Dysphagia

What is a dysphagia? What are the latest trends to deal with the case who has presented to you? This "Seminar Presentation" list some of the latest American College of Surgery guidelines, regarding the management of a case of dysphagia

1. Seminar Presentation DYSPHAGIA Dr. Davinder Pal Singh

2. Introduction and mechanis m Physiology of Swallowing

3. Normal Swallowing Mechanism Swallowing was previously described using a three-phase sequential model, whereby the position of the food bolus was used to identify the oral, pharyngeal, and esophageal phases. Later reports expanded this into a four-stage model by dividing the oral phase into preparatory and propulsive stages. These early models accurately depicted liquid swallows but were inadequate to model solid food boluses.

4. The process model accounts for swallowing of solid foods by expanding the oral phase into stage I, transport, food processing, and stage II, transport. The pharyngeal and esophageal phases are common to both liquid and solid transport.

5. Liquid Swallowing

6. Solid Swallowing

7. Pharyngeal Phase This rapid phase must achieve airway protection while facilitating passage of boluses through the upper esophageal sphincter (UES) to the esophagus proper. Airway protection is achieved by concerted closure of the vocal cords and laryngeal movement (anteriorly and superiorly) with concomitant tilting of the epiglottis to seal the laryngeal vestibule. Food propulsion follows: the soft palate rises to block retrograde passage into the nasopharynx.

8. Pharyngeal Phase Pharyngeal constrictors sequentially contract from cranial to caudal, shortening the pharynx and propelling the food bolus downward. This “ pharyngeal pump ” can generate pressures of up to 200 mm Hg. The UES has a resting pressure of between 16 and 118 mm Hg and is closed at rest. Active opening of the UES is achieved by relaxation of the cricopharyngeus, traction by the strap muscles, and distending pressure of the descending food bolus.

9. Esophageal Phase of Swallowing Both the UES and the lower esophageal sphincter (LES) remain closed at rest to prevent reflux. Autonomic efferent signals mediate peristalsis ; an initial wave of relaxation preceding the food bolus is followed by a wave of contraction, resulting in transit through the esophagus into the stomach.

10. Waves of Peristalsis - Esophagus Primary esophageal peristalsis is triggered by voluntary swallowing but autonomously propagates at 2 to 5 cm/s through the striated muscles of the upper third of the esophagus, slowing as peristalsis passes into the smooth muscles of the lower esophagus. Secondary esophageal peristalsis is involuntary and arises in response to esophageal distention or irritation. It is thought that these waves of peristalsis serve to keep the esophagus clear. Tertiary esophageal waves can arise normally between swallows but are often nonpropulsive.

11. It is evident that coordination between the different anatomic levels of the esophagus is required for effective swallowing. The high-pressure contraction in the pharynx is coordinated with full relaxation of the UES. This allows transfer of swallowed material into the esophagus proper, where peristaltic pressures of up to 80 mm Hg are noted.

12. Orderly progression of moderate-amplitude waves through the esophageal body occurs. The LES relaxes early in response to swallowing and slowly regains its resting pressure of 10 to 25 mm Hg in concert with distal esophageal transport.

13. Definition, causes, treatment Dysphagia

14. Definition The term dysphagia , derived from the Greek “ dys ” (with difficulty ) and “ phagia ” (to eat), describes difficulty in the transfer of food or liquid boluses from the mouth to the stomach. Dysphagia is a common complaint, with at least 35% of patients above the age of 50 complaining of weekly dysphagia and up to 60% of nursing home residents suffering feeding difficulties as a result of dysphagia.

15. Types of Dysphagia There are two forms of dysphagia. Oropharyngeal dysphagia results from a functional impairment in the initiation of swallowing, including the oral and pharyngeal phases and often results from systemic neurologic or myopathic syndromes. Esophageal dysphagia relates to intrinsic functional (motor) and anatomic abnormalities of the esophagus that result in swallowing difficulties.

