Narrowing of the oesophagus can be due to either stricture formation (benign or malignant), webs (mucosa and submucosa only), and rings (mucosa, submucosa and muscle), or from external compression from other structures in the neck or mediastinum.
Most oesophageal narrowings will present with dysphagia or symptoms suggestive of heartburn or indigestion, although some are found incidentally at endoscopy in patients with pathology elsewhere in the upper gastrointestinal (GI) tract.
Dysphagia is a 'red flag' warning sign that should be referred urgently for investigation, either by admission to hospital if severe, or as an outpatient under the 2-week rule (usually for upper GI endoscopy ± barium swallow) - see separate Dysphagia article.
In addition to webs, rings and strictures there are abnormalities of peristalsis, such as achalasia of the cardia that may cause dysphagia.
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- Presenting features include heartburn, dysphagia, impaction of food, weight loss, and chest pain. Less common presentations are persistent cough and wheeze due to aspiration of food or acid.
- Worsening strictures may cause progressive dysphagia - from hard food, such as meat, to sloppy food like porridge, to liquids - so ask about progression of symptoms and the time span.
- The degree of dysphagia may be more related to the degree of oesophagitis than the extent of narrowing, and correlation is rather poor.
- There may be a history of gastro-oesophageal reflux disease (GORD). This does not necessarily indicate a benign peptic structure, as Barrett's oesophagus may progress to adenocarcinoma.
Assess the nutritional status of the patient, examine the abdomen, noting any tenderness, organomegaly or lymphadenopathy (supraclavicular).
Thyroid disease, rheumatoid arthritis, graft-versus-host disease, Stevens-Johnson syndrome, psoriasis, blistering skin diseases, and pernicious anaemia.
- FBC and ferritin - may show evidence of chronic blood loss with iron deficiency anaemia, non-anaemic iron deficiency or even poor nutrition with iron and folate deficiency.
- Abnormal LFTs suggest metastasis to the liver.
- CXR may show a mass in the chest, impinging on the oesophagus. The gross dilatation of achalasia is characteristic. Lung disease due to inhalation may be seen.
- Endoscopy and barium swallow may both delineate the lesion but both have advantages and disadvantages:
- Endoscopy allows the lesion to be biopsied but it is also very easy to perforate the oesophagus, especially if the lesion is malignant.
- A barium swallow is usually required to clarify the nature and length of stricture before attempting to pass the scope through the stricture. It may be appropriate to arrange an urgent barium swallow before endoscopy, where dysphagia and weight loss are prominent.
- CT or endoscopic ultrasound are good ways to stage tumours and detect metastases.
- Oesophageal strictures may be benign or malignant:
- Benign oesophageal strictures are usually the result of scarring from acid reflux in severe and persistent gastro-oesophageal reflux disease (GORD). This represents about 70 to 80% of all oesophageal strictures and is 2 or 3 times more common in men. It may also follow ingestion of corrosives. About 25% of patients with peptic stricture give no history of heartburn.
- Postoperative strictures represent about 10% and corrosives account for fewer than 5%. Drugs that can cause strictures include alendronate, iron, non-steroidal anti-inflammatory drugs (NSAIDs), and potassium chloride. NSAIDs should be prescribed with caution in patients with known GORD.
- Malignant oesophageal strictures usually result from carcinoma of oesophagus but may ascend from carcinoma of stomach.
- Oesophageal webs are often about 2 or 3 mm wide. It is a smooth extension of normal oesophageal tissue, containing just mucosa and submucosa, and can occur anywhere along the oesophagus but, classically, at the anterior postcricoid area of the upper oesophagus. This is the Paterson Brown-Kelly syndrome, also known, in the USA, as Plummer-Vinson syndrome. It is associated with iron deficiency. There may be koilonychia (spoon nails), cheilosis and glossitis. Webs are more frequent in women and this may be related to propensity for iron deficiency. Most upper oesophageal webs are not associated with Paterson Brown-Kelly syndrome.
- Oesophageal rings are concentric, smooth, thin extension of normal oesophageal tissue, usually 3 to 5 mm thick. They consist of mucosa, submucosa and muscle. They may be an incidental finding at barium studies or endoscopy. Incidence is unknown as most are asymptomatic. There is no sex difference in the incidence of rings except that multiple rings are usually found in young men. Rings are classified as A, B and C:
- A is uncommon and is a muscular ring several centimetres proximal to the squamocolumnar junction. It may be an inconstant finding on barium swallow and there is some debate as to whether it really is an anatomical entity.
