Chapter 14. Benign Esophageal Disorders

The esophagus is a muscular tube whose function is to transport ingested material from the pharynx to the stomach. Its function is the result of a complex symphony of neuromuscular coordination. The esophagus is subject to a variety of disorders, both congenital and acquired. This chapter deals with the most common benign disorders encountered by the surgeon. These include paraesophageal hernias (PEHs), esophageal diverticula, and motility disorders. Our goal is to provide a logical and efficient approach to the evaluation and management of these disorders. Esophageal malignancy and gastroesophageal reflux disease (GERD) are addressed elsewhere in this book.

Paraesophageal hernias (PEHs) result from a defect at the diaphragmatic hiatus. Upward displacement of abdominal contents into the mediastinum occurs due to widening of the hiatal aperture between the right and left crura. The negative pressure of the chest creates a downward pressure gradient from the abdomen further facilitating this shift. Herniation may result from congenital anatomic causes, or it may be a result of trauma or iatrogenic causes. Prior surgery involving the gastroesophageal junction (GEJ), including esophageal mobilization, crural repair, or fundoplication, can result in PEH formation. The stomach is the organ most frequently involved; other organs including the colon, omentum, spleen, liver, and pancreas may also be associated.

Etiology and Anatomic Classification

Hiatal hernias are classified according to the location of the GEJ in relation to the diaphragmatic hiatus and also by the contents of the hernia sac. Type I hiatal hernias are by far most common and are characterized by cephalad displacement of the GEJ above the hiatus into the mediastinum (Fig. 14-1). Type I hiatal hernias are often referred to as sliding hiatal hernias and are typically reducible. Patients with type I hiatal hernias often suffer from gastroesophageal reflux (GER) as a consequence of the altered anatomy and mechanical function of the lower esophageal sphincter (LES) and hiatal complex. Loss of intra-abdominal esophageal length and alteration of the angle of His contribute to this. If type I hiatal hernias enlarge significantly, they may become fixed above the hiatus.

Type II hiatal hernias are considered true paraesophageal hernias and result from cephalad displacement of the fundus of the stomach into the mediastinum. The GEJ itself remains in its normal intra-abdominal location (Fig. 14-2). Dysphagia is a common symptom associated with a type II hiatal hernia, usually due to compression of the esophagus by the stomach. These types of hernia are also referred to as “rolling” hernias. Herniated portions of the stomach are typically found in the posterior mediastinum. These are the least common type of hiatal hernia.

Type III hiatal hernias are also true paraesophageal hernias and are best thought of as a combination of types I and II whereby both the GEJ and a portion of the stomach have herniated above the diaphragmatic hiatus (Fig. 14-3). These can become quite large and may involve complete herniation of the stomach into the thorax. The anchoring attachments of the stomach such as the gastrosplenic ligament and phrenogastric ligaments can become quite attenuated and stretched. This type of hernia can result in partial or complete outlet obstruction as well as in volvulus. Both organoaxial volvulus, where the stomach twists along its longitudinal axis, and mesoaxial volvulus, where the stomach flips anteriorly along its transverse axis, can occur. Organoaxial volvulus is more common. Patients will typically complain of reflux, dysphagia, regurgitation, and respiratory symptoms, all resulting from the displacement and altered mechanics of both the GEJ and the stomach.

Type IV hiatal hernias are distinguished by herniation of other abdominal viscera or omentum above the diaphragm. This can occur in association with either a type II or III PEH. The transverse colon and omentum are frequently involved, but other organs such as the spleen, liver, and pancreas may also be involved. Presenting symptoms can vary with particular organ involvement.

Clinical Presentation

Presentation of patients with PEH can vary widely from an incidental finding to an emergent presentation involving strangulation. Symptoms are often nonspecific and can include nausea, dysphagia, dyspnea, heartburn, regurgitation, bloating, chest pain, abdominal pain, early satiety, and aspiration leading to pneumonia. Severe pain is an ominous sign and usually indicates volvulus or incarceration evolving to strangulation. Symptoms can also be vague and intermittent with patients experiencing relief of their symptoms with shifting of their hernia contents or with relief of visceral torsion.

Iron deficiency anemia resulting from gastrointestinal (GI) bleeding due to mucosal ischemia is a common presenting finding in patients with PEH, affecting over one-third of patients with this condition.1 This results from mucosal irritation and ischemia occurring at the neck of the hernia sac where the crura are extrinsically compressing and rubbing against the gastric fundus, resulting in the linear gastric erosions known as Cameron's ulcers.1–3 It is usually not until after exhaustive workup for other causes of anemia that the diagnosis of PEH as the offending agent is obtained. Surgical correction of the hernia results in resolution of the anemia.4

While the natural history of paraesophageal hernias is not clearly known, it is known that many of these hernias are incidental findings. They are commonly discovered on chest x-rays, CT scans, or during upper endoscopies being performed for other reasons. This is revealing as it tells us that the true incidence remains unclear.

Diagnosis and Evaluation

The physical examination is frequently unimpressive and nonspecific in these patients. Abdominal examination is usually unremarkable. Chest examination with auscultation may reveal decreased breath sounds on the affected side or the presence of bowel sounds within the chest. It is not uncommon for patients to undergo extensive workup for noncardiac chest or abdominal pain, ultimately arriving at upper GI (UGI) evaluation with which the diagnosis is made. Upper GI endoscopy and imaging studies are the mainstays of diagnosis and evaluation.

Imaging Studies

Chest x-rays, whether obtained for entirely unrelated reasons or not, can give the diagnosis of PEH. Common findings on chest films include a retrocardiac air-fluid level, resulting from an intrathoracic stomach (Fig. 14-4). Coiling of a nasogastric tube above the diaphragm is another classic finding.

The upper GI barium swallow/esophagogram is an essential part of the workup for these patients and often gives the most accurate information regarding the hernia's anatomy and position, as well as the location of the gastroesophageal junction. It can also offer some functional information regarding esophageal peristalsis and reflux, though it is not the best test to evaluate esophageal function (Fig. 14-5).

Computed tomography (CT) is not typically used in the workup of PEH. Its frequent use in patient workup for other reasons often leads to diagnosis of PEH when present. CT is a good modality to differentiate from other hernias of the diaphragm such as Morgagni's hernia or to evaluate hernia contents in a type IV hiatal hernia.

Endoscopy

Flexible esophagogastroduodenoscopy (EGD) is an extremely useful diagnostic test and one that is necessary as part of the workup for PEH. EGD allows the operator to evaluate the gastroesophageal junction and the size of the hernia, which are both seen best on retroflexion. Navigating the anatomy can be a challenge as displacement and extrinsic compression of the esophageal lumen or stomach alter anatomic landmarks. An important part of the endoscopic evaluation is to screen for Barrett's esophagus and malignancy. The presence of either of these can alter therapy.

Manometry and pH Testing

Ambulatory pH testing and esophageal manometry may be useful adjuncts in the workup of PEH. They can be technically hard to perform, as intubation of the LES may be impossible to achieve due to anatomic distortion. Clarifying esophageal function in these patients with manometry, while useful, very rarely changes our operative plan. Only under circumstances of complete aperistalsis of the esophagus do we deviate from performing a routine Nissen fundoplication as part of the PEH repair. In these circumstances, either the fundoplication is omitted, or a partial fundoplication (Dor or Toupet) is performed. Because of the difficulty in successfully performing the test and because reflux is addressed surgically in our standard PEH repair with Nissen fundoplication, we do not mandate 24-hour pH testing. For those who perform fundoplication selectively, it may make even more sense to pursue 24-hour pH studies. That said, preoperative pH studies are probably a poor predictor of GERD after repair if a fundoplication is not done, because the hernia itself stretches the phrenoesophageal membrane and the other natural antireflux anatomy. In other words, even if patients do not have GERD before repair, most will develop GERD if a fundoplication is not employed. For patients whose symptoms are primarily related to suspected GERD, pH monitoring should be done to confirm the reason for surgical intervention.

