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    Achalasia

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    Jan.31.2022

    Achalasia

    Synopsis

    Key Points

    • Achalasia is a motor disorder of the esophagus characterized by aperistalsis and incomplete lower esophageal sphincter relaxation without evidence of mechanical obstruction
    • Results from degeneration of ganglion cells in myenteric plexus of esophageal body and lower esophageal sphincter, leading to incomplete relaxation of lower esophageal sphincter and aperistalsis in distal esophagus
    • Suspect diagnosis in patients with dysphagia to solids and liquids or with heartburn that does not resolve with an adequate trial of proton pump inhibitors
    • Diagnosis is established by esophageal manometry and may be supported by findings on barium esophagogram
    • All patients require esophagogastroduodenoscopy to exclude mechanical obstruction due to malignancy
    • Treatment may consist of pharmacotherapy or endoscopic or surgical procedures aimed at reducing tone of lower esophageal sphincter
    • Treat patients who are good surgical candidates with pneumatic dilation, peroral endoscopic myotomy, or laparoscopic surgical myotomy initially
      • Peroral endoscopic myotomy is recommended as the preferred treatment for management of patients with type III achalasia r1r2
    • Treat patients who are poor surgical candidates with endoscopic injection of botulinum toxin into lower esophageal sphincter or with pharmacotherapy (ie, calcium channel blockers and long-acting nitrates); these methods are less effective
    • Progressive disease may result in megaesophagus with end-stage achalasia; it is also associated with increased risk of esophageal cancer r3
    • Underlying process causing achalasia is incurable; treatment is aimed at resolving symptoms, and follow-up after therapy is lifelong r3

    Urgent Action

    • Patients should seek immediate medical attention if they experience severe chest pain after pneumatic dilation because it is a symptom of delayed esophageal perforation r3

    Pitfalls

    • Patients may report regurgitation as the primary symptom of achalasia, leading to a misdiagnosis as gastroesophageal reflux disease and a delay in appropriate treatment r4

    Terminology

    Clinical Clarification

    • Achalasia is a motor disorder of the esophagus characterized by aperistalsis and incomplete lower esophageal sphincter relaxation without evidence of mechanical obstruction r3

    Classification

    • Achalasia can be classified into 3 subtypes, based on high-resolution manometric findings using the Chicago classification of esophageal motility disorders (version 3); these have implications for management and prognosis: r5
      • Type I
        • No significant change in esophageal pressurization with swallowing
        • Elevated mean integrated relaxation pressure and 100% failed contractions (or premature contractions with distal contractile integrals less than 450 mm Hg/second/cm)
      • Type II
        • Swallowing associated with simultaneous pressurization throughout the entire esophagus
        • Elevated mean integrated relaxation pressure, 100% failed contractions, and panesophageal pressurization in 20% or more of swallows
        • Most responsive to treatment
      • Type III
        • Swallowing associated with abnormal esophageal contractions or spasms
        • Elevated mean integrated relaxation pressure, lack of normal peristalsis, premature contractions with distal contractile integrals more than 450 mm Hg/second/cm in 20% or more of swallows
        • Least responsive to treatment
    • Although there are no established criteria to define the severity of disease, size and conformation of esophagus within thoracic cavity have been used
      • End-stage achalasia has been defined by the presence of distal angulation, a sigmoid esophagus, and/or megaesophagus (2%-5% of patients diagnosed with and treated for achalasia go on to develop end-stage achalasiar6)

    Diagnosis

    Clinical Presentation

    History

    • Dysphagia to both solids and liquids is the defining symptom of achalasia (occurring in 90% of patientsr6) r3c1
      • Patients may compensate for dysphagia by eating slowly or performing various maneuvers while eating (eg, raising arms, lifting neck, arching back) r4
    • Regurgitation of undigested food or saliva, with or without dysphagia, occurs in 76% to 91% of patients, especially during recumbency at night,r6 which may result in aspiration or cough r3c2c3c4
    • Symptoms of gastroesophageal reflux, such as substernal chest pain, epigastric pain, or heartburn, occur in approximately half of patients; symptoms do not resolve with trial of proton pump inhibitorsr8r7c5c6c7
    • Other symptoms include odynophagia, halitosis, cough, hoarseness, wheezing, sore throat, and difficulty burping r8r9c8c9c10c11c12c13c14
    • Onset of symptoms is gradual, and symptoms may be present for many years before the patient seeks medical advice c15
    • May result in weight loss if severe r7c16

