Esophageal Diverticula (Pharyngoesophageal Diverticulum, Epiphrenic Diverticulum)

Esophageal diverticula are a rare disease of the esophagus, which is divided into the rarer true diverticula, a protrusion of all layers of the esophageal wall by traction, typically in the middle third of the thoracic esophagus, and the more common false or pseudodiverticula, a bulge without muscular covering, located in muscularly weak regions, such as the hypopharynx (Zenker’s diverticula) or the distal esophagus (epiphrenic diverticula), because of an emulsion caused by increased intraluminal pressure, which causes herniation. The prevalence of esophageal diverticula is between 0.015% and 3%, whereas Zenker’s diverticula is the most common form of esophageal diverticula, accounting for 70%.⁶ Genuine diverticula, which are usually caused by mediastinal disease, such as tuberculous lymphadenitis or sarcoidosis, are caused by motility disorders. Zenker’s diverticula in particular are mainly caused by insufficient relaxation of the UES.⁷ However, some studies show abnormal esophageal motility even in true esophageal diverticula, although it is unclear whether the motility abnormality is primarily or secondarily resulting from the presence of the diverticulum.⁸ Achalasia is also associated with epiphrenic esophageal diverticula followed by diffuse esophageal spasms.⁹

In general, dysphagia, regurgitation of undigested food, bad breath, and a history of silent aspiration with recurrent pneumonia are typical symptoms of esophageal diverticula. However, patients with small diverticula are often asymptomatic until the pouch becomes enlarged. In rare cases, patients with long-lasting dysphagia caused by esophageal diverticula experience weight loss and malnutrition. Large Zenker’s diverticula can lead to a complete obstruction of the esophagus because the retained contents press anteriorly. In addition, patients with Zenker’s diverticula are often associated with hiatal hernia and gastroesophageal reflux disease (GERD; 72%).¹⁰

The diagnosis is confirmed by radiologic imaging, such as barium esophagography, endoscopy, and esophageal manometry. In addition, transcutaneous sonography may be an alternative method for diagnosing Zenker’s diverticulum in experienced hands.

Despite an improvement in surgical therapy, mortality from esophageal diverticulum surgery is still 1% to 5%.¹¹ Surgical therapy is usually indicated in patients with esophageal diverticulum, but some authors recommend surgery only in symptomatic patients.¹² The Zenker’s diverticulum could be resected through an open left cervical or transoral access with rigid or flexible endoscopy. Diverticulectomy or diverticulopexy could be performed, including myotomy of the cricopharyngeal muscle to treat UES dysfunction.¹² Diverticula located in the middle of the esophagus require a transthoracic approach. The operation can usually be performed by thoracoscopy or thoracotomy from the left side.¹³ An additional myotomy may also be necessary. For diverticula of the lower esophagus, especially epiphrenic diverticula, transabdominal laparoscopic surgery with transhiatal access is recommended. Diverticulectomy and additional myotomy/cardiomyotomy imply the need for antireflux procedures, such as partial fundoplication. In some patients, dilatation of the inferior esophageal sphincter may be an alternative to myotomy and the required antireflux procedure, but the clinical evidence is not conclusive.^11–13

Esophageal Fistula (Tracheo- and Bronchoesophageal Fistula) (see Chapter 45)

A fistula from the airways to the upper gastrointestinal tract bypasses the normal protection provided by intact laryngeal reflexes and could lead to significant morbidity and mortality. There are connections between the esophagus and the trachea or large bronchi, all of which are grouped together as tracheoesophageal fistula (TEF). In adults, most acquired TEFs are associated with malignant diseases, such as esophageal or lung cancer, and surgical procedures, such as esophagectomy,¹⁴ radiotherapy, or chemotherapy.^15,16 TEFs rarely occur because of benign diseases, such as prolonged endotracheal intubation, dilatation tracheotomy, endoscopic procedures (e.g., endobronchial laser or cryotherapy, esophageal stenting), infectious diseases, or trauma. Particularly in anesthesia and intensive care units (ICUs), injury mechanisms, such as traumatic intubation, suction of the airways, and vascular compression of the tracheal wall, which is caused by prolonged overinflation of the endotracheal cuff leading to ischemia and subsequent ulceration. Most congenital TEFs are typically associated with esophageal atresia (90% with esophageal atresia have an esophageal fistula).