Indications for Treatment

The indications for operating on patients with PEH continue to evolve over time. Earlier surgical tenets deemed the diagnosis of PEH an indication for surgical correction. This was largely in part due to retrospective observations, published by Skinner, Belsey, and Hill in the late 1960s and early 1970s and from other small case series or reports in which a high incidence of complications and mortality associated with observation or emergent operation was observed. Skinner followed 21 asymptomatic patients, of which 6 (29%) developed complications of bleeding, perforation, or strangulation with observation. This, in addition to an observed pooled mortality rate of 17% with emergent operation but a 1% mortality rate with elective repair, led to the recommendation that all patients fit for surgery should undergo repair of PEH.5–7 We now know that these hernias are not always symptomatic, often discovered incidentally, less likely than previously thought to present with acute complications, and may not necessarily require repair.

Recently, Stylopoulos and colleagues performed a population-based study showing that mortality rates associated with observation to be lower than previously reported, and the mortality associated with emergent operation to be 5.4% rather than 17%. Their study used a population-based decision model to estimate the risk of watchful waiting versus repair in a cohort of 5 million patients, using available data from the 1997 Nationwide Inpatient Sample (NIS) on PEHs. They estimated that the perioperative mortality rate of those undergoing elective repair to be 1.4% and the annual risk of complications associated with watchful waiting to be 1%. As a result, they concluded that watchful waiting in an asymptomatic or minimally symptomatic population is a reasonable strategy.8 We believe that this is a reasonable approach, especially in elderly patients with multiple comorbidities and asymptomatic hernias. We have found their data to be consistent with our clinical experience. Young (<65 years) and fit patients, even if not symptomatic, should probably be considered candidates for repair because they have many years to become symptomatic or develop an acute volvulus. Obviously, patients who are symptomatic or who have demonstrated progression of their symptoms should be evaluated and taken for elective repair if their medical condition allows.

Therapeutic Controversies

Widely differing opinions exist regarding the aspects of surgical management for paraesophageal hernias. They center on the ideal approach, the use of mesh, management of the shortened esophagus, and the use of fundoplication. As one develops an operative strategy, it is important to keep in mind the fundamental steps that are considered universal in PEH repair: reduction of the stomach into the abdomen without tension, excision of the hernia sac, reapproximation of the crura, and anchoring of the stomach.

Operative Approach

Laparoscopic, open laparotomy, and transthoracic approaches have been described for PEH repair. Each has advantages and disadvantages. Thoracotomy offers excellent visualization and it is easier to perform gastroplasty for esophageal lengthening from this approach. Performing a fundoplication from the chest, however, is difficult to accomplish. This approach confers the most morbidity, with longer hospital stays, need for single-lung ventilation, and postoperative chest-tube drainage. Open laparotomy approach is familiar to most surgeons, avoids a chest incision and its associated morbidity, and may result in decreased operative time. Visualization of the mediastinal structures and exposure of the hiatus, however, are difficult with this approach.

The laparoscopic approach overcomes many of the drawbacks of the two conventional open approaches. It provides good visualization of the hiatus and mediastinal structures, and allows for easier creation of a fundoplication. High mobilization of the esophagus into the mediastinum is possible. Avoiding the chest as an open operative field eliminates the need for single-lung ventilation, chest tubes, and the postoperative pain associated with thoracotomy. Since the introduction of laparoscopic techniques, many studies have confirmed its feasibility, safety, quicker recovery, and shorter length of stay.9,10 While our approach is laparoscopic, we cannot overemphasize that these are complex operations to perform laparoscopically, even for those with advanced laparoscopic foregut experience. They should be left in the hands of experienced laparoscopic foregut surgeons. No randomized clinical trials have been performed comparing the various approaches.

Crural Repair

As recurrence is a major outcome measure with any hernia repair, it is important to examine the crural repair component of PEH repair. Crural repair and its longevity, as with any hernia repair, depends on a tension-free closure. Many strategies have been employed to overcome the tendency toward recurrence and improve the chances of healing at the hiatus. These have included the use of pledgets, relaxing incisions, and various types of prosthetic mesh. Two randomized trials comparing hiatal closure with and without mesh were performed by Frantzides et al and Carlson et al.11,12 Carlson's study randomized patients with PEH to simple suture cruroplasty or cruroplasty with polytetrafluoroethylene (PTFE) and all patients had a Nissen fundoplication. They showed a reduction in hiatal hernia recurrence in patients who received a mesh closure (18.8% recurrence with simple cruroplasty vs 0% recurrence with PTFE-reinforced cruroplasty). Frantzides performed a study that included patients with all types of hiatal hernia (type I–IV) in which patients with hernia defects of greater than 8 cm were randomized to simple suture cruroplasty or cruroplasty with PTFE mesh. At a median follow-up of 2.5 years, a 22% recurrence rate with simple cruroplasty was observed and no recurrences with mesh repair.

Initial enthusiasm and results, mostly from small series, have been tempered with increasing reports of complications at the hiatus because of prosthetic mesh placement, including migration, infection, dysphagia, and erosion into the esophagus. Polypropylene exhibits significant shrinkage due to hydrolysis and adhesions, and we do not recommend its use at the hiatus. PTFE produces fewer adhesions, but erosion into the esophagus can occur.13 Erosion into the esophagus is a serious complication that usually requires esophagogastrectomy for treatment and is a matter of high consequence.

Our preference has been to use a biologic mesh product to reinforce the primary closure. Biologic meshes act as a collagen-based absorbable bioscaffold into which native tissue ingrowth occurs. These materials potentially address the concerns of erosion, infection, and dysphagia associated with permanent prosthetic mesh placement at the hiatus.14 To test this approach, we conducted a multicenter randomized trial in which patients were randomized to primary repair (n = 57) or primary repair buttressed with a biologic prosthesis (n = 51, small intestinal submucosa [SIS]). The primary outcome measure was recurrence seen on UGI. Upon completion of our study, we observed a reduction in hernia recurrence rate from 24% down to 9% in 95 patients at 6 months.15 While our results only represent a 6-month follow-up, Jacobs and colleagues demonstrated excellent results with SIS mesh cruroplasty at a median follow-up of 28 months without complications and with similarly low recurrence rates.16 Desai and colleagues performed a histologic analysis at 1-year follow-up in a canine model in which SIS mesh was used to repair hiatal defects. They demonstrated that good tissue ingrowth occurred and that SIS mesh cruroplasty did not result in erosions or strictures.17 Problems with stenosis and fibrosis at the hiatus have been reported with biologic mesh, but not erosion. A recent review of mesh-related complications by Stadlhuber and colleagues summarizes the current literature in this regard.18 Like most surgical complications, we feel that the true incidence is likely underreported. At this time, we feel that biologic mesh offers the best efficacy and safety profile of the available hiatal mesh prostheses, and do recommend its use during PEH repair.

Routine Fundoplication

Routine partial or total fundoplication should be performed at the time of PEH repair for several reasons. First, there is a significant incidence of postoperative abnormal acid exposure as seen on 24-hour pH testing.19,20 In addition to the already abnormal gastroesophageal junction anatomy associated with PEH, further dissection at the time of surgery likely disrupts this natural barrier even more, eliminating its contribution to the natural antireflux mechanism of the hiatus. Second, the creation of a fundoplication acts as a gastropexy mechanism anchoring the stomach below the diaphragm, likely reducing recurrence rates. Total fundoplication (360 degrees) has not been associated with increased rates of dysphagia in patients with impaired peristalsis.21,22 In cases of complete aperistalsis, a partial fundoplication is a reasonable option.

