End-stage achalasia. Review articledote_1157

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1 1..12 Diseases of the Esophagus (2010), DOI: /j x Review articledote_1157 End-stage achalasia A. Duranceau, M. Liberman, J. Martin, P. Ferraro Department of Surgery, Université de Montréal, Division of Thoracic Surgery, Centre Hospitalier de l Université de Montréal, Montréal, Québec, Canada SUMMARY. Despite symptom improvement offered to achalasia patients by either pneumatic dilation or surgical myotomy, 10% to 15% of those so treated will present progressive deterioration of their esophageal function and up to 5% may eventually require an esophagectomy. The natural evolution of achalasia to its end stage as well as the timing of esophagectomy in these patients form the basis of this review. The optimal reconstruction for the decompensated resected esophagus will also be explored: gastric interposition, colon interposition, and jejunal interposition all have their respective advantages and disadvantages. Their use is examined in the exclusive context of resection for achalasia. KEY WORDS: achalasia, end stage, esophagectomy, failed treatment, reconstruction. INTRODUCTION Achalasia is the most frequent and best-studied primary motor disorder of the esophagus. It is characterized manometrically by aperistalsis in the esophageal body with a poorly relaxing lower esophageal sphincter that may show a normal or elevated resting pressure. Dysphagia is present in most patients, often accompanied by regurgitation of undigested food. Radiologically the condition has been clinically divided into stages where early achalasia (esophageal diameter <4 cm) moderate achalasia (esophageal diameter 4 6 cm), and severe achalasia (esophageal diameter >6 cm) are present with their respective symptom patterns. 1 Treatment of achalasia aims at palliating symptoms by reducing the lower esophageal sphincter (LES) gradient in order to improve esophageal emptying. Whether this treatment is achieved using pneumatic dilation or via surgical myotomy, the abnormal function present in the esophagus and its lower sphincter never return to normal. The rate of success of any treatment for functional esophageal disease Address correspondence to: Dr Andre Duranceau, MD, Department of Surgery, Université de Montréal, Division of Thoracic Surgery, Centre Hospitalier de l Université de Montréal, 1560, Sherbrooke Est, Montréal, QC, Canada, H2L 4M1. andre.duranceau@umontreal.ca Presented at the 12th World Congress of the International Society for Diseases of the Esophagus Kagoshima, Japan. should always be assessed on prolonged follow up (preferably more than 10 years). A surgical myotomy performed when the esophagus shows a straight axis results in a 90 95% early success rate. Over time, however, this success rate decreases. Pneumatic or hydrostatic dilation improves the symptoms of achalasia and esophageal emptying in 70% of patients. In rare patients, because of comorbidities or age, botulinum toxin injection may be used as a short-acting alternative. Despite these results, 10 15% of the treated population will undergo progressive deterioration of their esophageal function (Ellis, 2 Okike 3 ) and up to 5% of patients may eventually end up with an end-stage achalasia, and require an esophagectomy (Vela 4 ) The goal of this review is to define and analyze what constitutes end-stage achalasia in patients with the idiopathic form of the condition. MATERIALS AND METHOD A literature search for achalasia, end stage, esophagectomy, failed myotomy, failed dilatation was performed using the Medline database. Cross references from the reported papers helped to complete the list of reviewed series. The search selected papers exclusively dealing with idiopathic achalasia patients. Patients with the dilated esophagus secondary to Chagas disease were excluded, being considered a separate category of hypotonic motor dysfunction. 1

2 2 Diseases of the Esophagus The reviewed manuscripts focused on reasons for failure of the initial treatment. The definition of End-stage Achalasia was recorded as defined by each group in order to find a consensus for a unified description of what constitutes the condition. The treatment approach and the selection of a best solution was extracted from each individual experience. No statistical analysis was performed to reach any conclusion. PATHOPHYSIOLOGY AND DEFINITION The initial concept of decompensation was used by Olsen when describing the Mayo Clinic experience with cardiospasm. 1 Although the motor dysfunction remained unchanged, there was an evolution between clinical and radiological stages, where the esophagus lost its ability to contract and progressive dilatation occurred. The larger size esophagus was considered silent, decompensated, and paralyzed. Fekete 5 considered that the very large and flexuous dolichomegaesophagus was in Esophageal Asystole and emphasized that it was usually impossible to surgically straighten the angulation of the sigmoidlike esophagus. He considered that in these patients, there was a definite risk of failure when performing the modified Heller myotomy. Orringer 6 subsequently stimulated interest on this stage of esophageal achalasia when he described the tortuous megaesophagus that mechanically acted as a sinktrap. He then gave the opinion that the esophagus at this stage of the disease would simply not be relieved by incising its lower sphincter. Miller 7 also saw the markedly dilated esophagus as an advanced stage of a condition that might require resection. Peters 8 recognized an end-stage esophagus in patients where failure of previous treatments