An esophageal stricture refers to the abnormal narrowing of the esophageal lumen; it often presents as dysphagia commonly described by patients as difficulty swallowing. It is a serious sequela to many different disease processes and underlying etiologies. Its recognition and management should be prompt. Stricture formation can be due to inflammation, fibrosis or neoplasia involving the esophagus and often posing damage to the mucosa and/or submucosa.
The esophagus loses distensibility with stricture formation, and this may be localized or diffuse throughout the length of the esophagus. The luminal stricture itself may have abrupt or tapered margins. Recent advancement in the use of endoscopic procedures for diagnostic as well as therapeutic purposes has increased the occurrence of iatrogenic post-procedural esophageal stricture formation resulting from mucosal injury.
Generally, the term esophageal stricture is reserved for intraluminal esophageal disorders resulting in narrowing, although extrinsic esophageal compression and luminal compromise can sometimes occur, by direct invasion of malignancy or lymph node enlargement for example, and therefore result in esophageal stricture as well. Regardless of etiology, stricture disease is best managed promptly and aggressively to restore luminal patency; this is done for symptomatic improvement and/or palliative management in cases of cancer. New technological advancements in endoscopic therapy and different stent products have shown promising results with notable improvement in stricture management with low recurrence rates and fewer complications.
A stricture is either benign or malignant. Appropriate management depends on identifying the correct etiology for stricture. The majority of esophageal strictures result from benign peptic strictures from long-standing gastroesophageal reflux disease (GERD), which accounts for 70 to 80% of adult cases. Early and preventive use of proton pump inhibitors (PPI) has somewhat decreased the incidence of such peptic strictures. In addition to chronic poorly controlled acid reflux disease, other etiologies for esophageal stricture development exist. In young children and adolescent populations, corrosive substance ingestion is the leading cause of stricture formation in the esophagus. The following classification and list of common and uncommon causes for stricture formation in the esophagus can guide physicians in their approach to management:
Esophageal stricture formation is not common. There is an overall low disease prevalence for the condition. One study reported an incidence rate of 1.1 per 10000 person-years, which also increases with age. Peptic strictures, being the most common among them, have decreased in incidence from 1994 to 2000 along with a substantial increase in PPI use during this time. A history of GERD, hiatal hernia, prior dysphagia, peptic ulcer disease, and use of alcohol are known risk factors for peptic stricture formation.
Esophageal strictures can occur in any age group or population when one considers all the different possible etiologies. Strictures due to caustic esophagitis or eosinophilic esophagitis, however, are more common in children and young patients. Strictures related to acid reflux, iatrogenic or drug-induced esophagitis, on the other hand, are more common in adults. Malignant strictures are found in older people, as cancer prevalence is higher in older populations.
Peptic strictures are tenfold more common in Whites than Blacks or Asians. There is no clear association between sex genotype and esophageal stricture, but men are at higher risk than women for erosive esophagitis.
The normal esophagus measures up to 30 mm in diameter. A stricture can narrow this down to 13 mm or less, causing dysphagia. The pathophysiology of stricture development differs based on the underlying etiology, but the basic pathological changes include damage to the mucosal lining. Over time, this leads to chronic inflammatory changes in the wall of the esophagus. Chronic esophagitis progresses even further with subsequent development of intramural fibrosis and scarring, leading to luminal constriction.
In peptic stricture, these pathophysiological changes happen as a result of exposure of the esophageal mucosa to refluxed acid-peptic content from the stomach. This reflux can exacerbate with the weakening of lower esophageal sphincter or impaired esophageal motility or both. Hiatal hernia contributes in a significant way as an independent risk factor in the pathophysiology of peptic strictures, given that hiatal hernia presents in 85% of patients with gastroesophageal reflux disease with a stricture. Delayed gastric emptying and excessive pepsin secretion could contribute to the process, but these suggested factors lack good evidence for confirmation.
Similarly, most of all other benign strictures result from chronic, long-standing esophagitis secondary to different causes as described in the section on etiology above.
Malignant stricture develops from intrinsic direct proliferation and invasion of cancer cells from the luminal mucosa. Adenocarcinoma commonly arises from the lower part of the esophagus, and squamous cell carcinoma frequently occurs in the middle and upper part of the esophagus. Very rarely, the direct growth of lung tumor mass or mediastinal lymph node enlargement can also produce stricture.
Regardless of the nature of the stricture, patients typically present with one or all of the following symptoms: dysphagia, food impaction, odynophagia, chest pain, and weight loss. The most relevant symptom is progressive dysphagia to solid food, and this sometimes progresses to involve semisolid and liquid foods. The rate and type of symptom progression correlate with the underlying type of stricture. Physical examination findings are usually not significant in these patients.