Operative Technique: Laparoscopic Paraesophageal Hernia Repair

Positioning and Ports

The patient is positioned in the low lithotomy position, using a beanbag and gel pad to form a padded mold for support. The operation is performed in the steep reverse Trendelenburg's position (Fig. 14-6).

Access and insufflation are obtained per the surgeon's preference. We gain access at the left upper quadrant, immediately below the costal margin, using a Veress needle and an optical bladed trocar. Our ports are placed in what we refer to as our standard esophageal operating position (Fig. 14-7).

Dissection

The surgeon begins by gently reducing the stomach into the abdomen. The short gastric vessels are then divided using an appropriate energy source. These vessels are usually long and attenuated due to the fundus' displacement into the chest, and they will lead to the base of the left crus. We use a left crus approach as our group previously described.23 The hernia sac is sharply entered using electrocautery at the base of the left crus. It is important to stay in the correct plane and divide the entire hernia sac. Great care must be taken to avoid injuring the crural pillars, which are usually thin and attenuated. The dissection between the sac and the mediastinal structures is carried up and to the right, proceeding circumferentially around the hiatus. The anterior vagus nerve (as it is often displaced away from the esophagus by the sac and is easily divided) and the esophagus must be clearly identified. The esophagus is distorted, and identification is aided by the careful passage of a lighted bougie placed in the esophagus. The bougie should be pulled back once the esophagus is identified, as traction against it can lead to perforation.

If an aberrant left hepatic artery is encountered, all attempts should be made to preserve it. The sac is then dissected off the right crus, taking care to identify and not injure the posterior vagus nerve. Great caution must be taken to identify and avoid injuring the left gastric artery and vein as they may be stretched and entering the mediastinum. Once the hernia sac is released and reduced from the mediastinum, ½-in Penrose drain is placed around the esophagus at the gastroesophageal junction and used to provide traction. The assistant, through the left flank port, grasps the drain to manipulate the esophagus in order to provide exposure.

All of the hernia sac should be reduced, after which mediastinal dissection is carried out to free up the esophagus and gain length. Dissection of the esophagus is routinely carried up to the level of the pulmonary veins and can be taken higher if needed. The goal is to dissect enough so that the gastroesophageal junction lies easily and without tension within the abdomen. Once the sac is reduced and the esophagus mobilized, the sac is resected en bloc, beginning to the left of the anterior vagus nerve. We feel this aides with the creation of the fundoplication by keeping this extra tissue out of the wrap.

Crural Repair

Posterior crural reapproximation is the next step after mobilization of the esophagus is completed. Either intracorporeal suturing with free needles or a laparoscopic suturing device can be used. We begin inferiorly just above the median arcuate ligament and proceed up the pillars with our sutures. Depending on the size of the defect, three to eight no. 0 or 1 braided, nonabsorbable sutures are placed in interrupted fashion. The 52F bougie can be advanced into the stomach at this time to gauge the cruroplasty. The tip of a blunt instrument should pass in the space between the bougie-filled esophagus and the reapproximated crura. On rare occasions, the final posterior crural sutures, if placed, can cause excessive anterior angulation of the esophagus. In this case, we omit them and place anterior crural sutures using the same technique.

A biologic mesh is next used to buttress the crural repair. A U-shaped mesh is fashioned, using six-ply mesh, and affixed to the apex of the right and left crura with suture, then secured posteriorly with fibrin glue (Fig. 14-8).

Fundoplication

Fundoplication is then performed over a bougie to ensure appropriate sizing. To ensure correct geometry and positioning of the fundoplication, we first place a marking suture posteriorly on the fundus 3 cm below the gastroesophageal junction and 2 cm from the greater curve. This is brought to the patient's right side posteriorly through the retroesophageal window, at which point this suture is grasped. A mirror image is created with the anterior fundus, and they are brought together at the 10 o'clock position at the hiatus. A “shoeshine” maneuver is performed, bringing the posterior fundus back through the retroesophageal space to the left side, checking to see that an equal length of fundus is used on either side of the greater curve, as marked by the ligated short gastric vessels. This ensures that, when constructed, the sutures on the wrap should be 180 degrees opposite the greater curve.

Four sutures are placed, 1 cm apart, to create a 3-cm wrap. This is done over a 52F bougie. Three additional coronal sutures are placed. The first two are placed on the left and right sides respectively, through the top of the fundoplication, taking a good bite of the esophageal muscle, and finally through that respective right or left crus. The last suture is placed posteriorly where the fundoplication lies naturally against the now closed hiatus (Fig. 14-9).

For the open technique, essentially the same steps are taken but through a midline incision.

Spastic Motility Disorders

Primary esophageal motility disorders (PEMDs) encompass both spastic disorders and achalasia. Spastic disorders include nutcracker esophagus, diffuse esophageal spasm (DES), and hypertensive lower esophageal sphincter (HLES). These rare disorders can present with variable or nonspecific symptoms and can be difficult to diagnose and treat. Symptoms frequently include chest pain, GERD, regurgitation, and less commonly dysphagia. The clinician must pay careful attention to presenting complaints and beware of what symptoms typically respond to treatments and which do not. It is important to distinguish between the primary motility disorders just mentioned and those symptoms secondary to GERD. GERD is a significant cause of esophageal dysmotility and should be evaluated for its presence. Its successful treatment, medically or surgically, often mitigates the symptoms suffered by many patients. These patients may be initially diagnosed with esophageal motility disorders but in actuality have a significant reflux component to their problem.24–27 Secondary causes of esophageal motility disorders can include diabetes mellitus, Chagas' disease, collagen vascular diseases, and multiple sclerosis. If such conditions exist, their severity and prognosis should be taken into consideration when forming a diagnosis and prior to embarking on any therapy.

Patient Presentation and Evaluation

Prior to investigation of the esophagus as the cause of symptoms, a cardiopulmonary evaluation should be performed to rule out the heart or lungs as the cause. The evaluation of motility disorders should include a careful history taking. This may help clarify the diagnosis and should allow the examiner to pick up on any confounding psychiatric illness or disorder that may be responsible, such as rumination syndrome. A systematic workup should include endoscopy and UGI barium swallow to evaluate the anatomy and rule out malignancy or other lesions as a cause. Esophageal manometry is an essential component of the workup, and 24-hour pH study should be performed to evaluate for reflux (Fig. 14-10).

Diffuse Esophageal Spasm

Diffuse esophageal spasm (DES) was first described by Osgood in 1889.28 Typically, patients affected by DES will complain of chest pain and dysphagia, and may present with functional obstructive symptoms. Symptoms of DES can be difficult to distinguish from GERD; both pH and manometry should be performed as part of the workup to evaluate the patient. If abnormal reflux is found on testing, the first treatment strategy should be to control GERD with antisecretory therapy. The defining characteristics of DES on manometry include greater than 10% (but <100%) of wet swallows that are followed by simultaneous esophageal contractions of amplitude 30 mm Hg or greater (Fig. 14-11).29,30 LES dysfunction, manifested by improper relaxation and/or hypertensive state, is seen in over half of patients diagnosed with DES.31 Intermittent peristalsis and prolonged contractions are also findings seen on manometry. DES is a rare true finding and is estimated to be found only in 3–5% of patients evaluated for an esophageal motility disorder.32 Whereas previously there was thought to be little role for surgical treatment in DES, more recent reports of small series have shown good results for relief of dysphagia after esophageal myotomy in up to 80% of highly selected patients, while chest pain is more difficult to cure.24,31,33 A careful, thorough workup and exclusion of GERD as a confounding factor should be done before attempting to diagnose and surgically treat DES. Medical management is an appropriate initial approach.

