Zenker diverticulum originates from the posterior wall of the esophagus in a triangular area of weakness, limited inferiorly by the cricopharyngeus muscle and superiorly by the inferior constrictor muscles (ie, the Killian triangle). As the diverticulum enlarges, it tends to deviate from the midline, mostly to the left (Figure 36–5).
Zenker diverticulum results from either a lack of coordination between the pharyngeal contraction and the opening of the UES or a hypertensive UES (including cricopharyngeal spasm). Because of the increased intraluminal pressure, there is progressive herniation of mucosa and submucosa through the Killian triangle.
Dysphagia is the most common symptom. The regurgitation of undigested food from the diverticulum often occurs and can lead to aspiration both into the tracheobronchial tree and pneumonia. Patients frequently have halitosis and can hear gurgling sounds in the neck. About 30–50% of patients have associated GERD.
A barium swallow can clearly show the position and size of the diverticulum. It can also show a hiatal hernia.
Esophageal manometry can demonstrate a lack of coordination between the pharynx and the cricopharyngeus muscle, as well as a hypertensive UES. In addition, it can show a hypotensive LES and abnormal esophageal peristalsis. Ambulatory pH monitoring can determine whether abnormal esophageal acid exposure is present.
The differential diagnosis of Zenker diverticulum includes esophageal stricture, achalasia, esophageal cancer, and pneumonia. A sudden increase in pain and dysphagia symptoms or hematemesis in a patient with an existing Zenker diverticulum should raise suspicion for a squamous cell carcinoma arising within the diverticulum, which is a rare but reported event. Other diverticula can occur in the pharyngeal (pharyngeal pouch) and upper esophageal (Killiam–Jamieson diverticulum) areas and should be distinguished because the treatment is different.
The classic treatment consists of excision of the diverticulum and myotomy of the cricopharyngeus muscle, including the upper 3 cm of the posterior esophageal wall through a cervical incision. For small diverticula (ie, <2 cm), myotomy alone is sufficient.
In more recent years, transoral endoscopic management has gained popularity, using either rigid or flexible endoscopy. The wall of the diverticulum and the cricopharyngeus muscle is divided with a stapling device, electrocautery, or laser. Use of a stapling device has been preferred for diverticula between 3 and 6 cm because it effectively seals the cut edges of the wound as it divides the sac. The stapling device is mechanically limited in short segment (<3 cm) diverticula due to inability to advance the staple line to the end of the sac and thus incomplete division of the party wall, and this can also result in recurrence in patients with longer sacs. Division of short- and long-segment diverticula can be performed with CO2 laser or electrocautery, with some authors advocating mucosal closure with an endoscopic suturing device following division. Endoscopic injection of botulinum toxin into the cricopharyngeus has also been used with some success for small diverticula.
If GERD is present, it should be treated aggressively with either proton pump inhibitors or fundoplication in order to avoid aspiration into the tracheobronchial tree.
The prognosis is excellent in about 90% of cases.
Aly A, Devitt PG, Jamieson GG. Evolution of surgical treatment for pharyngeal pouch. Br J Surg
(Review article describing the evolution of treatment of Zenker's diverticulum.)
Ferreira LEVVC, Simmons DT, Baron TH. Zenker's diverticula: pathophysiology, clinical presentation, and flexible endoscopic management. Dis Esophagus
(Review of literature on flexible endoscopic management of Zenker diverticula.)
Mortensen M, Schaberg MR, Genden EM, Woo P. Transoral resection of short segment Zenker's diverticulum and cricopharyngeal myotomy: an alternative minimally invasive approach. Laryngoscope
(Description of methods for transoral cricopharyngeal myotomy and sac division.)
Counter PR, Hilton ML, Baldwin DL. Long-term follow-up of endoscopic stapled diverticulotomy. Ann R Coll Surg Engl
(Endoscopic, stapled diverticulotomy.)
Smith SR, Gendene M, Urken ML. Endoscopic stapling technique for the treatment of Zenker diverticulum vs standard open-neck technique: a direct comparison and charge analysis. Arch Otolaryngol Head Neck Surg
(Endoscopic stapled diverticulotomy is more cost effective than open surgery.)
- Diverticulum evident on barium swallow
- Esophageal motility disorder shown by esophageal manometry.
Epiphrenic diverticula are located just above the diaphragm (Figure 36–6). The diverticulum is not a primary anatomic abnormality but rather the consequence of an underlying motility disorder of the esophagus; achalasia is the most common, followed by diffuse esophageal spasm. The disorder causes an outflow obstruction at the level of the gastroesophageal junction, with a consequent increase in intraluminal pressure as well as progressive herniation of mucosa and submucosa through the esophageal muscle wall.