    Physical examination

    • Physical findings are typically normal
    • Weight loss may be noted c17

    Causes and Risk Factors

    Causes

    • Achalasia results from degeneration of ganglion cells in myenteric plexus of esophageal body and lower esophageal sphincter, leading to incomplete relaxation of lower esophageal sphincter and aperistalsis in distal esophagus r3
    • Exact cause of the degeneration leading to achalasia is unknown, but autoimmune, viral, or primary neurodegenerative processes are suspected r10c18

    Risk factors and/or associations

    Age
    • May occur at any age but is most frequent in those aged between 30 and 60 years r3c19c20c21c22c23c24
    Sex
    • Achalasia occurs equally in men and women r3
    Genetics
    • There is some evidence for genetic factors that increase susceptibility to achalasia, but familial cases are rare r4c25
    • May be associated with Allgrove syndrome, Down syndrome, and congenital central hypoventilation syndrome r8c26c27c28
    Ethnicity/race
    • There is no racial predilection for achalasia r11
    Other risk factors/associations
    • Patients with achalasia are more likely to have autoimmune conditions, including type I diabetes mellitus, hypothyroidism, Sjögren syndrome, systemic lupus erythematosus, and uveitis r12c29c30c31c32c33c34

    Diagnostic Procedures

    Primary diagnostic tools

    • Suspect diagnosis in patients with dysphagia to solids and liquids or heartburn that does not resolve with trial of proton pump inhibitors r3c35
    • Perform esophagogastroduodenoscopy in all patients to exclude mechanical obstruction due to malignancy r3c36c37
    • Obtain high-resolution esophageal manometry to establish diagnosis in all patients with suspected disease who do not have evidence of a mechanical obstruction on esophagogastroduodenoscopy r3r13c38
      • High-resolution manometry is preferred over conventional because it provides more information, is more comfortable for the patient, can simultaneously assess upper and lower esophageal sphincters and peristalsis, and allows achalasia to be classified into 3 specific clinical subtypes based on esophageal pressure topography r14r15
      • Functional lumen imaging probe is an alternative for studying the pressure geometry relationship and motor function of the esophagus that has an emerging role in diagnosing achalasia and assessing the esophageal gastric junction opening r3r10
        • May be useful in equivocal cases where manometry fails to diagnose achalasia despite clinical suspicion
    • Barium esophagography is indicated to assess esophageal emptying and gastroesophageal junction morphology in patients with equivocal manometric findings or if manometry is unavailable r3r14c39
      • A timed barium esophagogram is preferred over standard barium esophagography r14
    • Additional testing using CT or endoscopic ultrasonography is indicated for patients in whom there is a suspicion of malignancy (eg, aged more than 55 years, duration of symptoms fewer than 12 months, weight loss of more than 10 kg, severe difficulty passing the lower esophageal sphincter with an endoscope scope or suspicious endoscopic findings) r3r14

