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Query: UMLS:C0014848 (achalasia)
 2,804 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cardiospasm and megaesophagus are well known clinical and pathological entities but the cause remains obscure. Megaesophagus is probably congenital or developmental in origin. The majority of cases of cardiospasm can be successfully treated by forceful dilatation of the narrow area. Some cases of megaesophagus are best treated by a plastic surgical procedure which enlarges the esophageal gastric opening. Additional experience with this method is necessary before it can be correctly evaluated.
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PMID:Cardiospasm. 1814 84

A 6-month-old domestic shorthair female cat was presented with suspected diaphragmatic hernia (DH) that was later confirmed by thoracic radiography. The cat underwent exploratory celiotomy with a diaphragmatic rupture (DR) repair and recovered. Six days later, it was represented with vomiting and anorexia. Megaoesophagus (MO) and gastric dilatation were diagnosed by contrast radiography. A second celiotomy revealed no abnormalities and gastropexy was performed. Endoscopy demonstrated MO, oesophagitis and gastro-oesophageal reflux. MO persisted for several weeks and was an unexpected complication as no association between DR (or DH) and MO has never been described in the veterinary literature. The cat was treated medically with aggressive prokinetic and antacid therapy along with prolonged temporary oesophageal diversion (percutaneous endoscopic gastrostomy tube) with an excellent outcome.
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PMID:Transient megaoesophagus and oesophagitis following diaphragmatic rupture repair in a cat. 1824 47

Megaoesophagus resulting from achalasia is a rare but serious cause of airway obstruction. The exact aetiology remains unclear. Achalasia normally presents as weight loss, dysphasia and regurgitation but frequently considerable oesophageal distension can occur without complain and very rarely cause of achalasia presents with stridor or respiratory distress. The authors presents a 19 -year old young lady who had respiratory symptoms and had been treated as a cause of chronic asthma, was found by us to have oesophagus achalasia. She had complained of cough, dispneia and had a pulmonary function studies that showed a severe airway obstruction. After surgery the symptoms disappeared and she had a marked improvement in the flow-volume curve. Oesophagus achalasia should be considered as one of the differential diagnoses of airway obstruction.
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PMID:[Oesophagus achalasia: differencial diagnosis of asthma]. 1836 27

H-type tracheoesophageal fistula (TEF) may lead to chronic respiratory disease if the diagnosis is delayed. Long-standing fistula causes esophageal distention which is named as pneumoesophagus or megaesophagus and possibly affects the motility of the esophageal body which may also be encountered as a part of tracheoesophageal anomalies. Both dysmotility and megaesophagus may mimic achalasia radiologically and the patient may be advised an unnecessary esophagocardiomyotomy. The authors report a 15-year-old adolescent with H-type TEF who has been diagnosed during investigations for chronic respiratory disease due to presumptive diagnosis of achalasia. The authors emphasize that a complete anatomical and functional evaluation of the upper gastrointestinal tract should be done before recommending operation for achalasia in patients with chronic respiratory disease. H-type TEF should be investigated to avoid unnecessary cardiomyotomy.
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PMID:Achalasia-like findings in a case with delayed diagnosis of H-type tracheoesophageal fistula. 1858 87

Development of a megaesophagus with a sigmoid-shaped distal part in patients with achalasia--even in the course of successful myotomy with reduction of the resting pressure of the lower esophageal sphincter--is often the expression of an irreversible progression of the disease. Management of patients with end-stage achalasia and aperistaltic, dilated "burned-out" esophagus--with or without peptic stenosis--is a therapeutic challenge for gastroenterologists and surgeons. We report on a 37-year-old female patient with decompensated dolichomegaesophagus following multiple endoscopic and operative interventions at the lower and upper esophageal sphincters presenting with severe dysphagia and weight loss. Esophageal resection with gastric tube pull-up was indicated and performed as a two-stage procedure with ischaemic preconditioning of the esophageal substitute, with the intention of a microcirculatory improvement of the gastric fundus for the anastomosis, which was thought to be potentially compromised due to the previous interventions. The postoperative course was uneventful with dysphagia completely relieved and quality of life markedly improved. The concept of ischaemic preconditioning of the gastric tube offers, in particular, patients with esophageal resection for benign motility disorders, such as end-stage achalasia after multiple surgical procedures at the cardia and gastric fundus, a safe and practicable alternative to one-stage esophageal resection and reconstruction.
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PMID:[Two-stage esophageal resection with ischemic preconditioning of the gastric tube in decompensated dolichomegaesophagus and end-stage achalasia]. 1901

Achalasia is a neurodegenerative condition characterized by esophageal dysmotility and megaesophagus. Two cases are reported that demonstrate unexpected deaths associated with previously unsuspected achalasia. Case 1: A 66-year-old woman was found dead at her home. At autopsy significant stenosing coronary artery atherosclerosis was found with cardiac failure. In addition, a striking finding was narrowing of the distal esophagus with marked proximal dilatation. The esophagus was completely filled with a large amount of soft masticated food and was bulging anteriorly, compressing the left atrium. Death was attributed to ischemic heart disease complicated by previously unsuspected achalasia. Case 2: An 84-year-old man collapsed and suffered a respiratory arrest while eating. Internal examination revealed narrowing of the cardioesophageal junction with marked proximal dilatation of the esophagus that contained approximately 50 mL of soft semi-fluid masticated yellow food paste. Fragments of yellow masticated food remnants were present in upper and lower airways but not within the stomach. There was a history of dementia with symmetrical cerebral ventricular dilatation found at autopsy. Death was attributed to food asphyxia complicating previously unsuspected achalasia with dementia. Megaesophagus may, therefore, be a significant finding at autopsy that may either be a primary cause of unexpected death or else may exacerbate or compound the effects of pre-existing underlying disease.
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PMID:Megaesophagus and possible mechanisms of sudden death. 1901 34