occurred with persistent or recurrent dysphagia associated with a dilated and tortuous esophagus. Endoscopy showed retained food, a tree-barked aspect of the mucosa, and/or a reflux stricture. Banbury 9 considered end-stage achalasia to be present when disabling dysphagia (including aphagia) with regurgitation and weight loss from profound dietary restrictions occurred despite aggressive treatment of the primary disorder. This was usually associated with massive esophageal dilatation and tortuosity of the esophagus. In summary, the achalasic esophagus should be considered at an end stage when massive dilatation and retention have occurred despite an appropriate initial treatment. Incomplete esophageal emptying with mucosal damage from unresponsive reflux disease or despite multiple attempts at treatment should also be part of that end stage definition. PATHOLOGY The pathology of end-stage achalasia was reported by Lehman 10 and colleagues who examined 35 esophagectomy specimens. Their goal was to assess the morphologic alterations of the esophageal squamous mucosa. In their protocol, biopsies of the mucosa were taken 0.5 cm and 3.5 cm above the gastroesophageal junction. They compared their findings with a control group of 33 esophagectomy resections in pediatric autopsies where the same biopsies were obtained. Findings in the end-stage achalasia patients included first, diffuse squamous hyperplasia with papillomatosis and basal cell hyperplasia; second, an increased number of intra-epithelial lymphocytes that they termed Marked lymphocytic esophagitis ; and third, they observed no squamous dysplasia and no Barrett s esophagus in any of their 35 specimens. Those changes were interpreted as closely resembling the mucosal alterations of gastroesophageal reflux disease. The consistent finding of marked lymphocytic esophagitis is significantly more prominent than that seen in their control specimens. The infiltrate is composed almost exclusively of CD-3 positive lymphocytes with only rare CD-20 positive cells. The nature and significance of this esophagitis remains enigmatic as it may also be seen in gastroesophageal reflux disease, infectious esophagitis, and in mucosa adjacent to tumors. Stasis of luminal contents seems likely to be important in its development. In trying to associate lymphocytic esophagitis to the risk of esophageal squamous cell carcinoma (prevalence of 3.7% Streitz 11 ) these same authors looked at P53 tumor suppressor gene. They observed that 32 of 35 patients (91%) showed positive reactivity with extensive basal staining and even staining of supra basilar cells. This increase in P53 immunoreactivity is seen as early changes related to the increased risk of squamous cell carcinoma. Interestingly, Glatz and Richardson 12 reported two occult carcinomas in the squamous mucosal of their eight resected patients. These cancers were unsuspected at preoperative endoscopic assessment. This emphasizes the facts that achalasia patients are at risk of developing two types of malignant transformation. First, a squamous cell carcinoma in long-standing treated or untreated patients. Second, the development of reflux complications and potentially a columnar-lined esophagus with its known cascade of transformation toward an adenocarcinoma. Although there is no welldocumented benefit for endoscopic surveillance in achalasia patients, the prevalence of these two lesions dictates a prudent approach. Our surveillance recommendations to patients remain arbitrary: a detailed esophagoscopy every 2 to 3 years to document any change to the squamous mucosa or any evidence of reflux complications after treatment.

3 End-stage achalasia 3 When looking at muscle strips obtained at the time of myotomy, Clark 13 observed that pathologically, end-stage achalasia could be defined by complete absence of ganglion cells with severe neural fibrosis and minimal neuritis. While patients with early forms of achalasia showed prominent myenteric infiltrate with lymphocytes, patients with clinically advanced disease had a more variable degree of lymphocyte inflammation in the muscularis: the total number of lymphocytes appears to decrease with disease progression. When identified in the muscle wall, more lymphocytes are identified as CD3/CD8 activated or resting cytotoxic T cells; these cells decrease with disease progression. These observations support the immune-mediated etiology of achalasia with the progressive destruction of ganglion cells and inflammation. Both these pathologic abnormalities disappear in the end-stage achalasia esophagus. sigmoid-shaped esophagus seen in three patients. (vi) Para-esophageal hernia resulting from dividing the phrenoesophageal attachments in the hiatus (one patient). (vii) The development of a squamous cell cancer in the esophagus: four patients. Those failures of the initial myotomy were not all an indication for resection. The esophagus was considered at an end stage if: (i) it was dilated and sigmoid, with persistent obstruction or compressive symptoms, despite the initial myotomy; (ii) the complications of myotomy caused either obstruction or significant mucosal damage with active esophagitis with, or without stricture; (iii) the esophagus was perforated or bleeding; and (iv) severe dysplasia or cancer appeared in the esophageal mucosa. INDICATIONS TO PROCEED WITH RESECTION THE CAUSES FOR FAILED INITIAL TREATMENT The disabling recurrence of symptoms despite aggressive treatment of the primary disorder, usually associated with massive esophageal dilatation and tortuosity of the esophagus might be a definition that obtains a consensus among authors that have