AchalasiaAchalasia

BackgroundBackground Sir Thomas Willis first described achalasia in In 1881, von Mikulicz described the disease as a cardiospasm to indicate that the symptoms were due to a functional problem rather than a mechanical one. In 1929, Hurt and Rake realized that the disease was caused by a failure of the lower esophageal sphincter (LES) to relax. They coined the term achalasia, meaning failure to relax. Achalasia is a primary esophageal motility disorder characterized by failure of a hypertensive LES to relax and the absence of esophageal peristalsis. These abnormalities cause a functional obstruction at the gastroesophageal junction. Sir Thomas Willis first described achalasia in In 1881, von Mikulicz described the disease as a cardiospasm to indicate that the symptoms were due to a functional problem rather than a mechanical one. In 1929, Hurt and Rake realized that the disease was caused by a failure of the lower esophageal sphincter (LES) to relax. They coined the term achalasia, meaning failure to relax. Achalasia is a primary esophageal motility disorder characterized by failure of a hypertensive LES to relax and the absence of esophageal peristalsis. These abnormalities cause a functional obstruction at the gastroesophageal junction.

Pathophysiology:Pathophysiology: LES pressure and relaxation are regulated by excitatory (eg, acetylcholine, substance P) and inhibitory (eg, nitric oxide, vasoactive intestinal peptide) neurotransmitters. Persons with achalasia lack nonadrenergic, noncholinergic, inhibitory ganglion cells, causing an imbalance in excitatory and inhibitory neurotransmission. The result is a hypertensive nonrelaxed esophageal sphincter. LES pressure and relaxation are regulated by excitatory (eg, acetylcholine, substance P) and inhibitory (eg, nitric oxide, vasoactive intestinal peptide) neurotransmitters. Persons with achalasia lack nonadrenergic, noncholinergic, inhibitory ganglion cells, causing an imbalance in excitatory and inhibitory neurotransmission. The result is a hypertensive nonrelaxed esophageal sphincter.

Frequency: In the US: The incidence of achalasia is approximately 1 per 100,000 people per year. In the US: The incidence of achalasia is approximately 1 per 100,000 people per year. Internationally: Chagas disease may cause a similar disorder. Internationally: Chagas disease may cause a similar disorder. Sex: The male-to-female ratio of achalasia is 1:1. Age: Achalasia typically occurs in adults aged years. Fewer than 5% of cases occur in children. Frequency: In the US: The incidence of achalasia is approximately 1 per 100,000 people per year. In the US: The incidence of achalasia is approximately 1 per 100,000 people per year. Internationally: Chagas disease may cause a similar disorder. Internationally: Chagas disease may cause a similar disorder. Sex: The male-to-female ratio of achalasia is 1:1. Age: Achalasia typically occurs in adults aged years. Fewer than 5% of cases occur in children.

Mortality/Morbidity:Mortality/Morbidity: Failure of the LES to relax and decrements in esophageal peristalsis lead to stasis and the retention of foodstuffs and upper gastrointestinal secretions in the esophagus. The primary morbidity associated with this effect is esophagitis, which is readily recognized on endoscopic examination. The mass of pooled secretions and solid materials places the patient at risk for aspiration. According to Vantrappen and associates, as many as 30% of patients with achalasia report nocturnal coughing episodes Nocturnal coughing can lead to aspiration pneumonia, which is a serious complication. Patients with achalasia are at increased risk for esophageal carcinoma, compared with the general population. According to Wychulis et al and Lortat- Jacob et al, 2-7% of patients with achalasia have an esophageal carcinoma.Esophageal carcinoma and a greatly dilated esophagus are found more frequently in patients with long-standing disease than in patients with early- stage achalasia. Failure of the LES to relax and decrements in esophageal peristalsis lead to stasis and the retention of foodstuffs and upper gastrointestinal secretions in the esophagus. The primary morbidity associated with this effect is esophagitis, which is readily recognized on endoscopic examination. The mass of pooled secretions and solid materials places the patient at risk for aspiration. According to Vantrappen and associates, as many as 30% of patients with achalasia report nocturnal coughing episodes Nocturnal coughing can lead to aspiration pneumonia, which is a serious complication. Patients with achalasia are at increased risk for esophageal carcinoma, compared with the general population. According to Wychulis et al and Lortat- Jacob et al, 2-7% of patients with achalasia have an esophageal carcinoma.Esophageal carcinoma and a greatly dilated esophagus are found more frequently in patients with long-standing disease than in patients with early- stage achalasia.