Achalasia cannot be cured. Instead, our goal is to relieve symptoms of dysphagia and regurgitation, improve esophageal emptying and prevent the development of megaesophagus. The most definitive therapies are pneumatic dilation and surgical myotomy. The overall success of grade pneumatic dilation is 78%, with women and older patients performing best. Laparoscopic myotomy has an overall success rate of 85%, but can be complicated by the sequelae of severe acid reflux disease. Young patients, especially men, are the best candidates for surgical myotomy. There are no prospective, randomized studies comparing these two procedures. Botulinum toxin injections into the esophagus and smooth muscle relaxants are reserved for older patients or those with major comorbid illnesses. Some patients with end-stage achalasia will require esophagectomy.
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PMID:Update on the management of achalasia: balloons, surgery and drugs. 1907 91

Megaesophagus is the end-stage of achalasia cardiae. It is the result of peristaltic disorders and slow decompensation of the muscular layer of the esophagus. The aim of this article is to detail the diagnostic criteria and surgical management of megaesophagus. Criteria were acute bending of esophagus axis; lack of esophagus peristalsis, and no response to stimulation in the manometric test; and Los Angeles C/D esophagitis in the endoscopic examination. Between 1991 and 2004 seven patients (5 females, 2 males; age, 51-67 years; average age, 59 +/- 8 years) were treated. A bypass made from the pedunculated part of the jejunum connecting the part of esophagus above the narrowing with the praepyloric part of the stomach was made. Access was by an abdominal approach. A jejunum bypass was made in six patients with megaesophagus. A transhiatal esophageal resection was carried out, and in the second stage a supplementary esophagus was made from the right half of the colon on the ileocolic vessels in one patient who had experienced two earlier unsuccessful operations. Symptoms of dysphagia, recurrent inflammation of the respiratory tract, and pain subsided in all patients. Complications were not reported in the postoperative period. All patients survived. Subsequent radiographic and endoscopic examination showed very good outcome. The jejunum bypass gave very good results in the surgical treatment of megaesophagus.
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PMID:Diagnostic criteria and surgical procedure for megaesophagus--a personal experience. 1920 50

Megaoesophagus, or oesophageal achalasia, is a neuromuscular disorder characterized by an absence of peristalsis and flaccid dilatation of the oesophagus, resulting in the retention of ingesta in the dilated segment. The aetiology and pathogenesis of idiopathic (or primary) megaoesophagus are still poorly understood and very little is known about the genetic causes of megaoesophagus in humans. Attempts to develop animal models of this condition have been largely unsuccessful and although the ICRC/HiCri strain of mice spontaneously develop megaoesophagus, the underlying genetic cause remains unknown. In this report, we show that aged Rassf1a-null mice have an enhanced susceptibility to megaoesophagus compared with wild-type littermates (approximately 20%vs. approximately 2% incidence respectively; P = 0.01). Histological examination of the dilated oesophaguses shows a reduction in the numbers of nerve cells (both ganglia and nerve fibres) in the myenteric plexus of the dilated mid and lower oesophagus that was confirmed by S100 immunohistochemistry. There was also a chronic inflammatory infiltrate and subsequent fibrosis of the myenteric plexus and the muscle layers. These appearances closely mimic the gross and histopathological findings in human cases of megaoesophagus/achalasia, thus demonstrating that this is a representative mouse model of the disease. Thus, we have identified a genetic cause of the development of megaoesophagus/achalasia that could be screened for in patients, and may eventually facilitate the development of therapies that could prevent further progression of the disease once it is diagnosed at an early stage.
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PMID:Megaoesophagus in Rassf1a-null mice. 1933 48

Heller myotomy is recognized as the optimal treatment for achalasia. However, treatment of the markedly dilated esophagus has been debated in the literature. Although esophagectomy has been the standard treatment historically, several studies have examined successful treatment of achalasia with laparoscopic Heller myotomy in the setting of a markedly dilated esophagus (>6 cm). Patients with extreme megaesophagus (>10 cm) are often treated with esophagectomy. We report the successful treatment of 4 patients with extreme megaesophagus with laparoscopic Heller myotomy. Three of the 4 patients also had Toupet fundoplication. The average esophageal diameter was 11.2 cm (10 to 12 cm). In addition to severe dysphagia, all patients had preoperative signs, symptoms, and radiographic evidence of esophageal compression of their heart and lungs. All patients reported relief of their preoperative symptoms. Esophagectomy has not been required to maintain adequate clinical results in any of our patients. We conclude that laparoscopic Heller myotomy is an appropriate alternative to esophagectomy and can be offered to patients with extreme megaesophagus.
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PMID:Results of laparoscopic Heller myotomy for extreme megaesophagus: an alternative to esophagectomy. 1954 45


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