experience and interest in this topic. Most authors agree that aggressive treatment for end-stage achalasia should be considered only after all treatment options have been exhausted. Thus, documented failure of adequate treatment remains the first consideration when assessing the patient with end-stage achalasia. In the surgical literature, this is mostly reported in articles looking at reoperations in achalasia patients. The causes for failed treatment with this condition were reported by Fekete: 5 (i) the dolichomegaesophagus; (ii) the development of severe peptic esophagitis either by previously performed cardioplasties or side to side esophagectomy; (iii) the periesophageal sclerosis and hyalinization attributed to poor hemostasis and documented in 15 of their patients. In 1986, Ellis 14 added a more extensive classification of reasons where failed myotomies required re-operations. (i) Inadequate myotomy (incomplete or healed): 17 of their patients had this cause for failure. (ii) Gastroesophageal reflux complications after destruction of the LES by a complete myotomy. Fourteen of their patients, 10 with strictures and four with active esophagitis, were in this category. (iii) Antireflux operations causing obstruction: This was seen in six patients who underwent a total fundoplication after their myotomy. (iv) An incorrect diagnosis, where primarily, in four patients, a total fundoplication was created around an achalasic esophagus. (v) A megaesophagus described as a Even if most patients respond to either dilatation or myotomy, all the above reasons for considering subsequent resection of the esophagus are either the consequence of a functionally obstructed, decompensated, baggy esophagus that does not empty, or of irreversible damage to the mucosa with poor esophageal clearance, threatening complications during the patient s evolution. Fekete, 5 after reporting on their eight patients with a megaesophagus in whom their initial Heller myotomy failed, discussed their unwillingness to perform a resection in these patients because or their benign condition. He suggested that, with the technical advantages of the new mechanical esophagogastric anastomosis, a resection as the first approach to treatment in these patients might be perfectly acceptable as myotomy is not suitable for these forms of esophageal asystole. Orringer 6 in his initial report with esophagectomy for achalasia describes four of 26 patients with a megaesophagus who had resection as the primary treatment. Subsequently, Devaney 15 in reporting the same group s experience in 93 patients describes nine patients undergoing esophagectomy without prior esophageal surgical treatment. This primary esophagectomy approach has prompted significant discussion as most authors considered this form of therapy too drastic. The arguments favoring esophagectomy as first treatment are: a sigmoid shaped esophagus that has lost its axis where esophageal retention may still occur even after an adequate myotomy. At this stage, the esophagus is functionless with high risk of persistent retention, with regurgitations and aspirations in the tracheobronchial tree, and the additional threat of developing dysplasia and carcinoma. Although laparoscopic myotomy has been recommended as a first approach for these megaesophagus, in Orringer s experience, this operation has not provided a reliable relief of dysphagia.

4 4 Diseases of the Esophagus Furthermore, the multiple previous operations on the esophagus becomes a reason for insatisfaction when an esophagectomy is required. The arguments against esophagectomy as an initial approach is that it is considered too drastic. 6 Even if a large consensus exists on the need for an esophagectomy in what is called end-stage achalasia, the majority of surgeons and physicians emphasize that a modified Heller myotomy should always be attempted initially, even in patients with a large sigmoid esophagus, reserving esophagectomy for the failures. 4,16 Patrick and Payne 17 argue that even in the extremely dilated and tortuous esophagus of advanced achalasia, all patients still obtain quite satisfactory results with myotomy alone. Ellis 18 also mentions that his preference is to perform a myotomy first and consider more radical procedures at a second operation if symptoms persist. And this even if it is known that advanced disease patients show results that are not as good as those obtained with early achalasia. In the Mayo Clinic group, Miller 7 reports no patient undergoing esophageal resection as the initial operation. In the Cleveland Clinic report, Banbury 9 writes that esophagectomy for achalasia is considered only after alternative treatment options have been exhausted. Esophagectomy with gastric interposition reconstruction is reserved for patients with documented failure of adequate myotomy who have developed a sigmoid esophagus. Peters, 8 at the University of Southern California, observes that even with a markedly dilated and tortuous esophagus where the benefits of myotomy are reduced, myotomy should still be attempted as the primary option. For this group, the indications to proceed to an esophageal resection are: (i) massive dilatation of the esophagus with failed previous myotomy; (ii) achalasia with multiple previous operations; and (iii) reflux complications. Although it is true that gastroesophageal reflux disease may and does develop following myotomy, the need for a more radical treatment as an esophagectomy has to be weighted against an adequate acid suppression with proton pump inhibitors and anti-h 2 medication. Esophagectomy should be reserved for those patients who, in addition