Benign stricture follows a slow and insidious course, while malignant stricture develops rapidly. Sometimes dysphagia is combined with pain in the presence of acute esophagitis. Food impaction requires instant recognition and prompt management to avoid severe complications like aspiration or perforation. Patients presenting with such clinical symptoms have an underlying stricture in about 45% of cases diagnosed at endoscopy.
A careful clinical evaluation, including a directed history and physical examination, can suggest information about the underlying cause of a suspected or endoscopically diagnosed stricture. The physician should gather information about dysphagia pertaining to its nature, duration, onset, severity, and associated symptoms such as heartburn, vomiting, pain while swallowing, any upper respiratory symptoms or chest pain. Sometimes the patient may report having water brash, morning soreness of throat or asthma-like wheezing, which may be due to severe regurgitation. In the case of peptic stricture, weight loss is uncommon, and good appetite is usually present. Weight loss and anorexia along with long-standing weakness are more associated with malignant strictures or refractory strictures. The following historical information can also help understand the cause of stricture formation and guide management:
Once a thorough medical history and bedside evaluation are complete, and there is suspicion of esophageal stricture, the next best investigation would be an esophagogastroduodenoscopy or contrast-enhanced esophagogram. Both are principal diagnostic modalities for esophageal stricture. Depending on the severity of dysphagia and the presence of other clinical symptoms, an X-ray of the chest (PA and lateral views) may be acquired to assess for problems such as foreign body impaction or diaphragmatic hernia and rule out some other pulmonary conditions. Sometimes a CT scan is more helpful in patients with a history of caustic substance ingestion to rule of esophageal perforation. However, X-ray and CT imaging are not necessary in routine cases. CT is helpful in a patient who is found to have a malignant stricture on biopsy and helps with disease staging.
The majority of the patients are evaluated by endoscopy since it can provide overall information on esophageal anatomy and establish not just the diagnosis of a stricture but also allow for biopsy of the mucosa. Endoscopy affords an opportunity for therapeutic dilation of the stricture when indicated. Contrast fluoroscopy is only for those patients who have a complex stricture or when endoscopy is incomplete due to excessive narrowing of the lumen. Choosing a water-soluble contrast agent for first pass viewing is advised here to avoid inspissation of heavy agents such as barium and thereby minimizing the risk for obstruction and/or aspiration.
Upper GI endoscopy is the most important diagnostic and therapeutic intervention in the case of a stricture. After the presence of a stricture is confirmed, the most important step is to biopsy the stricture to rule out malignancy. Differentiation of benign stricture from malignant stricture is absolutely necessary to guide further management approaches. Endoscopy not only allows for the biopsy, but it also visualizes the area around the stricture for any mass or lesions. Benign esophageal strictures classify as simple or complex strictures based on the size, involved area, surface, luminal narrowing, and margins. Simple strictures are usually under 2 cm in size, straight and allow easy passage for the endoscope. Complex strictures, on the other hand, are typically longer than 2 cm, have an uneven surface, tortious margins, and a narrow diameter. Complex strictures are difficult to manage and require additional fluoroscopy or advanced thin-caliber endoscopes for further assessment.
Barium Contrast Swallow:
It can show the abnormality in the esophagus and provide an understanding of level, size, extent, and severity of strictures, especially when standard endoscopes cannot pass through the stricture and thin-caliber scopes are not available. Barium contrast swallows are found to have 95% sensitivity for diagnosing esophageal stricture. The radiographic appearance of a stricture differs based on the underlying etiology. Water-soluble contrast is used in patients with suspected perforation or to get a first image before passing barium. Currently, barium contrast fluoroscopy is the recommended first-line investigation in patients with suspected complex strictures such as patients having a history of radiation therapy or a history of caustic substance ingestion.
Endoscopic ultrasound (EUS) can provide high-resolution images of the esophageal wall, and it can provide detailed information about the extent of the esophageal injury in other benign causes of stricture. Sometimes multiple biopsy specimens of stricture are non-conclusive. These cases can also have normal mucosa with CT also showing only wall thickening. They pose difficult challenges for diagnosis. In such cases, EUS can provide critical information. Patients with malignant esophageal strictures have a thicker esophageal wall on EUS, with a loss of wall stratification compared to patients with benign esophageal strictures, who demonstrate preservation of wall stratification more frequently. With EUS, suspected malignancy cases could benefit from identification and elective surgical resection, rather than offering resection to all cases including indeterminate ones.