Symptoms when spontaneously breathing include postprandial coughing, recurrent pneumonia, hoarseness, and dysphagia. The Ohno sign, postprandial cough with fluid intake, is pathognomonic.¹⁷ Acquired TEF should be considered in any ventilated patient with recurrent chest infections and recurrent weaning disorders and weight loss.

Radiologic imaging, such as chest x-rays enhanced by barium swallowing, thoracic computed tomography (CT) with oral contrast agent, or magnetic resonance imaging, facilitates the localization of TEFs and the identification of their likely etiology. Bronchoscopy or gastrointestinal endoscopy should be performed to confirm TEFs, whereas oral instillation of methylene blue helps to identify even small TEFs.

The therapy of TEFs depends on the etiology and localization of the fistula. Spontaneous closure of TEFs is rare, and surgical therapy is therefore necessary. In the majority of patients with acquired, nonmalignant TEFs, successful closure could be safely achieved by a single repair of the esophagus and trachea supported by tissue flaps.¹⁸ Surgical access is via a cervical incision or thoracotomy, depending on the location of the TEF. Esophageal stenting is the dominant treatment of TEFs in malignant or critically ill patients with a high perioperative mortality rate.¹⁹ Stenting of the esophagus, trachea, or both (double stenting) may be necessary to achieve closure of the fistula. A congenital fistula, especially in combination with esophageal atresia, usually requires neonatal surgery, which is performed as a thoracotomy or thoracoscopy with comparable complications and a total mortality rate of 3.2%.²⁰ The outcome in patients with benign TEF who are successfully operated on is usually good; in patients with malignant TEF, the treatment may reduce dysphagia, repeated episodes of pneumonia, and improvement of palliative care.

Esophageal Rupture and Perforation

Esophageal perforation affects a wide range of conditions characterized by the transmural disruption of the esophagus.²¹ It occurs in 65% of cases as a result of iatrogenic interventions. These include endoscopy of upper gastrointestinal tract, endoscopic sclerotherapy, surgery to adjoining tissue, traumatic placement of esophageal dilators, nasogastric tubes, misplaced endotracheal tubes, or TEE. Esophageal perforation also results from external trauma or postoperatively, as well as by foreign bodies or chemically compounds—whereas alkaline compounds cause more damage than acidic compounds. Hospital mortality in patients with esophageal perforation tends to be around 17.5%, whereas 3-year survival rates are 67%.²²

Esophageal rupture results from a sudden increase of intraabdominal pressure while the relaxation of the cricopharyngeal muscle is incomplete. Spontaneous esophageal rupture during vomiting is known as Boerhaave syndrome because it was first described by the Dutch physician Hermann Boerhaave in 1724. It is a very rare pathology; a study in Iceland demonstrated an incidence of 3.1/1,000,000 per year of overall esophageal rupture and of that, only 24% of cases were categorized as spontaneous ruptures.²³ Spontaneous esophageal ruptures occur more frequently in men than women and typically affect men aged 40 to 70 years who overindulge in alcohol and food.²⁴ Only 5% of patients with Boerhaave syndrome are considered to be healthy with no underlying etiology. The perforation is mostly located on the left posterior part of the distal esophagus, 2 to 3 cm proximally to the gastroesophageal junction.²⁵

Patients with Boerhaave syndrome usually present with severe chest pain after an episode of violent vomiting. Although pain is often the primary symptom, other symptoms can include dyspnea, voice changes, dysphagia, and subcutaneous emphysema. Mackler’s triad, often associated with spontaneous esophageal rupture, includes chest pain, vomiting, and subcutaneous emphysema. To determine the diagnosis, a chest x-ray is helpful because patients with Boerhaave syndrome will have an abnormal x-ray in 81% to 90% of cases, although within the first 6 hours of the perforation, 10% to 33% of patients may have normal chest x-ray findings.²⁴ To confirm the diagnosis, gastrointestinal tract contrast CT has a better sensitivity than an upper gastrointestinal tract contrast study. Esophageal endoscopy also provides up to 100% sensitivity, but insufflation can be seen as an additional risk to the tear.²⁶

