Introduction

Achalasia is an idiopathic motility disorder of the esophagus characterized by aperistalsis of the esophageal body and failure of complete relaxation of the lower esophageal sphincter (LES) during deglutition.1 Treatment options are palliative in nature and are directed at decreasing the lower esophageal sphincter pressure (LESP) to promote enhanced esophageal emptying.2 Though Heller myotomy with partial fundoplication can achieve excellent long-term outcomes in 80–90% of patients with achalasia,3,4 the optimal therapy for patients with sigmoidal esophageal changes remains controversial (Fig. 1).

Figure 1
figure 1

Radiographic features of achalasia. a Barium esophagram demonstrating classic “bird’s beak” appearance consistent with achalasia. b Dilated and tortuous esophagus (sigmoidal esophagus) in a patient with chronic achalasia.

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Several authors advocate myotomy as the primary treatment in patients with sigmoidal esophagus, reserving esophagectomy for myotomy failure and persistent symptoms.5,6 Others recommend primary esophagectomy, citing concerns regarding the capacity of a dilated, sigmoidal esophagus to empty efficiently even with a myotomy.7–9 The aim of the current study is to review our experience with minimally invasive myotomy (MIM) and minimally invasive esophagectomy (MIE) in the treatment of patients with achalasia and sigmoidal esophagus.

Materials and Methods

Patients

Approval for this study was provided by the Institutional Review Board of the University of Pittsburgh. We performed a retrospective review of 250 patients undergoing minimally invasive Heller myotomy or MIE for achalasia at the University of Pittsburgh from 1992 to 2007. Sigmoidal esophageal changes were identified in 19 male and 11 female patients with a mean age of 59.1 years (range 25–83 years) (Table 1).

Table 1 Patient Demographics and Preoperative Data
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Preoperative Evaluation and Therapy

The diagnosis of achalasia was confirmed by barium esophagram demonstrating the classic appearance of achalasia (proximal esophageal dilation with a distal “bird’s beak”) and esophageal manometry (aperistalsis; LES with incomplete relaxation) when possible. In addition, each patient demonstrated sigmoidal esophageal changes by barium esophagram in varying geometrical configurations and with varying degrees of associated esophageal dilation. Preoperative manometry data is available in only 11 patients. Manometry was not required or was unable to be performed in the remaining patients due to difficulty in positioning the probe within the tortuous esophagus or to patient intolerance. All patients had a preoperative esophagogastroduodenoscopy to confirm the absence of other esophageal pathology prior to surgery. The most common presenting symptom was persistent or progressive dysphagia (95%), followed by regurgitation. Dysphagia scores were assessed preoperatively and postoperatively utilizing the following scale: 1 = no dysphagia; 2 = difficulty with hard solids; 3 = difficulty with soft solids 4 = difficulty with liquids; 5 = cannot swallow saliva. Mean preoperative dysphagia score was 3.0. Mean duration of symptoms for the entire patient cohort was 18.7 years. Endoscopic therapy (balloon dilation, botulinum toxin injection, or both) was performed in 19 out of 24 (79.2%) patients prior to Heller myotomy and in six out of six (100%) patients prior to MIE. Prior myotomy had been performed in three (10%) patients—two in the myotomy group and one in the esophagectomy group.

Operative Technique

A minimally invasive approach was performed in all patients. Minimally invasive esophagomyotomy (±partial fundoplication) was performed in 24 patients as described previously.10 A partial fundoplication was performed in 22 out of 24 (91.7%) of the patients undergoing myotomy. In 17 (70.8%) patients, a posterior (Toupet) fundoplication was performed. An anterior (Dor) wrap was performed in five (20.8%) patients. Two patients (8.3%) underwent myotomy alone (Table 2). MIE was performed in six patients, as described previously.11

Table 2 Postoperative Outcomes
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Postoperative Course

For MIM, patients were typically extubated in the operating room at the conclusion of the case. A barium swallow was performed on the first postoperative day and, if satisfactory, a clear liquid diet was initiated. Patients were typically discharged on the second postoperative day. Our current protocol is to evaluate the patients at 2 weeks for the first postoperative visit. Follow-up at 6 months, 1 year, and yearly thereafter is performed with repeat barium swallows. Manometry and esophageal transit studies are optional, based on surgeon and patient preference.

After MIE, patients remain nil per os with nasogastric tube decompression. Tube feeds are initiated at a low rate on postoperative day 3. A barium swallow is performed on postoperative day 5, after which a clear liquid diet is instituted. Patients are discharged home on a clear liquid diet and cycled night-time tube feeds. Patients are evaluated at 2 weeks, and their diet is advanced. The feeding tube is removed during their second postoperative visit if the diet is being tolerated well. Patients are then followed up yearly with repeat barium swallows.