Nutcracker Esophagus

Nutcracker esophagus (NE) was first described by Brand and associates34 in 1977 and named as such by Castell several years later.35 Typical presenting symptoms of patients with NE include chest pain and less frequently dysphagia. Its defining characteristics on manometry include hypertensive esophageal contractions of greater than 180 mm Hg (Fig. 14-12). Patti and colleagues performed myotomy for these patients and observed that dysphagia was controlled in 80% of patients, but that chest pain persisted in 50% of them.31 Interestingly, in the patients with recurrent pain, they developed dysphagia postoperatively, possibly because of weakening of peristalsis by performing the myotomy. Most patients with this manometric finding consistent with NE and presenting with chest pain do not need an operation, and consideration for surgery should rather be carefully given to patients with dysphagia as the presenting symptom. The best candidates may be a small subgroup in which manometry demonstrates a hypertensive LES in addition to NE findings as well as a functional obstruction on UGI. As with DES, one must evaluate for GERD and treat if present. GERD, when present in conjunction with hypertensive esophageal contractions, can be an inciting factor causing further esophageal irritation in a hypersensitive esophagus. Therapy aimed at correcting abnormal acid exposure and irritation can lead to significant improvement in symptoms. The mainstay of treatment is medical therapy. Calcium channel blockers have shown benefit in symptom improvement.36 Tricyclic antidepressants may also provide symptom relief and benefit to patients.

Hypertensive Lower Esophageal Sphincter

Hypertensive lower esophageal sphincter (HLES) is a condition defined as having a resting LES pressure of greater than 45 mm Hg with intact, normal peristalsis (Fig. 14-13). Incomplete relaxation of the LES may also be a feature. The condition was first described in 1960.37 Patients with HLES can be a heterogeneous group and can present with symptoms of chest pain and/or dysphagia. They may also have symptoms of a functional obstruction at the LES. Presentation can be with isolated symptoms or in association with GERD. Careful history taking and a thorough workup with manometry and pH testing are essential to clearly define the symptoms and the primary problem in terms of esophageal function. Therapy should be tailored to the presenting symptoms. Medical management to reduce LES pressures with calcium channel blockers, botulinum toxin, and phosphodiesterase inhibitors is typically the first-line approach in management. These drugs can have significant side effects and decreasing efficacy with time. In patients with GERD and manometric findings of HLES, Nissen fundoplication has shown good results in improvement of dysphagia and chest pain.38,39 This suggests that reflux disease may be the etiology in these patients. Patients with dysphagia or chest pain as their predominant symptom and workup findings of only isolated HLES without GERD are more likely to benefit from myotomy and partial fundoplication for symptom relief, suggesting a primary sphincter dysfunction as the etiology of their symptoms. Good results have been reported by several groups that have used myotomy and partial fundoplication to treat this subset of patients, with relief of symptoms persisting as far as 3 years out.31,38,40 While medical management is usually a reasonable first-line, conservative approach to treatment of HLES, in carefully selected and thoroughly worked up patients surgical treatment with either Nissen fundoplication or myotomy and partial fundoplication (depending on manometric and pH test findings) can produce good results. As HLES is a rare disease with heterogeneous presentation, the importance of carefully and thoroughly working up affected patients before embarking on therapy cannot be overemphasized.

Summary

Spastic PEMDs (NE, HLES, DES) represent a diagnostic and therapeutic challenge to the clinician. Careful attention to presenting symptoms and thorough workup of esophageal function are of utmost importance for both diagnosis and discussion with patients regarding treatment options. Overlap with these disorders and GERD is frequent, and GERD can significantly contribute to and exacerbate presenting symptoms. Medical and surgical therapies have been tried in the past, most of which share the goal of relieving functional obstruction at the GEJ to allow for improved esophageal clearance. In most cases, a less invasive therapeutic approach with smooth muscle relaxing agents is a prudent first line of therapy. Surgery may be offered to carefully selected patients in whom an operation can address a discrete etiology such as abnormal GERD or isolated LES dysfunction.

Achalasia

Idiopathic achalasia is a primary motor disorder affecting the esophagus. Achalasia, which is typified by complete aperistalsis of the esophagus, is the most frequently encountered motility disorder seen by surgeons. It is a rare condition, with an incidence of 1–3 per 100,000 population in the Western world.41 It is, however, of all the previously mentioned motility disorders, the most common PEMD. The histopathologic hallmark of the disease remains near complete or total loss of the myenteric plexus ganglion cells as a result of injury and fibrosis of these cells and myenteric nerves. Recent inquiries into the cause suggest an autoimmune disorder, as evidenced by CD3/CD8 lymphocyte markers seen on immunohistochemical analysis of the inflammatory infiltrate.42–44 The inciting event or trigger may relate to cytotoxic T-cell activation by latent herpes simplex virus 1 (HSV-1) antigen exposure.45,46 In addition, nitric oxide (NO) synthesis, a mediator of LES relaxation, is often impaired in the face of preserved cholinergic neuronal function.47–49 These two insults result in loss of peristalsis and impaired relaxation of the LES, which in turn lead to the pathophysiologic findings of impaired esophageal emptying, aperistalsis, and a nonrelaxing LES.

Patient Presentation

Achalasia can occur in patients of all ages but typically presents in patients in the second to fifth decades of life. It does not show a predilection toward either sex. Typical symptoms include dysphagia, regurgitation of indigested food, and complaints of food “sticking” in the chest. Symptoms often worsen after lying supine, with regurgitation occurring even the next day of the previous day's meal. Cold liquids frequently exacerbate symptoms, with inability to ingest cold water being a common complaint. Patients may give a history of various maneuvers they employ in attempts to allow passage of food through their nonrelaxing LES. These include raising their arms over their head, swallowing liquids to try to “wash down” food, or remaining upright for extended periods of time. It is only after overcoming the LES pressure with a column of food and liquid that exerts a greater hydrostatic pressure that the patient is able to swallow. Prior to severe progression of their disease, these maneuvers may work for them. As the disease progresses and the esophagus dilates, in effect acting like a stomach reservoir, one finds that regurgitation of the prior day's food contents becomes more common. Symptoms such as these can lead to avoidance of social situations by patients in which they fear regurgitating food in front of others. In addition, frequent regurgitation and aspiration can lead to pulmonary complications. Weight loss can occur with achalasia and tends to correlate with disease severity. However, in older patients (>60 years), recent onset of symptoms (<6 months) and significant weight loss (>10–20 lb) should stimulate concern for neoplastic causes otherwise known as pseudoachalasia. In such cases, patients should be worked up with a CT of the chest and abdomen and/or endoscopic ultrasound before therapy.

Evaluation

Patients should be worked up in a systematic, methodical fashion. The workup has several components. An upper GI esophagram should first be performed to assess the anatomy. This is a common element used early in the workup of dysphagia and is a good screening tool. Particular attention to the morphology of the esophagus (ie, is a sigmoid esophagus present?) and anatomic location of the LES should be given attention. Classic findings on barium esophagram include distal tapering to the GE junction, resulting in a “bird's beak” appearance. Air fluid levels are often seen (Figs. 14-14 and 14-15). While radiologic reports often will comment on the peristaltic quality of the esophagus, we reserve such categorization for manometry.

Manometry is used to confirm the diagnosis of achalasia. Aperistalsis of the esophageal body and impaired relaxation of the LES are the hallmark findings on manometry, with aperistalsis being a requisite finding. Waveforms are typically low amplitude and simultaneous (Fig. 14-16). Vigorous achalasia, a variant in which high-amplitude waveforms are present can be encountered, and it is usually found in patients with earlier stages of the disease before complete destruction of the myenteric ganglion cells ensues.

Endoscopy is an essential part of the workup for achalasia patients. This offers the chance to directly inspect the mucosa and evaluate the GE junction. Any abnormalities should be biopsied to rule out causes of pseudoachalasia, as well as evaluated with CT and/or endoscopic ultrasound.

We do not routinely perform 24-hour ambulatory pH monitoring on these patients, as it does not usually add to the clinical picture. False-positive results can occur as a result of fermentation of food within the esophagus.