16. Etiology – Oropharyngeal Dysphagia

17. Etiology – Esophageal Dysphagia

18. Evaluation of Dysphagia A systematic approach to the patient with dysphagia is mandatory. A thorough history and complete physical examination allow for an accurate assessment of likely etiologies. Confounding diagnoses, such as angina pectoris, thyroid goiter, and pharyngitis, should be eliminated .

19. Important Elements to elicit on history Timing of Dysphagia Immediate coughing, choking, or regurgitation suggests oropharyngeal causes for dysphagia. A sensation of food “sticking” or getting “caught” or the delayed regurgitation of food suggests esophageal causes of dysphagia. Patients reporting the constant presence of symptoms not associated with swallowing difficulties may have globus sensation , which is a benign, non painful fullness in the neck or throat.

20. History taking – Important Point Painful Swallowing Odynophagia is not typically associated with dysphagia; its presence should prompt consideration of infectious or inflammatory etiologies. Exposure and ingestion of caustic substances must be sought out as an expedient assessment of severity and consideration of immediate surgery must be made.

21. History taking – Important Points Location Patients will self-localize symptoms to the cervical, retrosternal , or epigastric regions. Studies have demonstrated accurate localization, by patient history, to within 4 cm of the culprit lesion in up to 74% of cases. Accuracy seems best for proximal lesions

22. History taking – Important Points Solid or Liquid Intolerance to both liquids and solids suggests a functional or neuromuscular cause of dysphagia. Difficulties with solid food only strongly implicates a mechanical or anatomic causes of dysphagia; a progression from purely solid food dysphagia to both solid and liquid dysphagia suggests narrowing attributable to an evolving mechanical obstruction .

23. History taking – Important Points Onset and Progression The temporal pattern of symptom onset and duration also gives valuable information as to possible causes for dysphagia. Intermittent , nonprogressive symptoms suggest an intrinsic motor dysfunction (such as diffuse esophageal spasm) or a mechanical cause such as a web or ring. If the symptoms have been present for a short period of time or are rapidly progressive, a malignant etiology must be ruled out.

24. History taking Associated Symptoms A history of anorexia or weight loss suggests an underlying malignancy. Passive regurgitation of food particles may arise from achalasia or a cricopharyngeal diverticulum. Retrosternal chest pain, once cardiac etiologies have been eliminated, may be present in cases of esophageal spasm or gastroesophageal reflux. However , dysphagia secondary to peptic strictures and adenocarcinoma are without symptoms of gastroesophageal reflux disease (GERD) in up to 25 to 35% of cases.

25. History Taking Drug History Medication lists must be examined to rule out culprit medications: alendronate, doxycycline, nonsteroidal anti-inflammatory drugs [NSAIDs], and mycophenolate mofetil [MMF]) These may cause drug-induced esophageal injury and hence dysphagia

26. Physical Examination The esophagus is a deep-seated structure that does not lend itself to direct physical assessment. Although a thorough physical examination should follow a complete history, information gathered is useful only in inferring potential diagnoses. A detailed and accurate history is the mainstay of clinical assessment in patients who present with dysphagia.

27. Physical Examination The head and neck are examined for the size of the thyroid gland, as well as for the presence of any lymphadenopathy or masses . A careful examination of cranial nerves may demonstrate deficits contributing to oropharyngeal dysphagia , and corresponding neurologic assessment may reveal signs of a cerebrovascular accident (CVA), myasthenia gravis, or Parkinson disease.

28. Diagnostic Tests Many diagnostic tests can be used to assess dysphagia , including endoscopic , radiologic, and manometric modalities . The application of these should be predicated by the history. In cases of suspected or confirmed caustic ingestion, the first test is emergent upper flexible endoscopy to assess the anatomic extent of damage and to grade the injury.

