Management of Achalasia: Surgery or Pneumatic Dilation
Management of Achalasia: Surgery or Pneumatic Dilation
Achalasia is an esophageal motility disorder of unknown cause, characterised by aperistalsis of the esophageal body and impaired lower esophageal sphincter relaxation. Patients present at all ages, primarily with dysphagia for solids/liquids and bland regurgitation. The diagnosis is suggested by barium esophagram or endoscopy and confirmed by esophageal manometry. Achalasia cannot be cured. Instead, our goal is to relieve symptoms, improve esophageal emptying and prevent the development of megaesophagus. The most successful therapies are pneumatic dilation and surgical myotomy. The advantages of pneumatic dilation include an outpatient procedure, minimal pain, return to work the next day, mild if any GERD, and can be performed in any age group and even during pregnancy. Pneumatic dilation does not hinder future myotomy, and all cost analyses find it less expensive than Heller myotomy. Laparoscopic myotomy with a partial fundoplication has the advantage of being a single procedure, dysphagia relief is longer at the cost of more troubling heartburn, and a myotomy may be more effective treatment in adolescents and younger adults, especially men. Over a two year horizon, the clinical success of pneumatic dilation and laparoscopic myotomy are comparable in a recent large European randomised trial. The prognosis for achalasia patients to return to near-normal swallowing and good quality of life are excellent, but few are "cured" with a single treatment and intermittent "touch up" procedures may be required.
Achalasia is a rare disorder with an estimated prevalence of 0.5–1 per 100 000 per year without a clear age predilection. Patients mainly present with symptoms such as dysphagia for both solids and liquids, regurgitation of undigested food, respiratory complications (nocturnal cough and aspiration), chest pain and weight loss. These symptoms result from impaired peristalsis and deficient or absent relaxation of the lower oesophageal sphincter which leads to stasis of food in the oesophagus. Treatment aims to disrupt or dissect the lower oesophageal sphincter to enhance transport of the bolus into the stomach. In this review, we will discuss the current treatment options and propose a therapeutic algorithm.
Why the enteric neurons of the oesophagus gradually disappear in patients with achalasia remains unclear. However, evidence is accumulating that an auto-immune response targeted against these neurons, triggered by an infectious agent, may be involved. This hypothesis fits with previous studies showing that, similar to auto-immune diseases, the risk of developing achalasia is associated with a certain genetic background, rendering individuals susceptible to develop an 'abnormal' immune response to an infectious event.
Genes that are associated with MHC genes, in particular class II MHC genes, are linked to auto-immunity. Several previous reports indicated a significant association of HLA-DR and DQ alleles with achalasia, and even showed that anti-neuronal antibodies were especially found in patients carrying the DQA1*0103 and DQB1*0603 alleles. Recently, Facco et al provided evidence that after HSV-1 infection, the virus persists in the neurons of the oesophagus triggering a persistent immune-activation, consisting of infiltration of the ganglia with cytotoxic CD8+ T cells and circulating anti-neuronal antibodies.
The first diagnostic step is to rule out anatomical lesions using endoscopy or radiology. In early stages, both endoscopy and radiology may be completely normal. In advanced cases, endoscopy may reveal a dilated oesophagus with retained food and some increased resistance at the gastro-oesophageal junction. Radiological examination may show a typical 'bird-beak' image at the junction, with a dilated oesophageal body, sometimes with an air-fluid level and absence of an intragastric air bubble. Endoscopy is diagnostic in about 1/3 and radiology in about 2/3 of the patients; diagnostic certainty is provided by manometry in over 90% of cases. Manometry typically shows an aperistaltic oesophageal body, sometimes with elevated intra-oesophageal pressure due to stasis of food and saliva, and incomplete relaxation of the lower oesophageal sphincter upon deglutition (residual pressure >10 mm Hg). In addition, resting tone of the lower oesophageal sphincter will often be elevated. Recently, Pandolfino et al proposed new diagnostic criteria using high resolution manometry with improved accuracy to diagnose achalasia and identifying three different types: type I (classical achalasia; no evidence of pressurisation), type II (achalasia with compression or compartimentalisation in the distal oesophagus >30 mm Hg) and type III (vigorous achalasia or two or more spastic contractions). Interestingly, the therapeutic response differs between the manometric subtypes suggesting that this classification may be useful to predict outcome.
Although most cases are idiopathic, it should be emphasised that Chagasic achalasia and pseudo-achalasia may lead to a similar clinical picture. Chagas' disease is endemic in South America as a consequence of an infection with the parasite Trypanosoma cruzi. Approximately 2–4% of patients suspected of achalasia suffer from pseudoachalasia. In general, patients with pseudoachalasia are older and have a shorter history of dysphagia and weight loss. The most common cause of pseudoachalasia is a malignancy infiltrating the gastro-oesophageal junction. Although the above mentioned symptoms may seem rather obvious and diagnostic evaluation has certainly improved, there is still a remarkable delay of approximately 5 years between the onset of symptoms and the diagnosis.
Interestingly, the frequent delay in the diagnosis is not due to an atypical clinical presentation of the disease, but rather to misinterpretation of typical findings by the physician consulted. Therefore, clinicians should be more alert for this diagnosis in patients presenting with dysphagia. In particular, differentiation between pseudo-achalasia and idiopathic achalasia is clinically of crucial importance and requires meticulous endoscopic inspection, endoscopic ultrasound examination or CT scanning to exclude an infiltrating malignancy. Certainly, in case of rapidly progressing dysphagia, significant weight loss and older age, this diagnosis should be suspected.