    Imaging

    • Timed barium esophagogram r3c40
      • Radiography of esophagus using barium as contrast medium
      • Aids in confirmation of diagnosis of achalasia in patients with equivocal findings on esophageal manometry, but it is not sensitive enough to confirm diagnosis on this basis alone r3
        • May also be used to assess factors that may impact treatment and to evaluate esophageal emptying after treatment
      • The following findings support the diagnosis of achalasia:
        • Narrow gastroesophageal junction with a bird-beakr3 or corkscrewr13 appearance
        • Aperistalsis
        • Retained food in esophagus r16
        • Delayed emptying of barium r3
        • Dilation of esophagus r7
        • Absence or diminution of gastric air bubbles r16
      • The following findings suggest long-standing outflow obstruction or end-stage achalasia:
        • Sigmoid esophagus, defined by either a distal diameter more than 10 cm or a tortuous esophageal course through the chest toward the gastroesophageal junction r7
        • Megaesophagus r7
        • Pulsion diverticula r7
    • Endoscopic ultrasonography r3c41c42
      • Indicated to exclude infiltrating carcinoma for patients in whom there is a strong suspicion of malignancy on endoscopic evaluation (eg, marked increase in resistance to passage of endoscope, mucosal changes) or in elderly patients with a short history of dysphagia and marked weight loss r3
        • Allows fine-needle aspiration biopsy of suspicious lesions
      • Finding of thickened circular muscle layer at lower esophageal sphincter and through smooth muscle of esophagus supports diagnosis of achalasia

    Procedures

    High-resolution esophageal manometry r3c43
    General explanation
    • High-resolution manometry with esophageal pressure topography has replaced conventional manometry; this is the gold standard test for diagnosing achalasia r3
    • A nasally placed pressure-sensing device is used to measure pressure in esophagus and at lower esophageal sphincter
    • Allows functional assessment of both lower esophageal sphincter and esophageal body (peristalsis)
    • Essential for establishing diagnosis of achalasia, regardless of findings on complementary investigations r8
    Indication
    • To establish diagnosis of achalasia
    Interpretation of results
    • In the presence of suggestive symptoms, diagnosis is confirmed by the combination of aperistalsis and incomplete lower esophageal sphincter relaxation without evidence of mechanical obstruction
    • Characteristics vary according to type of achalasia r3r17
      • Achalasia type I
        • 100% failed peristalsis (aperistalsis)
        • Abnormal integrated relaxation pressure
        • Absence of panesophageal pressurization to more than 30 mm Hg
      • Achalasia type II (most common)
        • 100% failed peristalsis (aperistalsis)
        • Abnormal integrated relaxation pressure
        • 20% or more of swallows with panesophageal pressurization to greater than 30 mm Hg
      • Achalasia type III (least common)
        • 20% or more of swallows with premature or spastic contractions
        • No evidence of peristalsis
        • Abnormal integrated relaxation pressure
        • Periods of panesophageal pressurization may or may not be present
    • The following findings support a diagnosis but are not required: r11
      • Complete relaxation of lower esophageal sphincter to gastric baseline with duration less than 6 seconds
      • Increased baseline pressure at lower esophageal sphincter (more than 45 mm Hg)
      • Increased baseline pressure in esophageal body
      • Simultaneous nonpropagating contractions
    Esophagogastroduodenoscopy c44c45
    General explanation
    • Direct visualization of upper gastrointestinal tract using a video gastroscope, with a biopsy specimen (either intact tissue or aspirate) obtained from any suspicious areasr3
    • Findings may support diagnosis of achalasia, but diagnosis cannot be made on this basis alone r11
    Indication
    • To evaluate for mechanical obstruction due to infiltrating gastric carcinoma in patients with findings suggestive of achalasia
    Contraindications
    • Clinical evidence of acute perforation
    • Medical instability
    Complications
    • Perforation d1
    • Bleeding
    Interpretation of results
    • Findings may support diagnosis of achalasia, but diagnosis cannot be made on this basis alone r3
      • Findings may range from normal (44%) to a tortuous dilated sigmoid esophagus (suggestive of end-stage achalasia) r11
      • Retained saliva, liquid, and food in esophagus without evidence of mechanical obstruction may be seen (suggestive of achalasia) r3
      • Appearance of lower esophageal sphincter may range from normal to thickened with a rosette configuration on retroflexion view r11
      • Appearance of mucosa may range from normal to exhibiting inflammatory changes or ulcerations r11
      • Stenosis causing a puckered or tight esophagogastric junction and/or abnormal contraction waves may be detected r3r16
        • Although slight resistance at esophagogastric junction is typical, strong resistance suggests malignancy r3