DifferentialDifferential Amyloidosis Amyloidosis Chagas disease Chagas disease Collagen-vascular disease Collagen-vascular disease Lymphoma Lymphoma Pseudoachalasia Pseudoachalasia Diffuse esophageal spasm Diffuse esophageal spasm Esophageal candidiasis Esophageal candidiasis Diabetes mellitus Diabetes mellitus Amyloidosis Amyloidosis Chagas disease Chagas disease Collagen-vascular disease Collagen-vascular disease Lymphoma Lymphoma Pseudoachalasia Pseudoachalasia Diffuse esophageal spasm Diffuse esophageal spasm Esophageal candidiasis Esophageal candidiasis Diabetes mellitus Diabetes mellitus

History: Achalasia is characterized by the following symptoms and signs: Dysphagia (most common) Dysphagia (most common) Regurgitation Regurgitation Chest pain Chest pain Heartburn Heartburn Weight loss Weight loss Physical: Physical examination is noncontributory. Causes: The cause of achalasia is unknown. History: Achalasia is characterized by the following symptoms and signs: Dysphagia (most common) Dysphagia (most common) Regurgitation Regurgitation Chest pain Chest pain Heartburn Heartburn Weight loss Weight loss Physical: Physical examination is noncontributory. Causes: The cause of achalasia is unknown.

Lab Studies: are noncontributory.

Imaging Studies: Barium swallow Barium swallow The esophagus appears dilated, and contrast material passes slowly into the stomach as the LES opens intermittently. The distal esophagus is narrowed and has been described as resembling a bird’s beak. Barium swallow Barium swallow The esophagus appears dilated, and contrast material passes slowly into the stomach as the LES opens intermittently. The distal esophagus is narrowed and has been described as resembling a bird’s beak.

Other Tests: Esophageal manometry findings include the following: Esophageal manometry findings include the following: Incomplete relaxation of the LES in response to swallowing Incomplete relaxation of the LES in response to swallowing High resting LES pressure High resting LES pressure Absent esophageal peristalsis Absent esophageal peristalsis Prolonged esophageal pH monitoring is important for the following reasons: Prolonged esophageal pH monitoring is important for the following reasons: To rule out gastroesophageal reflux disease (GERD) To rule out gastroesophageal reflux disease (GERD) To determine if abnormal reflux is being caused by treatment To determine if abnormal reflux is being caused by treatment Esophageal manometry findings include the following: Esophageal manometry findings include the following: Incomplete relaxation of the LES in response to swallowing Incomplete relaxation of the LES in response to swallowing High resting LES pressure High resting LES pressure Absent esophageal peristalsis Absent esophageal peristalsis Prolonged esophageal pH monitoring is important for the following reasons: Prolonged esophageal pH monitoring is important for the following reasons: To rule out gastroesophageal reflux disease (GERD) To rule out gastroesophageal reflux disease (GERD) To determine if abnormal reflux is being caused by treatment To determine if abnormal reflux is being caused by treatment

Perform an EGD to rule out cancer of the GE junction. Endoscopic ultrasound is also an option. Perform an EGD to rule out cancer of the GE junction. Endoscopic ultrasound is also an option.