The symptoms experienced by patients with epiphrenic diverticulum are in part due to the underlying motility disorder (eg, dysphagia or chest pain) and in part due to the diverticulum per se (ie, regurgitation with the risk of aspiration). Some diverticula, however, can be asymptomatic.
A chest radiograph can show an air-fluid level in the posterior mediastinum. A barium swallow clearly shows the position and size of the diverticulum (see Figure 36–6).
In most cases, esophageal manometry identifies the underlying motility disorder.
A paraesophageal hernia can be confused with an epiphrenic diverticulum. Barium swallow and endoscopy help in establishing the diagnosis.
The treatment is surgical and the laparoscopic approach is currently preferred. This procedure consists of (1) resection of the diverticulum, (2) a long myotomy, and (3) a partial fundoplication to prevent gastroesophageal reflux. The myotomy is performed on the side of the esophagus opposite to where the diverticulum is located. It should extend proximally to the upper border of the neck of the diverticulum and distally for 2 cm onto the gastric wall; a partial fundoplication is then performed.
A laparoscopic diverticulectomy, with myotomy and fundoplication, is successful in 80–90% of cases.
Kilic A, Schuchert MJ, Awais O et al. Surgical management of epiphrenic diverticula in the minimally invasive era. JLS
(Laparoscopic treatment of epiphrenic diverticula results in reduced morbidity compared to open treatment with similar symptom relief.)
Klaus A, Hinder RA, Swain J, Achem SR. Management of epiphrenic diverticula. J Gastrointest Surg
(Laparoscopic treatment of epiphrenic diverticula.)
Nehra D, Fumagulli W, Bona S et al. Physiologic basis for the treatment of epiphrenic diverticulum. Ann Surg
(A physiologic approach to the treatment of epiphrenic diverticulum.)
Tedesco P, Fisichella PM, Way LW et al. Cause and treatment of epiphrenic diverticula. Am J Surg. 2005;190(6):891.
- Recurrent food impactions
- Esophagitis on endoscopy with absence of reflux on pH monitoring
- Eosinophilic infiltration on biopsy.
Eosinophilic esophagitis (EE) is an uncommon but increasingly recognized and reported disease entity involving esophagitis in the setting of absent or controlled reflux. These patients tend to have a history of atopy and can present with dysphagia and recurrent food impactions. Currently it is a diagnosis of exclusion but diagnostic criteria are being developed.
This disease is not yet well understood. However, a strong association with hypersensitivity reactions to airborn alloantigens and particularly food has been found, suggesting a role for antigen-driven eosinophilic infiltration of the esophagus. This is supported by reports of success with food elimination strategies in reducing symptoms. Eosinophilic infiltration is thought to lead to smooth muscle dysfunction and resulting motor disturbances.
Dysphagia is the most common presenting sign, occurring in >90% of patients with EE. Patients can also present with recurrent food impaction, and in the setting of unimpressive endoscopy this should raise suspicion for EE. Heartburn is present in approximately a quarter of patients. Due to the association with atopy, a personal and family history of atopic diseases and reactions should be sought.
Findings consistent with but not pathognomonic for EE include: (1) concentric rings or “trachealization” of the esophagus; (2) longitudinal furrows; (3) friability and esophageal inflammation; (4) multicentric white patches; (5) narrowing or strictures; (6) absence of hiatal hernia or other findings more strongly suggestive of GERD. Biopsies should be performed of any suspicious areas, but should also be performed in any patient with symptoms and history suggestive of EE even in the setting of normal endoscopic findings.
An elevated level of eosinophils (>15/high-power field) has been proposed as a diagnostic criteria for EE. This finding can also be seen in GERD, though usually to a lesser degree. This is particularly true for patients who are currently on antireflux therapy, in whom high levels of esoinophils in the setting of refractory esophagitis should raise suspicion for EE. Patients with EE also tend to have elevated esosinophils in all areas of the esophagus, as compared to typically only the distal esophagus with GERD.
Skin prick testing has been used to identify food triggers in patients with suspected EE with some success. However not all sensitivities can be identified in this manner. Food elimination testing is the mainstay of food sensitivity testing but requires close partnership between the patient and physician and careful monitoring of food content and symptoms. Consultation with a dietician can be useful in the development of food elimination strategies.
It is important to rule out GERD and achalasia by esophageal manometry and pH monitoring tests. Other esophageal motility disorders and benign and malignant strictures can present in a similar way.
