In 90% or more of patients with this disease there is oesophageal involvement, with diminished peristalsis detected manometrically or by barium swallow. This is due to replacement of the smooth muscle layers by fibrous tissue. The LOS pressure is also decreased, allowing reflux; mucosal damage may occur as a consequence. Strictures may develop. Initially there are no symptoms, but dysphagia and heartburn occur as the oesophagus becomes severely involved. Similar motility abnormalities may be found in other connective-tissue disorders, particularly if Raynaud’s phenomenon is present. Treatment is as for reflux and stricture formation
Diffuse oesophageal spasm
This is a severe form of abnormal oesophageal motility that can sometimes produce retrosternal chest pain and dysphagia. Swallowing is accompanied by bizarre and marked contractions of the oesophagus without progression of the waves. On barium swallow the appearance may be of a ‘corkscrew’. However, asymptomatic changes in oesophageal motility are not infrequent, particularly in patients over the age of 60 years (presbyoesophagus). Care must therefore be taken that the symptoms, the manometry and X-ray findings of oesophageal spasm are not falsely attributed. A variant of diffuse oesophageal spasm is the nutcracker oesophagus, which is characterized by finding very highamplitude peristalsis (pressures >200 mmHg) within the oesophagus. Chest pain and dysphagia occur.
True oesophageal spasm producing severe symptoms is rare and treatment is often unhelpful. Antispasmodics, nitrates, or calcium channel blockers such as sublingual nifedipine 10 mg three times daily may be tried.Occasionally, balloon dilatation or even myotomy is necessary.
Miscellaneous motility disorders
Abnormalities of motility that are mostly asymptomatic but occasionally produce dysphagia are found in the elderly in diabetes mellitus, myotonica dystrophica and myasthenia gravis, as well as in any neurological disorder involving the brain stem.
OTHER OESOPHAGEAL DISORDERS
This is a pouch lined with epithelium that can produce dysphagia and regurgitation. It is usually asymptomatic and often detected accidentally on a barium swallow performed for other reasons. Diverticula can occur:
• Immediately above the upper oesophageal sphincter (pharyngeal pouch). If large, it may cause dysphagia as well as spillage of contents into the trachea.
• Near the middle of the oesophagus (traction diverticulum produced by extrinsic inflammation).
• Just above the LOS (epiphrenic diverticulum). Only when symptoms are severe should surgery be undertaken. Rings and webs.
A number of rings and webs have been described throughout the oesophagus. Lower oesophageal or Schatzki ring This is a narrowing of the lower end of the oesophagus due to a ridge of mucosa or a fibrous membrane. The ring may be asymptomatic, but it can very occasionally produce dysphagia after swallowing a large bolus of bread or meat. The narrowing or ring is seen on a barium swallow, with the oesophagus well distended with barium. The treatment is reassurance and dietary advice.
Upper oesophageal web
This is a constriction near the upper oesophageal sphincter in the postcricoid region and appears radiologically as a web. The web may be asymptomatic or may produce dysphagia. In the Plummer-Vinson syndrome (Paterson- Brown-Kelly syndrome) this web is associated with ironeficiency anaemia, glossitis and angular stomatitis. This rare syndrome affects mainly women and its aetiology is not understood. At oesophagoscopy the web may be difficult to see. Dilatation of the web is rarely necessary. Iron is given for the iron deficiency.
Benign oesophageal stricture
Peptic stricture secondary to reflux is the commonest cause of benign strictures. They also occur after the ingestion of corrosives, after radiotherapy, after sclerosis of varices and following prolonged nasogastric intubation. All strictures give rise to dysphagia. They are usually treated by dilatation, but occasionally surgery is necessary.
Infection is becoming increasingly recognized as a cause is painful swallowing, particularly in immunosuppressed debilitated patients and patients with AIDS. Infection can can with:
• Hiper simplex
It is occasionally difficult to distinguish between these either on barium swallow or oesophagoscopy, as only widespread ulceration is seen. In candidiasis the characteristic white plaques on top of friable mucosa are frequently found, but oral candidiasis is not always present.
The dianosis is of Candida can be confirmed by examining a direct smear taken at endoscopy, but often infections are mixed and cultures and biopsies must be performed.
Most patients on large doses of immunosuppressive agents are treated prophylactically with nystatin or amphotericin. Other antifungal or antiviral treatment is given appropriately