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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

Barium swallow. a Dilated esophagus with retained column of barium and “bird’s beaking” suggestive of achalasia. b End stage achalasia with retained food, barium and tortuous esophagus
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Fig3: Barium swallow. a Dilated esophagus with retained column of barium and “bird’s beaking” suggestive of achalasia. b End stage achalasia with retained food, barium and tortuous esophagus

Mentions: Barium swallow was initially used by Vantrappen et al. [67] in achalasia patients to determine the cause of persistent symptoms after treatment with pneumatic dilation. The characteristics of achalasia in barium esophagogram are the loss of primary peristalsis in the distal two third of the esophagus, and poor emptying with retained food and saliva producing an air-fluid level at the top of the barium column. In chronic stages of the disease, there is a dilated esophagus or sigmoid tortuosity and sometimes, in advanced cases, massive dilatation of the esophageal body that have implications for treatment [1, 5]. The typical finding in achalasia is the presence of smooth tapering of the lower esophagus leading to a closed LES, resembling a bird’s beak (Fig. 3).Fig. 3


Idiopathic (primary) achalasia: a review.

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

Barium swallow. a Dilated esophagus with retained column of barium and “bird’s beaking” suggestive of achalasia. b End stage achalasia with retained food, barium and tortuous esophagus
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Fig3: Barium swallow. a Dilated esophagus with retained column of barium and “bird’s beaking” suggestive of achalasia. b End stage achalasia with retained food, barium and tortuous esophagus
Mentions: Barium swallow was initially used by Vantrappen et al. [67] in achalasia patients to determine the cause of persistent symptoms after treatment with pneumatic dilation. The characteristics of achalasia in barium esophagogram are the loss of primary peristalsis in the distal two third of the esophagus, and poor emptying with retained food and saliva producing an air-fluid level at the top of the barium column. In chronic stages of the disease, there is a dilated esophagus or sigmoid tortuosity and sometimes, in advanced cases, massive dilatation of the esophageal body that have implications for treatment [1, 5]. The typical finding in achalasia is the presence of smooth tapering of the lower esophagus leading to a closed LES, resembling a bird’s beak (Fig. 3).Fig. 3

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