29. Diagnostic Tests In all other cases of dysphagia, the barium swallow is the ideal first test as it is readily available, cost effective , and rapidly performed. Information can be gained from the barium study regarding anatomic relations, esophageal transit patterns, and the presence or absence of mass lesions and diverticulae. The safety and diagnostic yield of subsequent upper endoscopy are enhanced.

30. UGI Endoscopy Upper endoscopy allows for a visual assessment of mucosa; diagnostic and therapeutic maneuvers such as biopsies, brushings, and dilatations can be performed.

31. Endoscopic Ultrasonography Endoscopic ultrasonography (EUS) is an emerging diagnostic modality that allows for assessment of the esophageal wall and surrounding tissues. This permits the characterization of esophageal masses (depth of invasion, T stage) and an assessment of adjacent lymphadenopathy (N stage), and guides endoscopic fine-needle aspiration biopsies. EUS guided biopsies have excellent predictive value in the assessment of lymph node involvement in cases of esophageal carcinoma.

32. Diagnostic Tests When reflux disease is suspected, extended pH monitoring is invaluable in assessing the presence and severity of GERD. Motility disorders are best diagnosed using manometric techniques. In cases where extrinsic compression is suspected or demonstrated, cross-sectional imaging using computed tomography (CT) or magnetic resonance imaging (MRI) may be useful in identification of malignant masses or vascular anomalies (aberrant subclavian vessels, aortic aneurysms, or Kommerell diverticulae ).

33. Dysphagia lusoria Dysphagia lusoria is a rare entity in which dysphagia results from extrinsic vascular compression of the esophagus from an aberrant right subclavian artery , which arises from the thoracic aorta and typically courses posterior to the esophagus.

34. Diagnostic Tests The assessment of esophageal cancer also requires cross-sectional imaging with CT and fluorodeoxyglucose–positron emission tomography (PET). The use of PET is highly sensitive for the detection of unsuspected metastatic lesions in patients deemed candidates for curative resection on the basis of CT alone.

35. Management of Esophageal Dysphagia M otor disorders Motility disorders affect the smooth muscle of the distal esophagus and the lower esophageal sphincter (LES). Symptoms typically include dysphagia to solids and liquids; non cardiac chest pain may also be present.

36. Brief overview Achalasia

37. Achalasia Ninety-eight percent of all cases of achalasia are idiopathic. The disease is thought to result from a loss of inhibitory neurons in the Auerbach plexus , altering neural input to the LES and preventing normal relaxation. Achalasia affects females and males equally at a rate of 1 per 100,000 individuals per year. The usual presentation is between 20 and 50 years, but it has been described in all age groups. The disease is slowly progressive, and presentation is typically at advanced stages.

38. Symptoms - Achalasia Symptoms include progressive dysphagia to both solids and liquids, accompanied by regurgitation of food particles, chest pain, and weight loss. GERD-like symptoms were present in up to 48% of patients in a study of 32 patients; these symptoms are a consequence of stasis esophagitis (secondarily to fermentation of retained food) rather than reflux of gastric acid.

39. Plain x-rays may reveal an air-fluid level in the distal esophagus, and a barium swallow will demonstrate a dilated and atonic esophagus with the pathognomonic “ bird’s beak ” narrowing of the gastroesophageal junction (GEJ ). Long-standing achalasia may manifest with an extremely dilated and tortuous esophagus (often described as a sigmoid esophagus ).

40. Barium swallow demonstrates the proximal dilatation and classic “bird’s beak” narrowing at the esophagogastric junction, consistent with achalasia, in a 22- year-old woman being evaluated for dysphagia.

41. Barium study demonstrates dilated esophagus with right-sided deviation and tortuous course of the distal esophagus. Treatment most often involves resection of diseased esophagus with conduit interposition.

42. Achalasia Manometric findings of aperistalsis and failure of LES relaxation are key in establishing the diagnosis. Resting LES pressures may be normal or elevated. Endoscopic assessment is required to visually assess mucosal appearance to rule out cancer.