    Differential Diagnosis

    Most common

    • Pseudoachalasia r3c46
      • Esophageal motility disorder most often caused by mechanical obstruction from tumors in the gastric cardia; however, malignant infiltration of the myenteric plexus by adenocarcinoma of the gastroesophageal junction or by cancer of the pancreas, breast, lung, or liver has also been implicated
        • May also be caused by extrinsic factors, such as tight fundoplication or peptic stricture or secondary to laparoscopic adjustable gastric banding for weight loss
      • Presents with classic symptoms of achalasia and similar endoscopic and manometric findings r3
      • Although the symptoms of achalasia and pseudoachalasia are the same, strong resistance to endoscope at gastroesophageal junction suggests pseudoachalasia r3
      • Differentiated by clinical presentation and findings on esophagogastroduodenoscopy and endoscopic ultrasonography
    • Gastroesophageal reflux disease r3c47d2
      • Condition characterized by backflow of gastric contents through an incompetent lower esophageal sphincter
      • Heartburn, substernal chest pain, regurgitation, and dysphagia symptoms may mimic achalasia r3
      • Differentiated by clinical history, lack of response to adequate trial of proton pump inhibitors, endoscopic findings (may show associated esophagitis, Barrett esophagus, or esophageal stricture), and manometry/impedance-pH testing
    • Chagas disease r11c48d3
      • Caused by infection with Trypanosoma cruzi; chronic infection may result in megaesophagus
      • As with achalasia, patients with Chagas disease may experience dysphagia, chest pain, relaxed lower esophageal sphincter, and aperistalsis
      • Unlike patients with achalasia, patients with Chagas disease often have other clinical features of enteric myenteric destruction, such as megacolon, heart disease, and neurologic disorders r11
      • Differentiated by clinical findings and serologic testing

    Treatment

    Goals

    • Alleviate symptoms, improve esophageal emptying, and prevent further dilation of esophagus

    Disposition

    Recommendations for specialist referral

    • Refer patients with suspected achalasia to a gastroenterologist for evaluation and management

    Treatment Options

    Treatment is palliative and may consist of pharmacotherapy or endoscopic or surgical procedures aimed at reducing tone of lower esophageal sphincter r3