A spontaneous esophageal rupture is rare, but it is a serious disease with a mortality rate as high as 80%, although current improvements in detection and treatment lower the mortality rates to 2% to 20%. The mortality rate doubles if treatment is delayed after 24 hours, so timely detection is a cornerstone of the therapy.²⁷ Surgical treatment is still a basic modality of therapy for esophageal perforation and rupture, but conservative treatment and endoscopic therapies can also be chosen. The treatment strategy should be carefully tailored according to individual severity of the disease and the location of perforation. In this context, a severity scoring system based on 10 clinical and radiologic factors—the Pittsburgh Classification—has been developed to stratify treatment options; an approach to esophageal perforation based on injury severity and the degree of mediastinal and pleural contamination is of paramount importance. Although operative management remains the standard in the majority of patients with esophageal perforation, nonoperative management may be successfully implemented in selected patients with a low morbidity and mortality.²⁸ The score was validated in a multinational study, and it was suggested that patients with a low score (≤ 2) could be considered for nonsurgical management. Perforation-related morbidity, length of stay, frequency of operative treatment, and mortality increased with increasing perforation severity score (PSS) strata. Patients with high PSS were 3.37 times more likely to be treated surgically compared with low PSS.²⁹ New interventional endoscopic techniques, including endoscopic clips, covered metal stents, and endoluminal vacuum therapy have been developed in recent years to manage esophageal perforation reducing the associated morbidity and mortality. These interventions demonstrated the usefulness of conservative treatment of esophageal rupture.^28,30,31 Endoscopic clip placement is currently the standard method for closing small (<2 cm) luminal perforations.³² Endoscopic treatment is also the gold standard for closing perforations in the esophagus that occur and are detected during an endoscopic procedure³³ (Fig. 44.2).³⁴

Endoscopically inserted stents (partially or fully covered self-expandable metal stents, self-expandable plastic stents) can be used to cover larger defects or to completely cover an unsatisfactory clip closure. Nonsurgical management of esophageal perforation may be considered in stable patients with early presentation, limited esophageal rupture, and minimal contamination of surrounding spaces if highly specialized monitoring is available.³³

Esophageal emergency guidelines published in 2019 defined that patients eligible for nonsurgical management (Table 44.2) should be kept empty stomach, and administered broad spectrum antibiotics and proton pump inhibitor therapy. The early introduction of enteral nutrition or total parenteral nutrition is also essential for esophageal healing. Therefore the endoscopic placement of a nasogastric tube is recommended. Although antiinfective treatment is considered a cornerstone of esophageal perforation management, there is no consensus on the optimal antibiotic regimen and duration of treatment.

Surgery should be performed in all patients who do not meet the criteria for nonsurgical management listed in Table 44.2.³³

If surgery is indicated for a perforation or rupture of the esophagus, patients should be taken to the operating room as soon as possible because slight delays in surgical treatment can increase morbidity and mortality. Mortality of patients treated within 24 hours of esophageal perforation is less than 10% compared with 30% after this time.^22,26,28 Surgical repair of esophageal perforation by a minimally invasive approach, such as laparoscopy or thoracoscopy, should be considered.

The goal of surgical treatment includes a lavage drainage and suture closure of the perforation, and in some cases the formation of a gastrointestinal fistula, depending on the patient’s clinical condition. Surgical therapy is generally divided into transthoracic or transhiatal procedures. To decide on a surgical method, it is extremely important to identify the location of the perforation. Thoracotomy is still mainly performed as the conventional surgical method. However, the number of reports of thoracoscopic procedures has increased recently, and are becoming more common and an effective therapy particularly for Boerhaave syndrome. Another approach to suture the tear is laparoscopic transhiatal simple closure. Laparoscopic transhiatal simple closure of all layers is considered a practical, less invasive surgical procedure for the treatment of esophageal rupture in patients who meet the following conditions: stable general condition, no intrathoracic perforation, and the perforation site is identified by preoperative examination at the lower esophagus. In summary, surgical approaches or conservative methods must be considered for thoracic or abdominal esophageal perforations. If the area of injury is diseased or direct repair of thoracic esophageal perforation is not feasible (hemodynamic instability, delayed surgical exploration, extensive esophageal damage), an emergent esophagectomy may be required, which is a safe option for the treatment of esophageal perforation with a mortality rate comparable to that of elective esophagectomy.^33,35

