Long Myotomy for Diffuse Esophageal Spasm
David D. Odell
James D. Luketich
DEFINITION
Diffuse esophageal spasm (DES) is a nonspecific motility disorder of the esophagus characterized by intermittent and disorganized smooth muscle contractions, often resulting in debilitating dysphagia and/or severe substernal chest pain.
DIFFERENTIAL DIAGNOSIS
DES is part of a spectrum of motility disorders affecting the esophagus. Consequently, a careful assessment of the clinical presentation and objective diagnostic testing is imperative in order to avoid misdiagnosis and inappropriate treatment. Several conditions may mimic the symptomatic presentation of DES.
Achalasia: Characterized by incomplete relaxation of the lower esophageal sphincter coupled with aperistalsis of the esophageal body. Focal distal (Heller) myotomy extending onto the gastric cardia is the treatment of choice for this condition.
Nutcracker esophagus: Primary motility disorder of the esophagus characterized by high-amplitude, peristaltic contractions of the distal esophagus. Patients present with pain but do not have dysphagia. Treatment is predominantly medical with the use of calcium channel blockers.
Connective tissue disorders: Connective tissue diseases such as scleroderma frequently impact esophageal motility. Patients presenting with primary symptoms of dysphagia should be evaluated for other systemic manifestations of these conditions.
Gastroesophageal reflux disease (GERD): Patients with untreated or poorly treated long-standing reflux may develop esophageal dysmotility from chronic inflammation caused by the refluxate. In these patients, control of the reflux (medically or surgically) will typically resolve the motility issues.
Pseudoachalasia: Mechanical obstruction of the esophagus from tumor, hiatal hernia, prior antireflux surgery, or foreign body may cause symptoms of dysphagia, esophageal dilation, and dysmotility. Correction of the underlying pathology is the appropriate treatment in these patients and esophageal motility often returns to normal once this is accomplished.
PATIENT HISTORY AND PHYSICAL FINDINGS
A careful history focused on symptoms of dysphagia is helpful in evaluating patients for DES. Patients should be asked to characterize the frequency (every meal, daily, weekly, etc.) and severity (solids, liquids, salivary secretions, etc.) of their dysphagia.
Patients should be asked about pain. The quality and duration of symptoms are important details to elicit as well as the relationship of symptoms to swallowing.
Typical and atypical reflux symptoms should be directly questioned.
Typical: heartburn, history of stricture/dysphagia, water brash, globus sensation
Atypical: cough, hoarseness of voice, aspiration, recurrent pulmonary infection
Cardiac history (prior myocardial infarction, history of exertional chest pain, review of any prior cardiac procedures or evaluations [i.e., catheterizations or stress testing])
Connective tissue disease history: Evaluation of cutaneous lesions, healing difficulty, and concomitant visual problems
IMAGING AND OTHER DIAGNOSTIC STUDIES
Barium swallow—will show a classic corkscrew appearance of the esophagus characteristic of the disease and corresponding to the simultaneous contractions.
Esophageal manometry—primary tool for establishing the diagnosis. The disease is typically characterized by simultaneous contractions (30 to 100 mmHg) with intermittent normal peristalsis. DES is typically defined as the presence of symptoms in concert with simultaneous contractions after greater than 10% to 30% of wet swallows. Amplitude of these simultaneous contractions should exceed 30 mmHg to support the diagnosis. The diagnosis of DES is more certain with a higher percentage of simultaneous contractions.
Esophagogastroduodenoscopy (EGD)—required to assess the anatomy prior to intervention and to rule out alternative pathology. EGD will allow causes of pseudoachalasia (tumor, stricture, obstruction/compression) to be fully assessed with biopsies taken as necessary. This also allows the surgeon the ability to assess for the presence of refluxrelated changes, such as Barrett’s esophagus, which may require further surveillance. In advanced stages of DES, the endoscopic appearance of the esophagus may be somewhat corrugated as hypertonic muscular bands tend to form in fixed positions.
Endoscopic ultrasound (EUS)—a useful adjunctive test in surgical planning, as the extent of the spasmodic muscular segment may sometimes be identified by the presence of thickening of the muscular layer on EUS.
pH studies—may confirm the presence of acid reflux and may help guide the decision to include a fundoplication at the time of surgical correction.
SURGICAL MANAGEMENT
The bulk of surgical treatment is discussed in the “Techniques” section. Here, consider indications and other more general concerns.
Preoperative Planning
Patients should be thoroughly counseled regarding the risks of the operation including esophageal perforation, persistent
dysphagia, and exacerbation of reflux symptoms. Alternative treatment options including pharmacotherapy with calcium channel blockers should be discussed.
A liquid diet should be administered for 2 days prior to surgery (longer if significant delay in esophageal clearance is expected).
Patient Positioning
The patient is turned to the left lateral decubitus position. A beanbag is placed underneath the patient to afford additional support and to fix the patient in position. An axillary roll is positioned at the base of the axilla to minimize nerve compression. Position of the double lumen endotracheal tube, placed prior to turning the patient lateral, is reconfirmed using a pediatric endoscope. The operating surgeon stands on the right side of the table (facing the patient’s back) while the assistant stands on the left side of the table.
Airway Management
The patient is intubated with a double lumen endotracheal tube. This allows for lung isolation on the operative side to provide adequate exposure.
TECHNIQUES
THORACOSCOPIC APPROACH
Port Placement
A total of five thoracoscopic ports are used (FIG 1). A 10-mm camera port is placed in the 8th or 9th intercostal space, just anterior to the midaxillary line. The working port is a 10-mm port placed in the 8th or 9th intercostal space, posterior to the posterior axillary line. Another 10-mm port is placed in the anterior axillary line at the 4th intercostal space, through which a fan-shaped retractor aids in retracting the lung to expose the esophagus. A 5-mm port is placed just inferior to the tip of the scapula for the surgeon’s left hand. A final 5-mm port is placed at the 6th rib, at the anterior axillary line for suction by the assistant.
Diaphragm Retraction
Adequate retraction of the diaphragm is essential to achieve visualization of the lower thoracic esophagus and to aid in the dissection. A retracting suture is used to facilitate this retraction (FIG 2). We place a 48-in, 0-Surgidac suture through the central tendon of the diaphragm using the Endo Stitch device. The suture is brought out through the lateral chest wall at the level of the insertion of the diaphragm through a small stab incision, retracting the diaphragm inferiorly in order to expose the distal esophagus.
Pleural Dissection and Esophageal Mobilization
The inferior pulmonary ligament is divided to the level of the inferior pulmonary vein to allow for maximal anterior lung retraction (FIG 3A).
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