Current therapy for EE consists of identification of food triggers and avoidance. This can be difficult because many patients are sensitized to multiple food allergens. Topical glutocorticoids has been successful in controlling symptoms and reducing inflammation in some patients. Acid reduction therapy should be considered if reflux is present to decrease irritation of the inflamed esophageal lining.
Despite food avoidance and pharmacotherapy demonstrating effectiveness in some patients, eosinophilic esophagitis remains a difficult disease to treat, with relapses in symptoms occurring in the majority of patients even after successful therapy.
Dellon ES, Gibbs WB, Fritchie KJ et al. Clinical, endoscopic, and histologic findings distinguish eosinophilic esophagitis from gastroesophageal reflux disease. Clinical Gastroenterol Hepatol
(Proposed criteria for distinguishing between EE and GERD.)
Hong S, Vogel NM. Food allergy and eosinophilic esophagitis: learning what to avoid. Cleve Clin J Med
(Overview of food allergy testing and treatment of EE.)
Rothenberg ME. Biology and treatment of eosinophilic esophagitis. Gastroenterol
(Current scientific understanding of the pathogenesis of EE and treatment strategies.)
Schopfer AM, Gonsalves N, Bussmann C et al. Esophageal dilation in eosinophilic esophagitis: effectiveness, safety, and impact on underlying inflammation. Am J Gastroenterol
. advance online publication 24 November 2009.
(Dilation in patients with EE can be carried out safely.)
Gastroesophageal Reflux Disease
- Sliding hiatal hernia on barium swallow
- Esophagitis on endoscopy
- Abnormal esophageal exposure on ambulatory pH monitoring.
GERD is the most common upper gastrointestinal disorder in the Western world and accounts for approximately 75% of esophageal diseases. Heartburn, usually considered synonymous with the presence of abnormal gastroesophageal reflux, is experienced by 20–40% of the adult population of Western countries. The incidence of reflux symptoms increases with age, and both sexes seem to be equally affected. Symptoms are more common during pregnancy, probably because of hormonal effects on the LES and the increased intraabdominal pressure of the enlarging uterus.
GERD is caused by the abnormal retrograde flow of gastric contents into the esophagus, resulting in symptoms, mucosal damage, or both. A defective LES is the most common cause of GERD. Transient LES relaxations account for most reflux episodes in patients either without mucosal damage or with mild esophagitis, whereas a short and hypotensive LES is more frequently found in patients with more severe esophagitis. In 40–60% of patients with GERD, abnormalities of esophageal peristalsis are also present. Because esophageal peristalsis is the main determinant of esophageal acid clearance (ie, the ability of the esophagus to clear gastric contents refluxed through the LES), patients with abnormal esophageal peristalsis have more severe reflux and slower clearance. Therefore, these patients often have more severe mucosal injury and more frequent atypical symptoms such as cough or hoarseness. A hiatal hernia also contributes to the incompetence of the gastroesophageal junction by altering the anatomic relationship between the LES and the esophageal crus. In patients with large hiatal hernias, the LES is usually shorter and weaker and the amount of reflux is greater.
Heartburn, regurgitation, and dysphagia are considered typical symptoms of GERD. However, a clinical diagnosis of GERD, based on typical symptoms such as heartburn and regurgitation, is correct in only 70% of patients when compared with the results of pH monitoring. A good response to therapy with proton pump inhibitors is instead a better predictor of the presence of abnormal reflux. In addition to the typical symptoms, patients with GERD can present with atypical symptoms such as cough, wheezing, chest pain, hoarseness, and dental erosions. These symptoms represent extraesophageal presentations of the disease, including respiratory disorders such as asthma, as well as ear, nose, and throat abnormalities such as laryngitis (Table 36–1). Two mechanisms have been postulated for GERD-induced respiratory symptoms: (1) a vagal reflux arc resulting in bronchoconstriction and (2) microaspiration into the tracheobronchial tree.
Table 36–1. Typical and Atypical Symptoms of GERD. ||Download (.pdf)
Table 36–1. Typical and Atypical Symptoms of GERD.
- Chronic laryngitis and sore throat
- Globus sensation
- Otitis media
- Dental erosions
- Noncardiac chest pain
- Chronic cough
- Aspiration pneumonia
Ear, nose, and throat symptoms such as hoarseness, globus sensation, or dental erosions are thought to be secondary to the upward extent of the acid with direct damage. This phenomenon has been termed laryngopharyngeal reflux.
A barium swallow provides information about the presence and size of a hiatal hernia, the presence and length of a stricture, and the length of the esophagus. This test, however, is not diagnostic of GERD since a hiatal hernia or reflux of barium can be present in patients who do not have GERD.
