Esophageal Achalasia
(repaired by Heller Myotomy)

Introduction

sophageal achalasia is a primary esophageal motility disorder of unknown etiology, characterized by absence of esophageal peristalsis and increased or normal resting pressure of the lower esophageal sphincter (LES), which fails to relax completely in response to swallowing.

Clinical Presentation

he most common symptom of Achalasia is difficulty of swallowing or,dysphagia. It is virtually experienced by all patients. Fluid and food moving back from the food pipe (esophagus) towards the throat also called regurgitation is the second most common symptom and is present in about 60% of patients. Regurgitation occurs more often when patients lie down in supine position or on their back. Regurgitation can lead to passage of fluid and food from food pipe into the wind pipe and cause infection or pneumonia. Chest pain occurs in about 40% of patients

Diagnosis

he diagnosis of Achalasia can be difficult. It is made more difficult by the rare nature of the disease. In addition to detailed medical evaluation, physicians use following tests for the diagnosis.

A barium swallow is a test performed in x-ray department. The patient is given a white liquid to drink and x-ray pictures of the food pipe and stomach are taken. It usually shows narrowing at the lower end of the food pipe ("bird beak"), and various degrees of esophageal dilatation.

Endoscopy is important to rule out the presence of a peptic stricture or cancer, and gastroduodenal pathology. Esophageal manometry is the key test for establishing the diagnosis. The classic manometric findings are: (a) absence of esophageal peristalsis, and (b) hypertensive or normotensive LES which fails to relax completely in response to swallowing.

The prolonged pH monitoring may be helpful preoperatively in patients who have previously failed treatment with pneumatic dilatation, Botulinum toxin (Botox), or surgical myotomy, for whom a myotomy is planned. Demonstration of reflux clearly indicates the need for a fundoplication in addition to the myotomy.

In patients older than 60 years of age, with recent onset of dysphagia and excessive weight loss, secondary or pseudo-achalasia should be ruled out. Because a cancer of the gastroesophageal junction is the most common cause of pseudo-achalasia, an endoscopic ultrasound or a CT scan of the gastroesophageal junction can help to establish the diagnosis.

Treatment

reatment is palliative, and it is directed toward elimination of the outflow resistance at the level of the gastroesophageal junction. The following treatment modalities are available to achieve this goal:

• Pneumatic dilatation: has a success rate between 70% and 80%. Gastroesophageal reflux occurs after dilatation in 25% to 35% of patients. Up to 5% of patients may sustain a perforation at the time of a dilatation. These patients may require open surgery to close the perforation and perform a myotomy
• Intrasphincteric injection of botulinum: results in initial relief of symptoms in about 60% patients, but this is transitory and symptoms will return in the majority of patients within a year. Subsequent injections are less effective and the benefit is of briefer duration. In addition, this treatment may cause an inflammatory reaction at the level of the gastroesophageal junction, which obliterates the anatomic planes. Consequently, a myotomy is more difficult, a mucosal perforation occurs more frequently, and the relief of dysphagia is less predictable. Because of these shortcomings, botulinum toxin should be reserved for elderly or high-risk patients who are poor candidates for dilatation or surgery
  
  Traditionally, pneumatic dilatation has been the first line of treatment for esophageal achalasia, while surgery was reserved for patients who had persistent dysphagia after multiple dilatations or who had suffered a perforation during dilatation

Laparoscopic Surgery For Achalasia

oday, minimally invasive surgery has completely changed this treatment algorithm and a laparoscopic Heller myotomy and partial fundoplication is preferred by most gastroenterologists and surgeons as the primary treatment modality. Critical details of the operation include a generous myotomy of the lower esophagus in a longitudinal fashion, extending well onto the gastric wall ( cutting the muscles of the esophagus or food pipe in the lower part of the food pipe all the way to the stomach). This procedure has a very high success rate in relieving the major symptom of achalasia i.e. dysphagia or difficulty swallowing. An anti reflux procedure is added to the operation to reduce the possibility of gastro esophageal reflux or GERD. Patients can usually eat the morning of the first postoperative day, and can be discharged home after one or two days. Surgical treatment of Achalasia provides the longest symptom relief compared to all the other modalities.

The need for esophagectomy (removal of esophagus or food pipe) for achalasia is very uncommon, and is reserved for extremely advanced cases.

All patients undergoing treatment for achalasia should be followed by surveillance endoscopy, because they are at increased risk for development of both squamous and adenocarcinoma.

Risks

spiration of retained food in the esophagus at the time of induction of anesthesia and perforation of the esophageal mucosa are the most common operative complications. Persistent or recurrent dysphagia occurs in 5% to 10% of patients. A complete work-up is necessary to evaluate the cause of the dysphagia in these patients, and either pneumatic dilatation or a second operation can often correct the problem. Up to 15% patients may experience gastroesophageal reflux after myotomy, as measured by 24-hour pH monitoring. In patients undergoing elective myotomy the mortality rate is less than 1%.

Expected Outcomes

bout 90% of patients have long-term relief of dysphagia after a myotomy, with a low incidence of symptomatic acid reflux. Patients should undergo 24-hour pH testing routinely after surgery, as reflux is often asymptomatic, and should be treated with proton pump inhibitors if abnormal acid reflux is present.

When To Call Our Office?

f you develop any of the following, be sure to call our office at (814) 877-6997 during business hours (9am-5pm Monday-Friday)

• Persistent fever (over 100°F)
• Bleeding
• Increased abdominal swelling or pain
• Persistent nausea or vomiting
• Chills
• Persistent cough or shortness of breath
• Difficulty swallowing that doesn't go away within a few weeks
• Drainage from any incision

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