to mucosal damage despite multiple attempts at treatment show a dilated and tortuous esophagus with incomplete emptying. cohort eventually required esophagectomy with an esophageal replacement as the only beneficial therapy. Once the decision to resect has been made, the discussion should focus on the advantages and disadvantages of the selected technique for reconstruction. Even today, the debate over best organ for long-term replacement of the esophagus is still unresolved. Cardioplasty or distal esophagectomy with lower chest reconstruction accompanied by antrectomy and Roux-en-Y diversion has been proposed early as a satisfactory solution. The types of reconstruction operations available and mostly selected today are gastric interposition with reconstruction in the apex of the right chest or in the neck. Colon interposition with a long transplant to the neck or the short colon transplant with reconstruction between the lower esophagus and stomach have also been proposed. Jejunal interposition between the distal esophagus and stomach is another available alternative. These reconstruction techniques and their use are frequently reported in series describing esophageal resection for a variety of benign conditions. Very few reports exist on management of the endstage achalasia esophagus. If evidence-based documentation is requested, even fewer reports document objectively the functional results after resection and reconstruction of the achalasic esophagus. A significant number of questions remain unanswered. In achalasia, the esophagus is and remains aperistaltic in its smooth muscle portion. Is it functionally acceptable to reconstruct with either stomach, colon, or jejunum on the distal still hypotonic and aperistaltic subaortic esophagus? Partial return of peristalsis is well documented in the proximal striated esophagus following myotomy. Should reconstruction always be completed in the apex of the chest or in the neck with the hope of reestablishing continuity with a functional esophagus? What reconstruction offers the safest blood supply and the most functional interposition while offering the best protection against further reflux damage to the esophageal remnant? With today s possibilities of efficient acid suppression for the stomach, where do reflux problems after myotomy become an indication for a more radical operation? And where bile is present, usually resulting in a mixed refluxate, should the solutions proposed be modified? TREATMENT DeMeester, 9 in his discussion of Banbury s presentation, mentions that the success of the initial therapy for achalasia cannot be assessed on symptoms alone. About 10% of treated patients will have progressive dilatation and end up with end-stage achalasia. Vela and Richter 4,16 documented that 5% of their patient CARDIOPLASTY WITH VAGOTOMY, ANTRECTOMY AND ROUX-EN-Y GASTROJEJUNOSTOMY (FIG. 1, TABLE 1) Braghetto and associates, 19 from Chile, reported on 12 patients with failed redomyotomies who were treated with an inverted Y gastroplasty, without any esophageal resection. They added a truncal vagotomy, a distal gastrectomy, and a Roux-en-Y

5 End-stage achalasia 5 distal esophagus. At endoscopy, for the whole group, there was no food retention, no erosive esophagitis, and no Barrett s metaplasia. Despite the wide and permanent opening of the esophagogastric junction, all patients showed improved emptying with a wide gastroplasty anastomosis and a decreased diameter of the esophageal body. DISTAL ESOPHAGECTOMY WITH ANTRECTOMY AND ROUX-EN-Y DIVERSION (FIG. 2, TABLE 1) Fig. 1 Cardioplasty with vagotomy, antrectomy, and Roux-en-Y gastrojejunostomy. duodenal diversion. These patients had a 2- to 10-year follow-up with radiologic endoscopic and clinical evaluation. One patient reported food retention and required reoperation to enlarge the sacular Payne 20 presented his extensive experience with distal esophageal resection with bilateral truncal vagotomy, antrectomy and long limb Roux-en-Y gastric drainage. In 17 patients with distal esophageal stricture 13 (77%) obtained good results from their operation (occasional dysphagia and rare heartburn were present). Twelve percent had fair results and 12% had poor results. This report was for the management of reflux disease complications and their opinion was that heartburn, esophagitis, and stenosis did abate following the use of that operation. Dumping and diarrhea did occur as side effects. During the same year, Washer 21 reported a randomized trial comparing the Roux-en-Y and antrectomy with total fundoplication, to treat esophageal complications of reflux disease. Their conclusions were that results are superior with the antrectomy and Roux-en-Y approach. Using the same principles for treatment, Ellis 14,17,18 and Payne 20 reported this approach with advanced achalasia patients. In Patrick s report, eight patients with achalasia developed reflux esophagitis strictures after their initial treatment: two of them were treated by distal esophageal resection with a low Table 1 End-stage achalasia: cardioplasty or cardiectomy with Roux-en-Y diversion (Figs 1 and 2) Author Year Approach Patients Follow-up Results Braghetto 2010 Inverted y gastroplasty years No food retention No esophagitis Roux-en-Y diversion No Barrett s Improved emptying Patrick 1971 Distal esophagectomy years Improved Antrectomy and Billroth I Cardiectomy years No heartburn excellent Low esophagogastrostomy Antrectomy and Roux y Ferguson (in discussion 1971 Cardiectomy years Comfortable of Patrick) Esophagogastrostomy Antrectomy + Roux-en Y No endoscopic esophagitis Ellis 1975 Cardiectomy months Good to excellent Low esophagogastrostomy Antrectomy, Roux en-y Ellis 1986 Cardiectomy 9 Excellent 6 Low esophagogastrostomy Good 1 Antrectomy Roux-en-Y Fair 1 Poor 1 Fekete 1982 Total duodenal diversion 4 18 months Excellent Gayet 1991 Total duodenal diversion 10 Avoided resection when severe esophagitis present

