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Midesophageal Pulsion Diverticulum Resulting From Hypercontractile (Jackhammer) Esophagus
Midesophageal Pulsion Diverticulum Resulting From Hypercontractile (Jackhammer) Esophagus Onkar V. Khullar, MD, Sagar R. Shroff, MD, Sonali S. Sakaria, MD, and Seth D. Force, MD Section of General Thoracic Surgery and Division of Digestive Diseases, Emory University School of Medicine, Atlanta, Georgia
We report a patient with significant dysphagia from hypercontractile “jackhammer” esophagus and a midesophageal pulsion diverticulum. This was treated with a thoracoscopic diverticulectomy and a long esophageal myotomy sparing the lower esophageal sphincter (LES). We describe the clinical diagnosis and surgical treatment of this uncommon esophageal motility disorder. To our knowledge, this is the first report in the literature of a midesophageal diverticulum caused by jackhammer esophagus. We propose that in the setting of normal LES function, successful treatment should include diverticulectomy with an LES-sparing myotomy. (Ann Thorac Surg 2017;103:e127–9) Ó 2017 by The Society of Thoracic Surgeons
E
sophageal motility disorders are highly varied, and include several diagnoses such as achalasia, distal esophageal spasm, esophagogastric junction outflow obstruction, and hypercontractile esophagus (jackhammer esophagus). Some of these disorders, in particular achalasia, are associated with a distal esophageal obstruction because of a hypertensive LES. Although pulsion diverticula have been associated with motility disorders, these are typically classified as distal esophageal, epiphrenic diverticulum. We report an unusual case of jackhammer esophagus with a midesophageal pulsion diverticulum, treated with diverticulectomy and esophageal myotomy sparing the LES.
unusual midesophageal pulsion diverticulum with a normal appearing LES. As pulsion diverticula are typically caused by a functional distal esophageal obstruction, most commonly from a motility disorder, esophageal manometry was obtained (Fig 3). High-resolution manometry revealed a normal LES, with a mean integrated residual pressure of 6.2 mm Hg. However, the mean distal contractile integral was 5025.6 mm Hg-cm-s with two contractions greater than 8000 mm Hg-cm-s. These findings are consistent with hypercontractile jackhammer esophagus with a normal LES. Therefore, we performed a right thoracoscopic stapled diverticulectomy and long esophageal myotomy, beginning several centimeters above the diverticulum down to just above the lower esophageal sphincter. This was completed using a standard three-port technique. On postoperative followup, the patient reported complete resolution of her chest pain and dysphagia.
Comment Esophageal motility disorders, such as achalasia, distal esophageal spasm, and esophagogastric junction outflow obstruction are relatively common. Some of these disorders, in particular achalasia, are often associated with a distal obstruction because of a hypertensive LES. The Chicago Classification version 3.0 defines hypercontractile esophagus, or jackhammer esophagus, as an esophageal motility disorder characterized by a distal contractile integral greater than 8000 mm Hg-s-cm in at least two of ten swallows on manometry. [1]. This can occur with or without involvement of the esophagogastric junction. Jackhammer esophagus is a relatively rare
A 49-year-old woman presented with dysphagia to solid food, intermittent emesis for 6 months, and intermittent severe substernal chest pain. She was treated with proton pump inhibitors without relief of symptoms. Esophagoscopy revealed a large midesophageal diverticulum (Fig 1). Notably, we identified no obstruction, resistance, or stricture at the LES, which could have resulted in formation of a diverticulum. These findings were confirmed with a barium esophageal showing a midesophageal diverticulum with no obstruction of contrast at the LES (Fig 2A). Finally, chest CT showed similar findings with no esophageal masses or calcified lymph nodes. Together, these findings were consistent with an
Accepted for publication July 13, 2016. Address correspondence to Dr Khullar, Emory University Hospital Midtown, 550 Peachtree St, 6th Flr, Atlanta, GA 30308; email: okhulla@ emory.edu.
Ó 2017 by The Society of Thoracic Surgeons Published by Elsevier
Fig 1. Esophagoscopy revealed a large midesophageal diverticulum (arrow). 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2016.07.030
e128
CASE REPORT KHULLAR ET AL JACKHAMMER ESOPHAGUS WITH DIVERTICULUM
Ann Thorac Surg 2017;103:e127–9
Fig 2. (A) Initial barium contrast esophagram revealed a large midesophageal pulsion diverticulum with no distal lower esophageal sphincter obstruction. (B) Postoperative esophagram showing resection of the diverticulum and successful myotomy.
disorder, occurring in only 4.1% of patients undergoing high-resolution manometry in a series of 2000 patients [2]. It is uniformly associated with dysphagia and chest pain. Epiphrenic diverticula have been associated with motility disorders resulting in obstruction of the LES. Recommended treatment typically consists of a diverticulectomy with esophageal myotomy including the LES with or without a fundoplication [3]. Several small case series of patients with esophageal epiphrenic
Fig 3. High-resolution esophageal manometry revealed hypercontractile esophageal body with two contractions with a distal contractile integral greater than 8000. Resting lower esophageal sphincter pressure was normal. These findings are consistent with jackhammer esophagus.
