Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0014848 (achalasia)
 2,804 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Diffuse esophageal leiomyomatosis is characterized by diffuse muscular hypertrophy chiefly marked in the lower end of the esophagus looks at a large tumor. It involves the entire length of the esophagus and down to the upper part of the stomach (esogastric leiomyomatosis). Diffuse leiomyomatosis is a extremely rare pathology encountered in children (13 previous report cases) and young adults. Il may be isolated or found in association with other intrathoracic and genital localisations or Alport syndrome (nephropathy, sensorineural deafness, ocular lesions). Extensive leiomyomatosis, engulfing the trachea and stem bronchi, may involve acute respiratory insufficiency. Genital localisations, exclusively in women (clitoral hypertrophy, vulvar leiomyomatosis), in association with esophageal leiomyomatosis, realize the esophago vulvar syndrome. Recently, familial diffuse leiomyomatosis cases were described with, in association, esophageal and extra-esophageal leiomyomatosis and Alport syndrome. The majority of the patients developed esophageal or respiratory symptoms. The radiological appearance is that of a mediastinal tumor or achalasia. CT Scans findings can give evidence diffuse muscular thickening of esophageal wall. Prognosis depends on the associated lesions. In that reports, two young adults (27 and 39 years old) died of inhabitual carcinomas (esophageal and gallbladder carcinoma). The only surgical treatment for symptomatic esogastric leiomyomatosis is subtotal esophagectomy with proximal gastrectomy and esocoloplasty. Myotomy is ineffective (2 cases). Five cases of esophageal leiomyomatosis are described (3 children, 2 young adults). Among these, two are familial leiomyomatosis cases. An esophageal resection was performed in four patients.
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PMID:[Diffuse esophageal leiomyomatosis. Apropos of 5 cases with 2 familial cases]. 261 74

Although esophagomyotomy is highly effective as the initial surgical treatment of most patients with achalasia, those with either recurrent symptoms after a previous esophagomyotomy or a megaesophagus do not respond as well to esophagomyotomy. Total thoracic esophagectomy was performed in 26 patients (average age, 49 years) with achalasia. Eighteen had a history of a previous esophagomyotomy, and 18 had a megaesophagus (esophageal diameter of 8 cm or larger). In 24 patients, a transhiatal esophagectomy without thoracotomy was the operative approach; 2 patients required a transthoracic esophagectomy because of intrathoracic adhesions from prior operations. The stomach was used as the esophageal substitute in all patients; it was positioned in the posterior mediastinum, and a cervical anastomosis was performed. Intraoperative blood loss averaged 765 mL. Major postoperative complications included mediastinal bleeding requiring thoracotomy (2), chylothorax (2), and anastomotic leak (1). There were no postoperative deaths. The average postoperative hospital stay was ten days. Follow-up is complete and ranges from 3 to 91 months (average duration, 30 months). All but 1 patient with severe psychiatric disease eat a regular, unrestricted diet without postprandial regurgitation. Early postoperative anastomotic dilation was required in 10 patients. Dumping syndrome has occurred in 5 patients. It is concluded that esophagectomy provides the most reliable treatment of esophageal obstruction, pulmonary complications, and potential late development of carcinoma in the patient with a megaesophagus of achalasia or a failed prior esophagomyotomy and that it is a far better option in these patients than esophagomyotomy, cardioplasty procedures, or limited esophageal resection.
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PMID:Esophageal resection for achalasia: indications and results. 264 31

Late results in 81 patients with achalasia treated in a prospective randomised study comparing forceful pneumatic dilatation with the Mosher bag and surgical anterior oesophagomyotomy by abdominal route, are reported. There were no deaths from either of the treatments. Two patients (5.6%) had a perforation of the abdominal oesophagus after pneumatic dilatation and were excluded from late follow up. In patients having surgery at radiological evaluation there was gullet diameter significantly increased at the oesophagogastric junction and decreased at the middle third of the oesophagus. One patient was lost from follow up and one died of an oesophageal carcinoma, leaving 95% of excellent results at the late follow up (median 62 months). Resting gastro-oesophageal sphincter pressure decreased significantly to approximately 10 mmHg; this was maintained five years after surgery. By contrast, in patients having pneumatic dilatation, there were good results in only 65% (follow up median 58 months), with 30% failures. One patient was lost from follow up and one developed oesophageal carcinoma. Measurement of resting gastro-oesophageal sphincter pressure after dilatation was highly predictive of the outcome. The study shows that surgical treatment offers a better final clinical result than pneumatic dilatation with the Mosher bag.
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PMID:Late results of a prospective randomised study comparing forceful dilatation and oesophagomyotomy in patients with achalasia. 280 99

We present the case of a 78-year-old patient who developed epidermoid carcinoma of the esophagus 5 years after Haller myotomy for achalasia, which produced an improvement in the clinical symptoms, as well as in the radiological, endoscopic and manometric manifestations. The appearance of carcinoma after a moderate period of time underlines the need for continued periodic follow-up of these patients.
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PMID:[Epidermoid carcinoma of the esophagus secondary to achalasia]. 266 74