6 6 Diseases of the Esophagus Fig. 2 Distal esophagectomy with antrectomy and Roux-en-Y diversion. same author reports that eight of 10 patients undergoing total duodenal diversion were free of symptoms with radiologic and endoscopic observations showing healing of esophagitis. One patient developed an ulcer on the gastrojejunal anastomosis after antrectomy without vagotomy. Thus, almost 90% of the patients were classified as improved, most of them reporting excellent results. The rate of improvement after this operation is striking, but symptoms and quality of life reports cannot be considered as objective results. Endoscopic and histologic documentation of successful protection of the remaining esophagus are needed as well as functional and emptying studies are required before reaching definitive conclusions. One of the main disadvantages that could be brought forward is the sacrifice of part of the stomach as a potential organ for reconstruction if more extensive esophageal resection becomes necessary. Despite this argument, a left cervical reconstruction with the stomach, followed by antrectomy and Roux-en-Y diversion to treat cervical remnant esophagitis has been reported by D Journo. 23,24 esophagogastrostomy, truncal vagotomy, antrectomy, and Billroth I anastomosis. Two more had the same esophageal resection with an antrectomy and a long limb Roux-en-Y. All patients experienced improvement and all but one obtained excellent results. The last two patients had fair results with no heartburn, regurgitation, or aspiration. Just as for the refluxing patients, incompetence of the cardia resulting from achalasia treatment can be treated by antrectomy, vagotomy, and long limb jejunal gastric drainage with satisfactory subjective improvement. Ferguson, 17 in discussing Patrick s presentation, mentions six additional patients who had a low esophagogastrectomy with a distal gastrectomy and a Rouxen-Y; after 10 to 19 years of evolution following the operation, all patients are comfortable, have maintained their weight, may present occasional reflux symptoms, but show no esophagitis endoscopically. Roux-en-Y gastrojejunostomy with cardiectomy was used in a further three patients by Ellis. 18 The three patients experienced good to excellent results after their operation. The same author 14 used antrectomy and a Roux-en-Y gastrojejunostomy with a distal esophagectomy and esophagogastrostomy in eight of nine patients. Six had an excellent result, while in one patient results were considered good and in one additional patient the results were fair. One patient had poor results. In Fekete s report, 5 four of their 32 patients requiring resection for achalasia underwent duodenal bypasses after their resection. The indication for resection was peptic strictures, but the extent of the antral resection was not mentioned. Excellent clinical results were obtained in all four patients with an 18 months follow-up. In a subsequent article, 22 the ESOPHAGECTOMY WITH GASTRIC INTERPOSITION IN THE APEX OF THE RIGHT CHEST OR IN THE LEFT NECK (FIG. 3, TABLE 2) Most of the more recent reports on management of end-stage achalasia refer to resection of the obstructed, damaged, or dilated sigmoid esophagus as the only solution to treat this condition. Early on, multiple and varied surgical solutions were proposed. Fig. 3 Esophagectomy with gastric interposition in the apex of the right chest or in the left neck.

7 End-stage achalasia 7 Table 2 Esophagectomy and gastric interposition (Fig. 3) Author Year Approach Patients Follow-up Results Fekete 1982 Esophagectomy 28/34 12 years 70% Good to excellent 10% Fair Gayet 1991 High esophagogastrostomy 5% Poor Waters 1988 Esophagectomy years 8/9 = Excellent or fair Neck anastomosis Orringer 1989 Esophagectomy months 95% on regular diet Early dilatation 46% Transhiatal 24 Regurgitation 42% Transthoracic 2 Excellent 29% Devaney 2001 Esophagectomy months Good 42% Transhiatal 87 Fair 27% Transthoracic 6 Poor 2% Miller 1995 Esophagectomy years Excellent to good for 26/32 High chest or neck anastomosis Banburry 1999 Esophagectomy months Significantly improved Transhiatal 21 Transthoracic 11 Frequent dilatations required Gockel 2004 Transhiatal 6 Improved Esophagectomy Glatz 2007 Esophagectomy years Weight gain No dysphagia High chest anastomosis No reflux Schuchert 2009 Minimally invasive months 10/14 = Good to excellent Esophagectomy During the 1960s and 1970s, gastric surgery aimed at reducing the refluxate or improving gastric emptying. Esophageal surgery aimed at removing mucosal damage while improving the emptying of both esophagus and stomach. Eventually, centers with more experience in esophageal surgery became more selective in their choice of reconstruction. In the early 1980s, Fekete 5 reported 32, and subsequently esophageal resections for what is now called endstage achalasia: 25 had reflux complications, six had massive dilatation including two with diverticulization of the myotomized zone, two had periesophageal sclerosis, and one had a perforated esophagus. Most of their patients underwent some type of esophageal resection with a high esophagogastric anastomosis to reduce the frequency of recurrent esophagitis. Their results over time (12 years) are considered good to excellent (70%), fair (16%), or poor (5%). Following this report, Fekete suggested a more