Achalasia and Esophageal Stricture

Achalasia is a progressive impairment of the autonomic motor function of the esophagus, leading to failure of relaxation of the LES with hypercontractility and loss of peristalsis in the distal esophagus because of a loss of function of the myenteric plexus ganglion cells in the distal esophagus. The degeneration of the myenteric plexus ganglion cells is suspected to be caused either by an autoimmune process or as a consequence of a systemic infectious disease, such as Chagas disease.³⁶ In addition, achalasia is associated with other systemic secondary movement disorders, such as neuromuscular diseases, scleroderma, amyloidosis, and Parkinson disease. Achalasia is a relatively rare disease with a prevalence of 10 cases per 100,000 people and an incidence of about one case per 100,000 people.³⁷

Because of the slow progression and unspecific symptoms of achalasia, patients with achalasia often seek medical advice in advanced stages of the disease. Typical esophageal symptoms are dysphagia, first with solid, later even with liquid foods, heartburn, regurgitation or vomiting, epigastric or chest pain. Other associated signs are unintentional weight loss or history of aspiration, such as chronic cough or asthma, pneumonia, or hoarseness.³⁶

The gold standard for the diagnosis of achalasia is high-resolution manometry with topographic pressure imaging. A barium esophageal is only recommended if a manometry is not conclusive because of its lower sensitivity.³⁸ In addition, endoscopy is recommended in all patients with symptoms of achalasia to exclude other associate diseases.

There is no curative medical therapy for achalasia by various conservative symptomatic therapeutic approaches. Surgical treatment consists of cardiomyotomy and esophagomyotomy (Heller myotomy), which aims to interrupt spastic LES, followed by a partial antireflux procedure (fundoplication). Laparoscopy is the preferred surgical approach because of its lower morbidity and comparable long-term outcome compared with esophagomyotomy via left-sided thoracotomy.³⁹ Peroral endoscopic myotomy is a surgical procedure that is gaining popularity worldwide as a less invasive endoscopic treatment with comparable efficacy to Heller myotomy.⁴⁰ The procedure consists of creating a submucous tunnel from the middle esophagus to the proximal stomach, followed by total or partial myotomy of the muscle without an accompanying antireflux procedure (Fig. 44.3).⁴¹ Therefore in patients at high risk of GERD, a Heller myotomy may be preferred after the procedure,³⁸ but the evidence is mixed.⁴² Because of the high risk of complications, esophagectomy should only be considered in the case of end-stage achalasia if all other treatments were permanently ineffective.⁴³

Gastroesophageal Reflux Disease and Hiatal Hernia

The transition between esophagus and stomach is formed by the LES, the diaphragm, the His angle, the Gubaroff valve (mucosal fold at the entrance to the stomach) and the phreno-esophageal membrane and acts as a barrier.⁴⁴ A malfunction of any of these barriers may lead to a reflux of stomach contents into the esophagus. Although some degree of gastroesophageal reflux is normal, the occurrence of frequent or severe symptoms is referred to as GERD. Lifestyle and diet, as well as increased intraabdominal pressure, such as obesity or pregnancy, sensomotoric disturbances, such as impaired esophageal peristalsis or, rarely, excessive secretion of stomach acid (Zollinger-Ellison syndrome) may all promote the symphyses of GERD. The incidence is high in developed countries and affects both the quality of life and socioeconomic aspects. Typical symptoms are heartburn, regurgitation, and dropsy.⁴⁵ Because of the disruption of various antireflux mechanisms, diaphragmatic hernia is a particular risk factor. Particularly large hiatal hernias are often associated with GERD, which is caused by an increased frequency of transient lower esophageal sphincter relaxation (TLESR), lower LES tone, and reduced esophageal motility. Hiatal hernias comprise four anatomic types. Type I, the sliding hiatal hernia, accounts for more than 90% of cases of hiatal hernia, with symmetrical ascent of the stomach through the diaphragmatic crust. They are often associated with severe degrees of esophagitis and Barrett esophagus. Types II–IV are called paraoesophageal hernias with asymmetrical ascent, and their main clinical significance depends on the anatomic deposition of the stomach, which carries the risk of ischemia, obstruction, or volvulus.⁴⁶

The management of GERD follows a step-by-step approach. Besides lifestyle interventions, the main principle is the medical reduction of esophageal luminal acid reflux by local neutralization or by suppressing gastric acid secretion. Surgery is rarely indicated but may be considered in refractory GERD or, if associated, large hiatal hernia.⁴⁷