- B ring or Schatzki's ring is really a web, as it involves only mucosa and submucosa. It tends to mark the proximal part of a hiatus hernia and usually presents in a patient aged over 50 whose main complaint is intermittent dysphagia to solid food, spanning months or years, and it is non-progressive.
- C ring is a rare X-ray finding of indentation caused by the diaphragmatic crura. It rarely causes symptoms.
- Eosinophilic oesophagitis is a recently described condition that is due to allergy. The symptoms resemble GORD and even barium swallow may suggest that diagnosis. Correct diagnosis is important as the treatment is different.
- Extrinsic lesions may compress the oesophagus from outside. It is usually a thoracic aortic aneurysm or carcinoma of lung. Other possible causes are a retrosternal thyroid or enlarged hilar lymph nodes (as in bilateral hilar lymphadenopathy of sarcoidosis, tuberculosis or lymphoma).
Failure of peristalsis occurs in achalasia and may present with dysphagia.
Urgent referralDysphagia is one of the features that may indicate malignancy, and demands urgent referral.
Other worrying features include:
- Rapidly progressive symptoms
- Weight loss
- Iron deficiency anaemia
- Abdominal mass
- Benign strictures or rings are managed by oesophageal dilation at endoscopy. This may be achieved under local anaesthetic spray or light sedation. Usually, an inflatable balloon or bougie is passed down a guidewire. Long-term use of proton pump inhibitors (PPIs) seems beneficial in reducing the frequency of repeated dilatations.
Those who need frequent dilatation initially are likely to continue with this pattern. They are more likely to suffer weight loss and less likely to suffer heartburn. Poor results will require consultation with a surgeon.
- Malignant strictures will require either surgical excision (oesophagectomy) or palliative management with an oesophageal stent (Atkinson's tube or similar).
- In Paterson Brown-Kelly syndrome, merely correcting iron deficiency may reverse symptoms.
- Eosinophilic oesophagitis will respond to topical steroids.
- Aspiration pneumonitis.
- Complete obstruction of food can occur. This requires removal at endoscopy. Barium studies must not be performed if this is suspected.
- Severe dysphagia can lead to malnutrition.
- Dilatation can cause bleeding or perforation, although spontaneous perforation of webs or rings is rare.
- Paterson Brown-Kelly syndrome is associated with risk of malignant change (post-cricoid carcinoma) but it seems that correction of the iron deficiency reverses both the disease and the risk. In Northern Sweden, where the risk in women has been traditionally very high, correction of deficiency has had a beneficial effect on both Paterson Brown-Kelly syndrome and post-cricoid carcinoma.
Further reading & references
- Zervos X et al; Esophageal Webs and Rings, eMedicine, Nov 2008
- Dakkak M, Hoare RC, Maslin SC, et al; Oesophagitis is as important as oesophageal stricture diameter in determining dysphagia. Gut. 1993 Feb;34(2):152-5.
- Heller SR, Fellows IW, Ogilvie AL, et al; Non-steroidal anti-inflammatory drugs and benign oesophageal stricture. Br Med J (Clin Res Ed). 1982 Jul 17;285(6336):167-8.
- Nosher JL, Campbel WL, Seaman WB; The clinical significance of cervical esophageal and hypopharyngeal webs. Radiology. 1975 Oct;117(1):45-7.
- Katzka DA; Eosinophilic esophagitis. Curr Opin Gastroenterol. 2006 Jul;22(4):429-32.
- Dyspepsia: Managing dyspepsia in adults in primary care, NICE Clinical Guideline (2004)
- Gastrointestinal (upper) cancer - suspected, Clinical Knowledge Summaries (2005)
- Agnew SR, Pandya SP, Reynolds RP, et al; Predictors for frequent esophageal dilations of benign peptic strictures. Dig Dis Sci. 1996 May;41(5):931-6.
- Bredenkamp JK, Castro DJ, Mickel RA; Importance of iron repletion in the management of Plummer-Vinson syndrome. Ann Otol Rhinol Laryngol. 1990 Jan;99(1):51-4.
- Arora AS, Perrault J, Smyrk TC; Topical corticosteroid treatment of dysphagia due to eosinophilic esophagitis in adults. Mayo Clin Proc. 2003 Jul;78(7):830-5.
- Larsson LG, Sandstrom A, Westling P; Relationship of Plummer-Vinson disease to cancer of the upper alimentary tract in Sweden. Cancer Res. 1975 Nov;35(11 Pt. 2):3308-16.
|Original Author: Dr Hayley Willacy, Dr Paul Scott||Current Version: Dr Huw Thomas|
|Last Checked: 18/03/2011||Document ID: 2537 Version: 23||© EMIS|
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