radical primary resection approach for the end-stage dilated esophagus. Later, Ellis 2 et al. after reporting on their excellent results in patients with cardiectomy, antrectomy, and Roux-en-Y, also suggested a more radical approach but only in patients who required reoperations, especially in those with reflux esophagitis with or without stricture and in patients with a megaesophagus. Payne, in discussing Ellis s work, 14 suggests the same aggressive approach at the first reoperation. He even suggests, in that same discussion, an operation that he considers even more aggressive: a transhiatal esophagectomy with a cervical esophagogastrostomy. Major reports on esophageal resection to correct complications of benign esophageal disease appeared during the late 1980s and 1990s, Waters reviewed 21 patients with benign esophageal problems treated by esophageal resection. Nine of their patients had achalasia and seven more had esophageal spastic disorders. For those nine achalasia patients, total esophagectomy with anastomosis to the neck was the preferred treatment, one patient died of sepsis after massive aspiration, eight had excellent (eat normally without aspiration and with no medication) or fair results (occasional dysphagia needing medication or dilation). Orringer 6 initially reported on esophageal resection for advanced achalasia in 26 patients where 18 had a megaesophagus of 8 cm or more. Of these 26 patients, 24 had a transhiatal esophagectomy, and two had their esophagectomy by an open thoracic approach. Four of these patients had their esophagectomy as primary treatment because of a massively dilated esophagus. In all patients, the gastric interposition was placed in the original esophageal bed, in the middle and posterior mediastinum. With an average follow up of 30 months, all but one patient could eat food of normal consistency without postprandial regurgitation. Ten patients complained of varying degrees of cervical dysphagia. Three patients mentioned nocturnal regurgitation, and one psychiatric patient complained of severe regurgitation. No complications from aspirations were observed. Miller 7 reported the Mayo experience with esophageal resection for achalasia. Thirty-seven out of 452 achalasia patients underwent resection, all of them after an initial surgical attempt at treating the condition. The indications for resection were obstructive symptoms in 30, perforation in two, bleeding in two, and the development of a cancer in three patients.

8 8 Diseases of the Esophagus Gastric interposition was the preferred reconstruction of the group. It was used for 70% of the patients treated, considering the stomach as a more reliable replacement organ with a good blood supply and requiring a single anastomosis. Postoperative reflux was not considered a problem in their experience. The results of esophagectomy followed by gastric interposition were considered excellent to good in the 26/32 patients so treated. These results were attributed to the fact that most of the nonfunctional esophagus was resected with a reconstruction completed high in the chest or in the neck. Transhiatal esophagectomy in their experience resulted in more bleeding (3/9) and more deaths (2/9), a morbidity and mortality possibly explained by the difficulties imposed by multiple prior operations. Gockel 28 proceeded to six esophagectomies by an abdominal and cervical approach in patients who had a large esophageal diameter and a failed prior operation. All showed improved dysphagia but little follow-up in time is offered. The vast majority of esophagectomy for end-stage achalasia were approached by open surgery. Orringer 6,15 and Gockel 28 used the abdominal and cervical approach for their patients while the standard laparotomy, thoracotomy, or the three hole approaches were used by the others. Schuchert 29 recently reported the Pittsburgh experience with minimally invasive esophagectomy in six patients who had primary esophageal resections with only balloon dilatation or botulinum toxin injections as prior treatment without any attempt at esophageal myotomy beforehand. To this group they added eight more patients offered the same treatment by the same approach 18 months after failure of a minimally invasive myotomy. No objective follow-up results are available in these patients. Banbury 9 presented the Cleveland Clinic experience with esophagectomy for achalasia. Their preferred reconstruction method was gastric interposition. Thirty-two patients underwent esophagectomy, two of them as an emergency resection because of bleeding. Transhiatal esophagectomy was the procedure of choice in 21/32 patients while 11/32 patients had their operation by open thoracotomy. Thirty-one of the patients had positioning of the gastric transplant in the esophageal bed, while in one patient, a substernal gastric interposition was completed. There was no in-hospital mortality. The reported results show significant improvement in dysphagia and food regurgitation. There was also a significant decrease in dietary restriction. Reflux disease after the transplant was not considered significant. However, over time, younger patients tended to complain of recurrent dysphagia more frequently while those who were operated with a smaller esophagus were more likely to mention reflux and regurgitation symptoms later. For 87% (26/32) of the patients, their condition was improved significantly. Although these authors consider that esophagectomy and gastric interposition offers the best potential for durable improved swallowing, 60% of their operated patients still required a mean of five dilatations per patient during their