Treatment

Therapy for achalasia is palliative in nature and may involve pharmacologic, endoscopic, and surgical therapies. It must be emphasized to the patient that therapies do not cure or address the pathophysiologic abnormality, but instead they are designed to relieve symptoms of obstruction and impaired esophageal emptying by relaxing or disrupting the muscle fibers of the LES.

Medical therapy has focused on drugs that relax smooth muscle and decrease LES pressure. Nitrates and calcium channel blockers are used. Because of their limited effectiveness and inconsistent absorption, their use is limited. Impaired esophageal emptying can affect their ingestion and absorption. In randomized controlled trials, calcium channel blockers have not shown significant success in improving clinical symptoms, despite lowering LES pressures.50,51 Sildenafil, a phosphodiesterase inhibitor, has been shown to have potent relaxing effects on the LES,52 but its clinical use is limited by poor tolerance and side effects. Nitrates, which can be used in sublingual formulation to overcome poor absorption, tend to work better than calcium channel blockers for symptom relief.53 Their use, however, can lead to undesired side effects such as hypotension and headache and, like all medical therapies, their efficacy decreases with time. Pharmacologic therapy should be pursued only in patients who, for medical reasons, are unable to undergo other therapies.

Endoscopic therapies include balloon dilation and botulinum toxin injection. Botulinum toxin injection works by inhibiting acetylcholine release at cholinergic nerve endings, thereby decreasing LES pressure. A recent meta-analysis of therapies for achalasia, by Campos et al, reviewed 315 patients in 9 studies who underwent botulinum toxin injection and found initial symptom relief of 78.7% at 1 month postprocedure. This steadily declined over time to 40.6% at 12 months, with 46.6% of patients requiring repeat injection.54 While botulinum toxin injection therapy may offer temporary relief of symptoms, its effects are not durable as with surgery and repeat treatments are often needed. Moreover, when compared with myotomy, the results for botulinum toxin seem inferior. Zaninotto et al, in a randomized trial comparing botulinum toxin injection with laparoscopic Heller myotomy with fundoplication, observed at 1 year 60% remained asymptomatic in the botulinum injection arm compared with 87% of patients in the surgical arm being symptom free. At 2 years, only 34% of patients in the botulinum injection arm remained without symptoms, while 87% in the surgical arm remained symptom free.55 Multiple injections can further complicate future surgical therapy due to the submucosal fibrosis that can result, making myotomy more difficult and increasing the risk of perforation.56 Endoscopic botulinum toxin injection may offer an alternative to those unwilling or unable to undergo more invasive procedures but has a limited role in the treatment of the disease.

Endoscopic balloon dilation, which creates a controlled tear in the LES muscle, is another endoscopic therapy that has been used to treat achalasia and is probably the main alternative to surgery. Different types of dilations have been used in the past, including fixed diameter dilators, mercury-weighted balloons, and water-filled balloons. The most controlled and consistent results are seen with the use of noncompliant pneumatic balloon dilators, such as the Rigiflex balloon dilator (Boston Scientific, Boston, MA).57 Campos et al, in their meta-analysis, evaluated 15 studies involving 1065 patients using new generation pneumatic dilators, and observed symptom relief rates of 84.8% at 1 month postprocedure, 73.8% at 6 months, and 68.2% at 12 months. After 36 months, the symptoms relief rate declined to 58.4%. One quarter of all patients required repeat endoscopic balloon dilation therapy.54 Balloon dilation has more long-term efficacy than botulinum toxin injection but still shows significant rates of symptom recurrence and the need for repeat therapy. It does carry more risk than botulinum toxin injection due to the risk of perforation, which is nearly 2% with pneumatic dilation methods.54 This risk increases with the presence of significant esophageal dilation, hiatal hernia, and epiphrenic diverticula (ED). These should be considered relative contraindications to pneumatic dilation. Between the endoscopic therapies mentioned, pneumatic balloon dilation is a more efficacious procedure but has greater risk of perforation compared to botulinum toxin injection.

Surgical myotomy was first described by Ernst Heller in 1913.58 His original description involved performing both an anterior and posterior myotomy. This has evolved in most centers to performing an anterior myotomy only. Esophageal myotomy for achalasia is associated with good long-term results and relief from dysphagia. Long-term follow-up studies have demonstrated symptom relief in nearly 75% of patients at 20 years out. Shorter-term follow-up studies demonstrate that nearly 90% of patients are symptom free approximately 3 years postprocedure.41,54,59 Prior hesitancy for referring patients for Heller myotomy was partially due to the invasive nature of the procedure, which in the past was performed via laparotomy or thoracotomy, as well as a long hospital stay and long recovery. These approaches eventually evolved to the minimally invasive approaches via thoracoscopy or laparoscopy. Shimi et al reported the first laparoscopic Heller myotomy in 1991, while Pellegrini et al reported the first thoracoscopic approach in 1992.60,61 Drawbacks to this thoracoscopic approach included the need for single-lung ventilation, postoperative chest tubes, and being unable to perform an antireflux procedure. The minimally invasive approach has moved predominantly to the laparoscopic myotomy approach that has eliminated these drawbacks of the thoracoscopic approach. Laparoscopy offers excellent visualization of the hiatus and the mediastinal structures, does not require single-lung ventilation or postoperative chest tube drainage, and makes creation of an antireflux technically straightforward. In addition, the laparoscopic performance of myotomy, when compared with the thoracoscopic technique, has shown better symptomatic improvement (89.3 vs 77.6%) and a lower incidence of reflux symptoms when combined with a partial fundoplication (14.9 vs 28.3%).54

The two main debates surrounding surgical myotomy have centered on whether or not to include an antireflux procedure (and if so which one) and what the optimal length and extent of myotomy are that should be performed. GER symptoms and esophagitis represent common causes of treatment failure after myotomy if a fundoplication is not added. Addition of an antireflux procedure to a standard Heller myotomy has been thought to reduce these symptoms and improve outcomes.

This issue has been studied in a prospective, randomized trial by Richards et al, comparing Heller myotomy with Heller myotomy plus Dor (anterior) fundoplication. They demonstrated that the pathologic occurrence of GER, as defined by distal esophageal acid exposure of greater than 4.2% on 24-hour pH monitoring at 6 months postoperatively, was reduced from 47.6 to 9.1% with creation of a Dor fundoplication.62 Some surgeons have advocated in the past for inclusion of a floppy Nissen fundoplication, rather than partial fundoplication, to prevent GER. Concern for postoperative dysphagia due to poor esophageal clearance and weak or absent propulsive force is clearly warranted in this instance. Rebecchi et al recently published the results of their study in which patients were randomized to Heller myotomy plus Dor fundoplication or Heller myotomy plus floppy-Nissen fundoplication. At 60 months of follow-up, no statistically significant difference in GER symptoms between the two groups were seen; the rate of dysphagia, however, was found to be significantly higher in the floppy-Nissen fundoplication group when compared to the Dor fundoplication group (15 vs 2.8%). They concluded that both antireflux procedures offered adequate protection from GER, but that recurrence of dysphagia was significantly higher when Nissen fundoplication was performed.63 Toupet and Dor fundoplications with EM are being compared in a randomized, multicenter trial at this time, and the hope is that the data will help answer which is a superior antireflux procedure. Until the data can conclusively demonstrate superiority of one technique over the other, surgeon's preference and experience should guide which one is performed in conjunction with myotomy.