Medical Care: The goal of therapy for achalasia is to relieve symptoms by eliminating the outflow resistance caused by the hypertensive and nonrelaxing LES. The goal of therapy for achalasia is to relieve symptoms by eliminating the outflow resistance caused by the hypertensive and nonrelaxing LES. Calcium channel blockers and nitrates are used to decrease LES pressure. Calcium channel blockers and nitrates are used to decrease LES pressure. Approximately 10% of patients benefit from this treatment. Approximately 10% of patients benefit from this treatment. This treatment is used primarily in elderly patients who have contraindications to either pneumatic dilatation or surgery. This treatment is used primarily in elderly patients who have contraindications to either pneumatic dilatation or surgery. The goal of therapy for achalasia is to relieve symptoms by eliminating the outflow resistance caused by the hypertensive and nonrelaxing LES. The goal of therapy for achalasia is to relieve symptoms by eliminating the outflow resistance caused by the hypertensive and nonrelaxing LES. Calcium channel blockers and nitrates are used to decrease LES pressure. Calcium channel blockers and nitrates are used to decrease LES pressure. Approximately 10% of patients benefit from this treatment. Approximately 10% of patients benefit from this treatment. This treatment is used primarily in elderly patients who have contraindications to either pneumatic dilatation or surgery. This treatment is used primarily in elderly patients who have contraindications to either pneumatic dilatation or surgery.

Endoscopic treatment includes an intrasphincteric injection of botulinum toxin to block the release of acetylcholine at the level of the LES, thereby restoring the balance between excitatory and inhibitory neurotransmitters. Endoscopic treatment includes an intrasphincteric injection of botulinum toxin to block the release of acetylcholine at the level of the LES, thereby restoring the balance between excitatory and inhibitory neurotransmitters. This treatment has limited value. Only 30% of patient’s treated endoscopically still have relief of dysphagia 1 year after treatment. This treatment has limited value. Only 30% of patient’s treated endoscopically still have relief of dysphagia 1 year after treatment. This treatment can cause an inflammatory reaction at the level of the gastroesophageal junction, making a subsequent myotomy very difficult. This treatment can cause an inflammatory reaction at the level of the gastroesophageal junction, making a subsequent myotomy very difficult. Use this treatment in elderly patients who are poor candidates for dilatation or surgery. Use this treatment in elderly patients who are poor candidates for dilatation or surgery. Endoscopic treatment includes an intrasphincteric injection of botulinum toxin to block the release of acetylcholine at the level of the LES, thereby restoring the balance between excitatory and inhibitory neurotransmitters. Endoscopic treatment includes an intrasphincteric injection of botulinum toxin to block the release of acetylcholine at the level of the LES, thereby restoring the balance between excitatory and inhibitory neurotransmitters. This treatment has limited value. Only 30% of patient’s treated endoscopically still have relief of dysphagia 1 year after treatment. This treatment has limited value. Only 30% of patient’s treated endoscopically still have relief of dysphagia 1 year after treatment. This treatment can cause an inflammatory reaction at the level of the gastroesophageal junction, making a subsequent myotomy very difficult. This treatment can cause an inflammatory reaction at the level of the gastroesophageal junction, making a subsequent myotomy very difficult. Use this treatment in elderly patients who are poor candidates for dilatation or surgery. Use this treatment in elderly patients who are poor candidates for dilatation or surgery.

Pneumatic dilatation by a qualified gastroenterologist is the recommended treatment. Pneumatic dilatation by a qualified gastroenterologist is the recommended treatment. A balloon is inflated at the level of the gastroesophageal junction to blindly rupture the muscle fibers while leaving the mucosa intact. A balloon is inflated at the level of the gastroesophageal junction to blindly rupture the muscle fibers while leaving the mucosa intact. The success rate is 70-80%, and the perforation rate is approximately 5%. The success rate is 70-80%, and the perforation rate is approximately 5%. If a perforation occurs, emergency surgery is needed to close the perforation and perform a myotomy. If a perforation occurs, emergency surgery is needed to close the perforation and perform a myotomy. As many as 50% of patients may require more than 1 dilatation. As many as 50% of patients may require more than 1 dilatation. The incidence of abnormal gastroesophageal reflux after the procedure is approximately 25%. The incidence of abnormal gastroesophageal reflux after the procedure is approximately 25%. Pneumatic dilatation by a qualified gastroenterologist is the recommended treatment. Pneumatic dilatation by a qualified gastroenterologist is the recommended treatment. A balloon is inflated at the level of the gastroesophageal junction to blindly rupture the muscle fibers while leaving the mucosa intact. A balloon is inflated at the level of the gastroesophageal junction to blindly rupture the muscle fibers while leaving the mucosa intact. The success rate is 70-80%, and the perforation rate is approximately 5%. The success rate is 70-80%, and the perforation rate is approximately 5%. If a perforation occurs, emergency surgery is needed to close the perforation and perform a myotomy. If a perforation occurs, emergency surgery is needed to close the perforation and perform a myotomy. As many as 50% of patients may require more than 1 dilatation. As many as 50% of patients may require more than 1 dilatation. The incidence of abnormal gastroesophageal reflux after the procedure is approximately 25%. The incidence of abnormal gastroesophageal reflux after the procedure is approximately 25%.