    • Treat patients who are good surgical candidates using pneumatic dilation, peroral endoscopic myotomy, or laparoscopic surgical myotomy initially r3
      • Generally considered equally effective;r11choice may be based on type of achalasia, patient preference, and institutional expertiser1
        • American Society for Gastrointestinal Endoscopy recommends peroral endoscopic myotomy as the preferred treatment for management of patients with type III achalasia; they suggest that the three procedures are comparable options for management of patients with achalasia types I and II r1
        • American College of Gastroenterology also recommends any of the 3 procedures as reasonable options for types I and II achalasia and recommends tailored surgical myotomy or peroral endoscopic myotomy for type III achalasia r3
        • American Gastroenterological Association suggests that, if the expertise is available, peroral endoscopic myotomy should be considered as primary therapy for type III achalasia r2
        • Society of American Gastrointestinal and Endoscopic Surgeons recommends peroral endoscopic myotomy or laparascopic Heller myotomy for type I and II achalasia and peroral endoscopic myotomy for type III achalasia r18
          • In patients who wish to avoid use of proton pump inhibitors postoperatively, peroral endoscopic myotomy or pneumatic dilatation are recommended
      • Peroral endoscopic myotomy is an effective minimally invasive technique for treatment of all types of achalasia and is preferred over surgical myotomy at most institutions
        • Equivalent in efficacy as surgical myotomy and more effective than pneumatic dilation in the short term, with symptom relief reported as more than 90%; however, long-term efficacy data are lacking r19r20r21r22r23
          • A 2019 randomized control trial suggested that, among treatment-naive patients with achalasia, treatment with peroral endoscopic myotomy compared with pneumatic dilation resulted in a significantly higher treatment success rate at 2 years r24
        • Indicated for patients who do not wish to undergo surgery or those in whom prior treatments (eg, botulinum toxin injection, pneumatic dilation, laparoscopic myotomy) have failed r25
        • Safe and very well tolerated, with lower complication rates, shorter duration of hospitalization, and faster recovery compared with surgical myotomy; however, there is an increased risk of postoperative symptomatic gastroesophageal reflux disease r1r26
          • Performing the procedure with a short esophageal myotomy is as effective as standard-length myotomy and results in a lower incidence of postoperative erosive esophagitis r27
      • Surgical myotomy (Heller myotomy) is an effective treatment of achalasia, particularly type III achalasia r3
        • Laparoscopic myotomy is preferred over open or thoracoscopic approaches owing to decreased morbidity and faster recovery r3
        • Provides initial symptom relief in approximately 90% of patients; results are better in patients with types I and II achalasia compared with type III r28
        • After a complete myotomy, educate patients that they can never lie flat because of risk of aspiration and that they should chew their food well and swallow only small quantities r6
        • Development of gastroesophageal reflux is common after myotomy r3
          • An antireflux fundoplication procedure is recommended in conjunction with myotomy to reduce the incidence of distal esophageal acid exposure r29
      • Graded pneumatic dilation is an effective nonsurgical treatment of achalasia and is an option for patients who do not wish to undergo surgery or those in whom surgical myotomy has failed; however, peroral endoscopic myotomy is recommended over pneumatic dilatation for most patients r3r18
        • This approach has been associated with symptom relief in 50% to 93% of patients and with a lower risk of perforation compared with surgical myotomy r11
        • Less effective for type III achalasia; peroral endoscopic myotomy or laparoscopic surgical myotomy are preferred r3
        • Patients undergoing pneumatic dilation should also be candidates for surgical myotomy because esophageal perforation is a complication of pneumatic dilation and may require surgical repair r3
      • Esophagectomy with gastric interposition is indicated for patients with end-stage achalasia in whom myotomy has failed and who are candidates for surgery r3
    • Treat patients who are poor surgical candidates with endoscopic injection of botulinum toxin into lower esophageal sphincter or with pharmacotherapy r3
      • Botulinum toxin injection is indicated for patients who are unable or unwilling to undergo pneumatic dilation or surgical myotomy or as adjunct therapy for those with residual spastic contractions after myotomy r3
        • Botulinum toxin therapy is a relatively simple procedure with a low complication rate that is associated with about 50% decrease in basal pressure at lower esophageal sphincter;r3 however, relapse at 2 years is commonr29
        • Therapeutic effect often wanes with time, so repeated injection is required at 6 to 24 months in about half of patients r3r11
        • Botulinum toxin injections may produce a more prolonged response in older patients (older than 65 years), patients with vigorous achalasia (compared with those with classic achalasia), and those with lower esophageal pressures (50% or less of the upper reference limit) r11r29
      • Pharmacotherapy is indicated for patients with very early disease who are not able to undergo surgical therapy and for whom botulinum toxin therapy was unsuccessful; it may also be used as a temporary treatment for patients waiting for more definitive therapies r3
        • Pharmacotherapy is considered the least effective therapy for achalasia, and use is limited by short duration of action, incomplete symptom relief, decreased efficacy during long-term use, and adverse effects (eg, headache, hypotension, edema)
        • Calcium channel blockers and long-acting nitrates are the most commonly used agents r3
        • Other agents include anticholinergics (atropine, dicyclomine, and cimetropium bromide), terbutaline and theophylline, and sildenafil r3
      • Placement of a self-expanding biodegradable esophageal stent, as for benign esophageal strictures, may be an alternative option in older patients with achalasia in whom other treatments are high risk; a series in high-risk octogenarians demonstrated good short- and long-term clinical results with this approach r30r31
        • Metallic removable stent placement is not recommended for management of long-term dysphagia in patients with achalasia r3r14
    • Patients who develop end-stage achalasia (megaesophagus or significant dilation and tortuosity of esophagus) may ultimately require esophagectomy if pneumatic dilation or surgical myotomy fails to improve symptoms
      • Consists of partial removal of esophagus with gastric interposition r3
      • Over 80% of patients report symptomatic improvement r21