The length and extent of myotomy is another area of debate. Most surgeons agree that the proximal extent of the myotomy should extend 6–7 cm above the GE junction. This is carried out in a safe manner with appropriate dissection of the anterior esophagus. Distally, a standard myotomy has typically been performed and carried 0.5–1.5 cm below the GE junction. This length was chosen with the intent of being long enough to relieve the functional obstruction to the esophagus, while in an effort to preserve an antireflux barrier.64 The result proved to fall short on both counts, with dysphagia and/or GERD being fairly common. In 1998, based on observations that reoperations for thoracoscopically performed myotomies that extended the myotomy onto the stomach resulted in improvement of dysphagia, we changed our practice to carry out the myotomy a full 3 cm below the GE junction (an extended myotomy) completely obliterating the LES fibers. We compared our extended myotomy/Toupet patients with standard myotomy/Dor patients and observed that patients who underwent extended myotomy had lower LES pressures (9.5 vs 15.8 mm Hg), less frequent and less severe dysphagia, and lower rates of recurrent severe dysphagia requiring interventions (3 vs 17%).65 We continued to follow and then compared a cohort of 52 of these patients at a median follow-up of 46 months. No significant differences in heartburn frequency, esophageal acid exposure, or LES pressure were observed. However, dysphagia severity was reduced, and relief was improved in the EM and Toupet fundoplication group. Only 5% of patients who underwent EM/Toupet required reintervention (dilation) versus 18% of SM/Dor patients (10% required endoscopic treatment, 8% required reoperation).66 We feel that the Toupet is a better antireflux operation in combination with extended myotomy. Reasons for its superior efficacy may stem from its more physiologic angulation of the GE junction with its construction and its ability to stent open the myotomy and prevent reapproximation of muscle fibers and symptom recurrence. Our study compared two different operations (SM and Dor vs EM and Toupet), and we cannot answer which wrap is superior. At this time, we recommend performing either anterior or posterior fundoplication with extended myotomy. We continue to routinely perform extended myotomy and have seen excellent results and low rates of dysphagia. Rarely do we need to consider dilation, and we have essentially eliminated the need for reoperation with this approach. Because of this more complete obliteration of the LES, this should be used in conjunction with an antireflux procedure. We feel that extended myotomy of 3 cm below the GE junction should be a routine practice when performing a Heller myotomy.

Operative Technique

Laparoscopic Heller Myotomy.

The setup is the same as previously described in this chapter for PEH repair, utilizing our standard esophageal operating position regarding patient positioning and trocar placement. We use a 10-mm, 30-degree laparoscope to ensure the best possible image for performing the myotomy. This is especially important during the creation of a myotomy. In contrast, we use 5-mm, 30-degree laparoscopes for PEH repairs and first-time Nissen fundoplications. Patients are instructed to remain on a liquid diet for 2 days prior to surgery to minimize the amount of retained food within the esophagus and decrease the risk of aspiration at the time of surgery.

We begin by dividing the phrenogastric ligament sharply and then divide the short gastric vessels with ultrasonic shears. A left crus approach is employed as previously described, and left, right, and anterior mediastinal dissection of the esophagus is performed. It is not necessary to significantly dissect the posterior attachments of the esophagus, except to provide enough intra-abdominal esophagus to perform a good fundoplication. The main goal is to gain as much length as possible anteriorly to later perform the myotomy. It is important to identify and preserve the anterior (left) vagus nerve. This nerve and GEJ fat pad are carefully dissected away from the esophageal body and preserved so that a continuous myotomy can be performed, starting below on the stomach and extending above the vagus as it crosses from left to right on the anterior aspect of the GEJ. The anterior GEJ fat pad to the left of the anterior vagus nerve is resected. This allows for accurate identification of the GEJ at the time of myotomy.

At this time, a 50F lighted bougie is passed into the body of the stomach. The transillumination provided aids in identification of the submucosal plane. A laparoscopic Babcock clamp, first applied partially opened over the bougie, is used to gently drag the tissue over and around the bougie to provide tension and exposure. The myotomy is started on the anterior stomach 3 cm below the GEJ. We prefer an L-shaped hook to perform the myotomy, but other devices can be used as well. We employ gentle use of cautery to start the myotomy and then use the L-shaped hook to gently tease the muscle fibers apart, exposing the submucosa. Entering the correct plane takes patience and careful dissection. The submucosa of the stomach contains a rich plexus of vessels that can be used as a visual identifier. Once the appropriate plane is identified, the myotomy is carried cephalad. Only minimal electrocautery is used during performance of the myotomy (Fig. 14-17).

The correct plane may be difficult to identify on the stomach, as the sling fibers of the cardia cross in variable directions and the mucosa tends to be thin. Once the GEJ is reached, the plane becomes easier to identify due to the organized outer, longitudinal, and inner circular muscle fibers of the esophagus. We first divide the outer longitudinal muscle fibers and then the inner circular layer. The myotomy is carefully extended and taken above the level of the anterior vagus nerve as it crosses from left to right over the esophagus. The extent of the myotomy is to take it as proximally as is safe. Typically, one can get 6–8 cm above the GE junction. The assistant repositions the Babcock clamp as needed to continually provide exposure and tension on the tissues over the bougie. As the myotomy is carried cephalad, the assistant can switch over to using an atraumatic grasper to hold the left side of the divided muscle fibers on tension, with the surgeon's left hand holding the right-sided fibers. In this fashion, the myotomy is completed.

Bleeding from submucosal vessels that are mistaken for muscle fibers occasionally occurs but is self-limited; gentle pressure is usually adequate to control and stop it. One must be very cautious in applying electrocautery as an unrecognized injury may result leading to delayed perforation, and thus should be avoided. If mucosal perforation occurs during the dissection, it is usually evident as saliva or gastric secretions or the light from the bougie will be seen coming forth. Intraoperative endoscopy can be used to confirm injury as well as evaluate it after it has been repaired. Mucosal injuries should be repaired immediately with 4-0 absorbable suture, and consideration given to performing an anterior, buttressing fundoplication.

Intraoperative endoscopy is carefully performed to evaluate for the completeness of myotomy and to evaluate for injury. If all muscle fibers have been correctly divided, an open GE junction will clearly be visible on endoscopy, without indentations from undivided fibers. In addition, with gentle insufflation, injury to the mucosa can be seen both endoscopically and laparoscopically.

A Toupet (posterior) fundoplication is performed for the antireflux procedure as the final part of the operation. A suture is placed on the posterior portion of the fundus, 3 cm below the GE junction and 2 cm away from the line of the divided short gastric vessels. This is used as a reference point to ensure a symmetric posterior wrap. The fundus is brought posteriorly behind the GE junction, and the reference suture is grasped and brought up to the edge of the myotomy. The fundus is sutured to the right crus to alleviate tension, using 2-0 silk suture. The edge of the wrap is then sutured to the myotomized edge with three sutures. In similar fashion, the left component of the wrap is sutured to the edge of the myotomy and the left crus (Figs. 14-18 and 14-19). After completing the wrap, the ports and liver retractor are removed and the port sites are closed, concluding the case.

A Dor fundoplication is an acceptable antireflux procedure and is technically easier to perform than the Toupet, as it requires less dissection, especially of the posterior stomach (Fig. 14-20). The Toupet does a better job of stenting open the divided muscle fibers and with this mechanism may lead to lower rates of recurrence and dysphagia. For this reason, we prefer this posterior fundoplication. Figure 14-21 depicts the construction and geometry of full and partial fundoplications. In patients with a very tortuous or sigmoid shaped esophagus, we omit the antireflux portion of the procedure because of the high incidence of postoperative dysphagia we have observed when performing fundoplication in these patients.

Postoperatively, patients are started on a clear liquid diet and advanced slowly. We do not use nasogastric tubes. Nausea is controlled aggressively to prevent retching or emesis. Patients are typically discharged home on postoperative day 1. On routine follow-up, we assess for symptoms of reflux and dysphagia. At 4–6 months postoperatively, we request patients to repeat manometry and obtain 24-hour pH testing to evaluate acid exposure. If abnormal acid exposure is present or the patient has symptoms of GER, a proton pump inhibitor (PPI) is started to ameliorate symptoms and to reduce the risk of peptic stricture formation.