Follow-Up

Follow-up data was successfully acquired in all patients. The primary postoperative outcome variables included length of stay, morbidity, mortality, and need for reoperation. Myotomy failure was defined as patients with no improvement in dysphagia score or those requiring reoperation (redo myotomy or esophagectomy). Postoperative dysphagia scores were assessed at each clinic or hospital visit and compared with preoperative values. Mean follow-up for all patients (MIM and MIE) was 25.3 months.

Statistical Analysis

Data were summarized with descriptive statistics (mean, standard deviation, median, and range for continuous variables, frequency, and percentage for categorical variables). A log rank test was used to assess the association between time to failure and age, duration of symptoms, previous myotomy, prior endoscopic treatment, and preoperative LESP. Univariate Cox regression model was used to assess the association between time to failure and age, duration of symptoms, and preoperative LESP as continuous variables, respectively. Multivariate Cox regression model was used to assess the association between time to failure and age, duration of symptom, and previous myotomy simultaneously.

Results

Perioperative Outcomes

Demographics and preoperative data are detailed in Table 1. Patients undergoing primary MIE were younger, had a higher rate of preoperative interventions, a lower LESP, and a higher incidence of “end-stage” sigmoidal megaesophagus on barium esophagram. None of these features attained statistical significance. Duration of symptoms and mean preoperative dysphagia scores were similar between the two groups. There were no perioperative deaths. MIM was associated with a similar improvement in symptoms compared with MIE, but had a significantly shorter length of hospital stay. There were trends towards less morbidity and reduced need for postoperative endoscopic interventions, though these did not attain statistical significance (Table 2). Complications were few and are detailed in Table 3. Pneumonia was the most common complication among the MIM group (8.3%). Complications in the MIE group included a contained anastomotic leak treated with opening of the cervical wound, a hemothorax necessitating drainage by video-assisted thoracoscopic surgery, and a pleural effusion.

Table 3 Complications
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Surgical Management of Sigmoidal Esophagus

From 1992 to 2007, 30 patients with sigmoidal esophageal changes in the setting of achalasia underwent surgical intervention. Among these, minimally invasive Heller myotomy was performed in 24 patients (Fig. 2). Partial fundoplication was performed in 22 (91.7%) of these patients (17 Toupet, five Dor). The remaining two patients underwent myotomy alone. Six patients received primary MIE. The principal criterion for primary esophagectomy was an end-stage sigmoidal esophagus in fit patients where long-term functionality of the esophagus was in doubt (Fig. 3). Among the 24 patients undergoing MIM, 15 patients (62.5%) had durable relief of dysphagia and required no further surgical intervention. Seven patients (29.2%) developed recurrent dysphagia and/or regurgitation, requiring subsequent esophagectomy. One patient required takedown of the partial wrap and extension of the myotomy, with improvement in her symptoms. One patient had no durable improvement after MIM and is considered a failure of therapy, but refused further surgical intervention and has been managed with periodic dilations (Fig. 2).

Figure 2
figure 2

Clinical outcomes of patients with sigmoid esophagus.

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Figure 3
figure 3

End-stage sigmoidal esophagus. Significant dilation is noted with food and fluid retention.

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Analysis of Myotomy Failures

During follow-up (mean 30.5 months; range 0.3–105.4 months), nine patients (37.5%) undergoing MIM were considered clinical failures due to the need for subsequent operative intervention (n = 8) or lack of symptomatic improvement (n = 1). Mean time to myotomy failure was 18.4 months. Interestingly, there was no apparent correlation between preoperative radiographic findings and outcomes in either group. Similarly, there was no apparent correlation with surgical approach and rate of failure following myotomy. There were two failures with the Dor approach (40%), six with the Toupet approach (35.3%), and one with myotomy alone (50%).

A comparison of the clinical features between successful and failed myotomy is shown in Table 4. All patients who failed myotomy had prior endoscopic interventions. Both patients with a history of previous myotomy undergoing a redo myotomy failed and required subsequent esophagectomy. Myotomy failure was associated with a trend toward lower preoperative LESP (19.5 versus 33.8 mmHg) and longer duration of symptoms (24.8 versus 13.1 years) when compared with patients with successful outcomes after MIM (Table 4). Univariate analysis to assess the association between myotomy failure and age, duration of symptoms, prior myotomy, preoperative LESP, and prior endoscopic therapy was performed. Univariate analysis showed that previous myotomy and duration of symptoms were significant predictors of failure of MIM, with patient age demonstrating a strong trend toward significance. Multivariate analysis identified patient age and symptom duration as independent predictors of myotomy failure (Table 5).

