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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

a) Conventional water perfused manometric findings of classic achalasia. Isobaric simultaneous esophageal body contractions (lower four tracings) with incomplete LES relaxation (upper most tracing). b) High resolution manometry (HRM) findings in achalasia (simultaneous pan esophageal pressurization with incomplete LES relaxation)
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Fig1: a) Conventional water perfused manometric findings of classic achalasia. Isobaric simultaneous esophageal body contractions (lower four tracings) with incomplete LES relaxation (upper most tracing). b) High resolution manometry (HRM) findings in achalasia (simultaneous pan esophageal pressurization with incomplete LES relaxation)

Mentions: Manometry is the gold standard for establishing the diagnosis of achalasia and is essential for the diagnosis regardless of the findings on barium esophagram and esophagogastroduodenoscopy (EGD). The manometric findings of aperistalsis and incomplete LES relaxation is characteristic on conventional manometry. Wet and dry swallows are followed by simultaneous contractions [1]. The amplitude of the contractions is low (10–40 mm Hg) and repetitive in most cases [9] (Fig. 1). The LES displays high pressure at rest and fails to relax, or relaxes only partially with swallowing (Fig. 1). Up to 40 % of the patients with achalasia have normal LES pressure (10–40 mm Hg); however, low pressure LES is not seen in untreated achalasia patients [62].Fig. 1


Idiopathic (primary) achalasia: a review.

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

a) Conventional water perfused manometric findings of classic achalasia. Isobaric simultaneous esophageal body contractions (lower four tracings) with incomplete LES relaxation (upper most tracing). b) High resolution manometry (HRM) findings in achalasia (simultaneous pan esophageal pressurization with incomplete LES relaxation)
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Fig1: a) Conventional water perfused manometric findings of classic achalasia. Isobaric simultaneous esophageal body contractions (lower four tracings) with incomplete LES relaxation (upper most tracing). b) High resolution manometry (HRM) findings in achalasia (simultaneous pan esophageal pressurization with incomplete LES relaxation)
Mentions: Manometry is the gold standard for establishing the diagnosis of achalasia and is essential for the diagnosis regardless of the findings on barium esophagram and esophagogastroduodenoscopy (EGD). The manometric findings of aperistalsis and incomplete LES relaxation is characteristic on conventional manometry. Wet and dry swallows are followed by simultaneous contractions [1]. The amplitude of the contractions is low (10–40 mm Hg) and repetitive in most cases [9] (Fig. 1). The LES displays high pressure at rest and fails to relax, or relaxes only partially with swallowing (Fig. 1). Up to 40 % of the patients with achalasia have normal LES pressure (10–40 mm Hg); however, low pressure LES is not seen in untreated achalasia patients [62].Fig. 1

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