Upper GI demonstrating epiphrenic diverticulum and esophageal dysmotility in a patient with type II achalasia
It is often difficult to distinguish between symptoms of an ED and the underlying motility disorder. Dysphagia , regurgitation , chest pain, and aspiration can all be seen in both conditions, and in fact there does not seem to be a correlation between the size of an epiphrenic diverticulum and symptom severity [9]. All patients with ED should therefore undergo esophageal manometry in order to ascertain the extent of the disordered peristalsis and to guide treatment options and expectations. Conventional manometry fails to identify motility disorders in up to 40% of patients with epiphrenic diverticula [5, 10].
Rarely ED may be due to traction, typically from an extrinsic tumor, or pulsion against “pseudoachalasia” from an obstructing esophageal mass. There is at least one case report of diverticulum arising from pseudoachalasia due to laparoscopic adjustable gastric banding for obesity [13]. Upper endoscopy allows for detection of mucosal lesions, including premalignant lesions (Barrett’s), ulcers, or malignancy in the esophagus, diverticulum, or stomach. Performing the UGI prior to the EGD allows the endoscopist to be aware of the epiphrenic diverticulum and therefore reduces the risks of blindly intubating and potentially perforating it. If there is any concern for possible traction diverticula, chest imaging is critical to evaluate for thoracic infection or malignancy. This is more common with mid-esophageal diverticula than with true epiphrenic diverticula.
Watchful Waiting
Up to 40% of diverticula are asymptomatic or minimally symptomatic [14]. This is relevant as an asymptomatic diverticulum may be left in place without treatment. There are no surveillance recommendations for patients with known ED that are being observed with watchful waiting [14]. There have been rare case reports of spontaneous rupture [15], bleeding [16], and squamous cell carcinoma (SCC) in epiphrenic diverticula [17]. The risk of SCC in ED has been estimated to be 0.6% and likely attributable to stasis and inflammation within the diverticulum [17]. Patients with a known ED presenting with worsening odynophagia, regurgitation, or hematemesis should be evaluated for malignant transformation.
Even if a patient is symptomatic, management of the motility disorder alone without diverticulectomy may be sufficient, if these symptoms are thought to be due to the underlying motility disorder and not the diverticulum. For example, Zaninotto et al. [5, 10] reported on 19 patients with ED for whom they either did not intervene [15] or performed only a pneumatic dilation [3]. The patients who underwent pneumatic dilation had improved symptom scores. Two of the non-intervention group eventually progressed on to need surgery. In the remaining 14 patients, however, symptoms were unchanged over the follow-up period (median 46 months).The authors concluded that surgery could be avoided in patients who were asymptomatic or minimally symptomatic with small diverticula. There are also case reports of botulinum toxin [18] or esophageal stents [19] used in symptomatic patients thought to be too high risk to undergo definitive surgical management.
Laparoscopic Management
The key element of surgical management of ED is myotomy of the esophagogastric junction with extension to at least to the base of the diverticulum. Although the historic management of esophageal diverticula was through the chest, with the advent of laparoscopy, most foregut surgeons now prefer a laparoscopic approach. The laparoscopic approach avoids single-lung ventilation, the morbidity of a thoracotomy or thoracoscopy, and the need for a chest tube. In addition to reduced trauma and faster recovery, the laparoscopic approach allows easier access to the GE junction and therefore an extended myotomy onto the cardia of the stomach, which in achalasia has been shown to reduce the risk of recurrent dysphagia [20]. A laparoscopic approach also allows for a more-easily performed partial fundoplication if desired.
There are a number of controversies in laparoscopic management of diverticula, including the extent of the myotomy, the need for diverticulectomy , and the need for antireflux procedure. Because this is a relatively rare disorder, most case series are small, and there is little evidence to guide these decisions.
Given the diversity of motility disorders that have been associated with epiphrenic diverticula, including disorders of the esophageal body with a normal lower esophageal sphincter, there has been disagreement among surgeons about the extent of the myotomy that is required. In particular, when a thoracic approach was favored, there was enthusiasm for sparing the lower esophageal sphincter (LES) in patients without achalasia or other disorders of the LES in the hopes of avoiding postoperative reflux. However, with the growing popularity of the laparoscopic approach with its easier extension onto the stomach and ability to add an antireflux procedure, complete myotomy of the LES even in non-achalasia patients has become well-established [5].
In our opinion, and based on the achalasia literature [20], the myotomy should extend distally for 3 cm on to the stomach. Proximally the myotomy should extend at least to and preferably beyond the base of the diverticulum. If the myotomy does not extend proximally past the base of the diverticulum, there is increased risk of recurrence [21]. There are some anecdotal reports of using intraoperative esophageal distensibility to guide the extent of myotomy using the Endoflip TM device (Medtronic Inc.); however to our knowledge, this has not yet been published in the literature or shown to improve outcomes. The myotomy itself should not go to the base of the diverticulum itself, as this risks perforation of the diverticulum. Instead, most surgeons will perform the myotomy at least 1–2 cm lateral to the base of the diverticulum.
The need for a diverticulectomy is also debated, as in many patients symptoms are not due to the diverticulum but instead to the underlying motility disorder. In some patients, diverticulectomy may not add any benefit and only add risk due to the potential morbidity of a staple line leak. The actual rate of staple line leak is unclear but is reported to be 0–23% [3, 5, 22, 23]. On the other hand, a large diverticulum that does not drain well may cause trapping of food with fermentation, chest pressure and pain, and regurgitation . Patti’s group reported a series of 13 patients undergoing myotomy for diverticulum, 6 with diverticulectomy and 7 without [24]. In the patients not undergoing resection, 3 were because the diverticulum was small and 4 were due to the diverticulum being too proximal or too adherent to fully dissect. Symptom control was excellent and equivalent in both groups, indicating that myotomy alone may be sufficient in selected patients.
In our practice, we carefully examine the preoperative dynamic cine films from the upper GI series. In a patient with a named motility disorder like achalasia, classic achalasia symptoms, and a small wide-mouthed diverticulum, we may plan to leave the diverticulum alone. We may also elect for myotomy alone if the patient is frail, and we judge that they could not tolerate a complication like a staple line leak. Intraoperatively we may decide on myotomy alone if operation is too technically challenging, for example, with a diverticulum higher in the chest. On the other hand, in patients where the primary symptoms seem to be from the diverticulum itself or if the diverticulum is large, is poorly draining, or has a narrow neck, we will be more aggressive in performing a diverticulectomy .
Because we routinely perform our myotomy across the GE junction onto the cardia of the stomach, we feel that a fundoplication is required to minimize the risk of postoperative reflux. A randomized trial of Dor vs. Toupet fundoplication in achalasia showed no difference in postoperative reflux, so either procedure may be performed based on the surgeon’s preference [25]. Although that study showed no significant differences, there were non-significant trends in favor of posterior (Toupet) fundoplication. In our practice, we prefer a Toupet except in the rare circumstance of concern for possible mucosal injury, in which case we perform a Dor to help cover the mucosa.
Outcomes of laparoscopic management of ED are generally good. In one of the larger case series, Heniford’s group at Carolinas reported on 27 patients over 20 years [5], all of whom underwent diverticulectomy , 90% with myotomy and 85% with antireflux procedure. There were no leaks, no mortality, and no recurrence with median follow-up of 36 months. Symptoms resolved in 89% of patients, with 11% having some residual dysphagia .