Cases reported "Esophageal Achalasia"

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1/34. Achalasia: diagnosis and management.

    Achalasia is a primary esophageal motor disorder of unknown cause that produces complaints of dysphagia, regurgitation, and chest pain. The current treatments for achalasia involve the reduction of lower esophageal sphincter (LES) pressure, resulting in improved esophageal emptying. calcium channel blockers and nitrates, once used as an initial treatment strategy for early achalasia, are now used only in patients who are not candidates for pneumatic dilation or surgery, and in patients who do not respond to botulinum toxin injections. Because of the more rigid balloons, the current pneumatic dilators are more effective than the older, more compliant balloons. The graded approach to pneumatic dilation, using the Rigiflex (boston Scientific Corp, boston, MA) balloons (3.0, 3.5, and 4.0 cm) is now the most commonly used nonsurgical means of treating patients with achalasia, resulting in symptom improvement in up to 90% of patients. Surgical myotomy, once plagued by high morbidity and long hospital stay, can now be performed laparoscopically, with similar efficacy to the open surgical approach (94% versus 84%, respectively), reduced morbidity, and reduced hospitalization time. Because of the advances in both balloon dilation and laparoscopic myotomy, most patients with achalasia can now choose between these two equally efficacious treatment options. Botulinum toxin injection of the LES should be reserved for patients who can not undergo balloon dilation and are not surgical candidates.
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2/34. Achalasia presenting as acute airway obstruction.

    Achalasia presenting as acute airway obstruction is an uncommon complication. We report the case of an elderly woman with previously undiagnosed achalasia who presented with acute respiratory distress due to megaesophagus. Emergency endotracheal intubation and insertion of a catheter into the esophagus, with continuous aspiration was required. Upon introduction of the esophageal catheter an abruptand audible air decompression occurred, with marked improvement of the clinical picture. Endoscopic injection of botulinum toxin was chosen as the definitive treatment with good clinical result. The pathophysiology of the phenomenon of esophageal blowing in achalasia is unclear, but different hypothetical mechanisms have been suggested. One postulated mechanism is an increase in upper esophageal sphincter (UES) residual pressure or abnormal UES relaxation with swallowing in achalasia patients. We reviewed the UES manometric findings in 50 achalasia patients and compared it with measurement performed in 45 healthy controls. We did not find any abnormalities in UES function in any of our achalasia patients group, or in the case under study. An alternative hypothesis postulates that airway compromise in patients with achalasia results from the loss UES belch reflex (abnormal UES relaxation during esophageal air distension), and in fact, an abnormal UES belch reflex was evidenced in our case.
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3/34. Manometric heterogeneity in patients with idiopathic achalasia.

    BACKGROUND & AIMS: In certain cases of achalasia, particularly those in early stages with minimal endoscopic or radiographic abnormalities, the diagnosis may rely on manometry, which is the most sensitive test for the disease. The aim of this study was to critically evaluate the manometric criteria in a population of patients with idiopathic achalasia. methods: Clinical histories and manometric recordings of 58 patients with idiopathic achalasia and 43 control subjects were analyzed with regard to esophageal body contraction amplitude, peristaltic effectiveness in terms of both completeness and propagation velocity, lower esophageal sphincter (LES) resting pressure, LES relaxation pressure, and intraesophageal-intragastric pressure gradient. Variants of achalasia were defined by finding manometric features that significantly differed from the remainder of achalasia patients, such that the diagnosis might be questioned. RESULTS: Four manometrically distinct variants were identified. These variants were characterized by (1) the presence of high amplitude esophageal body contractions, (2) a short segment of esophageal body aperistalsis, (3) retained complete deglutitive LES relaxation, and (4) intact transient LES relaxation. In each instance, the most extreme variant is discussed and compared with the remainder of the achalasia population and with controls. CONCLUSIONS: The significance in defining these variants of achalasia lies in the recognition that these sometimes confusing manometric findings are consistent with achalasia when combined with additional clinical data supportive of the diagnosis. Furthermore, such variants provide important clues into the pathophysiology of this rare disorder.
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4/34. Three-dimensional pressure imaging: a novel method for intraoperative manometry during laparoscopic esophagomyotomy for esophageal achalasia.

    With the introduction of computerized manometry, the creation of the three-dimensional (3-D) pressure image with measurement of pressure vector volume (PVV) has become available to assess the pressure structure of the high-pressure zone (HPZ) of the distal esophagus. We have applied this technique to intraoperative manometry during laparoscopic surgery for esophageal achalasia (EA). A laparoscopic esophagomyotomy with anterior fundoplication was performed in a 12-year-old boy with EA. Computerized 3-D images were obtained by a stepwise pullback of a manometric assembly with eight radial side-holes to quantitate the PVV of the HPZ of the distal esophagus in each stage of the operation for EA. Virtual abolishment of the peak pressure image and a marked reduction in the PVV after esophagomyotomy suggested appropriate decompression of the HPZ. Intraoperative manometry using computerized 3-D pressure imaging with measurement of the PVV is thus useful to assess the HPZ during laparoscopic surgery for EA.
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5/34. Abnormal elevation of resting pressure at the upper esophageal sphincter of Parkinson's disease patients.

