Etiology

Clinical Presentation

Diagnosis

Treatment

Calcium channel blockers and nitrates relax the LES and are effective in 70% of patients. Botulinum toxin injection into the LES works in 80%, but the effect lasts for only 6 months. Pneumatic dilation of the LES relieves the obstruction by tearing its fibers. It is effective in 85%, and the effect lasts for up to 3 years.

Surgical: Heller’s myotomy of the LES with partial fundoplication by Laparoscopic technique is currently preferred, where the LES is divided and a partial fundoplication performed. Position of the patient and port placement is similar to that for fundoplication. A gastroscope is passed to clear the esophagus and the GE junction and is left crossing the junction. The left lobe of the liver is retracted, exposing the hiatus, and the stomach is retracted inferiorly. This exposes the GE junction, and the gastroheaptic ligament and the peritoneum over the GE junction are then divided. The left crus is completely dissected. The retroesophageal space is then dissected and the esophagus encircled with a vascular tape or penrose drain. The phrenoesophageal ligament is divided, and 6 cm of the esophagus is mobilized. With the cautery on low setting, a myotomy is then performed starting 6–8 cm proximal to the phrenoesophageal ligament and carried through the GE junction and for at least 2 cm onto the stomach. The muscle is swept off the mucosa bluntly with a peanut dissector or graspers for approximately 180 degrees. Care is taken to avoid the vagus nerves. The gastroscope is then used to check for perforation and the GE junction for patency. The left edge of the muscle is then sutured to the left crus and the medial side of the fundus with three 2-0 silk sutures. The tape or drain encircling the esophagus is now removed and the fundus anchored to the apex of the right crus. The rest of the fundus is rolled over the lower esophagus and sutured to the right crus including the right edge of the esophageal myotomy with three sutures of 2-0 silk. The short gastric vessels can be divided if the fundoplication is under tension.

Perforation should be recognized intraoperatively and repaired primarily and covered with the stomach.

Barium swallow is done on POD1 to rule out perforation and assess patency of the GE junction. Clear liquids are started, and the patient is discharged on a liquid diet.

Success rates are higher than dilation, and the recurrence rate is less. The 5-year probability of being asymptomatic is 90%. Recurrence, when it occurs, is usually within 12 months and can be treated by dilatation. Reflux can occur in 30% of patients and is managed medically.

Both balloon dilatation and myotomy have equivalent results over the long term, but younger patients have a higher chance of failure with dilatation. Dilatation has to be performed up to three times over a period of 2 years to yield equivalent results to surgery and has a 4% perforation rate.

Per oral endoscopic myotomy is a new treatment where a myotomy of the LES is performed endoscopically with good initial results.

Patients are managed medically first and then may need dilatation or surgery, with surgery being preferable in patients younger than 40. Botulinum injection is preferred for those who are at higher risk, that is, the elderly or those with many co-morbidities.

Testing

Nonsurgical management: Patients whose primary symptom is chest pain – calcium channel blocker, diltiazem 180–240 mg/day or a tricyclic antidepressant, imipramine 25–50 mg at bedtime as the initial treatment. For patients who do not respond to this treatment, isosorbide 10 mg or sildenafil 50 mg taken as needed for pain should be tried. Following which, botulinum toxin injection at the Z-line (100 units given circumferentially in 20-units doses) can be given with relief in 70% of patients and a duration of effect of 7 months. Pneumatic dilatation may also be effective. In patients whose primary symptom is dysphagia, treatment with a calcium channel blocker (diltiazem 180–240 mg/day) is tried initially, followed by botulinum injection or pneumatic dilation.

Indications for surgery: Patients with diffuse esophageal spasm who continue to be symptomatic after medical management, injection, or dilatation with severe dysphagia or chest pain are candidates for surgical treatment. Patient’s in whom manometry reveals less than 30% of contractions are effective benefit from surgery. Manometry identifies the proximal extent of the myotomy, which should be high enough to include the entire length of the disordered motility. Surgery for Nutcracker esophagus and hypertensive LES is not clearly beneficial and is probably best avoided.

Operations: A left posterolateral thoracotomy is performed through the sixth space. The inferior pulmonary ligament is divided. The mediastinal pleura is opened over the esophagus from the hiatus to the arch of the aorta and, if needed (based on manometry), to the thoracic inlet. A long myotomy is performed starting where it is easiest, dividing the muscular wall of the esophagus sharply or with cautery on a low setting until the mucosa is seen. The mucosa can then be bluntly or sharply dissected from the muscular layer, and division of the muscle can proceed superiorly and inferiorly to the LES. A strip of muscle can be removed to prevent reapproximation of the muscle layer. The mucosal integrity is tested by air insufflation or methylene blue instillation via the nasogastric tube. A partial fundoplication is then performed. The chest is drained by a single chest tube. A barium swallow can be performed on POD2 prior to allowing the patient to resume oral intake.

A right-sided thoracoscopic approach is preferred because the entire esophagus is readily accessable. A camera incision is placed in the line of the anterosuperior iliac spine in the seventh space, an access incision is placed in the same or sixth space in the anterior axillary line, and the working incision is placed in the fifth space between the middle and anterior axillary line. The inferior pulmonary ligament is divided, and the mediastinal pleura over the esophagus is opened. A myotomy is commenced below the carina dividing the muscle sharply or with cautery on a low setting. The muscle can be held with Hunter graspers or with retraction sutures. The division of the muscle can then be done sharply, bluntly, or with a harmonic scalpel taking care to protect the mucosa. This is carried superiorly to the inlet and distally to the hiatus and the lower esophageal sphincter. A partial fundoplication is then performed laparoscopically.

Complications: Mucosal perforation should be recognized intraoperatively and repaired primarily and buttressed with an intercostal muscle flap.

Results: Relief of symptoms occurs in 80–90% of patients with the myotomy extending to the arch or the thoracic inlet.