diverticulum have noted jackhammer esophagus. One series of high resolution manometry in nine patients with esophageal epiphrenic diverticulum identified a segmental hypercontractile zone in one patient [4], whereas in another series of 19 patients with distal esophageal diverticulum, two were identified as having jackhammer esophagus [5]. Treatment of pulsion epiphrenic diverticulum owing to an esophageal motility disorder typically consists of a diverticulectomy and long esophageal myotomy, which includes the
Ann Thorac Surg 2017;103:e127–9
LES and extends onto the cardia of the stomach (i.e., Heller myotomy). This can be completed surgically using transthoracic or, more commonly, laparoscopic approaches. The described case was unique in that the pulsion diverticulum was located in the midesophagus, and not in the epiphrenic region. Thus, it would not have been accessible laparoscopically. Furthermore, destruction of the LES in a patient with normal resting LES pressure would likely result in significant reflux with no additional benefit in terms of symptom management. Therefore, we believed that the ideal treatment in this patient would consist of a minimally invasive thoracoscopic diverticulectomy with an extended myotomy of the areas of affected esophagus above and below the diverticulum, while sparing the LES. This resulted in complete resolution of symptoms in the described patient with no postoperative reflux. We propose that patients with midesophageal pulsion diverticulum owing to motility disorders that spare the LES, such as jackhammer
CASE REPORT KHULLAR ET AL JACKHAMMER ESOPHAGUS WITH DIVERTICULUM
e129
esophagus, can be successfully treated with a thoracoscopic long myotomy sparing the LES.
References 1. Kahrilas PJ, Bredenoord AJ, Fox M, et al. International High Resolution Manometry Working Group. The Chicago Classification of esophageal motility disorders, v3.0. Neurogastroenterol Motil 2015;27:160–74. 2. Roman S, Pandolfino JE, Chen J, Boris L, Luger D, Kahrilas PJ. Phenotypes and clinical context of hypercontractility in highresolution esophageal pressure topography (EPT). Am J Gastroenterol 2012;107:37–45. 3. Macke RA, Luketich JD, Pennathur A, et al. Thoracic esophageal diverticula: a 15-year experience of minimally invasive surgical management. Ann Thorac Surg 2015;100:1795–803. 4. Vicentine FP, Herbella FA, Silva LC, Patti MG. High resolution manometry findings in patients with esophageal epiphrenic diverticula. Am Surg 2011;77:1661–4. 5. Carlson DA, Gluskin AB, Mogni B, et al. Esophageal diverticula are associated with propagating peristalsis: a study utilizing high-resolution manometry. Neurogastroenterol Motil 2016;28:392–8.
We report a patient with significant dysphagia from hypercontractile “jackhammer” esophagus and a midesophageal pulsion diverticulum. This was treated with a thoracoscopic diverticulectomy and a long esophageal myotomy sparing the lower esophageal sphincter (LES). We describe the clinical diagnosis and surgical treatment of this uncommon esophageal motility disorder. To our knowledge, this is the first report in the literature of a midesophageal diverticulum caused by jackhammer esophagus. We propose that in the setting of normal LES function, successful treatment should include diverticulectomy with an LES-sparing myotomy. (Ann Thorac Surg 2017;103:e127–9) Ó 2017 by The Society of Thoracic Surgeons
E
sophageal motility disorders are highly varied, and include several diagnoses such as achalasia, distal esophageal spasm, esophagogastric junction outflow obstruction, and hypercontractile esophagus (jackhammer esophagus). Some of these disorders, in particular achalasia, are associated with a distal esophageal obstruction because of a hypertensive LES. Although pulsion diverticula have been associated with motility disorders, these are typically classified as distal esophageal, epiphrenic diverticulum. We report an unusual case of jackhammer esophagus with a midesophageal pulsion diverticulum, treated with diverticulectomy and esophageal myotomy sparing the LES.
unusual midesophageal pulsion diverticulum with a normal appearing LES. As pulsion diverticula are typically caused by a functional distal esophageal obstruction, most commonly from a motility disorder, esophageal manometry was obtained (Fig 3). High-resolution manometry revealed a normal LES, with a mean integrated residual pressure of 6.2 mm Hg. However, the mean distal contractile integral was 5025.6 mm Hg-cm-s with two contractions greater than 8000 mm Hg-cm-s. These findings are consistent with hypercontractile jackhammer esophagus with a normal LES. Therefore, we performed a right thoracoscopic stapled diverticulectomy and long esophageal myotomy, beginning several centimeters above the diverticulum down to just above the lower esophageal sphincter. This was completed using a standard three-port technique. On postoperative followup, the patient reported complete resolution of her chest pain and dysphagia.