The value of endoscopy is mostly limited to the detection of the complications of GERD (eg, esophagitis, Barrett esophagus, and stricture) and to the exclusion of other pathology (esophageal, gastric, or duodenal). The value of endoscopy in diagnosing GERD is limited because only 50% of patients with GERD have esophagitis. In addition, there is major interobserver variation among endoscopists for the low grades of esophagitis.
Esophageal manometry provides information about the LES, including the resting pressure, length, and relaxation, as well as about the quality of esophageal peristalsis. In about 40% of patients with GERD, the pressure of the LES and the peristalsis are normal. In addition, manometry is essential for proper placement of the pH probe for ambulatory pH monitoring (5 cm above the upper border of the LES).
Ambulatory pH monitoring is the most reliable test in the diagnosis of GERD, with a sensitivity and specificity of about 92%. Acid-suppressing medications must be stopped 3 days (eg, H2-blocking agents) to 14 days (eg, proton pump inhibitors) prior to the study. Diet and exercise are unrestricted during the test in order to mimic a typical day of the patient's life. This test should always be performed (1) in patients who do not respond to medical therapy; (2) in patients who relapse after the discontinuation of medical therapy; (3) before antireflux surgery; (4) when atypical symptoms are present. In patients with atypical symptoms a pH probe with two sensors (5 and 20 cm above the LES) is used to determine the upward extent of the reflux. The tracing should be analyzed for a temporal correlation between symptoms and episodes of reflux (Figure 36–7).
Ambulatory pH monitoring. (A) Two sensors located 5 and 20 cm above the lower esophageal sphincter. (B) Correlation between episodes of reflux and cough (c).
Irritable bowel syndrome, achalasia, eosinophilic esophagitis, cholelithiasis, and coronary artery disease can present with heartburn. Esophageal manometry and pH monitoring are essential to determine with certainty whether GERD is present.
Esophagitis is the most common complication. Barrett esophagus (ie, metaplastic changes from squamous to columnar epithelium) is found in about 12% of patients with reflux documented by pH monitoring. This complication may lead to the development of adenocarcinoma. Asthma, aspiration pneumonia, laryngitis, chronic sinusitis, and dental erosions can also occur.
Patients should eat frequent, small meals during the day to avoid gastric distention. They should also avoid fatty foods, spicy foods, and chocolate, because these foods lower the LES pressure. The last meal of the day should be no less than 2 h before going to bed. To increase the effect of gravity, the head of the bed should be elevated over 4- to 6-inch blocks. Patients should also be counseled to lose weight if obese.
Other Nonsurgical Measures
Antacids are useful for patients with mild intermittent heartburn. Acid-suppressing medications are the mainstay of medical therapy. H2 blocking agents are usually prescribed for patients with mild symptoms or mild esophagitis. Proton pump inhibitors are superior to H2 blocking agents because they exert a more profound control of acid secretion—healing of the esophagitis occurs in 80–90% of these patients. However, both the symptoms and esophagitis tend to recur in most patients after therapy is discontinued so that most patients need chronic maintenance therapy. In addition, about 50% of patients on maintenance proton pump inhibitors require increasing doses to maintain healing of the esophagitis. Medical therapy is largely ineffective for the treatment of the extraesophageal manifestations of GERD due to the upward extension of the gastric contents. In these patients, acid-suppressing medications only alter the pH of the gastric refluxate, but reflux and aspiration still occur because of an incompetent LES and an ineffective esophageal peristalsis.
Recent concern has been raised about potential adverse effects of long-term therapy with proton-pump inhibitors, in particular calcium malabsorption leading to osteoporosis, increased risk of gastrointestinal infections and pneumonia. Overall the safety profile of proton pump inhibitors is excellent, but it is recommended that patients are treated with the lowest effective dose to control symptoms.
The goal of surgical therapy is to restore the competence of the LES. A laparoscopic total fundoplication (360°) is considered the procedure of choice because it increases the resting pressure and length of the LES and decreases the number of transient LES relaxations (Figure 36–8).
Laparoscopic Nissen fundoplication. (A) Position of the trocars. (B) Completed fundoplication.
A laparoscopic fundoplication provides the same excellent results of open surgery, with symptom resolution in more than 90% of patients. It now requires a 1- to 2-day hospital stay and results in both minimal postoperative discomfort and a fast return to regular activity.
