Cases reported "Esophageal Achalasia"

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1/112. leiomyosarcoma of the esophagus in a patient with chagasic megaesophagus: case report and literature review.

    leiomyosarcoma constitutes approximately 0.5% of the malignant neoplasias of the esophagus and its association with megaesophagus has not been described. We report on a case of a woman with dysphagia that was slowly progressive from the age of 19 due to chagasic megaesophagus. The woman was subjected to cardiomyotomy at the age of 49. She presented a rapid worsening of the dysphagia due to leiomyosarcoma at the age of 61, and was subjected to subtotal esophagectomy with cervical esophagogastroplasty. She developed pulmonary and hepatic metastases 14 months after surgery and died six months later.
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2/112. Achalasia and Down's syndrome: coincidental association or something else?

    Achalasia is an uncommon esophageal motor disorder. It has been associated with other diseases such as Parkinson's disease and depressive disorders, but coincidence of achalasia and Down's syndrome is rare. We report five cases of achalasia in Down's syndrome patients seen in our institution. Two of the five cases were diagnosed at pediatric age. Respiratory symptoms and growth retardation were the main clinical manifestations in pediatric patients, whereas adult patients mainly complained of dysphagia. Taking into account the prevalence rate of both disorders, the association seems higher than that expected by chance. The possible etiopathogenic implications of this association, as well as its clinical relevance, are discussed.
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3/112. 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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4/112. Secondary achalasia due to a mesenchymal tumour of the oesophagus.

    A rare case of a secondary achalasia or pseudoachalasia due to a mesenchymal tumour of the oesophagus is presented. A 67-year-old Caucasian man had symptoms including dysphagia, odynophagia, and weight loss for 8 months. Radiological examination revealed no signs of neoplasia but an exploratory laparotomy revealed the presence of a mesenchymal tumour of the oesophagus. Tucker's criteria constitute an important tool in the differential diagnosis of secondary achalasia from primary achalasia with clinical value, but in this case, exploratory laparotomy rather than non-invasive diagnostic procedures provided the final and definite diagnosis.
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5/112. Dysphagia with multiple autoimmune disease.

    myasthenia gravis (MG) and polymyositis (PM) are organ-specific autoimmune diseases. Occasional reports describe patients with clinical and pathologic features of both. Achalasia is idiopathic in nature, but autoimmune and inflammatory mechanisms have been proposed for this disorder as well. We describe a patient with dysphagia who was diagnosed at different points in time with all these three rare conditions. Despite at least putatively having immune mechanisms in common, an association between the three has not been previously described.
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6/112. Recurrent gastric carcinoma causing pseudoachalasia: case report.

    We report the case of a patient with a gastric remnant relapse of an antral carcinoma resected 5 years before and presenting with the clinical feature of a secondary achalasia (pseudoachalasia). In spite of the patient's 4-month history of dysphagia and weight loss that suggested a malignant lesion, barium swallow, repeated endoscopic biopsies and computed tomography (CT) scan of the upper abdomen did not reveal any abnormalities to indicate a recurrence. However, in the following months, because of worsening symptoms, a further CT scan was performed and revealed thickening of the cardia and gastric wall. The patient underwent an exploratory laparotomy that showed an unresectable lesion involving the gastric fundus, the diaphragm and penetrating into the mediastinum, and therefore a palliative jejunostomy was performed.
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7/112. Gastric adenocarcinoma mimicking achalasia in a 15-year-old patient: a case report and review of the literature.

    Although adenocarcinoma of the cardia is extremely rare in adolescent patients, the endoscopist should be alert to this disease in patients of any age with dysphagia, even if symptoms, and results of a barium study, upper endoscopy, and esophageal manometry are suggestive of primary achalasia, especially if family history is negative for achalasia. In addition, secondary achalasia should be suspected in patients who do not respond to therapy with botulinum toxin within 2 months. Because none of the mentioned tests can distinguish between primary achalasia and secondary forms due to carcinoma of the cardia, biopsy specimens should be obtained. It appears that, although there is a minimal risk for complications, a diagnostic procedure such as biopsy would be appropriate when the information obtained could be essential. In some cases EUS can be an additional diagnostic tool, because lesions of the submucosa and the surrounding area can be identified by EUS.
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8/112. Allgrove syndrome in adulthood.

    A 35-year-old man with a past history of achalasia developed progressive spastic tetraparesis, distal limb atrophy, dysarthria, and dysphagia. A clinical diagnosis of amyotrophic lateral sclerosis (ALS) was considered before neurophysiological investigation, which disclosed a polyneuropathy and a prolonged central conduction time. One year later, the patient developed dysautonomic symptoms. Following confirmation of adrenal insufficiency, a diagnosis of Allgrove syndrome was made. This is a rare case, and we emphasize its clinical similarity with ALS.
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9/112. Concomitant manifestation of achalasia and sphincter of oddi dysfunction.

    We report the case of a 44-year-old woman who suffered from biliary-type pain after cholecystectomy, dysphagia, and weight loss. Examinations revealed sphincter of oddi dysfunction (SOD) and achalasia. Complete relief of symptoms was achieved by endoscopic sphincterotomy and pneumatic dilation of the distal esophagus. This case report demonstrates for the first time the concomitant manifestation of two motility disorders of the upper gastrointestinal tract, i.e. achalasia and SOD. At present, any causal relationship seems speculative; however, both diseases were successfully treated using endoscopic procedures.
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10/112. Achalasia developing years after surgery for reflux disease: case reports, laparoscopic treatment, and review of achalasia syndromes following antireflux surgery.

    Two case reports demonstrate the paradoxical occurrence of achalasia many years after the successful surgical treatment of gastroesophageal reflux disease (GERD). These patients had remedial surgery laparoscopically. The three types of achalasia syndromes that can follow antireflux surgery are discussed. In type 1, primary achalasia is misdiagnosed as GERD and inappropriate antireflux surgery causes worsening dysphagia immediately after surgery without any symptom-free interval. In type 2, secondary iatrogenic achalasia is seen early after antireflux surgery and is characterized by the presence of stenosis and scar formation at the site of the fundic wrap. Although the motility studies resemble achalasia, the repair needs only to be taken down and refashioned when there is no response to balloon dilatation. In type 3, illustrated by the case reports, primary achalasia follows antireflux surgery after a significant symptom-free interval. There is complete absence of any stenosis or fibrosis of the esophagus and periesophageal tissues at remedial surgery. Moreover, surgical treatment of this condition needs to include esophageal myotomy.
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