Any stricture requires treatment to establish adequate luminal patency. Various methods and instruments are used to achieve this goal. Treatments include the use of dilators, stent placement, surgical resection, and medical management. The technique most utilized for benign stricture management is endoscopic dilation using a bougie or a balloon dilator. The main objective is to improve symptoms, mainly in relieving patients of dysphagia. In clinical practice, treatment outcome is evaluated by a dysphagia scoring system. Ogilvie et al. first introduced such a scoring system, but they applied it in the context of esophageal malignancy palliated with stent placement to relieve dysphagia. However, it has application to almost all types of benign and malignant stricture management situations. The following describes the clinical dysphagia scoring system:
Upon establishing the type of stricture, the dilation management plan involves the following considerations:
Benign strictures commonly receive treatment with endoscopic dilators followed by disease-specific management approaches to treat the underlying inflammatory process.
Esophageal Stricture Dilation:
There are currently two main types of dilators in use in clinical practice. Each has its own advantage and disadvantage.
Stricture dilation is an ambulatory outpatient procedure that requires certain levels of expertise from an endoscopist. Appropriate selection of dilator is determined usually based on the complexity, size, and site of the stricture. A lower esophageal stricture is commonly peptic in nature; due to their simple characteristic and small size, mechanical dilators are safe and effective in treating them. Complex strictures tend to undergo management with balloon dilators.
First, the size of the dilator that is going to be used is estimated endoscopically by assessing the diameter of the stricture area. The first dilation performed should be the same size as the stricture. It is advanced in increments. No excessive force is used. The majority of surgeons or endoscopists follow the “rule of three,” performing up to three dilations per session while successively increasing the diameter of the dilator by 2 mm (6Fr). Use of fluoroscopy is controversial, but can certainly be helpful in complex strictures. It can play a contributory role based on the endoscopist’s experience.
Use of Adjunctive Methods:
At present two main adjunctive treatment methods are employed based on preference: Intra-lesion injection of steroid, or oral steroid gel use and endoscopic stricturoplasty. Steroids help in decreasing the inflammation related to injury from dilation and, hence, reduce the chance of restenosis. However, long-term data are needed to establish its standard use. Other studies have shown better outcomes based on the lower recurrence of stenosis and the achievement of larger diameter patency. Four-quadrant stricturoplasty can be one option to consider in highly fibrotic strictures.
Long-term success with dilation can often be challenging to achieve in all cases of the stricture. Unfavorable outcomes are more common in strictures from corrosive injury. Dilation is successful in only about 25% of such cases. Successful outcomes here refer to the ability to swallow solid food without intervention for 6 months after the first procedure.
The major problem one faces in stricture management is a recurrence. A stricture is recurrent when there is an inability to maintain a satisfactory luminal diameter for 4 weeks after achieving the target diameter of 14 mm. A stricture is refractory when there remains a persistent dysphagia score of 2 or more, as a result of an inability to successfully achieve a diameter of 14 mm over five sessions of dilation done at 2-week intervals.
Stents are often reserved for malignant stricture and refractory benign strictures. The goal of stent placement is to hold the stricture open for prolonged periods, causing the stricture, or the tissue around it, to remodel so that the stricture does not recur after stent removal. In malignant stricture, this could be either used for complete palliation in case of advanced cancer or temporary palliation in cases of ongoing neoadjuvant treatment.
Stents are the breakthrough inventions considering the extent of benefits they can provide in terms of symptom improvement and quality of life enhancement, especially in patients who are suffering from terminal cancer. Over the years, esophageal stents have evolved from rigid plastic conduits to self-expanding metal stents (SEMS). Improvements continued to address the disadvantages of previous models. This trend is notable with SEMS, where initially non-covered stents were the initial offering. Later, partially and then fully covered stents were developed to correct the issue of epithelialization and tumor growth in the previous older uncovered SEMS, allowing feasibility of stent removal. Covered stents, however, show a higher displacement rate (20%) compared to there metal counterparts. To resolve this, biodegradable stents and suture fixation techniques are under clinical evaluation, and initial evidence shows promising outcomes. Biodegradable stents show superiority in outcomes and symptomatic improvements in patients with a corrosive esophageal stricture.
Benefits, safety, and feasibility of different stents have undergone comparison in various clinical and randomized controlled trials. Currently, no clear outcome benefit is apparent between the use of partial vs. full covered SEMS for palliative management of malignant strictures in regards to recurrent obstruction and symptomatic success (the COPAC Study). The FDA has approved the use of SEPS (Self-expanding plastic stents) for the indication of benign esophageal stricture.
