Diffuse esophageal spasm (DES) is one of several nonspecific smooth muscle esophageal motility disorders associated with intermittent debilitating dysphagia and chest pain. The efficacy of long esophageal myotomy for the surgical treatment of DES has not been as favorable as myotomy has been for achalasia (see Chapter 33). Surgical treatment in this patient group therefore remains controversial. Long esophageal myotomy was first described for the treatment of DES in 1950 by Professor Lortat-Jacob of Paris, France.¹ Although the surgical principles remain the same, a few modifications have been made in the approach to diagnosis and treatment.

The pathophysiology of DES is still poorly understood. It is thought to be a neuropathy involving the smooth muscle layer of the esophageal wall that causes an inhibitory effect in the distal esophagus and hypotension of the lower esophageal sphincter (LES), giving rise to incomplete LES relaxation after a wet swallow. Gastroesophageal reflux disease (GERD) also has been implicated in the etiology of DES.² Dysphagia to solids and liquids, sometimes exacerbated by very cold or very hot foods, and intermittent chest pain are the predominant symptoms. The dysmotility, which is characterized by the presence of incoordinated nonperistaltic esophageal contractions of high, medium, or low amplitude, may progress or, in rare cases, has been observed to normalize. Progression of DES to achalasia is uncommon but has been reported in a prospective cohort study. No manometric or demographic predictors of progression have been identified.³

The condition continues to represent a therapeutic challenge. For some patients, medical therapy with smooth muscle relaxants (e.g., long-acting nitrates), calcium channel antagonists, and/or psychotropic drugs has proved beneficial.^4,5 Anticholinergics, pneumatic dilation, and botulinum toxin (Botox) injections produce brief and partial symptomatic relief.

Surgery is offered when symptoms are refractory to medical treatment or when complications arise. In general, surgical therapy can control symptoms in approximately 80% of patients. Patients with spastic disorder in addition to a pulsion diverticulum will benefit more from surgery than patients with spastic disorder only.⁶ Long myotomy is often combined with an antireflux operation.

A successful outcome for surgical treatment of DES requires the elimination or reduction of episodes of dysphagia and/or chest pain and prevention of postoperative GERD. Some consider long esophageal myotomy for DES to be a palliative procedure because swallowing is never perfectly restored.

Since esophageal motility dysfunction is thought to represent a continuum of disease, establishing the correct diagnosis is important to selecting the most appropriate treatment, reducing the incidence of postoperative complications, and achieving the best therapeutic outcome. The standard diagnostic work-up for DES includes barium swallow, esophageal manometry, esophagogastroduodenoscopy (EGD), and endoscopic ultrasound (EUS). pH studies can confirm the presence or absence of gastroesophageal reflux and may guide therapeutic maneuvers.

Radiologic examination with barium swallow reveals the characteristic corkscrew appearance of the esophagus first described by Moersch and Camp (Fig. 35-1).⁷ The contractions are relatively constant in their location and usually confined to the lower half of the intrathoracic esophagus. On esophageal manometry, DES is characterized by the presence of intermittent simultaneous contractions (30–100 mm Hg) intermixed with normal peristalsis (Fig. 35-2). The disorder is variously defined as the presence of 10% to 30% or more simultaneous contractions after 10 wet swallows.^8,9 The greater the percentage of simultaneous contractions, the more certain is the diagnosis. The mean simultaneous contraction amplitude in DES should exceed 30 mm Hg. Other manometric findings may include spontaneous contractions and repetitive contractions (multiple-peaked contractions).^8,10

EGD is indicated to exclude organic pathology such as an obstructing lesion, benign stricture, or cancer. Endoscopic observation of DES also has revealed that the tighter muscular rings tend to be fixed in location and that the less constricting spastic muscle causes the mucosa to have a corrugated appearance (Fig. 35-3).^11,12

EUS can be quite useful for assessing the extent of the spastic segment when used in combination with barium esophagram and manometric analysis. The spastic segment is revealed by a thickened muscle layer in the esophageal wall (Fig. 35-4).¹³ The esophageal wall muscle is thicker at rest (baseline) in patients with DES than in normal subjects, and there is a significant correlation between baseline muscle thickening and peak pressure.^11,14

Anesthesia and Positioning

Long esophageal myotomy is usually performed via thoracotomy or minimally invasive thoracoscopy. Whenever a thoracotomy is planned, an epidural catheter should be placed. With the minimally invasive approach, depending on the surgeon’s experience and confidence that the procedure can be completed without conversion to open technique, the epidural may not be necessary.¹⁵ A double-lumen endotracheal tube permitting lung isolation is necessary, regardless of approach. A nasogastric tube is inserted, and the patient is placed in right lateral decubitus position. The position of the endotracheal tube is reconfirmed before draping.

Open Thoracotomy Approach

A left posterolateral thoracotomy is preferred because it is easier to perform an antireflux procedure if needed through the left chest. The left lung is isolated. The sixth or preferably seventh intercostal space is entered (Fig. 35-5). The inferior pulmonary ligament is mobilized up to the inferior pulmonary vein. The lung is retracted medially and cephalad, and the mediastinal pleura is incised over the esophagus. A Penrose drain is placed around the esophagus to assist with mobilization. Care should be taken to identify and preserve both vagal nerve trunks. A complete longitudinal extramucosal myotomy in the spastic segment of the esophagus is made using scissors, knife, diathermy, LigaSure (Valleylab, Boulder, CO), or Harmonic Scalpel (Ethicon Endosurgery, Inc.). The segment of spastic smooth muscle usually is located in the middle and lower esophageal wall in patients with DES. The myotomy thus is usually performed inferior to the level of the aortic arch.