    Drug therapy

    • Calcium channel blocker r3
      • Nifedipine c49
        • Nifedipine Oral capsule; Adults: Not FDA-approved but has been studied. 10 to 30 mg PO, 30 to 45 minutes before meals, is a usual dosage. Nifedipine inhibits lower esophageal muscle contraction and resting pressure; side effects and tolerance may limit its utility. Reserve for those not accepting/not candidates for definitive procedural therapies or who fail to respond to botulinum toxin injection.
        • Off-label use for this indication
    • Nitrates
      • Isosorbide dinitrate c50
        • Product not available in the United States
        • Isosorbide Dinitrate Sublingual tablet; Adults: 5 mg SL given 10 to 15 minutes before meals. Reserve for those refusing/not candidates for more definitive procedural therapies or who fail to respond to botulinum toxin injections.
        • Off-label use for this indication
    • Botulinum toxin (see procedures section for more details)
      • OnabotulinumtoxinA Solution for injection; Adults: Use not FDA-approved; per guidelines, reserved as an option for patients who are not candidates for definitive treatments. A usual dose is 20 to 25 units per injection, diluted in 0.5 to 1 mL of preservative-free saline, injected 1 cm above the squamocolumnar junction into 4 or more quadrants of the lower esophageal sphincter (LES); total dosage is not to exceed 100 units/treatment. A sclerotherapy needle is used to achieve placement during upper-GI endoscopy. Repeat injections may be necessary.

    Nondrug and supportive care

    Procedures
    Pneumatic dilation c51

    Intraluminal dilation of lower esophageal sphincter typically using a nonradiopaque polyethylene balloon guided by fluoroscopy or endoscopy r3

    Serial dilation is recommended beginning with dilation using 3-cm balloon followed by assessment and a 3.5-cm dilation if necessary at 2 to 6 weeks, with a subsequent 4-cm dilation if there is insufficient relief r3r32

    • May use 3.5-cm balloon initially in younger male patients r3

    Presents small risk of esophageal perforation; therefore, perform the procedure only in patients who are candidates for surgery and in an institution in which surgical intervention is available

    Patients should seek immediate medical attention if they experience severe chest pain after pneumatic dilation because it is a symptom of delayed esophageal perforation

    Routine gastrografin esophagogram no longer recommended after dilation; gastrografin or barium esophagogram and/or CT scan of abdomen and chest is reserved for patients with a clinical suspicion for perforation r3

    Indication
    • Indicated as a first line therapy for patients with achalasia who are good surgical candidates and whose condition has not yet progressed to end-stage achalasia r3
    Contraindications
    • Non–surgical candidate status (because complication of perforation may require surgical repair)
    Complications
    • Esophageal perforation r3
    • Intramural hematoma r29
    • Gastroesophageal reflux disease r3
    • Chronic active or ulcerating esophagitis r29
    Peroral endoscopic myotomy r21c52
    General explanation
    • Combination endoscopic and surgical procedure that uses esophagogastroduodenoscopy to perform surgical myotomy
    Indication
    • Minimally invasive option for all patients with achalasia who are good surgical candidates; second line treatment for patients in whom pneumatic dilation therapy fails
    • Particularly useful for patients in whom laparoscopic myotomy is technically difficult, such as those with prior major upper abdominal surgery, prior laparoscopic myotomy, or morbid obesity
    • American Society for Gastrointestinal Endoscopy recommends peroral endoscopic myotomy as the preferred treatment for management of patients with type III achalasia r1
    • American Gastroenterological Association suggests that, if the expertise is available, peroral endoscopic myotomy should be considered as primary therapy for type III achalasia r2
    Complications r33
    • Mucosal perforation
    • Subcutaneous emphysema
    • Pneumoperitoneum
    • Pneumothorax
    • Pneumomediastinum
    • Pleural effusion
    • Pneumonia
    • Postprocedure gastroesophageal reflux disease r1
    Laparoscopic surgical esophagomyotomy (Heller esophagocardiomyotomy) c53
    General explanation
    • Division of lower esophageal sphincter muscles followed by fundoplication procedure to reduce the risk of postsurgical reflux and minimize the risk of treatment failure; partial fundoplication is favored over total fundoplication r29
    Indication
    • Indicated for all patients with achalasia who are willing and able to undergo surgery, particularly those with end-stage achalasia (in whom the laparoscopic form is first line treatment); indicated as a second line treatment for patients in whom pneumatic dilation therapy fails r3
    Complications
    • Mucosal perforation r3
    • Incomplete myotomy r29
    • Postprocedure gastroesophageal reflux disease r3
    Botulinum toxin injection r3c54
    General explanation
    • Injection of up to 100 units of botulinum toxin in 0.5- to 1-mL aliquots into at least 4 quadrants of the lower esophageal sphincter just above the squamocolumnar junction and in the lower third of the esophagus r3r34
    • Sclerotherapy needle is used to achieve placement during esophagogastroduodenoscopy; repeated injections (at 6- to 24-month intervals) may be necessary if the patient has good response
    Indication
    • Second line therapy for achalasia (for those who are unable or unwilling to undergo surgery) or adjunct therapy for those with residual spastic contractions after myotomy; not recommended as first line therapy in patients who are fit to undergo surgery r3
    • American Society for Gastrointestinal Endoscopy recommends botulinum toxin injection be reserved for patients who are not candidates for other definitive therapies r1
    Complications
    • Chest pain r11
    • Less common complications include mediastinitis, esophageal mucosal ulceration, pleural effusion, cardiac conduction defects,r29 and allergic reactions related to egg proteinr11
    • Repeated treatments may cause an inflammatory reaction r11

