CAG PRACTICE GUIDELINES

Canadian Association of Practice Guidelines: Evaluation of

Alan W Cockeram MD FRCPC

DEFINITION Dysphagia may be defined as difficulty in swallowing. SPONSORS AND VALIDATION Dysphagia may be classified as oropharyngeal or esophag- eal; arises from a structural or This practice guideline was developed by Dr Alan W functional abnormality in the oropharynx, and esophageal Cockeram MD FRCPC and was reviewed by dysphagia occurs as a result of structural or functional ab- · normalities in the . Practice Affairs Committee (Chair – may be further subclassified Dr A Cockeram): Dr T Devlin, Dr J McHattie, symptomatically as dysphagia for solids alone, which usu- Dr D Petrunia, Dr E Semlacher and Dr V Sharma ally suggests a mechanical problem, versus dysphagia for · Canadian Association of Gastroenterology (CAG) liquids and solids, which is more suggestive of a neuromus- Endoscopy Committee (Chair – Dr A Barkun) cular problem. Dysphagia may be described by the patient as a sensation of food ‘sticking’ or as a sensation of food · Dr N Diamant, Dr N Marcon and Dr W Paterson passing slowly through the esophagus. True dysphagia al- ways indicates organic disease and always warrants inves- · CAG Governing Board tigation and consultation if no cause is found in initial studies. These symptoms should be distinguished from those of a persistent foreign body-type sensation or a sen- reaches the lower esophageal sphincter, the sphincter re- sation of a lump, which is more typical of globus sensation. laxes to allow the food to progress into the stomach. Odynophagia, defined as pain with swallowing, may occur in association with esophageal dysmotility or as a result of OROPHARYNGEAL DYSPHAGIA mucosal disease in the esophagus. The majority of cases of oropharyngeal dysphagia occur as a result of neuromuscular degeneration or damage such as PHYSIOLOGY OF SWALLOWING a cerebral vascular accident. In addition to strokes, degen- Normal swallowing is accomplished by a complex series of erative diseases such as amyotrophic lateral sclerosis, my- interconnected events. After mechanical crushing of a asthenia gravis and Parkinson’s disease are the most food bolus in the mouth, the tongue forces the food bolus common neuromuscular causes. Anatomical causes are to the posterior oropharynx. The upper esophageal sphinc- less frequent but include Zenker’s diverticulum, proximal ter relaxes, allowing the food bolus to enter the proximal esophageal webs, strictures, tumour and, less commonly, esophagus. The food bolus is then propelled down the vertebral osteophytes. A more complete listing of causes is esophagus by peristaltic contraction. As the food bolus included in Table 1 (1). Patients with oropharyngeal dysphagia typically de-

Hilyard Place, St John, New Brunswick Correspondence: Dr Alan Cockeram, Hilyard Place, Building A, 270–560 Main Street, St John, New Brunswick E2K 1J5. Telephone 506-634-7742, fax 506-632-1107, e-mail [email protected]