43. Treatment Modalities AIM: Treatment modalities for achalasia must achieve enhanced LES compliance and lower resting LES pressures. Medical management with calcium channel blockers or nitrates has no meaningful benefit. Endoscopic management includes endoscopically injected botulinum toxin, or balloon dilatation , to mechanically disrupt the lower esophageal muscle fibers.

44. Recurrent dysphagia (up to 50%) has been noted in some studies at 5 years after balloon dilatation, with a 5% periprocedural risk of esophageal rupture. In comparison, a laparoscopically performed “ Heller esophagomyotomy with partial anterior ( Dor ) fundoplication” is considered to be the standard of care in terms of both durable outcomes (90 to 95% resolution of dysphagia) and low complication rates.

45. Long-standing achalasia is a risk factor for esophageal squamous cell carcinoma, and tumors of the GEJ may present with symptoms similar to those of achalasia

46. Brief overview Dysmotility Syndromes

47. Dysmotility Syndromes Motility disorders can be considered within a spectrum that includes: Diffuse esophageal spasm (DES), Nutcracker esophagus , and Hypertensive LES These disorders are traditionally considered separate entities ; however, the manometric findings and the mainstays of medical treatment are similar.

48. Diffuse Esophageal Spasm DES is a dysmotility syndrome of unknown etiology. It is characterized in 50% of patients by intermittent dysphagia to solids and liquids. Up to 5% of patients with unexplained chest pain are found to have DES on manometric testing .

49. Evidence for DES on manometry includes: periodic prolonged , multipeaked , high-amplitude contractions in more than one in five wet swallows, with observation of normal peristalsis in intervening periods. Incomplete LES relaxation or hypertensive LES may also be observed.

50. DES The classic corkscrew appearance of the esophagus is evident in this barium study in a middle-aged patient presenting with dysphagia and intermittent chest pain.

51. Nutcracker Esophagus Nutcracker esophagus presents more commonly with chest pain rather than dysphagia. Manometry also forms the mainstay of diagnosis: a normal peristaltic pattern is noted, with extremely increased pressure amplitudes of more than 180 mm Hg. In contrast to DES, normal peristalsis is not observed within trains of high-pressure waves. Barium swallow is of normal appearance

52. Hypertensive LES Hypertensive LES may be found in isolation but often coexists with other dysmotility syndromes. Resting pressures at the LES by manometry are found to be 45 mm Hg or greater.

53. Treatment Treatment for DES, nutcracker esophagus, and hypertensive LES is based on smooth muscle relaxation using nitrates such as isosorbide dinitrate or calcium channel blockers such as diltiazem. Balloon dilatation may be effective for isolated hypertensive LES.

54. Brief overview Esophageal Diverticulae

55. Diverticulae Diverticulae are classified according to the degree to which the esophageal wall is involved in the outpouching: T rue diverticulae involve all layers of the esophageal wall , F alse diverticulae involve only the mucosal layer.

56. Both types of diverticulae can also be classified by the mechanism underlying their formation: True diverticulae usually form in the mid-esophagus and are most often related to extrinsic traction from extramural inflammation in adjacent mediastinal lymph nodes. These are also referred to as “ Traction” type diverticulae . False diverticulae relate to dysmotility and consist of the mucosa being extruded through external muscular layers above a high-pressure zone and are thus “ Pulsion” type diverticulae.

57. Zenker diverticulum Pharyngoesophageal diverticulae (Zenker diverticulum) are the most common diverticulae observed. These pulsion type false diverticulae arise in the Killian triangle and are located just superior to the cricopharyngeus muscle. The basis for the formation of a Zenker diverticulum is pharyngo- cricopharyngeal dyscoordination. When the cricopharyngeal sphincter fails to immediately and fully relax during swallowing , the pharyngeal pump mechanism produces extremely high pressures. As a result of these high pressures, progressive bulging of the mucosa occurs in the posterior midline through a potential gap between the pharyngeal constrictors and the cricopharyngeus muscles (the Killian space).

