ACHALASIA Is a motor disorder of the esophageal smooth muscle in which the lower esophageal sphincter does not relax

normally with swallowing, and the esophageal body undergoes non-peristaltic contractions. associated with nerve impulse cannot pass

REVIEW OF ANATOMY AND PHYSIOLOGY

Swallowing/ Deglutition Swallowing is a method of ingestion of food, from the mouth to the stomach. Swallowing has 3 stages: Voluntary stage, Pharyngeal Stage, and Esophageal Stage. Voluntary Stage- Food is pushed by the tongue muscles into the posterior end of the pharynx. Pharyngeal stage- Involuntary stage of swallowing and constitutes passage of food through the pharynx into the esophagus. Esophageal Stage- Involuntary phase that transports food from the pharynx to the stomach.

Esophageal Stage of Swallowing The esophagus functions primarily to conduct food rapidly from the pharynx to the stomach, and its movements are organized specifically for this function. The esophagus normally exhibits two type of peristaltic movements: 1. Primary peristalsis- continuation of the peristaltic wave that begins in the pharynx and spreads into the esophagus during the pharyngeal stage of swallowing. This wave passes all the way from the pharynx to the stomach in about 8 to 10 seconds. **Food swallowed by a person who is in upright position is usually transmitted to the lower end of the esophagus even more rapidly than the peristaltic wave itself, in about 5 to 8 seconds, because of the additional effect of gravity pulling the food downward. 2. Secondary peristalsis- results from distention of the esophagus itself by the retained food; these waves continue until all the food has emptied into the stomach. They are initiated partly by intrinsic neural circuits in the myenteric nervous system and partly by reflexes that begin in the pharynx and are then transmitted upward through vagal afferent fibers to the medulla and back again to the esophagus through glossopharyngeal and vagal efferent nerve fibers. The musculature of the pharyngeal wall and upper third of the esophagus is striated (skeletal) muscle. Therefore, the peristaltic waves in these regions are controlled by skeletal nerve impulses from the glossopharyngeal and vagus nerves. In the lower two thirds of the esophagus, the musculature is smooth muscle, but this portion of the esophagus is also strongly controlled

by the vagus nerves acting through connections with the esophageal myenteric nervous system. When the vagus nerves to the esophagus are cut, the myenteric nerve plexus of the esophagus becomes excitable enough after several days to cause strong secondary peristaltic waves even without support from the vagal reflexes. Therefore, even after paralysis of the brain stem swallowing reflex, food fed by tube or in some other way into the esophagus still passes readily into the stomach.

Receptive relaxation When the esophageal peristaltic wave approaches toward the stomach, a wave of relaxation, transmitted through myenteric inhibitory neurons, precedes the peristalsis. Furthermore, the entire stomach and, to a lesser extent, even the duodenum become relaxed as this wave reaches the lower end of the esophagus and thus are prepared ahead of time to receive the food propelled into the esophagus during the swallowing act.

Function of the Lower Esophageal Sphincter (Gastroesophageal Sphincter) At the lower end of the esophagus, extending upward about 3 centimeters above its juncture with the stomach, the esophageal circular muscle functions as a broad lower esophageal sphincter. This sphincter normally remains tonically constricted with an intraluminal pressure at this point in the esophagus of about 30 mmHg, in contrast to the mid portion of the esophagus, which normally remains relaxed. When a peristaltic swallowing wave passes down the esophagus, LES becomes relaxed ahead of the peristaltic wave, which allows easy propulsion of the swallowed food into the stomach.

Pathophysiology Exact mechanism is unknown. But pathological studies have shown damage in the neural network of the myenteric plexus in the lower two thirds of the esophagus. As a result, the musculature of he lower esophagus remains spastically contracted, and the myenteric plexus has lost its ability to transmit a signal to cause receptive relaxation of the gastroesophageal sphincter as food approaches this sphincter during swallowing.

ASSESSMENT Incidence: Occur in the 2nd or 3rd decade of life

Etiology: UNKNOWN but may be related to: o Hereditary o Autoimmune disorder - associated with HLA-DQw1 - antibodies to enteric neurons o Chronic infections with herpes zoster or measles viruses o Chagas disease (Trypanosoma cruzi) can result in a loss of intramural ganglion cells leading to aperistalsis and incomplete LES relaxation

Risk Factors: Annual incidence of approximately 1 case per 100,000. Men and women are affected with equal frequency (1:1 ratio). Usually diagnosed between the ages of 25 and 60 years. (mean age of 25)

Manifestations: Dysphagia (initial and major symptom) Substernal pain (early stage) Nocturnal regurgitation of undigested food eaten many hours earlier (33%); may lead to aspiration pneumonia Regurgitation of large amounts of mucus Avitominosis Weight loss

Medical Diagnosis: Patients typically experience symptoms for years before seeking medical attention. In one series of 87 patients with newly diagnosed achalasia, the mean duration of symptoms was 4.7 years. Patients who have a clinical history suggestive of achalasia require radiographic, manometric, and endoscopic evaluation to confirm the diagnosis.

Diagnostic Procedures: Barium swallow - diagnostic accuracy is approximately 95%; reveal non propulsive waves, can determine presence of esophageal dilation, can see absence of persistalsis. In some patients, spastic contractions in the esophageal body is present (vigorous achalasia).

Manometry- measures pressure in the esophagus. Can determine elevated resting LES pressure, incomplete LES relaxation, and presence of simultaneous esophageal contractions. Endoscopy- allows the physician to observe the physical form and condition of the esophagus. Can determine dilated esophagus, a residual material, presence of inflammation and ulceration, stasis of bacteria/ fungi (commonly Candida Albicans), the LES does not open spontaneously, traversed easily with gentle pressure, and rule out malignancies.