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TABLE 1 scribe difficulty in initiating swallowing. Frequently this Selected causes of oropharyngeal dysphagia may be associated with regurgitation or symptoms of aspi- Structural lesions ration. Change in speech is often associated. All of these Intrinsic pharyngoesophageal lesions latter features suggest neuromuscular degeneration, par- Oropharyngeal carcinoma ticularly when the symptoms are progressive. Other neu- rological manifestations should be sought. Proximal esophageal carcinoma Benign esophageal tumour ESOPHAGEAL DYSPHAGIA Esophageal web Patients with esophageal dysphagia typically complain of Zenker’s diverticulum dysphagia for solids and/or liquids. Gastroesophageal re- High esophageal stricture flux is a frequent cause of esophageal stricture. The major- Inflammatory disease (eg, , tonsillar abscess) ity of patients with stricturing secondary to reflux have a history of ; however, this may be absent or re- Postsurgical change mote in older patients and in those with Barrett’s esopha- Foreign body gus. may indicate reflux with or without Extrinsic lesions gastroparesis. Typically dysphagia from reflux is slowly Thyroid enlargement or tumour progressive. Intermittent dysphagia for solids suggests a Vertebral spur Schatzki’s ring. Esophageal or proximal gastric tumours Cervical lymphadenopathy often produce rapidly progressive dysphagia for solids in Vascular anomalies the absence of a history of any significant heartburn. Sig- nificant weight loss favours a neoplastic cause although Neuromuscular disease weight loss may occur with esophageal dysmotility, usually Central nervous system diseases at a slower pace. Localization by the patient of the site of Cerebrovascular accident dysphagia in the lower retrosternal area usually accurately Parkinson’s disease indicates a more distal esophageal site of obstruction. More Brain stem tumour proximal localization of the site of dysphagia does not pre- Amyotrophic lateral sclerosis and other motor neuron diseases dict the true site of obstruction as accurately (2). Dysphagia for liquids as well as solids is more suggestive Congenital and degenerative disorders of an esophageal motility disorder but solid-only dysphagia Huntington’s chorea may also occur with motor disorders, often in a sporadic Multiple sclerosis pattern (versus the more predictable pattern noted with Tabes dorsalis mechanical causes). Patients with dysmotility may note Poliomyelitis aggravation of dysphagia with colder foods. Odynophagia Cranial nerve diseases may occur with esophageal dysmotility. A history of heart- Diabetes mellitus burn in addition to dysphagia for both solids and liquids may suggest reflux-induced dysmotility, or a disease pro- Recurrent laryngeal nerve palsy (eg, mediastinal tumour, postsurgical onset) cess such as scleroderma with reflux superimposed upon esophageal dysmotility. Transection or injury An alternative approach is to classify motility disorders Skeletal muscle diseases as hypomotile versus spastic. Hypomotile disorders with Inflammatory myopathies decreased activity in the body and/or sphincter include Polymyositis/dermatomyositis scleroderma and other collagen vascular disorders. These Scleroderma and mixed connective tissue disease often result in gastroesophageal reflux. Spastic motor dis- Inclusion body myositis orders with increased contraction in the body and/or Muscular dystrophies sphincter include achalasia and other motor disorders listed in the primary category in Table 2 (3). These may Oculopharyngeal muscular dystrophy result in functional obstruction leading to dysphagia Myotonia dystrophica and/or pain. Other muscular disorders Thyroid disease CLINICAL EVALUATION Cricopharyngeal dysfunction Patient history is critically important in evaluating dys- Other neuromuscular disorders phagia (4). The pertinent details of the history are alluded Myasthenia gravis to above. Historical features that should be sought include the following: Amyloidosis · Botulism difficulty in initiating versus completing swallowing; · Modified from reference 1 timing of symptoms after initiating swallowing;

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TABLE 2 Etiologies of esophageal dysphagia Dysphagia Neuromuscular (motility) disorders Difficulty initiating swallows Food stops or ‘sticks’ after Primary (includes coughing, choking swallowing Achalasia and nasal regurgitation) Other primary motility disorders OROPHARYNGEAL ESOPHAGEAL Diffuse esophageal spasm DYSPHAGIA DYSPHAGIA Nutcracker esophagus (hypertensive peristalsis)

Hypertensive lower esophageal sphincter Solid food only Solid or liquid food Nonspecific esophageal dysmotility Mechanical obstruction Neuromuscular disorder Secondary Motor disorder secondary to reflux Intermittent Progressive Intermittent Progressive Scleroderma Other collagen disorders Chagas’ disease Chronic heartburn Chronic heartburn Mechanical lesions – intrinsic No weight loss Most common Peptic stricture Bread/ Age >50 Chest Bland regurgitation Lower esophageal (Schatzki’s) ring steak Weight loss pain Weight loss Carcinoma Other Lower Peptic Carcinoma Diffuse Sclero- Achalasia Esophageal webs esophageal stricture esophageal derma Esophageal diverticula (often with an associated esophageal ring spasm motor disorder) Benign tumours Figure 1) Algorithm for symptomatic assessment of the patient with dyspepsia. Important differentiating symptoms are within Foreign bodies boxes. Modified and reproduced with permission from reference 5 Medication-induced injury Mechanical lesions – extrinsic Vascular compression cranial nerve lesions or other more generalized motor ab- Mediastinal abnormalities normalities, eg, weakness or gait disturbance. The chest Cervical osteoarthritis should be examined to rule out the possibility of aspira- Modified from reference 3 tion pneumonia. The patient with esophageal dysphagia should be care- · other neurological symptoms, ie, voice change, fully examined for any cervical lymphadenopathy, organo- , aspiration and/or weakness; megaly, and evidence of weight loss, all of which may suggest a neoplasm. Skin lesions such as telang- · dysphagia for solids versus liquids; iectasia, sclerodactyly or calcinosis may occur in associa- · intermittent versus progressive dysphagia; tion with scleroderma and esophageal dysmotility. · sporadic versus predictable dysphagia; Occasionally a succussion splash may be heard in the chest in patients with achalasia. · history of remote or recent heartburn; · weight loss; APPROACH TO INVESTIGATION · odynophagia; The screening procedures of choice classically have been an esophagram and upper gastrointestinal series, and many · foreign body sensation; and family physicians follow this route. However, upper gas- · prior history of any caustic ingestion. trointestinal series can miss significant lesions, especially Figure 1 provides a diagnostic algorithm for symptom- in early disease and particularly if the esophageal lumen is based diagnosis (5). not well distended during filming. Specialized techniques may improve the sensitivity of the examination. Video PHYSICAL EXAMINATION studies may be more useful in evaluating esophageal dys- In patients with oropharyngeal dysphagia, the physician motility, particularly in cases of oropharyngeal dysphagia should make particular note of other neurological manifes- where the x-ray is truly the only way to evaluate this area. tations, such as abnormal speech pattern, drooling, other Slow motion viewing may help to identify more minor dis-

