Achalasia is inevitably a progressive and incurable disease. All therapy is at best palliative, and the results of surgical treatment continue to deteriorate with the passage of time. This observation is supported in a number of publications reporting long-term follow-up in series of patients managed by a short esophagomyotomy with or without the addition of an antireflux reconstruction.1–5
Long-Term Results of Esophagomyotomy
Ellis and colleagues reported long-term results in a large number of patients managed by a transthoracic short esophagomyotomy without antireflux reconstruction.6 At the end of 1 year, 91% of patients registered good to excellent results. However, when 57 of these patients were evaluated over the long term (median 13.6 years), only 67% still registered good to excellent outcomes. Ten percent were classified as poor. These failures largely presented with disabling dysphagia, which Ellis and colleagues attributed to the natural progression of achalasia over time.
Malthaner and colleagues reported on 22 patients with newly diagnosed achalasia managed at Toronto General Hospital by a transthoracic esophagomyotomy combined with a Belsey Mark IV antireflux reconstruction.7 All 22 patients were followed for longer than 10 years (10–23 years). At the end of 1 year, all 22 patients were improved, and 21 of the 22 (96%) registered good to excellent categories. The good to excellent results diminished to 69% at the end of 10 years and to 67% after 20 years. The deterioration in results almost exclusively was a result of the development and progression of GERD with gross endoscopic esophagitis and stricture. Three of the twenty-two patients ultimately were managed by esophagectomy and gastric replacement (cervical anastomosis) was performed 7, 19, and 23 years after the initial esophagomyotomy. A fourth patient was palliated successfully with antrectomy, vagotomy, and Roux-en-Y diversion at 23 years.
Ruffato and colleagues, from Bologna, reported the long-term results of extended esophagomyotomy reinforced with a Dor fundoplication.8 Of the 173 patients, 68 were followed for more than 15 years. The authors reported subsequent esophageal cancer in four patients performed 27, 46, 96, and 144 months after initial myotomy. Poor results were managed by additional surgery in 22 of these patients, and in 15 of these 22, failed outcome was due to the development of disabling GERD. Management included esophagectomy in two, antrectomy and Roux-en-Y diversion in two, and a second antireflux reconstruction in two. The remaining 7 of these 22 failures were patients with a grossly dilated sigmoid esophagus. Six of these seven patients underwent esophagectomy. It was noted that 26 patients had a grossly dilated sigmoid esophagus at initial presentation. The authors proposed that primary surgical treatment with esophagectomy may be a reasonable consideration when patients present with advanced degrees of esophageal dilatation and tortuosity.
Gaissert and colleagues, from the Massachusetts General Hospital, reviewed 64 consecutive patients with achalasia managed by esophagomyotomy (with and without fundoplication).9 Thirty-one of these patients were followed for more than 10 years. The authors recorded good to excellent short-term results in 91% of patients, but this category deteriorated to 63% at and beyond 10 years. Dysphagia persisting after operation was a harbinger of late failure. Four of the thirty-one long-term patients developed a peptic stricture, and two of these thirty-one ultimately were managed by esophagectomy.
Glatz and colleagues, in Louisville, Kentucky, reported their experience with eight patients managed by Ivor Lewis esophagectomy after prior unsuccessful esophagomyotomy.10 Five of these eight patients had advanced esophageal dilatation with a sigmoid configuration. The mean time from esophagomyotomy to esophagectomy was 12.5 years (range 2–18 years, median 14 years). At final pathology, two of the patients were found to have an occult squamous carcinoma (invasive in one and in situ in the other, both located in the middle third of the esophagus).
Indications for Esophagectomy in Achalasia
It is difficult to identify precise indications or categories of failure that would prompt the decision for esophagectomy and reconstruction in patients with achalasia. The earliest reports in the medical literature are found in the early 1980s.11,12 Since that time, there has been an increasing number of reports documenting experience from several well-known surgical centers with a significant interest in the management of benign motor disorders of the esophagus.1,2,13–17
Most patients for whom esophagectomy is indicated have undergone one or more operations, beginning with esophagomyotomy performed with or without an antireflux reconstruction. The reasons for subsequent failure leading to esophagectomy are disabling obstruction with dysphagia and regurgitation, disabling GERD exacerbated by the concomitant achalasia, or recognition of malignancy in the squamous epithelium of the esophagus.
Dysphagia is the most common indication prompting a decision for esophagectomy. The origin of dysphagia may prove difficult to determine even after extensive evaluation with contrast examinations, endoscopy, manometry, pH studies, or observations made at surgical exploration.