Summary

Motility disorders of the esophagus share the hallmark symptom of dysphagia. Careful history taking, in conjunction with physiologic testing with pH and manometry, and appropriate imaging lead to the diagnosis. With the exception of achalasia, many of these disorders can be managed medically, especially after careful evaluation and control of GER. Achalasia is a disease best treated surgically with laparoscopically performed extended myotomy and partial fundoplication. Though endoscopic therapies exist, they have inferior outcomes and durability, and should be reserved for patients unwilling or unable to undergo surgery. Minimally invasive techniques have shown great promise in treating achalasia both in terms of patient recovery and long-term outcomes.

Diverticula of the esophagus are relatively uncommon. They are classified based upon their location: proximal or pharyngoesophageal, midesophageal, and distal or epiphrenic, with the latter being located within 10 cm of the GE junction. Midesophageal diverticula, which are usually traction diverticula and thus are true diverticula, are rare and usually do not require surgical treatment. They result from an extrinsic “pulling” inflammatory process and in the past were often associated with tubercular or granulomatous disease. Proximal and distal diverticula are more common and are false, or pulsion-type diverticula, as they are not composed of all layers of the esophageal wall but rather are outpouchings of mucosa. This chapter focuses on these types, specifically Zenker's diverticulum (ZD) and epiphrenic diverticulum (ED), and their management.

Zenker's Diverticulum

Originally described by Ludlow in 1769,67 this proximal esophageal diverticula was named by German pathologist Friedrich Albert Von Zenker, who, more than 100 years later in 1877, described their etiology as being that resulting from increased pharyngeal pressure leading to formation.68 The anatomic location of this lesion is proximal to the upper esophageal sphincter (UES) and in the posterior hypopharynx. The area in the posterior wall of the pharynx between the cricopharyngeus muscle and the inferior constrictor muscles is known as Killian's triangle. The weakest point in this space is the area between the two muscles, and it is here that herniation or outpouching of the mucosa and submucosa occurs, resulting in a Zenker diverticulum (ZD) formation (Fig. 14-22). In addition to a weak posterior wall, inelasticity and higher resting tone from fibrosis of the cricopharyngeus muscle are thought to contribute to the dysfunction of the pharyngoesophageal segment, leading to ZD formation.69,70 A complete understanding of the causes for ZD formation does not exist, despite decades of research.

ZD usually presents in the seventh to eighth decades of life. It is not uncommon for significant lengths of time to elapse between the start of symptoms and presentation to a surgeon, because the symptoms are often vague, innocuous, with a lot of overlap with other benign conditions. Its incidence is difficult to estimate as the number of patients with ZD who are asymptomatic is unknown. Estimates in the United Kingdom place its incidence at 2 per 100,000 population per year.71 Common symptoms include dysphagia, globus sensation, halitosis, aspiration, and regurgitation of undigested food. Physical examination findings are largely absent but may infrequently reveal a palpable mass, most often located in the left side of the neck.

Workup for ZD consists of barium swallow to delineate size, as measured in the craniocaudal dimension, and position. Only after this has been done, should endoscopy be attempted as perforation by blind intubation of the false lumen can lead to significant morbidity. Endoscopy largely serves to exclude other diagnoses, including tumors, mucosal abnormalities, synchronous esophageal lesions, malignant neoplasia within the diverticulum, and GERD. Manometry has not shown specific findings associated with ZD, though UES dysfunction may be present.

Treatment

Therapeutic options have evolved over the last century from open diverticulectomy and myotomy or diverticulopexy toward perioral endoscopic methods, including mucomyotomy with staplers, CO2 laser, argon plasma coagulation (APC), and needle-knife. No randomized trials have been conducted comparing the methods. Most methods have comparable symptomatic improvement ranging near or above 90% and with low morbidity and mortality. Choice of therapy is often a matter of patient and physician choice. Trends in therapy, following the European experience, seem to be shifting toward endoscopic management due to its low morbidity and mortality, avoidance of an open surgical procedure, and good outcomes.69,72 Despite trends toward endoscopic therapy, there are patients for when the standard open surgical approach should be used, including those with narrow mandibles or small oral cavities, those in which the diverticulum cannot accommodate the scope, and instances in which the diverticulum is not posterior, for example Killian-Jamieson diverticula.73 The instances in which open surgical resection should always be sought are with diverticula in which mucosal neoplastic changes are known to exist and those very large diverticula that cannot be approached safely with a perioral technique.

Operative Technique

Open Cervical Diverticulectomy.

Patients with ZD are placed on a liquid diet for 2 days prior to surgery, to minimize the risk of retained food and aspiration. The patient is placed supine with the neck fully extended and the head turned to the right, exposing the left neck. The left cervical approach is used as the majority of diverticula occur posteriorly and on the left. In addition, the esophagus is most accessible here as the trachea has a natural slight rightward shift. An oblique cervical incision, overlying the anterior border of the sternocleidomastoid muscle (SCM) is made. The SCM is retracted laterally as is the carotid sheath, while the thyroid gland is retracted medially. Ligation of the middle thyroid vein and omohyoid muscle is necessary to gain medial retraction of the thyroid and exposure of the tracheoesophageal groove and esophagus. The left recurrent laryngeal nerve should be identified and preserved. A left-sided approach is also more desirable from this aspect as the recurrent laryngeal nerve on this side has better exposure and more consistent anatomy compared to the right.

Dissection is carried distally and cephalad. A true ZD will be encountered in the posterior midline at Killian's triangle; it is grasped and its neck is dissected free. Next, a 50F bougie is placed under palpation and direct vision of the surgeon into the distal esophagus. A myotomy is performed, which must include the cricopharyngeus muscle and come down several centimeters onto the esophagus, which can be identified by its outer longitudinal and inner circular muscle fibers. The diverticulum is excised using a reticulating linear stapler (Fig. 14-23A–C). Resection of the diverticulum should be performed with the bougie in place to avoid narrowing of the esophagus. A drain may be placed at the discretion of the surgeon. The platysma is closed and the wound is closed in layers. If the patient is doing well clinically, he or she is started on a liquid diet the next day and can be discharged within 48 hours.

Surgical open diverticulectomy and myotomy are associated with excellent relief from symptoms in up 82–94% of patients and low recurrence rates of 3.6–7%.69 Mild to severe complications, including staple-line leak, fistula formation, stenosis, recurrent laryngeal nerve palsy, mediastinitis, pneumonia, and hemorrhage have an occurrence rate of up to 25%. Mortality associated with the surgical approach ranges between 1.2 and 3.4%.69,74–78

Endoscopic Treatment.

Endoscopic treatment of ZD was first described by Mosher79 in 1917 and lost favor, because of complications, until revived by Dohlman and Mattsson in 1960.80 They reintroduced the concept with use of electrocoagulation techniques. Collard et al in 1993 described the endoscopic stapled diverticulectomy (ESD) that is the predominant endoscopic method of treatment today.81 Endoluminal treatments for ZD are the least invasive methods of treatment and the various forms all share the common principle of performing mucomyotomy by dividing the septum between the diverticulum and the esophageal lumen. CO2 laser, electrocautery, needle-knife, clips, and staplers have been described and used.80–83 The stapled diverticulectomy offers the additional advantage over these other methods of wound closure with the staples after division of the septum is completed. This is thought to decrease the risk of bleeding and possible perforation. Both rigid and flexible endoscopy platforms can be used. Rigid endoscopy is usually performed in the operating room by ENT (ear, nose, and throat) surgeons, and it incorporates the use of the stapler and a diverticuloscope, which can intubate both the esophageal lumen and diverticulum simultaneously (Fig. 14-24). Flexible endoscopic techniques employ various methods of cautery, cutting or clipping, or laser to divide the septum. Flexible endoscopy offers some advantages over the rigid method in that it can be done with sedation and analgesia, avoiding a general anesthetic, and can be performed in an outpatient setting with reduced stay and potential cost savings. It is associated with higher recurrence rate when compared to rigid endoscopy and ESD (up to 35% in some series vs up to 15.4% for ESD and rigid endoscopy). Head-to-head randomized trials comparing the differing endoluminal approaches have not been performed.