    We investigated the oral and pharyngeal swallowing function in seven Parkinson's disease (PD) patients, using videomanofluorometry, which is videofluorographic and manometric evaluation conducted simultaneously. Abnormal elevations of resting pressure were found at the upper esophageal sphincter (UES) in three of the seven cases, when they were asked to hold a bolus in the mouth and initiate swallowing. One of these three cases showed an abnormal elevation of resting pressure, intermingled with a normal pressure pattern. Since the UES showed complete relaxation in these three cases, it is inappropriate to suppose that irreversible pathophysiological changes at the level of peripheral nerves had occurred. Our results suggested that altered resting pressure resulted from dysfunction at a more central level, such as a lack of dopaminergic stimulation at the supramedullary level causing skeletal muscle rigidity. Since a tonic abnormality of the UES cannot be measured by only videofluorography, both videofluorographic and manometric evaluation will be necessary to assess the pharyngeal phase of swallowing in PD patients.
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6/34. Treatment of achalasia with botulinum A toxin.

    Achalasia is an idiopathic neuromuscular disorder of the esophagus which is associated with absence of esophageal peristalsis and incomplete relaxation of a normal or raised lower esophageal sphincter (LES). Dysphagia is the most commonly associated symptom. Conventional therapeutic approaches are directed to reducing LES pressure and include orally-administered smooth muscle relaxants, forceful sphincter dilation with balloon dilators, and open or laparoscopic-assisted myotomy of the LES. Pharmacologic therapies have a low success rate. Forceful dilation has a perforation complication rate of 2% to 5%, and myotomies may precipitate significant gastroesophageal reflux, a complication minimized when a partial fundal wrap is employed simultaneously. In recent years, botulinum toxin, utilized widely as a striated muscle relaxant in managing blepharospasm, anal sphincter spasm, and muscle spasm complicating CVAs, and in smoothening facial wrinkles, has been extended to the management of achalasia on the basis that it impairs smooth muscle responsiveness to acetylcholine. Eighty units of Botox (botulinum toxin) are injected directly into the endoscopically (endoscopic ultrasound techniques may facilitate localization) located LES region (20 units into each of 4 quadrants). Symptom relief lasting 6 months on average is experienced in more than 65% of treated patients, and the complication rate is negligible. This therapeutic option is reserved for patients too ill to undergo any surgical procedure and is most effective when the lower esophageal region is hypertonic.
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7/34. Return of esophageal peristalsis in idiopathic achalasia.

    A 47-year-old male was diagnosed as having idiopathic achalasia on the basis of clinical, roentgenographic, and manometric criteria. He was on no medication and had no disorders known to impair esophageal motility. He was treated by pneumatic dilation with a good clinical response. On reexamination 7 years later, several features considered to be typical of achalasia were no longer present. Changes included return of peristaltic activity throughout most of the body of the esophagus, failure of a direct-acting cholinergic agent to produce an increase in base line intraesophageal pressure, and failure of a direct-acting cholinergic agent to produce a heightened response at the lower esophageal sphincter (LES). Incomplete LES relaxation in response to swallowing persisted. This represents the first reported case of return of esophageal peristalsis in idiopathic achalasia.
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8/34. Diffuse oesophageal spasm masking achalasia.

    Several reports have suggested that esophageal motility disorders may progress from one type to another. A 41-year-old female patient underwent thoracoscopic esophagomyotomy for diffuse esophageal spasm (DOS) with normal resting pressure and complete relaxation of the LOS; findings were confirmed in two preoperative esophageal manometries. Postoperatively, she developed severe dysphagia, and a new esophageal manometry concluded achalasia. She underwent a laparoscopic Heller's myotomy and a posterior (180 degrees) Toupet's fundoplication. Since the second operation, she remains asymptomatic and does not experience any difficulty in swallowing. We concluded that DOS and achalasia might coexist in this case. Through multiple synapses and several nervous roots in the esophageal wall, the inhibitory neurons at the level of LOS were effective before esophagomyotomy and did not show symptoms and manometric findings suggestive for achalasia. Esophagomyotomy, causing disruption of these synapses and lost of inhibitory innervation, finally resulted in symptoms and manometric findings of achalasia.
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9/34. Oesophageal achalasia causing respiratory obstruction.

    A patient with complete respiratory obstruction due to massive oesophageal dilatation is described. Immediate relief of symptoms and disappearance of the oesophageal swelling occurred after administration of sublingual glyceryl trinitrate. nitrates cause a reduction in the lower oesophageal sphincter pressure in patients with oesophageal achalasia and in this case it is presumed that spasm of the lower oesophageal sphincter had been a major factor preventing decompression of the dilated oesophagus.
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10/34. Long-term outcomes of laparoscopic heller cardiomyotomy without an anti-reflux procedure.

    Certain technical features of laparoscopic Heller cardiomyotomy (LHM) remain controversial, including the extent of the myotomy and the indication for an antireflux procedure. We completed a retrospective chart review of all patients who underwent LHM for achalasia at 1 tertiary care institution to review our institutional experience with LHM without an antireflux procedure. Forty patients underwent a LHM performed by 2 surgeons, 65% of whom had previous medical management (Botox: 12 patients, LES dilatation: 14). The operating time was significantly increased in patients with Botox injections (98.3 vs. 71.1 minutes, P = 0.005). There were 3 intraoperative complications (mucosal injury in 3 patients, 2 had Botox injections). Postoperative evaluation demonstrated a mean dysphagia score of 0.2, a mean heartburn score of 3.2, and a mean LES pressure of 6.32 mm Hg. Thirty-two patients are maintained on acid-suppressing medications with good control of reflux symptoms. LHM without an antireflux procedure achieves excellent clinical outcomes in most patients with achalasia regardless of previous medical management. Previous medical management may present a greater technical challenge and may place patients at increased risk of mucosal injury.
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