Esophageal resection and reconstruction in patients with primary or secondary motility disorders of the esophagus are very uncommon. Often, consideration of esophagectomy is the final decision in a long and difficult plan of care by both the gastroenterologist and esophageal surgeon. Fortunately, the majority of motility disorder patients are seen in expert centers, where they usually undergo extensive evaluation and are treated appropriately with reasonable palliation of their symptoms. Patients who are not well palliated usually present with disabling symptoms associated with obstruction or pseudo­obstruction, uncontrollable pain with eating, and/or refractory gastroesophageal reflux disease. In addition, these patients may have undergone not one but several prior esophageal and/or gastroesophageal surgeries.

Esophagectomy in these situations is viewed as a “Hail Mary” and may not be given enough credit owing to the significant risk of mortality and morbidity that have been attributed to this operation. However, recent technical improvements have lessened these risks and perhaps esophagectomy should be considered earlier in the treatment course rather than after repeated attempts at repair. In skilled hands, the improvement in quality of life (QOL) and swallowing function after esophagectomy may outweigh the risks of the operation for many of these patients.

This chapter briefly reviews the features, initial treatment(s), complications of treatment, and long-term outcomes of the most common primary and secondary motility disorders. It outlines the indications for esophagectomy, discusses current controversies in management, describes the technique of vagal-sparing esophagectomy (VSE) for reconstruction in these settings, and finally, summarizes the outcomes of esophagectomy in patients with benign disease.

Detailed review of the primary and secondary motility disorders is beyond the scope of this chapter. For meaningful discussion of the role of esophagectomy, however, it is helpful to have some idea of the salient features of the most common primary and secondary motility disorders, as well as the recommended diagnostic evaluation, manometric findings, appropriate primary surgical therapy, and long-term complications. This information is summarized in Table 36-1.

Aside from collagen vascular disorders such as scleroderma, progressive systemic sclerosis, and systemic lupus erythematosus, the other causes of secondary motility disorders such as diabetes mellitus, alcohol, amyloidosis, myxedema, and psychiatric diseases are exceptionally rare and few if any reports exist that outline the role of esophagectomy let alone primary surgical therapy in these situations. As a principle, treatment is directed at the underlying cause of the motility disorder followed by careful evaluation with upper endoscopy, manometry, and pH testing to ensure the appropriate course of treatment may be undertaken. Secondary motility disorders are commonly associated with GERD and often complicate antireflux surgery. Whether these disorders result from, cause, or exacerbate GERD is unclear. The management of recurrent GERD and failed antireflux surgery is discussed in Chapter 43.

Patients with esophageal motility disorders who contemplate esophagectomy present with a wide variety of symptoms. In one large series, the most common symptoms include dysphagia (90%), regurgitation (57%), heartburn (52%), weight loss (32%), and chest pain (25%).³ In some instances, the symptoms will be germane to the original disease process such as chest pain in nutcracker esophagus or dysphagia in achalasia. Symptoms such as regurgitation, aspiration, and dysphagia may be derived from the previous medical and surgical therapies. Rarely, patients present acutely with hemorrhage, ulceration, perforation, or fistulization. In most instances, these symptoms will be the reason to consider additional surgery, but it is important to recognize that some of these symptoms may indicate a more significant underlying process such as a benign reflux stricture that is refractory to dilation or a cancer of the esophagus. Indications for surgery that may be secondary to the motility disorder or the previous surgical therapy are listed in Table 36-2.

The preoperative evaluation in some ways is more thorough and complex than a similar evaluation would be for a patient with esophageal cancer considering esophagectomy. This is largely because of the number of surgical options for benign disease. Although staging investigations are not required, the patient must undergo a complete physiologic esophageal and gastric evaluation. The primary goal of this evaluation is to determine whether the patient’s symptoms may be palliated with esophagectomy or if a lesser intervention, surgical or not, will suffice.

Esophageal Function Testing

A comprehensive history by an experienced esophageal surgeon is likely to reveal a significant amount of information about the indications for esophagectomy before the objective evaluation begins. Obvious lines of questioning relate to aspiration events, food bolus impaction, severe dysphagia and odynophagia, and weight loss. It is often enlightening to ask the patient about their daily routines since they may have modified or adapted to their disorder in significant ways which seem normal to them but will strike outsiders as being somewhat unusual. Some examples include eating while standing up to make the food pass easier, keeping a bucket by the bedside for nighttime regurgitation, or eating the last meal of the day at 3 pm to avoid evening and nighttime symptoms of GERD.