MEDICAL MANAGEMENT PHARMACOLOGIC MANAGEMENT (PALLIATIVE) o o o o o Calcium channel blocker relax LES Non-narcotic and narcotic analgesics substernal pain Antacids Pain H2 Receptor Antagonist relief Proton Pump Inhibitor

**Pharmacotherapy is often ineffective, and associated with side effects. **Used primarily for patients who are unwilling or unable to tolerate more effective invasive forms of therapy.

SURGICAL MANAGEMENT o Pneumatic/ Ballon Dilatation- Weaken the LES by tearing its muscle fibers.

Short to Medium Term Results (Less than 10 years) 899 patients - 65% success rate, mean follow-up of 6.5 years Single PD - effective in 85% of 144 patients, followed for an average of 6.5 years. A systematic review of the literature published in 1998: o o 2418 patients 2/3 of patients had good to excellent improvement after one or more dilations during a mean follow-up of 4.6 years

Long Term Results (More than 10 years) Few studies One study 50% developed recurrent symptoms after 10 years In a prospective follow-up of 54 patients (Single pneumatic dilation) 5 year remission rate of 40%

10 year remission rate of 36% Repeated dilations only mildly improved the clinical response.

Predictors of Outcome A decrease in LES pressure to approximately 10 mmHg Young age (<40 years) predicts a poor response to pneumatic dilatation Sex Retrospective study including 49 male pts and 16 female pts, young men required repeat treatment > young women.

Complications of Surgery Esophageal perforation 3% - 5% Intramural hematomas Esophageal mucosal tears Fever (resolves spontaneously) Severe postprocedural chest pain 15% Gastroesophageal reflux disease - incidence of 2% o Botulinum Toxin- Reduces the LES pressure by selectively blocking the release of acetylcholine from presynaptic cholinergic nerve terminals in the myenteric plexus.

Short Term Response (Less than 5 years) 21 patients were randomly assigned to BTX injection or placebo One week after treatment, the mean decrease in LES pressure was significantly greater in patients who received BTX (33% versus 12%),

Similar results in a number of other series - overall efficacy has ranged from 65-90% after one injection, lasting from 3 months to more than 1 year

Predictors of Outcome 31 pts who were treated with BTX were followed prospectively for a median of 2.4 years A response beyond three months was significantly more likely in patients older than age 50 (82 versus 43 percent) and in patients with vigorous compared to classic achalasia (100 versus 52 percent)

Complications of Treatment Post-procedural transient chest pain 25% Heartburn 5% Esophageal wall injury and paraesophageal tissue inflammation are rare Botulism - the low dose of BTX has virtually no risk of causing generalized neuromuscular blockade. o Modified Heller Approach/ Esophagocardiomyotomy

Results in good to excellent relief of symptoms - 70-90% with few serious complications The mortality rate - approximately 0.3% Reflux esophagitis - 10% The few long-term studies available suggest that surgical myotomy results in: o sustained remission rates of approximately 85% at 10 years, and 65% at 20 years.

Advantages of Each Treatment Surgical myotomy - may provide a more permanent solution For the high-risk patient, a trial of BTX is a reasonable approach For most younger patients, a laparoscopic myotomy offers the best chance for a single permanent procedure

DIETARY MANAGEMENT o o o o o Small frequent feeding Offer semi-soft warm foods (Soups, Pastas, Porridge) Avoid hot, spicy and iced foods Avoid alcohol and tobacco gastric irritants Chew food thoroughly to add saliva-lubrication

OTHER MANAGEMENT o o o Try different positions to reduce pressure while eating Sleep with HOB elevated semi fowlers position (X) Constrictive clothing

Nursing Diagnoses 1. Altered Nutrition : less than body requirements r/t duysphagia PRE-OP a. Drink fluids with meals using valsalva maneuver. b. Eat small frequent meals rather than large meals. c. Eat slowly,chew food thoroughly with arched back while swallowing to relieve pain. d. Soft bland diet. e. Instruct to avoid lying down immediately after meals. POST-OP a. NPO for atleast 24 hours before tube feeding begins b. Maintain tube feeding (PEG, PEJ) c. Check patency of tubes q 4 d. Elevate HOB at all times continous feeding Intermittent : 1 after feeding e. Change enteral feeding bag and tube every 24 hours f. Flush the tube with 50 to 100 ml NSS/ water after feeding 2. Pain r/t irritation of the esophageal wall secondary to regurgitation of esophageal content a. Give medications as ordered (CI: Anticholinergics) reflux b. Modify diet c. Elevate HOB especially at night 3. Risk for impaired skin integrity POST-OP a. Remove initial dressing of the surgical incision for tube placement after 12 to 24 hours b. Wash site with soap and water c. Report any manifestations of infection d. Teach about proper home care management of the incision site 4. Risk for injury a. Notify the physician immediately for any signs of perforation -fever, chest/ b. Maintain chest tube drainage and NGT shoulder c. Manage pain analgesics, splint pain, emphysema 5. Prepare for Surgery a. Explain that a local anesthetic may be sprayed in the throat; may receive analgesics or tranquilizer before esophageal dilatation. b. Instruct to take long, slow breaths during the passage of bougles. c. General anesthesia is administered before esophagomyotomy d. Instruct on usual pre-op procedures 6. OTHER NURSING DIAGNOSES a. Risk for aspiration r/t regurgitation of esophageal contents b. Risk for fluid volume deficit r/t dysphagia