follow-up, the alluded cause suggested for this being reflux disease. Devaney 15 with the University of Michigan group gave a follow-up presentation to their 1989 experience with 93 patients considered to have end-stage achalasia and treated by esophagectomy. Their indications were a megaesophagus wider than 6 cm (59 patients), reflux with strictures (six patients), and multiple previous operations for the others. Their approach was transhiatal for 87 patients (94%) while six had an open thoracic operation. Nine patients of their group had their esophagectomy without prior esophageal surgery. All of them had a massively dilated esophagus. In this report, attention is given to the potential technical difficulties that should be expected with a transhiatal approach in the end-stage achalasia patient: (i) the deviation of the megaesophagus to the right; (ii) the hypertrophied esophageal arteries emerging from the aorta; (iii) the molding of the esophageal submucosa to the adjacent aorta and lung; and (iv) the more difficult mobilization of the larger cervical esophagus. The authors still emphasize the safety, reliability, and clinical efficacy of esophagectomy followed by a gastric interposition positioned in the posterior mediastinum. The morbidity was mostly related to the esophagogastric anastomosis in the neck with leakage documented in nine patients (10%) and recurrent nerve paralysis seen in five patients (5%). Bleeding occurred in three patients (3%) and chylothorax in two patients (2%). Patients were assessed over time for their symptoms: 95% ate a regular diet without regurgitation. Early dilatation was required in 46% of the group and nocturnal regurgitations occurred in 42% of the patients. Twenty-nine percent of the patients consider their results as excellent, 42% classified them as good. Results are reported as fair for 27% and poor for 2% of this cohort. Glatz and Richardson 12 reported their experience with eight patients treated by esophagectomy completed by laparotomy and right thoracotomy; they had no leaks and no mortality. Their main indications were retained food in a markedly dilated esophagus for seven of their eight patients without any mechanical cause for obstruction. The incidental finding of two occult squamous cancers in the esophageal specimens is revealing of the abnormal mucosa in these patients. ESOPHAGECTOMY WITH SHORT AND LONG COLON INTERPOSITION (FIGS. 4 AND 5, TABLE 3) Colon interposition is the preferred approach for a few groups when reconstructing the esophagus

9 End-stage achalasia 9 esophagectomy and a short isoperistaltic colon interposition based on the left colic artery. The proximal anastomosis was at the level of the inferior pulmonary vein and the distal anastomosis was on the posterior wall of the stomach, with 15 cm of the transplant in the chest and 15 cm in the abdomen. One of the transplants infarcted and an esophagogastrostomy was completed in the left chest. There was no mortality and the morbidity was minimal. Six of the patients eat regular meals, 2/9 eat a soft rice diet with an occasional liquid diet. Three patients of the group are on regular antacids and prokinetics. Six of eight patients are considered to have good results, and one fair. One patient is considered worse. Fig. 4 Esophagectomy with short and long colon interposition. following resection of the end-stage achalasic esophagus. Fekete, 5 Ellis, 18 Thomas, 30 and Gaissert 31 alluded to a small number of patients where they used this reconstruction. Curet-Scott 27 mentions that eight of their 53 patients undergoing esophagectomy for benign esophageal disease had advanced motility disorders. Six of them were achalasia patients and three of these six had a stricture as an indication for resection. Among treated patients, there was one postoperative death in a patient initially resected after a perforation and reconstructed with a long colon interposition 2 months later. Infarction of the transplant was the cause of death. These authors suggest that a short segment colon interposition rather than a long one is preferred if it is going to accommodate the patient s needs. It is considered safer. The results in those patients are considered excellent or good for 72% while 9% of patients consider their results poor. Peters 8 reported on 19 patients with achalasia requiring resection. Seven had multiple previous operations, six had a stricture, five had a massively dilated and tortuous esophagus, and one suffered an upper gastrointestinal hemorrhage. All were treated using an isoperistaltic long colon interposition pedicled on the inferior mesenteric artery. There were no deaths, no anastomotic leak, and no graft necrosis. There were two reoperations, one for bleeding and one for chylothorax. Fifteen patients were assessed by standardized questionnaires. The results were excellent or good in 12/15, improved in two, and without improvement in one. Fourteen out of 15 were satisfied with the improvement of their symptoms. Hsu 32 reported on nine achalasia patients treated by JEJUNAL INTERPOSITION (FIG. 6, TABLE 4) Merendino and Dillard 33 recommended resection for selected cases of megaesophagus with a jejunal interposition to restore the continuity between esophagus and stomach. An occasional report using this approach can be found in surgical series; however, the operation has not been met with wide acceptance. Picchio 34 analyzed their experience retrospectively in 21 patients who developed peptic stricture following myotomy for achalasia. All their patients had severe mucosal damage at endoscopy. Fig. 5 Esophagectomy with short and long colon interposition.