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TABLE 3 the two are complementary and both are operator- Manometric features of primary esophageal motility dependent. disorders If patient history or barium studies suggest esophageal Diagnosis Required features Sometimes present dysmotility one may choose to proceed directly with eso- Achalasia Esophageal body – Incomplete LES relaxation phageal manometry, but in most cases it is preferable to lack of peristalsis Elevated LES pressure proceed with endoscopy before manometry to ensure that (>45 mmHg) no mechanical lesion is present. In some cases dysmotility Elevated intraesophageal may be related to unsuspected reflux esophagitis. Rarely pressure esophageal dysmotility may arise as a result of lesions in Nutcracker Increased amplitude Increased duration (>6 s) the cardia which may be poorly identified by upper gastro- esophagus (>180 mmHg) with normal peristalsis intestinal series. After mechanical obstruction has been excluded one may then proceed with esophageal ma- Diffuse Simultaneous Repetitive contractions esophageal contractions Spontaneous contractions nometry. Several recording systems are available; discus- spasm (>10% wet Increased duration(>6 s) sion of the relative merits of each is outside the scope of swallows) and/or amplitude this Practice Guideline. The typical manometric features Intermittent normal (>200 mmHg) of the major causes of motility disturbances of the esopha- peristalsis gus are outlined in Table 3. Hypertensive Elevated LES pressure LES (45 mmHg) THERAPY Normal LES relaxation Normal peristalsis The outcome of the diagnostic procedures dictates the therapeutic approach. Nonspecific Any combination of Nonperistaltic contractions esophageal features in Prolonged contractions Patients with oropharyngeal dysphagia may be helped motility ‘Sometimes present’ (>6 s) by consultations with speech pathologists and dieticians disorder column Multiphasic contractions and by dietary changes including predominantly soft, pu- Retrograde contractions réed or jellied foods and avoidance of liquids that may be Low amplitude peristalsis aspirated. As a short term measure in patients with oro- (<3 mmHg) Absent peristalsis with normal pharyngeal dysphagia, nasoenteric feeding may be useful. LES Oropharyngeal dysphagia from cerebrovascular accidents may gradually improve with time; however, if there is no LES Lower esophageal sphincter improvement after more than a month or in cases where neuromuscular degenerative disease has caused oropha- coordination or aspiration. After objective clinical evalua- ryngeal dysphagia, then management with gastrostomy is tion the speech pathologist may assist the clinician in the indicated. Gastrostomy may be performed using several evaluation of oropharyngeal dysphagia. Modified barium different techniques. swallow using barium impregnated marshmallows or pud- Patients with mechanical dysphagia arising from eso- ding may be useful. Upper gastrointestinal series is not al- phageal lesions require appropriate dilation. Benign dis- ways required first if endoscopy is to be undertaken. ease usually responds to single or repeated dilations. Certainly a negative esophagram should not be considered Surgery is rarely indicated to treat the obstruction per se, sufficient to rule out organic disease nor should it be a rea- although patients with severe reflux with or without a son to cease investigation of the patient with persisting stricture may be considered for antireflux surgery. The dysphagia. majority of patients respond well to medical management In the patient with a significant history suggesting me- with dilation as necessary. There is evidence that treat- chanical obstruction, endoscopy is more likely to be help- ment with a proton pump inhibitor lessens stricture recur- ful in establishing a diagnosis because it is more sensitive rence (7,8). in detecting mucosal lesions such as minor ulcerations and Patients with neoplastic lesions causing stricturing re- less severe changes of reflux. In addition, endoscopy offers quire appropriate investigation to stage the disease. Surgi- the added benefit of biopsy and/or brushings, and offers cal resection offers the only possibility of cure. If resection the possibility of therapy in conjunction with the diagnos- is not considered feasible, internal and/or external radio- tic test. If significant mechanical narrowing is identified therapy may be considered for squamous cell neoplasms. one may proceed with dilation at the same setting. Endo- Chemotherapy may also be considered. Patients with un- scopy is quite sensitive at detecting strictures less than resectable disease who require frequent dilation benefit 10 mm in diameter but slightly less sensitive for wider from endoprosthesis. While there is a significant risk with strictures (6). Endoscopy may not detect tapered narrow- placement of rigid esophageal endoprosthesis, such mo- ing of the esophagus when the lumen is wider than the en- dalities offer good palliation of symptoms. Newer expend- doscope and there is no associated mucosal abnormality. able stents can be placed with less risk. Laser or thermal Neither endoscopy nor radiography are totally accurate ablation of tumours are other alternatives. in all situations and they are not interchangeable. Often Patients with esophageal dysmotility may respond to