Heller myotomy is the appropriate treatment for patients in whom pneumatic dilatation fails. Heller myotomy is the appropriate treatment for patients in whom pneumatic dilatation fails.

Surgical Care Because of excellent results, a short hospital stay, and fast recovery time, a laparoscopic Heller myotomy and partial fundoplication is considered the primary treatment. Because of excellent results, a short hospital stay, and fast recovery time, a laparoscopic Heller myotomy and partial fundoplication is considered the primary treatment. Under general anesthesia, make a controlled division of the muscle fibers (myotomy) of the lower esophagus (5 cm) and proximal stomach (1.5 cm) followed by a partial fundoplication to prevent reflux Under general anesthesia, make a controlled division of the muscle fibers (myotomy) of the lower esophagus (5 cm) and proximal stomach (1.5 cm) followed by a partial fundoplication to prevent reflux Patients remain hospitalized for hours and return to regular activities in about 2 weeks. Patients remain hospitalized for hours and return to regular activities in about 2 weeks. The operation relieves symptoms in 85-95% of patients, and the incidence of postoperative reflux is 10-15%. The operation relieves symptoms in 85-95% of patients, and the incidence of postoperative reflux is 10-15%. Patients in whom surgery fails may need a dilatation, a second operation, or removal of the esophagus (ie, esophagectomy). Patients in whom surgery fails may need a dilatation, a second operation, or removal of the esophagus (ie, esophagectomy). Because of excellent results, a short hospital stay, and fast recovery time, a laparoscopic Heller myotomy and partial fundoplication is considered the primary treatment. Because of excellent results, a short hospital stay, and fast recovery time, a laparoscopic Heller myotomy and partial fundoplication is considered the primary treatment. Under general anesthesia, make a controlled division of the muscle fibers (myotomy) of the lower esophagus (5 cm) and proximal stomach (1.5 cm) followed by a partial fundoplication to prevent reflux Under general anesthesia, make a controlled division of the muscle fibers (myotomy) of the lower esophagus (5 cm) and proximal stomach (1.5 cm) followed by a partial fundoplication to prevent reflux Patients remain hospitalized for hours and return to regular activities in about 2 weeks. Patients remain hospitalized for hours and return to regular activities in about 2 weeks. The operation relieves symptoms in 85-95% of patients, and the incidence of postoperative reflux is 10-15%. The operation relieves symptoms in 85-95% of patients, and the incidence of postoperative reflux is 10-15%. Patients in whom surgery fails may need a dilatation, a second operation, or removal of the esophagus (ie, esophagectomy). Patients in whom surgery fails may need a dilatation, a second operation, or removal of the esophagus (ie, esophagectomy).

Port Placement

The myotomy is performed at the 11 o'clock position. The myotomy is performed at the 11 o'clock position. The myotomy is started about 3 cm above the esophagogastric junction. Before it is extended upward and downward. The myotomy is started about 3 cm above the esophagogastric junction. Before it is extended upward and downward. The proper submucosal plane should be reached at a single point; in this way, the likelihood of subsequent mucosal perforation can be reduced. The proper submucosal plane should be reached at a single point; in this way, the likelihood of subsequent mucosal perforation can be reduced. The total length of the myotomy is typically about 8 cm. The total length of the myotomy is typically about 8 cm.

Dor Procedure - Dor Procedure - Anterior Partial Fundoplication The uppermost stitch in the first row incorporates the fundus, the esophageal wall, and the left pillar of the crus. - anterior 180° wrap

Dor Procedure - Dor Procedure - Anterior Partial Fundoplication The second and third stitches in the first row incorporate only the fundus and the left side of the esophageal wall..