    Monitoring

    • Evaluate patients with symptoms following treatment with objective testing r3
      • Continued or recurrent symptoms after definitive therapy may be related to incomplete disruption of the lower esophageal sphincter during myotomy and dilation, anatomical distortion related to dilation, tortuosity, diverticulum formation, gastroesophageal reflux, or spastic contractions r3
      • Timed barium esophagogram is the preferred modality for evaluating continued or recurrent symptoms after definitive therapy for achalasia r3
        • Follow closely if evidence of continued bolus retention and consider repeat treatment if retention worsens or dilatation increases
      • High-resolution manometry can assess the completeness of myotomy and determine whether spastic contractions are present but cannot accurately determine bolus retention or assess for gastroesophageal reflux r3
      • Findings on barium esophagography or manometry alone should not drive the decision to intervene; however, these tools may be used to identify patients who may require a closer follow-up to prevent progression r13
      • Endoscopy is recommended to screen for gastroesophageal reflux disease in patients treated with surgical myotomy without a concomitant antireflux procedure r14
    • Lifelong follow-up is required to monitor for development of megaesophagus or esophageal cancer r36(particularly squamous cell carcinoma) r35
      • Most guidelines do not recommend routine endoscopic cancer surveillance because the absolute risk is lowr36; however, some experts do advocate esophagogastroduodenoscopy every 3 years beginning 10 to 15 years after initial diagnosis of achalasia r3
      • Endoscopic surveillance may have a role in the assessment of gastroesophageal reflux folllowing peroral endoscopic myotomy or assessing for progression to megaesophagus in longstanding disease r35

    Complications and Prognosis

    Complications

    • Aspiration pneumonia r37c55d4
    • Megaesophagus or end-stage achalasia r37c56c57
    • Postmyotomy gastroesophageal reflux; may require lifelong proton pump inhibitor therapy r14
    • Esophageal cancer (both squamous cell carcinoma and adenocarcinoma) r36c58d5

    Prognosis

    • Achalasia is considered a chronic incurable disorder; treatments aimed at reducing hypertonicity of lower esophageal sphincter are generally effective but do not represent a permanent cure r3
      • Approximately 10% to 15% of patients will progress to megaesophagus or end-stage achalasia despite treatment r11
    • Prognosis of achalasia depends in part on subtype of disease
      • Type II achalasia is most likely to respond to treatment (myotomy or pneumatic dilation) and least likely to require further treatment within 12 months of therapy r38
      • Type III achalasia has the worst prognosis after treatment r6
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