412 Can J Gastroenterol Vol 12 No 6 September 1998 CAG Practice Guidelines pharmacotherapy using nitrates or calcium channel an- respond to pharmacological or endoscopic therapy may be tagonists, although there are no controlled trials demon- considered for myotomy or rarely for esophagectomy. strating a benefit in motor disorders other than achalasia. REFERENCES Esophageal dilation is sometimes helpful. In patients with 1. Clouse RE. Gastrointestinal disease. In: Sleisenger MH, Fordtran JS, eds. Pathophysiology/Diagnosis/Management, 5th edn. achalasia, treatment with pneumatic dilation remains the Philadelphia: WB Saunders Co, 1993:341-77. standard therapy, but in patients who would not tolerate a 2. Kahrilas PJ. Disorders causing oropharyngeal dysphagia. complication of dilation, the recent advent of botulinum In: Sleisenger MH, Fordtran JS, eds. Pathophysiology/Diagnosis/ Management, 5th edn. Philadelphia: WB Saunders Co, toxin may offer an alternative. Current evidence suggests 1993:205-18. that the response may not be sustained. Patients failing to

Canadian Association of Gastroenterology Practice Guideline Disclaimer

This clinical practice guideline has been developed by the useful to all physicians, it is recognized that specialists may author on behalf of the Canadian Association of rely less on practice guidelines than those in more general Gastroenterology (CAG) in order to outline the clinical practice. These guidelines are intended to give a practical approach to management problems or training issues. After approach to a problem based on the current literature, but preparation by the author, based on a review of the are not intended to be state-of-the-art reviews with literature, each guideline is extensively reviewed by the extensive references. CAG Practice Affairs Committee, composed of practitioners Practice guidelines are developed to be of assistance to from across Canada. Changes are made, and once the practising clinicians and are not intended to be the only guideline is felt to be appropriate, it is then circulated for approach to the management of clinical problems, nor are further review by recognized Canadian experts and then they intended to be considered as a ‘standard of care’. The amended further. Finally, the guideline is presented to the CAG Practice Affairs Committee recognizes that clinical CAG Governing Board for further review and final approval. circumstances may at times justify an approach different Practice guidelines are intended to give an understanding of from that outlined in a practice guideline. It is also a clinical problem and outline one or more preferred recognized that new developments in medical research and approaches to investigation and management of the clinical practice may require subsequent changes to the problem. While practice guidelines are intended to be practice guideline.

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3. Bennett JR, Castell DO. The Esophagus, 2nd edn. Boston: Little 6. Ott DJ. Endoscopy sensitivity in the detection of esophageal Brown and Co, 1995:29-39. strictures. J Clin Gastroenterol 1985;7:121-5. 4. Richter JE. Heartburn, dysphagia, odynophagia, and other 7. Klinkenberg-Knol EC, Festen HP, Jansen JB, et al. Long-term esophageal symptoms. In: Sleisenger MH, Fordtran JS, eds. treatment with omeprazole for refractory reflux esophagitis: efficacy Pathophysiology/Diagnosis/Management, 5th edn. Philadelphia: and safety. Ann Intern Med 1994;121:161-7. WB Saunders Co, 1993:331-40. 8. Marks RD, Richter JE, Rizzo J, et al. Omeprazole versus 5. Castell DO, Donner MW. Evaluation of dysphagia: a careful H2-receptor antagonists in treating patients with peptic stricture history is crucial. Dysphagia 1987;2:65-71. and esophagitis. Gastroenterology 1994;106:907-15.

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