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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

Management algorithm for patients with achalasia
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Fig5: Management algorithm for patients with achalasia

Mentions: A proposed algorithm for the management of patients with achalasia is depicted in Fig. 5. The choice of initial therapy should be guided by patients’ age, gender, preference, and local institutional expertise. Surgical myotomy and PD remain the key treatment options for patients. When deciding on myotomy vs PD, it is important to consider the complications, durability, and cost-effectiveness, as well as the experience of the surgeons and gastroenterologists. Botulinum toxin therapy is recommended for patients who are not surgical candidates or are high-risk, and pharmacologic therapy is reserved for patients who cannot undergo definitive treatment or have failed botulinum toxin injections.Fig. 5


Idiopathic (primary) achalasia: a review.

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

Management algorithm for patients with achalasia
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Fig5: Management algorithm for patients with achalasia
Mentions: A proposed algorithm for the management of patients with achalasia is depicted in Fig. 5. The choice of initial therapy should be guided by patients’ age, gender, preference, and local institutional expertise. Surgical myotomy and PD remain the key treatment options for patients. When deciding on myotomy vs PD, it is important to consider the complications, durability, and cost-effectiveness, as well as the experience of the surgeons and gastroenterologists. Botulinum toxin therapy is recommended for patients who are not surgical candidates or are high-risk, and pharmacologic therapy is reserved for patients who cannot undergo definitive treatment or have failed botulinum toxin injections.Fig. 5

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