Abstract and Introduction
Abstract
Achalasia is an esophageal motility disorder of unknown cause, characterised by aperistalsis of the esophageal body and impaired lower esophageal sphincter relaxation. Patients present at all ages, primarily with dysphagia for solids/liquids and bland regurgitation. The diagnosis is suggested by barium esophagram or endoscopy and confirmed by esophageal manometry. Achalasia cannot be cured. Instead, our goal is to relieve symptoms, improve esophageal emptying and prevent the development of megaesophagus. The most successful therapies are pneumatic dilation and surgical myotomy. The advantages of pneumatic dilation include an outpatient procedure, minimal pain, return to work the next day, mild if any GERD, and can be performed in any age group and even during pregnancy. Pneumatic dilation does not hinder future myotomy, and all cost analyses find it less expensive than Heller myotomy. Laparoscopic myotomy with a partial fundoplication has the advantage of being a single procedure, dysphagia relief is longer at the cost of more troubling heartburn, and a myotomy may be more effective treatment in adolescents and younger adults, especially men. Over a two year horizon, the clinical success of pneumatic dilation and laparoscopic myotomy are comparable in a recent large European randomised trial. The prognosis for achalasia patients to return to near-normal swallowing and good quality of life are excellent, but few are "cured" with a single treatment and intermittent "touch up" procedures may be required.
Introduction
Achalasia is a rare disorder with an estimated prevalence of 0.5–1 per 100 000 per year without a clear age predilection. Patients mainly present with symptoms such as dysphagia for both solids and liquids, regurgitation of undigested food, respiratory complications (nocturnal cough and aspiration), chest pain and weight loss. These symptoms result from impaired peristalsis and deficient or absent relaxation of the lower oesophageal sphincter which leads to stasis of food in the oesophagus. Treatment aims to disrupt or dissect the lower oesophageal sphincter to enhance transport of the bolus into the stomach. In this review, we will discuss the current treatment options and propose a therapeutic algorithm.
Pathophysiology
Why the enteric neurons of the oesophagus gradually disappear in patients with achalasia remains unclear. However, evidence is accumulating that an auto-immune response targeted against these neurons, triggered by an infectious agent, may be involved. This hypothesis fits with previous studies showing that, similar to auto-immune diseases, the risk of developing achalasia is associated with a certain genetic background, rendering individuals susceptible to develop an 'abnormal' immune response to an infectious event.
Genes that are associated with MHC genes, in particular class II MHC genes, are linked to auto-immunity. Several previous reports indicated a significant association of HLA-DR and DQ alleles with achalasia, and even showed that anti-neuronal antibodies were especially found in patients carrying the DQA1*0103 and DQB1*0603 alleles. Recently, Facco et al provided evidence that after HSV-1 infection, the virus persists in the neurons of the oesophagus triggering a persistent immune-activation, consisting of infiltration of the ganglia with cytotoxic CD8+ T cells and circulating anti-neuronal antibodies.
Diagnosis
The first diagnostic step is to rule out anatomical lesions using endoscopy or radiology. In early stages, both endoscopy and radiology may be completely normal. In advanced cases, endoscopy may reveal a dilated oesophagus with retained food and some increased resistance at the gastro-oesophageal junction. Radiological examination may show a typical 'bird-beak' image at the junction, with a dilated oesophageal body, sometimes with an air-fluid level and absence of an intragastric air bubble. Endoscopy is diagnostic in about 1/3 and radiology in about 2/3 of the patients; diagnostic certainty is provided by manometry in over 90% of cases. Manometry typically shows an aperistaltic oesophageal body, sometimes with elevated intra-oesophageal pressure due to stasis of food and saliva, and incomplete relaxation of the lower oesophageal sphincter upon deglutition (residual pressure >10 mm Hg). In addition, resting tone of the lower oesophageal sphincter will often be elevated. Recently, Pandolfino et al proposed new diagnostic criteria using high resolution manometry with improved accuracy to diagnose achalasia and identifying three different types: type I (classical achalasia; no evidence of pressurisation), type II (achalasia with compression or compartimentalisation in the distal oesophagus >30 mm Hg) and type III (vigorous achalasia or two or more spastic contractions). Interestingly, the therapeutic response differs between the manometric subtypes suggesting that this classification may be useful to predict outcome.
Although most cases are idiopathic, it should be emphasised that Chagasic achalasia and pseudo-achalasia may lead to a similar clinical picture. Chagas' disease is endemic in South America as a consequence of an infection with the parasite Trypanosoma cruzi. Approximately 2–4% of patients suspected of achalasia suffer from pseudoachalasia. In general, patients with pseudoachalasia are older and have a shorter history of dysphagia and weight loss. The most common cause of pseudoachalasia is a malignancy infiltrating the gastro-oesophageal junction. Although the above mentioned symptoms may seem rather obvious and diagnostic evaluation has certainly improved, there is still a remarkable delay of approximately 5 years between the onset of symptoms and the diagnosis.
Interestingly, the frequent delay in the diagnosis is not due to an atypical clinical presentation of the disease, but rather to misinterpretation of typical findings by the physician consulted. Therefore, clinicians should be more alert for this diagnosis in patients presenting with dysphagia. In particular, differentiation between pseudo-achalasia and idiopathic achalasia is clinically of crucial importance and requires meticulous endoscopic inspection, endoscopic ultrasound examination or CT scanning to exclude an infiltrating malignancy. Certainly, in case of rapidly progressing dysphagia, significant weight loss and older age, this diagnosis should be suspected.
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