Endoluminal therapies demonstrate excellent symptom improvement in 80–96% of patients. Mild complications such as subcutaneous emphysema or mild hemorrhage are seen in up to 23% of patients; severe complications occur less frequently when compared to surgical treatment, ranging from 0 to 3.8%.69,84 The most feared of these are esophageal or pharyngeal perforation. Mortality rates are low (0–0.4%) and no mortalities have been reported with the flexible endoscopic methods. Recurrence rates, however, are significantly higher than with surgical therapy and range between 3.3 and 35%.69 Between the methods, the flexible platform has the highest recurrence rates; this can often be addressed with repeat therapy. While this is a drawback, the overall safety and lower-risk profile of endoluminal therapies may be a desirable factor when treating elderly patients, in which ZD most commonly presents. All patients are not candidates for endoluminal treatment of ZD, such as those with small oral cavities, large osteophytes, and small diverticula (<3 cm).73 Small diverticula are difficult to engage with the stapling device and diverticuloscope.

Summary

ZD is a rare disorder in which a pulsion-type diverticulum occurs in the posterior pharyngoesophageal region. Both surgical and endoscopic treatments (using rigid or flexible endoscopy) are employed. All therapies are associated with greater than 90% symptom relief. Endoscopic therapies are associated with shorter length of stay, can be performed as outpatient procedures, and may avoid general anesthetic administration. However, they are associated with higher recurrence rates, which can often be addressed with repeat endoscopic treatment. These are important factors to consider as ZD usually presents in elderly patients who are more likely to be infirm. Both open surgical technique and perioral endoscopic methods are valid treatment methods and the choice of which to pursue should be based upon anatomic considerations, available expertise, and patient comorbidities.

Epiphrenic Diverticula

Diverticula present in the distal third of the esophagus, usually within 10 cm of the GE junction, are referred to as epiphrenic diverticula (ED). It is a rare condition, and its true incidence remains unclear as the number of patients with asymptomatic diverticula is not known. These are pulsion-type false diverticula, similar to ZD, as they are only outpouchings of mucosa and submucosa through the muscular wall of the esophagus. The pathophysiology underlying the disease, first recognized by Mondiere in 1833,85 is assumed to be elevated esophageal intraluminal pressures, as a result of an underlying esophageal motility disorder, in conjunction with functional distal obstruction. With the advent of esophageal manometric testing, it is clear that EDs are commonly associated with a heterogeneous group of motility disorders and LES dysfunction, including achalasia, DES, Nutcracker esophagus, hypertensive LES, as well as nonspecific esophageal motility disorders (NSMD),86,87 although often there is no detectable underlying motility disorder. As such, these patients may present with symptoms similar to the aforementioned motility disorders, most commonly to include dysphagia, chest pain, heartburn, and regurgitation. Intermittent nocturnal aspiration is frequently seen and occurs in nearly 45% of patients.88,89 The workup of these patients should include a complete history and examination, upper GI barium swallow, esophageal manometry and pH testing, and endoscopy (Fig. 14-25). Some controversy exists regarding who should be treated surgically. Those with mild symptoms and small-size diverticula, or those to whom surgery presents significant risk, can be safely observed. If symptoms of GERD are present, they may be controlled with medications. These patients should be followed for progression of their symptoms.90 Those with severe symptoms who are candidates for surgery should be treated surgically.

Treatment

Surgical treatment focuses on the concepts of resection of the diverticulum and treatment of the underlying esophageal motility disorder to relieve the functional obstruction, typically with long esophagogastric myotomy. Historically, this was performed through a left thoracotomy to provide optimal exposure to the distal esophagus, GE junction, and cardia.91 This was associated with excellent symptom relief in 76–94% of patients, but with mortality rates of up to 15% and complication rates of nearly 40%. Leak rates of 6–18% were observed.92–94 Just as with treatment of achalasia and other benign esophageal disorders, the dominant operative technique has now shifted to the minimally invasive approach. Both the video-assisted thoracoscopic surgery (VATS) and laparoscopic approach have been described95,96; neither these nor the open approach have been compared in a randomized prospective fashion. When compared to thoracotomy approach, both minimally invasive techniques (VATS and laparoscopy) are associated with lower perioperative mortality rates (0–7.7%), shorter length of stay, and lower leak rates (14% cumulative rate); morbidity still ranges as high as 50%.94,97,98 With the minimally invasive approaches, excellent relief of symptoms is seen in 83–100% of patients.94 The laparoscopic approach, which has been the minimally invasive technique predominantly reported, capitalizes on these advantages and avoids single-lung ventilation and postoperative chest tubes. In addition, performing fundoplication is technically easier with the laparoscopic approach compared to the VATS approach. The laparoscopic method is the one we have adopted, in which we perform stapled resection of the diverticulum, long myotomy, and Toupet fundoplication.

Operative Technique

Laparoscopic Epiphrenic Diverticulectomy

Patients are placed on a liquid diet for 48 hours prior to operation to minimize retained food in the diverticulum. At the time of operation, endoscopy is performed prior to commencing, while the patient is under general anesthetic, to remove any retained debris and avoid its incorporation into the eventual suture line. The standard esophageal operating position is used, as previously described in this chapter. A 10-mm, 30-degree laparoscope is used to obtain the best image, and a liver retractor is placed to visualize the hiatus. The short gastric vessels are ligated, and the left crus technique is used to begin the division and dissection of the phrenoesophageal membrane. A Penrose drain is placed around the esophagus to aid with retraction. The esophagus is circumferentially dissected up into the mediastinum. The diverticulum usually becomes apparent at this time. Most diverticula are encountered on the right side. The diverticulum is freed from surrounding structures and dissected using both blunt and sharp dissection, taking care to cleanly expose the neck. The least obvious, but most important aspect of this is separating the diverticulum from the surrounding esophagus by dividing adhesions connecting the two. This is often underappreciated and can lead to incomplete resection of the diverticulum. An appropriately sized bougie is carefully placed in the esophagus (50–60 F) ensuring it does not enter the mouth of the diverticulum. The diverticulum is resected using an articulating laparoscopic stapling device, keeping the stapler parallel to the esophagus and using the bougie for guidance and to help avoid narrowing (Figs. 14-26A–C). The laparoscopic approach typically affords better visualization and stapler alignment than either VATS or open thoracotomy. The stapled edge is inspected to make sure there is no bleeding or disruption. A myotomy is performed opposite the suture line in the same fashion as previously described for achalasia, with extension of 3 cm onto the cardia. The separation of muscle fibers from the myotomy allows the suture line to be oversewn in Lembert fashion using interrupted sutures placed in intracorporeal fashion to protect it. Finally, a Toupet fundoplication is then performed as previously described to complete the operation, which provides protection from GER and in most cases will buttress the staple line. Endoscopy is performed to evaluate for leak or narrowing. Patients are admitted and an UGI barium study is performed on postoperative day 2. If no leak or stricture is seen, the patient is started on liquids and discharged home.

Summary

Epiphrenic diverticula are a rare type of pulsion diverticula that occur in the distal third of the esophagus. Treatment should be offered to those with symptomatic diverticula who are medically fit for surgery. Therapy should include surgical resection of the diverticulum and address the motility disorder to provide relief of distal obstruction, typically with long myotomy. Minimally invasive methods with either VATS or laparoscopy have demonstrated excellent symptomatic relief and lower mortality and morbidity compared to the older approach with thoracotomy. The laparoscopic method offers excellent visualization of the distal esophagus and is a technically easier approach for performing myotomy and partial fundoplication. The technique chosen should be based on anatomic considerations and surgeon experience.