The disabling symptoms frequently are attributed to the late complications of GERD. These include intractable peptic ulceration, peptic stricture, and Barrett's columnar replacement with Barrett ulcer.2–6,10–14 Malthaner and colleagues found that the incidence of severe GERD increased with the passage of time after esophagomyotomy.7 Severe manifestations of GERD were identified in 15% of patients followed beyond 15 years. Esophagectomy in these patients is done most frequently 10 or more years after the original surgery.3–5
Disabling dysphagia may persist or progress in patients, with the most advanced changes occurring in a grossly dilated, redundant “sigmoid” megaesophagus. In such patients, the distended distal esophagus may lie in the posterior mediastinum at a level well below the diaphragmatic hiatus. This large distal reservoir is rarely, if ever, empty of food and fluid. Although such redundancy may be diminished with the operation of open transthoracic myotomy (some of the redundant distal esophagus can be positioned below the hiatus), such correction is difficult, if not impossible, with the laparoscopic myotomy that prevails today. Orringer and colleagues were the first to suggest that when such advanced cases are seen in patients at suitable risk, esophagectomy may be the preferred primary operation.12 A similar recommendation was made by Ruffato and colleagues in 2006.8
Ellis and colleagues in 198613 and again in 199218 attributed poor results to the natural progression of this incurable disease with the passage of time. The 1992 paper by Ellis and colleagues18 reported 10- and 20-year follow-up results in a significant number of patients. Follow-up, however, did not include a critical evaluation of the role of GERD.
In addition to obstruction, GERD may create intractable symptoms of painful retrosternal distress, acid regurgitation, and aspiration. Esophagectomy may be advised if some form of local antireflux operation is considered likely to fail. A safer alternative to resection in such patients may be an antrectomy, vagotomy, and diverting Roux-en-Y jejunal gastroenterostomy. Favorable results with this operation have been reported by Fekete and colleagues,11 Ellis and colleagues,13 Gayet and Fekete,2 and Miller and colleagues.15
Carcinoma of the Esophagus
Squamous cell carcinoma is a known complication of long-standing achalasia. The cancer usually develops in the middle third of the esophagus and frequently is reported as an asymptomatic and unsuspected incidental finding at pathology. When such malignancies do become symptomatic, they are almost always advanced and incurable. Obstruction resulting in dysphagia owing to malignancy develops late in the grossly dilated esophagus of achalasia. Furthermore, the mucosal changes of early malignancy are difficult or impossible to identify at endoscopy. Early abnormalities are obscured by retained food and secretions, the presence of leukoplakia, and inflammatory changes of retention esophagitis, all of which are seen in long-standing achalasia. Skinner and Belsey provide a concise but comprehensive review of malignancy secondary to achalasia. In Belsey's personal series, the incidence of squamous cell cancer was 8% (12 of 158 consecutive cases followed for more than 6 years). All 12 cases were at middle third level, and only one patient survived beyond 5 years.
Type of Esophagectomy Used for Achalasia
A detailed review of the options for esophagectomy and reconstruction can be found in Chap. 21. Part 2 also contains individual chapters that describe the many different approaches to esophagectomy that have evolved over the years.
When the dominant reason for esophagectomy is dysphagia with obstruction and regurgitation, a total thoracic esophagectomy is advised, with the esophagogastric anastomosis in the neck or high in the posterior mediastinum (high Ivor Lewis procedure). All the various techniques of total thoracic esophagectomy have been used and include the transhiatal esophagectomy popularized by Orringer and Stirling.1 A low intrathoracic anastomosis is highly susceptible to damage by reflux, which is aggravated by the remaining aperistaltic thoracic esophagus.
If operation is indicated for squamous cell cancer, which almost invariably develops in the middle third of the thoracic esophagus, a total thoracic esophagectomy is the obvious choice.19 In patients suffering primarily from the disabling symptoms of GERD, an attractive and less morbid alternative to total thoracic esophagectomy is antrectomy, vagotomy, and bile diversion with Roux-en-Y gastrojejunostomy. This operation has been popularized and is increasingly promoted by both Fekete and colleagues in Paris11,20 and Ellis and colleagues in Boston.13,18
Diffuse esophageal spasm is a relatively rare condition of obscure etiology that is difficult to treat medically or surgically with predictably good outcomes. The generally accepted surgical operation consists of a long esophagomyotomy extending from the esophagogastric junction below to the lower border of the aortic arch (or higher) above. The condition is very well reviewed in a report by Ellis published in 1998.21
Odynophagia is the most disabling symptom and is the main indication for esophagectomy. When pain becomes intractable (unbearable to the patient), despite the best available medication and failure of long esophagomyotomy, esophagectomy may be the “court of last resort.” Orringer and Orringer reported on 22 patients with esophageal neuromotor dysfunction who were managed by esophagectomy.12 Eight of these twenty-two patients had primary esophageal spasm. Young and colleagues reported the Mayo Clinic experience with esophagectomy for benign disease between the years 1956 and 1997.16 In a total of 255 patients, they had 10 who were resected for diffuse esophageal spasm. Here again, the resection should remove all malfunctioning thoracic esophagus, which will require a high intrathoracic or cervical anastomosis.