Comment Esophageal motility disorders, such as achalasia, distal esophageal spasm, and esophagogastric junction outflow obstruction are relatively common. Some of these disorders, in particular achalasia, are often associated with a distal obstruction because of a hypertensive LES. The Chicago Classification version 3.0 defines hypercontractile esophagus, or jackhammer esophagus, as an esophageal motility disorder characterized by a distal contractile integral greater than 8000 mm Hg-s-cm in at least two of ten swallows on manometry. [1]. This can occur with or without involvement of the esophagogastric junction. Jackhammer esophagus is a relatively rare
A 49-year-old woman presented with dysphagia to solid food, intermittent emesis for 6 months, and intermittent severe substernal chest pain. She was treated with proton pump inhibitors without relief of symptoms. Esophagoscopy revealed a large midesophageal diverticulum (Fig 1). Notably, we identified no obstruction, resistance, or stricture at the LES, which could have resulted in formation of a diverticulum. These findings were confirmed with a barium esophageal showing a midesophageal diverticulum with no obstruction of contrast at the LES (Fig 2A). Finally, chest CT showed similar findings with no esophageal masses or calcified lymph nodes. Together, these findings were consistent with an
Accepted for publication July 13, 2016. Address correspondence to Dr Khullar, Emory University Hospital Midtown, 550 Peachtree St, 6th Flr, Atlanta, GA 30308; email: okhulla@ emory.edu.
Ó 2017 by The Society of Thoracic Surgeons Published by Elsevier
Fig 1. Esophagoscopy revealed a large midesophageal diverticulum (arrow). 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2016.07.030
e128
CASE REPORT KHULLAR ET AL JACKHAMMER ESOPHAGUS WITH DIVERTICULUM
Ann Thorac Surg 2017;103:e127–9
Fig 2. (A) Initial barium contrast esophagram revealed a large midesophageal pulsion diverticulum with no distal lower esophageal sphincter obstruction. (B) Postoperative esophagram showing resection of the diverticulum and successful myotomy.
disorder, occurring in only 4.1% of patients undergoing high-resolution manometry in a series of 2000 patients [2]. It is uniformly associated with dysphagia and chest pain. Epiphrenic diverticula have been associated with motility disorders resulting in obstruction of the LES. Recommended treatment typically consists of a diverticulectomy with esophageal myotomy including the LES with or without a fundoplication [3]. Several small case series of patients with esophageal epiphrenic
Fig 3. High-resolution esophageal manometry revealed hypercontractile esophageal body with two contractions with a distal contractile integral greater than 8000. Resting lower esophageal sphincter pressure was normal. These findings are consistent with jackhammer esophagus.
diverticulum have noted jackhammer esophagus. One series of high resolution manometry in nine patients with esophageal epiphrenic diverticulum identified a segmental hypercontractile zone in one patient [4], whereas in another series of 19 patients with distal esophageal diverticulum, two were identified as having jackhammer esophagus [5]. Treatment of pulsion epiphrenic diverticulum owing to an esophageal motility disorder typically consists of a diverticulectomy and long esophageal myotomy, which includes the
Ann Thorac Surg 2017;103:e127–9
LES and extends onto the cardia of the stomach (i.e., Heller myotomy). This can be completed surgically using transthoracic or, more commonly, laparoscopic approaches. The described case was unique in that the pulsion diverticulum was located in the midesophagus, and not in the epiphrenic region. Thus, it would not have been accessible laparoscopically. Furthermore, destruction of the LES in a patient with normal resting LES pressure would likely result in significant reflux with no additional benefit in terms of symptom management. Therefore, we believed that the ideal treatment in this patient would consist of a minimally invasive thoracoscopic diverticulectomy with an extended myotomy of the areas of affected esophagus above and below the diverticulum, while sparing the LES. This resulted in complete resolution of symptoms in the described patient with no postoperative reflux. We propose that patients with midesophageal pulsion diverticulum owing to motility disorders that spare the LES, such as jackhammer
CASE REPORT KHULLAR ET AL JACKHAMMER ESOPHAGUS WITH DIVERTICULUM
e129
esophagus, can be successfully treated with a thoracoscopic long myotomy sparing the LES.
References 1. Kahrilas PJ, Bredenoord AJ, Fox M, et al. International High Resolution Manometry Working Group. The Chicago Classification of esophageal motility disorders, v3.0. Neurogastroenterol Motil 2015;27:160–74. 2. Roman S, Pandolfino JE, Chen J, Boris L, Luger D, Kahrilas PJ. Phenotypes and clinical context of hypercontractility in highresolution esophageal pressure topography (EPT). Am J Gastroenterol 2012;107:37–45. 3. Macke RA, Luketich JD, Pennathur A, et al. Thoracic esophageal diverticula: a 15-year experience of minimally invasive surgical management. Ann Thorac Surg 2015;100:1795–803. 4. Vicentine FP, Herbella FA, Silva LC, Patti MG. High resolution manometry findings in patients with esophageal epiphrenic diverticula. Am Surg 2011;77:1661–4. 5. Carlson DA, Gluskin AB, Mogni B, et al. Esophageal diverticula are associated with propagating peristalsis: a study utilizing high-resolution manometry. Neurogastroenterol Motil 2016;28:392–8.