58. There has been a long-standing appreciation of an association between the pathologic severity of gastroesophageal reflux and the development of Zenker diverticulae: It is thought that repeated exposure of the proximal esophagus and pharynx to low pH refluxate results in cricopharyngeal spasm and loss of normal coordination.

59. Symptoms Symptoms of a cricopharyngeal diverticulum include: D ysphagia , H alitosis , T hroat discomfort, a palpable mass, and R egurgitation of undigested food. Some patients may suffer from recurrent aspiration pneumonia and in severe cases may develop lung abscesses.

60. Diagnostic Evaluation The first diagnostic test should be a barium swallow, which will delineate the size and position of the diverticulum. Initial assessment by endoscopic means is not recommended as there is a sizable risk of perforating the pouch with the endoscope.

61. Pharyngoesophageal diverticulum A large Zenker diverticulum is shown in an elderly patient who presented with dysphagia, recurrent pneumonia, and regurgitation

62. Treatment Treatment is surgical and must include the division of the cricopharyngeus muscle. Smaller pouches may be treated by myotomy alone, whereas those larger than 2 cm should be excised. Endoscopic ( transoral ) approaches to diverticulostomy have also been described.

63. Treatment Midesophageal diverticulae are not typically associated with dysphagia. These true diverticulae are formed by traction from extraesophageal inflammation, most often granulomatous disease in subcarinal lymph nodes. Midesophageal diverticulae are usually asymptomatic, and treatment is focused on the underlying inflammatory process.

64. Treatment Epiphrenic diverticulae arise in the distal esophagus. These pulsion-type diverticulae are associated with underlying esophageal dysmotility and are also occasionally an isolated finding. In the absence of symptoms, expectant management is appropriate.

65. Surgical Treatment When dysphagia is present, surgical management is necessary. The surgical approach, should incorporate “triple therapy,” which must include: Excision of the diverticulum, An esophageal myotomy , and A n antireflux procedure

66. Brief overview Secondary Motor Disorders

67. Secondary Motor Disorders In secondary dysmotility syndromes, the esophageal symptoms are a manifestation of a generalized systemic process. The etiology is thought to be progressive neuropathy and subsequent fibrosis . Common diseases associated with secondary dysmotility include: R heumatologic syndromes, such as scleroderma, and D iabetes mellitus

68. Brief overview Mechanical Obstruction

69. Webs A web is a thin mucosal fold that protrudes into the esophageal lumen . Congenital webs are rare and usually restricted to the pediatric population . These are located in the middle and lower thirds of the esophagus. Acquired webs are normally located in the postcricoid cervical esophagus and are mostly asymptomatic .

70. Etiologies for acquired webs include iron deficiency anemias ( Plummer-Vinson and Paterson-Kelly syndromes) and dermatologic diseases. Webs are twice as common in female patients.

71. Symptoms Dysphagia occurs intermittently with solids, and when symptoms arise, the orifice of the web is found to be less than 1.3 cm.

72. Treatment Diagnosis is by barium swallow, and treatment involves mechanical dilatation using Savary bougies or endoscopic balloons. Underlying anemias and dermatologic conditions should also undergo assessment and appropriate treatment

73. Rings Esophageal rings are typically located in the lower third of the esophagus . Two types are typically described: Muscular rings and M ucosal or Schatzki rings. Muscular rings are rarely associated with dysphagia and are often found incidentally in children undergoing barium swallow for other reasons.

74. Schatzki Rings Schatzki rings are located at the Z-line ( squamo-columnar junction) and are almost always seen in patients with GERD; consequently, the upper surface of a Schatzki ring is covered by squamous epithelium, whereas the lower surface is covered by columnar epithelium. Associations with eosinophilic esophagitis and GERD have been proposed. Diagnosis and treatment are as for esophageal webs.