There are several surgical approaches to treat GERD and associated hiatal hernia. Laparoscopic transabdominal Nissen fundoplication (total fundoplication), which creates a 360-degree wrap of the fundus around the lower esophagus, is the most common surgical procedure for the treatment of GERD.⁴⁸ Partial fundoplication, such as 180-degree fundoplication anterior or 270-degree fundoplication posterior (toupee fundoplication) has been developed to reduce postfundoplication symptoms. In patients with previous abdominal surgery, long disease duration with severe peptic strictures, or extremely large hiatal hernia, transthoracic fundoplication through a left lateral thoracotomy is preferred. In this case, the anesthetist must advance an esophageal dilator through the gastroesophageal junction to bring the stomach into the thorax after mobilization of the distal esophagus by the surgeon. After fundoplication, the dilator is removed and the fundoplication wrap is placed under the diaphragm.

The combination of GERD and hiatal hernia, especially paraoesophageal hernia, implies the need for surgical treatment. The repair of paraoesophageal hernia can be challenging, depending on its size and the abnormalities associated with the organ hernias. The standard procedure is currently hiatal repair and laparoscopic fundoplication.⁴⁶ The combination of this procedure with a collis gastroplasty to lengthen the esophagus contracted by the chronic inflammation to allow tension-free fundoplication may be necessary and is classically performed transthoracically.⁴⁹ In addition, the use of a net for hiatal hernia repair is favored to reduce the recurrence rate of hernia even if the evidence is weak. Robotic surgery is also developing in hiatosurgery, offering improved visualization and optimized ergonomics with low complication rates. Arcerito M et al. evaluated 75 patients (23 males and 47 females), preoperatively diagnosed with a large paraesophageal hiatal hernia, who were treated with a robotic approach. Biosynthetic tissue absorbable mesh was applied for hiatal closure reinforcement. Some 58 patients underwent total fundoplication, 11 patients had partial fundoplication, and one patient had a Collis-Nissen fundoplication for acquired short esophagus. The authors reported that all procedures were completed robotically, without laparoscopic or open conversion. Mean operative time was 223 minutes, mean length of stay was 38 hours. Moderate postoperative dysphagia was noted in eight patients, all of which resolved after 3 months without esophageal dilation. No mesh-related complications were detected. They concluded that robotic surgical treatment of GERD with large paraoesophageal hernias may afford the surgeon increased dexterity and is feasible with comparable outcomes compared with traditional laparoscopic approaches.⁵⁰

Alternatively, endoscopic treatments for GERD have been developed in recent decades, such as transoral incisionless fundoplication or the delivery of radiofrequency energy to the LES. The Stretta procedure is a minimally invasive endoscopic procedure for the treatment of GERD that delivers radiofrequency energy in the form of electromagnetic waves through electrodes at the end of a catheter to the LES and the gastric cardia.⁵¹ The absence of an abdominal incision and short intervention times are the advantages of these new methods, and comparable short-term results have been demonstrated.

Surgical Approaches (Endoscopic Resection, Open Esophagectomy, Hybrid Minimally Invasive Esophagectomy, Total Minimally Invasive Esophagectomy, Robotic Surgery)

Esophagectomy represents the current standard of care for patients with localized esophageal cancer of stage T1sm/N+ or higher.⁶⁷ Other indications include curative resection of high-grade dysplasia and severe nonmalignant diseases, such as esophageal injury, nondilatable stricture, severe recurrent GERD, and achalasia.^68,69 The standard surgical approach to esophagectomy has always been a laparotomy or a combined laparotomy and thoracotomy.⁷⁰ The three classical approaches to perform an open esophagectomy (OE) are (Table 44.4): (1) transhiatal laparotomy; (2) two-incision techniques via laparotomy and right thoracotomy (Ivor Lewis approach); and (3) three-incision techniques via laparotomy, right thoracotomy and a cervical incision for anastomosis (McKeown approach). Today, most centers perform esophagectomies using hybrid or totally minimally invasive techniques—replacing either the laparotomy with a laparoscopy or the thoracotomy with a thoracoscopy (hybrid minimally invasive esophagectomy [HMIE]). The most common surgical procedure today is the combination of laparoscopy to mobilize the stomach and the lower esophagus followed by right thoracoscopy to complete the resection and to carry the intrathoracic anastomosis (total minimally invasive esophagectomy [TMIE]). Robot-assisted minimally invasive thoracolaparoscopic esophagectomy (RAMIE) is also becoming increasingly common (Fig. 44.4).⁷¹

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