Dor Procedure - Dor Procedure - Anterior Partial Fundoplication The uppermost stitch in the second row incorporates the fundus, the esophageal wall, and the right crus.

Dor Procedure - Dor Procedure - Anterior Partial Fundoplication The second and third stitches in the second row incorporate only the fundus and the right side of the esophageal wall. Two final stitches are placed between the superior portion of the wrap and the anterior rim of the hiatus.

Guarner Procedure: Posterior Partial Fundoplication - a posterior 220° fundoplication

Any perforation found should be repaired with 5-0 absorbable suture material, with interrupted sutures employed for a small perforation and a continuous suture for a larger one. When a perforation has occurred, an anterior fundoplication is usually chosen in preference to a posterior one because the stomach will offer further protection against a leak. Any perforation found should be repaired with 5-0 absorbable suture material, with interrupted sutures employed for a small perforation and a continuous suture for a larger one. When a perforation has occurred, an anterior fundoplication is usually chosen in preference to a posterior one because the stomach will offer further protection against a leak.

Postoperative Complications Delayed esophageal leakage Delayed esophageal leakage During the first 24 to 36 hours after operation During the first 24 to 36 hours after operation The characteristic signs are chest pain, fever. The characteristic signs are chest pain, fever. The diagnosis is confirmed by an esophagogram. The diagnosis is confirmed by an esophagogram. Treatment options depend on the time of diagnosis and on the size and location of the leak. Early, small leaks can be repaired directly. If the site of the leak is high in the chest, a thoracotomy provides the best approach for the repair; If the site of the leak is high in the chest, a thoracotomy provides the best approach for the repair; If the site is at the level of the GE junction, a laparotomy provides the best approach, and the stomach can be used to reinforce the repair. If the site is at the level of the GE junction, a laparotomy provides the best approach, and the stomach can be used to reinforce the repair. If the damage to the esophagus is too extensive to permit repair, a transhiatal esophagectomy If the damage to the esophagus is too extensive to permit repair, a transhiatal esophagectomy Delayed esophageal leakage Delayed esophageal leakage During the first 24 to 36 hours after operation During the first 24 to 36 hours after operation The characteristic signs are chest pain, fever. The characteristic signs are chest pain, fever. The diagnosis is confirmed by an esophagogram. The diagnosis is confirmed by an esophagogram. Treatment options depend on the time of diagnosis and on the size and location of the leak. Early, small leaks can be repaired directly. If the site of the leak is high in the chest, a thoracotomy provides the best approach for the repair; If the site of the leak is high in the chest, a thoracotomy provides the best approach for the repair; If the site is at the level of the GE junction, a laparotomy provides the best approach, and the stomach can be used to reinforce the repair. If the site is at the level of the GE junction, a laparotomy provides the best approach, and the stomach can be used to reinforce the repair. If the damage to the esophagus is too extensive to permit repair, a transhiatal esophagectomy If the damage to the esophagus is too extensive to permit repair, a transhiatal esophagectomy

Postoperative Complications Dysphagia may either persist after the operation or recur after a symptom-free interval. In either case, a complete workup is necessary, and treatment is individualized on the basis of the specific cause of dysphagia. Reoperation may be indicated. Dysphagia may either persist after the operation or recur after a symptom-free interval. In either case, a complete workup is necessary, and treatment is individualized on the basis of the specific cause of dysphagia. Reoperation may be indicated. Gastroesophageal reflux occurs in 7% to 20 % of patients after operation. Reflux should be treated with acid-reducing medications Gastroesophageal reflux occurs in 7% to 20 % of patients after operation. Reflux should be treated with acid-reducing medications Dysphagia may either persist after the operation or recur after a symptom-free interval. In either case, a complete workup is necessary, and treatment is individualized on the basis of the specific cause of dysphagia. Reoperation may be indicated. Dysphagia may either persist after the operation or recur after a symptom-free interval. In either case, a complete workup is necessary, and treatment is individualized on the basis of the specific cause of dysphagia. Reoperation may be indicated. Gastroesophageal reflux occurs in 7% to 20 % of patients after operation. Reflux should be treated with acid-reducing medications Gastroesophageal reflux occurs in 7% to 20 % of patients after operation. Reflux should be treated with acid-reducing medications