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 Chap. 26). 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 workup 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 Camp7 (Fig. ...