The ideal patient is one who has a good response to proton pump inhibitors. A patient who is nonresponsive to medical therapy requires a thorough work-up to elucidate the cause of the foregut symptoms, and an alternative diagnosis ranging from irritable bowel syndrome to gallbladder disease is frequently found. Young patients might also choose an operation early in the course of their disease to avoid a life-long commitment to lifestyle changes and medications. Long-term follow-up of patients treated with either fundoplication or proton pump inhibitors demonstrates surgery to be superior to medical management with regards to control of symptoms.
Patients who have regurgitation with respiratory symptoms or hoarseness are also ideal candidates for a fundoplication. Even the complete elimination of gastric acid secretion by proton pump inhibitors frequently fails to control these symptoms, since it only alters the pH of the gastric refluxate but does not prevent the regurgitation and upward extent of the reflux. Analyzing the pH tracing for a correlation between the symptoms and the episodes of reflux helps to predict the surgical outcome (see Figure 36–7).
Many surgeons also consider the presence of Barrett esophagus as an indication for surgical rather than medical treatment, based on the following considerations: (1) Proton pump inhibitors, although effective in controlling the acid component of refluxate, do not eliminate the reflux of bile, which is a major contributor to the pathogenesis of Barrett epithelium. (2) Patients with Barrett esophagus have a lower LES pressure and a defective peristalsis more often than patients without Barrett esophagus. As a consequence, their mucosa is exposed to larger amounts of gastric refluxate. (3) Evidence suggests that an effective antireflux operation can prevent the progression from metaplasia to dysplasia. The definite answer, however, awaits the results of further randomized control studies; therefore, endoscopic surveillance after laparoscopic fundoplication is recommended.
After a fundoplication, the control of typical symptoms is obtained in about 90% of patients. The success rate is in the range of 70–90% for patients with atypical symptoms, since it is often more difficult to establish, preoperatively, a strong correlation between gastroesophageal reflux and symptoms.
Cote GA, Howden CW. Potential adverse effects of proton pump inhibitors. Curr Gastroenterol Rep
(Review of emerging data on adverse effects of proton pump inhibitor therapy.)
Campos GM, Peters JH, DeMeester TR et al. Multivariate analysis of factors predicting outcome after laparoscopic Nissen fundoplication. J Gastrointest Surg
(Preoperative factors that predict a good outcome after laparoscopic fundoplication.)
Diener U, Patti MG, Molena D, et al. Esophageal dysmotility and gastroesophageal reflux disease. J Gastrointest Surg
(Patients with abnormal esophageal peristalsis have more acid reflux, slower clearance, and worse mucosal injury.)
Garrett CG, Cohen SM. Otolaryngological perspective on patients with throat symptoms and laryngeal irritation. Curr Gastroenterol Rep
(Ear, nose, and throat manefestations of GERD/LPR.)
Jailwala JA, Shaker R. Oral and pharyngeal complications of gastroesophageal reflux disease: globus, dental erosions, chronic sinusitis. J Clin Gastroenterol
(Ear, nose, and throat manifestations of gastroesophageal reflux disease.)
Kahrilas PJ. Clinica practice: gastroesophageal reflux disease. NEJM
(Review of current diagnosis and treatment strategies for GERD.)
Lundell L, Miettinen P, Myrvold HE et al. Comparison of outcomes twelve years after antireflux surgery or omeprazole
maintenance therapy for reflux esophagitis. Clin Gastroenterol Hepatol
(Surgery is superior to omeprazole
for control of reflux symptoms.)
Patti MG, Arecerito M, Tamburini A et al. Effect of laparoscopic fundoplication on gastroesophageal reflux disease-induced respiratory symptoms. J Gastrointest Surg
(Laparoscopic fundoplication is an effective treatment for GERD-induced respiratory symptoms.)
Patti MG, Diener U, Tamburini Aet al. Role of esophageal function tests in the diagnosis of gastroesophageal reflux disease. Dig Dis Sci
(Esophageal manometry and pH monitoring are necessary to establish the diagnosis of gastroesophageal reflux disease.)
Patti MG, Robinson T, Galvani C et al. Total fundoplication is superior to partial fundoplication even when esophageal peristalsis is weak. J Am Coll Surg
(Study comparing total and partial fundoplication for gastroesophageal reflux disease.)
Patti MG, Tedesco P, Golden J et al. Idiopathic pulmonary fibrosis. How often is it really idiopathic? J Gastrointest Surg
(Gastroesophageal reflux disease has probably a cause-and-effect relationship with idiopathic pulmonary fibrosis.)
Richter JE. Extraesophageal presentations of gastroesophageal reflux disease: an overview. Am J Gastroenterol
(Atypical symptoms of gastroesophageal reflux disease.)