Endoscopic ultrasonography (EUS) was performed prospectively in 38 patients with various esophageal disorders. Twenty-four had a histologically proven carcinoma and EUS was done to assess its ability in preoperative staging. In 9 of 24 patients (37.5%), tumor stenosis could be passed with the endoscope and EUS preoperative findings regarding tumor extension and the presence of enlarged periesophageal lymph nodes were confirmed in those operated on (n = 4). In five patients with achalasia, a periesophageal tumor was reliably excluded by EUS. In one of four patients with Barrett's esophagus, EUS demonstrated a small (less than 1 cm) carcinoma that could not be visualized with any conventional technique. For differentiation of cancer recurrence after operation from periesophageal scar tissue EUS-guided transmural biopsies are needed. Our experience shows that at the present time EUS is the most reliable method to demonstrate small (less than or equal to 1 cm) intra- and extramural esophageal lesions and that it should therefore be applied early in the work-up of patients with dysphagia.
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PMID:Endoscopic ultrasonography in esophageal diseases. 266 1

In the US, the cumulative lifetime risk of developing carcinoma of the upper gastrointestinal tract is less than 1 per cent, premalignant conditions are uncommon, and esophageal and gastric malignancies are rarely curable even when identified early. Endoscopic screening of the upper gastrointestinal tract in asymptomatic persons thus cannot be justified. Surveillance of persons with certain uncommon conditions associated with a higher risk of upper gastrointestinal cancer may be of benefit. These conditions include achalasia, Barrett's esophagus, chronic atrophic gastritis with intestinal metaplasia, familial polyposis coli, gastric polyps, lye stricture, Plummer-Vinson syndrome, and tylosis. In the lower gastrointestinal tract, however, the lifetime risk of developing carcinoma is 5 per cent, premalignant conditions and lesions are common, and carcinoma is curable when detected at an early stage. Sigmoidoscopic screening of asymptomatic adults has been advocated by the American Cancer Society but has not become widely practiced because of its cost, required physician effort, low overall yield, and poor patient compliance. Surveillance by flexible sigmoidoscopy is recommended for persons at slightly increased risk of colorectal carcinoma who have prior breast or gynecologic malignancy or a family history of colorectal malignancy. Colonoscopic surveillance is recommended for patients with high risk of colorectal cancer who have had prior colorectal carcinoma or adenoma or who have inflammatory bowel disease or a ureterosigmoidostomy.
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PMID:Endoscopic screening and surveillance for gastrointestinal malignancy. 268 51

Esophageal involvement in systemic amyloidosis is common. Manometric studies have been few in number and have revealed a variety of findings compatible with deposition of amyloid in myopathic as well as neuropathic patterns. This report describes a patient with primary amyloid whose esophageal dysfunction was limited to the lower esophageal sphincter, with both a hypertensive sphincter and impaired relaxation after swallows. Pharmacologic testing confirmed a mild abnormality of the inhibitory pathway to the LES with an intact excitatory pathway and sphincter muscle. This case suggests that amyloidosis, like idiopathic achalasia and carcinoma of the lower esophagus, can produce dysphagia by selective impairment of the inhibitory neural pathway to the lower esophageal sphincter.
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PMID:Amyloidosis mimics achalasia's effect on lower esophageal sphincter. 270 92

Secondary or "pseudo" achalasia of the esophagus can mimic idiopathic achalasia radiographically and can be difficult to diagnose. Typically, it is due to invasive carcinoma involving the gastroesophageal junction, usually gastric adenocarcinoma. Occasionally, an achalasialike condition can be produced by tumors not involving the gastroesophageal junction. We report 2 cases, 1 of lung carcinoma and the other of hepatoma, in which the patients had radiographic and endoscopic changes compatible with achalasia. However, the onset of symptoms was abrupt and the patients were elderly; these are unusual features for primary achalasia. There have been several other reports of nongastrointestinal neoplasms producing a clinical and radiographic picture similar to achalasia. Although there are several theories as to the cause, our cases would support the concept that direct tumor involvement of the gastroesophageal junction is not necessary to produce significant esophageal dysmotility.
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PMID:Achalasia secondary to nongastrointestinal malignancies. 299 89

Respiratory symptoms due to compression of the trachea by the dilated esophagus in achalasia are extremely rare. A patient is presented whose respiratory manifestations included engorged neck veins and a neck swelling that fluctuated with respiration. He also had two malignant tumors in his dilated esophagus, a squamous cell carcinoma and an adenoid cystic carcinoma.
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PMID:Acute thoracic inlet obstruction in achalasia with adenoid cystic and squamous cell carcinoma. 299 91

The principal radionuclide procedures involved in the evaluation of esophageal disorders that are amenable to surgery are illustrated and briefly described. The role of the radionuclide esophagogram (RE) in the diagnosis and management of achalasia, oculopharyngeal muscular dystrophy and its complications, tracheoesophageal fistulae, pharyngeal and esophageal diverticulae, gastric transposition, and fundoplication is discussed. Detection of columnar-lined esophagus by Tc-99m pertechnetate imaging and of esophageal carcinoma by Ga-67 citrate and Tc-99m glucoheptonate studies also is presented.
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PMID:Nuclear medicine and esophageal surgery. 301 86


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