Table 4 Analysis of Failures After MIM
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Table 5 Univariate and Multivariate Analyses
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Discussion

MIM has been established as a highly effective treatment modality in the management of patients with achalasia, with a greater than 80–90% long-term success rate.12,13 The development of sigmoidal esophageal changes in the setting of achalasia presents a unique set of challenges in surgical management. As achalasia progresses, the esophagus can progressively dilate and acquire tortuous undulations due to persistent obstruction to forward flow, retained foodstuff, and pressurization from swallowed air.8 These anatomic features further impair esophageal emptying and promote food retention and/or impaction, resulting in recurring dysphagia as well as regurgitation. The progressive nature of these findings has led some to believe that myotomy alone is unlikely to succeed long-term in patients with dilated, sigmoidal esophageal changes where a functionless esophagus fails to empty efficiently even after myotomy, promoting the risk of retention esophagitis, regurgitation, and carcinoma.9,14 From this perspective, esophagectomy provides a definitive “curative” solution for advanced or refractory achalasia with excellent relief of symptoms in the majority of patients.9,15,16

Concern is routinely raised regarding the morbidity and mortality of esophagectomy for benign disease. In experienced centers, mortality rates range from 1% to 5%.17 MIE is a particularly useful technique in the setting of advanced achalasia. Several technical concerns encountered during open esophagectomy for achalasia include difficulty encircling the dilated esophagus, deviation of the esophagus into the right chest, enlarged aortoesophageal arteries, and the adherence of the exposed esophageal submucosa to the adjacent aorta subsequent to myotomy.9 The enhanced visualization achieved with a laparoscopic/thoracoscopic approach can augment the successful management of each of these issues with acceptable morbidity and low mortality (1.4%).11

Several studies have shown, however, that esophageal resection may not be necessary in all patients with a sigmoid esophagus. Patti and colleagues evaluated the outcomes of seven patients with sigmoidal esophageal changes treated primarily with laparoscopic Heller myotomy and Dor fundoplication.5 They were able to achieve good–excellent results in 100% of the patients with complete resolution or significant improvement in dysphagia, regurgitation, and chest pain. None of the patients in their study required further operative intervention. Mineo and coworkers evaluated 14 patients with achalasic sigmoid esophagus treated with an open or minimally invasive Heller myotomy and Dor fundoplication by the same surgeon.6 In this series, ten out of 14 patients (71.4%) achieved good–excellent results with similar morbidities and hospital stays after the operation compared to those patients without a dilated esophagus. In addition, a substantial reduction in postoperative LESP and esophageal width was noted secondary to the relief of distal obstruction. Importantly, quality of life measures (SF-36) improved in all measured domains.

Currently, there are no randomized data available to definitively establish who should undergo primary myotomy or esophagectomy in the setting of advanced achalasia with sigmoid esophagus. The Practice Parameters Committee of the American College of Gastroenterology currently recommends graded pneumatic dilatation or laparoscopic myotomy as primary therapy for patients with achalasia (including early sigmoidal changes) and propose esophagectomy for those patients with megaesophagus (>8 cm) and those with low LESP with persistent symptoms.18 In addition to sigmoid esophagus, several preoperative features have been postulated to impact negatively upon the results of myotomy including low preoperative LESP,19,20 prior endoscopic therapy,21,22 and longer duration of symptoms.23 In the current series, each of these variables was seen more commonly in patients who failed primary myotomy (Table 4). Multivariate analysis confirmed age and duration of symptoms as independent risk factors for myotomy failure (Table 5).

Despite this, the majority (15 out of 24; 62.5%) of patients with sigmoidal esophagus in the present study achieved durable improvement in dysphagia and regurgitation, requiring no further operative intervention (redo myotomy or esophagectomy) at a mean follow-up of 30.5 months. In addition, myotomy was associated with reduced length of stay and a trend toward decreased morbidity compared with esophagectomy (Table 2). Similar to the observations of Mineo and associates,6 the relief of distal obstruction not only improved patient symptoms, but was associated with improved anatomic features (decreased width and curvature, improved emptying) of the esophagus on barium esophagram in several cases (Fig. 4).

Figure 4
figure 4

Radiographic improvement after Heller myotomy and relief of distal esophageal obstruction (a, c preoperative; b, d postoperative). a Sigmoidal esophagus prior to Heller myotomy. b Improved emptying of sigmoidal esophagus immediately following Heller myotomy. c Significant dilation and sigmoidal changes prior to Heller myotomy. d Decreased distension and sigmoidal angulation, as well as improved emptying, 3.5 years after Heller myotomy.

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Conclusion

Minimally invasive Heller myotomy can be performed safely in patients with sigmoidal esophageal changes and can be successful in many patients with this condition. Significant symptomatic improvement can be achieved in approximately two thirds of these patients, without need for further operative intervention. Younger patients with longer duration of symptoms are at higher risk for myotomy failure, and consideration should be given to primary esophagectomy in this setting. Failure of myotomy in the setting of sigmoidal esophageal changes is likely multifactorial in nature, however, and the decision to perform primary myotomy or esophagectomy should thus be individualized based on patient characteristics as well as surgeon experience and judgment. Further prospective studies with longer-term follow-up are required to define the optimal treatment algorithm in these patients.