10 10 Diseases of the Esophagus Table 3 End-stage achalasia: colon interposition (Figs. 4 and 5) Author Year Approach Patients Follow up Results Long colon interposition Curet-Scott 1987 Esophagectomy 8 Not specified Not specified Long or short interposition Gayet 1991 Belsey colon interposition 5 Not specified Not specified Thomas 1997 Long interposition (isoperistaltic) 3 Not specified Not specified Peters 1995 Long interposition (isoperistaltic) years In 12/15: Excellent to good Improved: 2 Not improved: 1 Short colon interposition Hsu 2003 Distal esophagectomy 9 6 years 6/9: Regular meals Isoperistaltic short colon 2/9: Soft rice diet Gaissert 1993 Distal esophagectomy 2 87 months Not specified Isoperistaltic short colon Table 4 End-stage achalasia : jejunal interposition (Fig. 6) Author Year Approach Patients Follow up Results Picchio 1997 Esophago-jejunogastroplasty years (1 16) 20 patients Esophagojejunal strictures in all Excellent: 0 Good: 17 Fair: 2 Poor: 1 Kiss 1996 Esophago-jejunogastrostomy 14 Unclear Excellent or good in most They used Merendino s technique with a cm. jejunal interposition between the esophagus and the anterior wall of the stomach. Patients were followed for 1 to 16 years (mean: 11 years). Based on symptom evaluation, none of their patients were considered to have excellent results. For 85% of their patients (17/21) results were good with mild occasional dysphagia and regurgitation. Two patients (10%) were considered to have fair results mainly because of post prandial fullness and diarrhea. One patient had poor results with a redundant jejunal loop. The mortality was 4% (one patient) and was related to heart failure. Kiss et al. 35 report excellent to good results in their Fig. 6 Jejunal interposition. 14 patients who received an isoperistaltic jejunal interposition between the distal esophagus and the stomach. CONCLUSION When the esophagus is rendered unusable by natural evolution of the disease or from complications following attempts at treatment, a resection may become necessary. From the existing literature discussing management of the End-stage achalasia requiring resection, there are lessons to be learned. The ideal method of reconstruction is not yet established. Resection followed by gastric interposition has the advantage of a single anastomosis (when not counting or adding a pyloroplasty). The operation however remains morbid. The vascular supply to the proximal stomach is rarely perfect when considering the prevalence of anastomotic complications in the neck. Furthermore, reflux disease with its mucosal damage is virtually always present on the esophageal remnant, 23,24 making this reconstruction an ideal clinical model for reflux disease. As suggested early by Ellis and Payne, 14,17,18 this situation can be improved by an antrectomy and a Roux-en-Y reconstruction. There is very little objective information on the functional response of the stomach after this operation in achalasia patients. When the esophagectomy and esophagogastrostomy are distal, the reconstruction is on an achalasic distal esophagus that

11 End-stage achalasia 11 cannot regain function. Well quantified emptying studies with meticulous endoscopic and histologic documentation of the remaining esophagus are needed. If the Roux-en-Y is added primarily to a gastric interposition, with a reconstruction high in the chest or in the neck, the complexity of the operation might be compared to that of a long limb colon interposition. Adding the antrectomy and bile diversion to an already performed gastric interposition has its own complexity although the endoscopic results on the esophageal remnant are excellent. 24 Colon interposition is more complex because of the vascular anatomy of the transplant and because of the three anastomoses required to complete the operation. Once again, objective function, endoscopic, and histologic evaluation of the transplant over time are still needed, especially for achalasia patients. Is a short interposition, as emphasized, if it serves its needs, a good selection for the achalasia patient? Possibly; however, the achalasic esophagus as well as the transplanted organ need close follow-up for their respective long-term evolution results and evolution of complications. Jeyasingham reported in ,37 the Bristol series with 365 colon interpositions, possibly the largest experience available with this reconstructive approach. Sixty-nine of these reconstructions were long interposition while 296 were short interpositions between the subaortic esophagus and the posterior stomach. For long interpositions, ischemia at the distal extremity of the transplant is perceived as a risk for fistula and stricture formation at the cervical anastomotic level. In addition, redundancy at the supraaortic, supradiaphragmatic, and subdiaphragmatic levels becomes responsible for functional and mechanical dysfunction that may require reintervention in time. Neoplastic transformation at the cologastric junction is also important. The short segment colonic transplant is an isoperistaltic colon replacement based on either the middle colic or the left colic arterial supply. It is perceived as an excellent operation with minimal and mostly preventable morbidity. Jejunal interposition was initially reported by Merendino 33 to give satisfactory results. But this experience was mostly in patients with idiopathic reflux strictures. In achalasia patients, Pischio 34 reports favorable follow-up comparable with that of colon interpositions. The vascular supply however is less reliable, and limits the reconstruction to the distal esophagus, unless microvascular revascularisation is added. The problem of redundency persists. At present, from the published literature, when achalasia has reached an end stage, gastric interposition remains, in written reports, the first choice for reconstruction. 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