Surgical resection is reserved for malignant disease-causing esophageal stricture or benign conditions recalcitrant to less aggressive forms of medical and/or endoscopic therapy. When surgery is necessary for benign refractory peptic strictures, an antireflux procedure is selectively done to prevent further stenosis. Extensive surgery may be necessary in cases of malignant stricture, where concurrent removal of a mass also takes place if staging is favorable. In such cases, partial or complete esophagectomy, with gastric tube pull-up or bowel loop interposition and anastomosis is performed. Otherwise, palliative surgical approaches are considered to relieve symptoms or obstruction and to provide a route for enteral nutrition distal to a stricture, usually via gastrostomy tube placement.
In the workup and management of suspected esophageal stricture disease, it is prudent to assess for the concomitant presence of esophageal motility disorders that may influence management. The following conditions are important to consider:
Esophageal stricture develops over time, and prognosis depends on the timing of evaluation and management as well as the underlying cause of stricture. While esophageal dilation is the first line management in cases of benign esophageal stricture, regardless of the underlying cause, it poses a 10 to 30% chance of re-stenosis. Stricture recurrence is the primary concern resulting in added risks and costs. Peptic stricture shows excellent prognosis when treated promptly with endoscopic dilation and long-term PPI therapy. Surgical correction of a hiatal hernia, if present, produces excellent results with minimal risk for re-stenosis and significant symptomatic improvement. To improve prognosis in terms of decreasing stricture recurrence, concurrent steroid injection therapy or steroid pill therapy have been used and are showing promising clinical outcomes. Stent placement is kept reserved for benign stricture cases where repeated dilation is not adequate and symptomatic control is poor. In the case of malignant stricture, the prognosis depends on the cancer type, tumor invasion, and disease stage. Surgical resection demonstrates better prognosis for cancer, which hasn’t yet invaded lymph nodes and surrounding tissue. When stricture management is with stent placement for palliation, the prognosis is almost always poor.
Untreated esophageal stricture related complications:
Stricture is a complicating disease process and thus requires a thorough understanding and cooperation from patients for adequate management. The patient should know the potential complications which could happen during the stricture treatment since they could be more severe involving esophageal perforation and bleeding. Patients should receive education about the long term management of the underlying disease process and chances of recurrence and regarding seeking immediate medical attention when symptoms like dysphagia, regurgitation recur. Patients should receive immediate attention when they are diagnosed with any of the etiologies which could cause stricture, about preventive therapies to decrease the chances of developing such severe complications.
Etiology behind stricture formation can be managed with appropriate treatment modalities to prevent future stricture formation as well as the progression of the pathology. Peptic strictures are best managed with long term PPI therapy. Eosinophilic esophagitis should undergo a trial of a PPI, as the clinical response to the PPI trial indicates GERD associated eosinophilia rather than eosinophilic esophagitis. If repeat endoscopy still shows eosinophilia, they are diagnosed as true eosinophilic esophagitis and best treated medically with vaporized glucocorticoids such as fluticasone or budesonide. Pill-induced esophageal stricture requires proper measures in regards to pill ingestion. Patients should be advised to take plenty of water while taking pills, take one pill at a time and avoid lying down for 30 min after pill ingestion. Such measures help in preventing further damage to mucosa and recurrence of stricture.
Esophageal stricture is an emergency condition, which requires prompt recognition and management by health care providers in the emergency room or clinic setting. Outcomes are enhanced when primary care teams recognize concerning signs and symptoms and take immediate measures to minimize complications for patients with partial or complete obstruction of the esophagus. Being selective with the diagnostic workup is essential, and appropriate, timely referrals to an endoscopist merit consideration. A solid clinical knowledge base and endoscopic skill level are needed for the endoscopist to successfully diagnose and treat patients who present with dysphagia or other esophageal related symptoms. In addition to early treatment, stricture requires long-term clinical observation and management to prevent a recurrence. Nursing personnel and pharmacists familiar with the etiologies for esophageal stricture formation can help deliver appropriate treatment and guide patients to seek specialty consultations when needed. Their knowledge of medications that could potentially cause esophagitis and stricture is important in assisting them in modifying care and suggesting preventive measures such as drinking plenty of water and staying upright for a minimum amount of time when taking pills orally. Malignant stricture requires a team approach to management with involvement of medical, radiation and surgical oncologists in addition to GI consultants, nutritional specialists, and hospice nursing staff. It is important to recognize that most benign strictures should have initial treatment with endoscopic dilation. If refractory or malignant, they can be candidates for stent placement for palliative relief in individual cases. All members of the interprofessional healthcare team (physicians, specialists, specialty-trained nurses, and pharmacists) need to communicate across disciplines to achieve optimal clinical outcomes. [Level V]
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