75. Schatzki ring Barium swallow demonstrates a ring in a middle-aged man with severe gastroesophageal reflux disease symptoms and recent-onset dysphagia

76. Peptic Stricture Peptic stricture was previously found in up to 10% of patients with GERD and represents the end stage of reflux associated ulcerative esophagitis. The incidence of peptic strictures has been drastically reduced with the increased use of effective antireflux medications, chiefly the proton-pump inhibitors (PPIs). Symptoms are described as progressive in nature and involve initial solid food dysphagia, progressing to liquid dysphagia.

77. Peptic Stricture Initial assessment is by barium swallow. Peptic strictures are short segment, circumferential, and located at the squamo-columnar junction. A high index of suspicion for concomitant Barrett esophagus or frank cancer at the site of stricture must be kept.

78. Peptic Strictures Treatment of isolated peptic strictures includes acid suppression and endoscopic dilatation. In the past, peptic strictures were usually an indication for surgical correction; however, recent clinical experience with careful repeated dilatations combined with effective acid suppression therapy (using PPIs) has rendered surgical treatment an uncommon occurrence.

79. Cancer Dysphagia is the presenting complaint in the majority of patients with esophageal cancer. The temporal course is usually short and rapidly progressive. Weight loss is a prominent feature. Locally advanced cancers causing airway fistulization may present with aspiration (swallow-cough sequence ). Evaluation and staging should proceed as per established guidelines.

80. Mid-esophageal squamous cell carcinoma Shown is the classic appearance of a midesophageal squamous cell carcinoma. Mucosal irregularity is apparent within the lesion, along with proximal dilatation and shouldering at the upper and lower borders. Bronchoscopy confirmed anterior penetration of tumor into the airway mucosa

81. Brief overview O dynophagic S yndromes

82. Caustic Ingestion Caustic agents are found in many household cleaning products. Ingestion is typically accidental but may be related to a suicide attempt. The pH of the offending agent (less than 2 or greater than 12), the volume ingested, and the total contact time with the esophageal muscosa are the determinants of the severity of the esophageal injury.

83. Immediate flexible endoscopy is required to assess the degree and severity of the injury. Frank perforation or instability mandates immediate surgical exploration and resection. Sites most commonly affected include the distal esophagus and stomach; there is relative sparing of the upper pharynx and esophagus because of rapid transit through these regions.

84. Injuries will mature into strictures, which require serial assessment by barium studies starting 4 weeks after the initial injury. Repeated dilatations are typically required, although these procedures are technically challenging and sometimes dangerous as a result of the intense fibrosis, which virtually obliterates the esophageal lumen.

85. A large majority of these patients do not have satisfactory results with dilatation as a result of an inability to establish an esophageal lumen or an inability to maintain an adequate lumen despite repeated dilatations. Resection and reconstruction using colonic interposition may become necessary.

86. Eosinophilic Esophagitis This rare inflammatory condition is characterized by eosinophilic infiltrates isolated to the esophagus. Intermittent dysphagia to solid food and pain are commonly noted, and associated manometric abnormalities ( hypercontractility ) are found in up to 60% of patients. Barium studies and endoscopy are often normal in appearance. Glucocorticoids and leukotriene antagonists represent currently accepted treatment .

87. Infections Infectious causes of dysphagia associated with odynophagia include intrinsic infections such as esophageal candidiasis, herpetic infections, and cytomegaloviral illness. Treatment is based on the infectious agent involved. Rarely, extrinsic infection of the esophagus (originating in neighboring necrotizing mediastinal lymph nodes) can occur, causing dysphagia, odynophagia, and other potentially disastrous complications.

88. Conclusion Evaluation of the patient presenting with dysphagia represents a challenge for the surgeon. A careful history is key in determining likely etiologies. The barium swallow should be the first diagnostic test to be considered, endoscopy to follow. Esophageal manometry represents the gold standard for diagnosing benign, functional (motor) disorders. Treatment is varied and depends on the etiology of the dysphagia.

89. References: ACS Surgery 7 th Edition

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