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Idiopathic (primary) achalasia: a review.

Patel DA, Kim HP, Zifodya JS, Vaezi MF - Orphanet J Rare Dis (2015)

Bottom Line: Current medical and surgical therapeutic options (pneumatic dilation, endoscopic and surgical myotomy, and pharmacologic agents) aim at reducing the LES pressure and facilitating esophageal emptying by gravity and hydrostatic pressure of retained food and liquids.Either graded pneumatic dilatation or laparoscopic surgical myotomy with a partial fundoplication are recommended as initial therapy guided by patient age, gender, preference, and local institutional expertise.Most patients who are appropriately treated have a normal life expectancy but the disease does recur and the patient may need intermittent treatment.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Nashville, TN, USA.

ABSTRACT
Idiopathic achalasia is a primary esophageal motor disorder characterized by loss of esophageal peristalsis and insufficient lower esophageal sphincter relaxation in response to deglutition. Patients with achalasia commonly complain of dysphagia to solids and liquids, bland regurgitation often unresponsive to an adequate trial of proton pump inhibitor, and chest pain. Weight loss is present in many, but not all patients. Although the precise etiology is unknown, it is often thought to be either autoimmune, viral immune, or neurodegenerative. The diagnosis is based on history of the disease, barium esophagogram, and esophageal motility testing. Endoscopic assessment of the gastroesophageal junction and gastric cardia is necessary to rule out malignancy. Newer diagnostic modalities such as high resolution manometry help in predicting treatment response in achalasia based on esophageal pressure topography patterns identifying three phenotypes of achalasia (I-III) and outcome studies suggest better treatment response with types I and II compared to type III. Although achalasia cannot be permanently cured, excellent outcomes are achieved in over 90 % of patients. Current medical and surgical therapeutic options (pneumatic dilation, endoscopic and surgical myotomy, and pharmacologic agents) aim at reducing the LES pressure and facilitating esophageal emptying by gravity and hydrostatic pressure of retained food and liquids. Either graded pneumatic dilatation or laparoscopic surgical myotomy with a partial fundoplication are recommended as initial therapy guided by patient age, gender, preference, and local institutional expertise. The prognosis in achalasia patients is excellent. Most patients who are appropriately treated have a normal life expectancy but the disease does recur and the patient may need intermittent treatment.

No MeSH data available.


Related in: MedlinePlus

Three sub-types of achalasia on high resolution manometry. a Quiescent esophageal body (Type I); b isobaric pan-esophageal pressurization (Type II); c simultaneous contractions (Type III)
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Fig2: Three sub-types of achalasia on high resolution manometry. a Quiescent esophageal body (Type I); b isobaric pan-esophageal pressurization (Type II); c simultaneous contractions (Type III)

Mentions: Aperistalsis is defined as lack of propagating esophageal contractile activity and presents with different pressure patterns including quiescent esophageal body (Type I), isobaric pan-esophageal pressurization (Type II), or simultaneous contractions (Type III), and can now be easily identified with high-resolution manometry (HRM) (Fig. 2) [63]. Although both conventional manometry or HRM can be used for diagnosis, new data is emerging to suggest that HRM may have increased sensitivity in diagnosing achalasia compared to conventional manometry techniques [64]. More importantly, new space-time analysis paradigms with HRM that portrays the pressure signal through the esophagus in a seamless dynamic space-time continuum in the form of esophageal pressure topography can help characterize the motor patterns with treatment outcome implications. Based on three retrospective studies, subtype II has the best prognosis, whereas subtype I is somewhat lower and subtype III can be difficult to treat [63, 65, 66].Fig. 2


Idiopathic (primary) achalasia: a review.

Patel DA, Kim HP, Zifodya JS, Vaezi MF - Orphanet J Rare Dis (2015)

Three sub-types of achalasia on high resolution manometry. a Quiescent esophageal body (Type I); b isobaric pan-esophageal pressurization (Type II); c simultaneous contractions (Type III)
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4509143&req=5

Fig2: Three sub-types of achalasia on high resolution manometry. a Quiescent esophageal body (Type I); b isobaric pan-esophageal pressurization (Type II); c simultaneous contractions (Type III)
Mentions: Aperistalsis is defined as lack of propagating esophageal contractile activity and presents with different pressure patterns including quiescent esophageal body (Type I), isobaric pan-esophageal pressurization (Type II), or simultaneous contractions (Type III), and can now be easily identified with high-resolution manometry (HRM) (Fig. 2) [63]. Although both conventional manometry or HRM can be used for diagnosis, new data is emerging to suggest that HRM may have increased sensitivity in diagnosing achalasia compared to conventional manometry techniques [64]. More importantly, new space-time analysis paradigms with HRM that portrays the pressure signal through the esophagus in a seamless dynamic space-time continuum in the form of esophageal pressure topography can help characterize the motor patterns with treatment outcome implications. Based on three retrospective studies, subtype II has the best prognosis, whereas subtype I is somewhat lower and subtype III can be difficult to treat [63, 65, 66].Fig. 2

Bottom Line: Current medical and surgical therapeutic options (pneumatic dilation, endoscopic and surgical myotomy, and pharmacologic agents) aim at reducing the LES pressure and facilitating esophageal emptying by gravity and hydrostatic pressure of retained food and liquids.Either graded pneumatic dilatation or laparoscopic surgical myotomy with a partial fundoplication are recommended as initial therapy guided by patient age, gender, preference, and local institutional expertise.Most patients who are appropriately treated have a normal life expectancy but the disease does recur and the patient may need intermittent treatment.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Nashville, TN, USA.

ABSTRACT
Idiopathic achalasia is a primary esophageal motor disorder characterized by loss of esophageal peristalsis and insufficient lower esophageal sphincter relaxation in response to deglutition. Patients with achalasia commonly complain of dysphagia to solids and liquids, bland regurgitation often unresponsive to an adequate trial of proton pump inhibitor, and chest pain. Weight loss is present in many, but not all patients. Although the precise etiology is unknown, it is often thought to be either autoimmune, viral immune, or neurodegenerative. The diagnosis is based on history of the disease, barium esophagogram, and esophageal motility testing. Endoscopic assessment of the gastroesophageal junction and gastric cardia is necessary to rule out malignancy. Newer diagnostic modalities such as high resolution manometry help in predicting treatment response in achalasia based on esophageal pressure topography patterns identifying three phenotypes of achalasia (I-III) and outcome studies suggest better treatment response with types I and II compared to type III. Although achalasia cannot be permanently cured, excellent outcomes are achieved in over 90 % of patients. Current medical and surgical therapeutic options (pneumatic dilation, endoscopic and surgical myotomy, and pharmacologic agents) aim at reducing the LES pressure and facilitating esophageal emptying by gravity and hydrostatic pressure of retained food and liquids. Either graded pneumatic dilatation or laparoscopic surgical myotomy with a partial fundoplication are recommended as initial therapy guided by patient age, gender, preference, and local institutional expertise. The prognosis in achalasia patients is excellent. Most patients who are appropriately treated have a normal life expectancy but the disease does recur and the patient may need intermittent treatment.

No MeSH data available.


Related in: MedlinePlus