gravity and esophageal pressurization promote esophageal emptying.
Peristalsis rarely, if ever, recovers
Reduced LES pressure by pharmacologic therapy, pneumatic balloon dilatation, or surgical myotomy
No large, controlled trials of the therapeutic alternatives exist, and the optimal approach is debated
Pharmacologic therapies are relatively ineffective but are often used as temporizing therapies

13. Nitrates or calcium channel blockers ,caution because of their effects on blood pressure
Botulinum toxin, injected into the LES under endoscopic guidance, inhibits acetylcholine release from nerve endings and improves dysphagia in about 66% of cases for at least 6 months
Sildenafil and alternative phosphodiesterase inhibitors effectively decrease LES pressure
The only durable therapies for achalasia are pneumatic dilatation and Heller myotomy

14. DIFFUSE ESOPHAGEAL SPASM (DES)
Episodes of dysphagia and chest pain due to abnormal esophageal contractions with normal deglutitive LES relaxation
The pathophysiology and natural history of DES are ill defined
Radiographically : tertiary contractions …“corkscrew esophagus (indicative achalasia)
Manometrically : Uncoordinated (“spastic”) activity in the distal esophagus, spontaneous and repetitive contractions, or high amplitude and prolonged contractions
DES is actually much less common than achalasia
Esophageal chest pain closely mimics angina pectoris , pain that is nonexertional, prolonged, interrupts sleep, meal-related, relieved with antacids, and accompanied by heartburn, dysphagia, or regurgitation.

15. Both chest pain and dysphagia are also characteristic of peptic or infectious esophagitis
Dx : Manometry , Endoscopy (useful to identify structural and inflammatory lesions that may cause chest pain)
Radiographically : “corkscrew esophagus,” “rosary bead esophagus,” pseudodiverticula, or curling can be indicative of DES, but these are also found with spastic achalasia.
Tx : Medical (nitrates, calcium channel blockers, hydralazine, botulinum toxin, and anxiolytics)
Surgical therapy (long myotomy or even esophagectomy) in severe weight loss or unbearable pain
These indications are extremely rare