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)

Cancer excepted all other diseases of the esophagus are rare. Diverticula, benign tumors, perforations and the pathology of the cardia (hiatus hernia, achalasia and esophageal varices) are not studied here. We took into consideration the following diseases only: spasm of the cricopharyngeal muscle, Plummer-Vinson or Kelly-Paterson syndrome, cervical osteophytosis, dysphagia lusoria, benign and malignant mediastinal lymphatic nodes, Schatzki ring of the lower esophagus and esophageal duplications.
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PMID:[Some rare diseases of the esophagus (author's transl)]. 22 8

A 19-year-old man first presented with clinical, radiological, and manometric features of diffuse spasm. Within a year his motility disorder progressed to "vigorous" and, finally, classic achalasia. After pneumatic dilatation and subsequent long myotomy, the features of classic achalasia disappeared and he again exhibited radiological and manometric evidence of "vigorous" achalasia. The evolution of this patient's disease provides evidence that diffuse spasm and achalasia are different stages of the same disease and lie at opposite ends of a spectrum of related esophageal motility disorders.
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PMID:Transition from diffuse esophageal spasm to achalasia. 26 36

Most investigators agree that the most important goal in correcting gastroesophageal reflux is restoring or developing a competent lower esophageal sphincter. Although the sphincter can be incompetent in its normal intra-abdominal position and rarely a patient may have a competent sphincter in the thorax, generally the sphincter is much more effective in the positive pressure abdominal position. The choice of operative technique will depend upon the abnormal conditions present and the general condition of the patient. The thoracic approach is elected if there is associated intrathoracic disease warranting surgical correction, such as diffuse spasm of the esophagus, achalasia, epiphrenic diverticulum, or a pulmonary lesion requiring biopsy and possible resection. Very obese patients, patients with recurrent hernias, and patients with shortened esophagus are better managed by the thoracic approach. Patients with an essentially normal esophagus are treated with a Mark IV Belsey procedure. If shortening of the esophagus is present, a combination Collis-Nissen technique with fixation below the diaphragm is preferable. The abdominal approach is indicated when there is another intraabdominal disease known or suspected warranting surgical correction. This approach is also useful for the thin or poor risk patient. Usually, through an abdominal incision, we elect to use a modified Nissen fundoplication, with fixation of the fundoplication to the median arcuate ligament or the right crus of the diaphragm. The crural sling is returned to normal dimensions with interrupted sutures. Reflux in the absence of an hiatal hernia initially is treated medically. If symptoms are significant and intractable, a competent lower esophageal sphincter is restored, or developed by the modified Nissen procedure just described. Most reflux strictures at the esophagogastric junction are reversible by dilatation and restoration of a competent sphincter. Firm, fixed, fibrous strictures occasionally cannot be safely dilated. These may be managed by a Thal procedure to correct the stricture and a Nissen fundoplication to prevent recurrent reflux.
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PMID:Surgical treatment of gastroesophageal reflux. 39 Jul 43

From 1972 to 1977, the authors observed 156 patients with primary esophageal motility disorders which caused such a severe degree of dysphagia that treatment by pneumatic dilatation was deemed necessary. Before dilatation, 24% of the patients presented with motility disorders that did not fit well into the two classical disease entities, diffuse esophageal spasm and achalasia (absence of peristalsis with presence of lower esophageal sphincter (LES) relaxations or presence of peristalsis with absence of LES relaxations). After treatment with pneumatic dilatation, these "intermediate" forms constituted 45% of the motor disorders. This was due mainly to the reappearance, on manometric tracings, of peristaltic contractions and of LES relaxations. Radiologic and manometric observations suggest that in many patients, this "return of peristalsis" may be an apparent change in pressure pattern rather than a real change in motility. In 6 of the 156 patients, a deterioration of the esophageal motility disorder was observed, which was characterized by the loss of peristalsis and of LES relaxations over a period of a few months or years. The frequent occurrence of intermediate types of motility disorders and the transition from diffuse spasm to achalasia suggest that achalasia and diffuse esophageal spasm are part of a spectrum of related motor disorders.
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PMID:Achalasia, diffuse esophageal spasm, and related motility disorders. 42 3

Six patients with myelomeningocele and the Arnold-Chiari malformation developed cricopharyngeal achalasia and lower cranial nerve deficits. Diagnosis is established by cine-esophagram. Distortion of the brain stem or cranial nerves secondary to the Arnold-Chiari malformation may produce the autonomic imbalance necessary for cricopharyngeal achalasia. Treatment is supportive and includes verification of cerebral spinal fluid shunt function. Suboccipital craniectomy may reverse progressive lower cranial nerve deficits and reduce cricopharyngeus spasm. Cricopharyngeal myotomy may be considered when the cranial nerve deficits and cricopharyngeal achalasia are fixed, irreversible, and continue to cause disability.
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PMID:Cricopharyngeal achalasia associated with Arnold-Chiari malformation in childhood. 63 1

The use of endoscopic procedures in the evaluation of primary motor disorders, or functional diseases, of the esophagus is filled with both risks and benefits. Since both flexible and open-tube esophagoscopy carry a significant risk factor, it is necessary to have a clear concept of the indications and value of endoscopy in the management of functional diseases of the esophagus. A review of the literature reveals very little documentation on the value of endoscopy in diagnosing esophageal functional diseases other than Zenker's diverticulum and achalasia. Based on the current literature and the experience of the authors, observations and recommendations concerning the role of endoscopy in functional diseases of the esophagus are presented. These are: 1) In Phase I or upper esophageal sphincter dysfunctions, endoscopy contributes little to their understanding, is difficult to perform, and may be hazardous. In this group, esophagoscopy should be reserved for indications beyond the dysfunction itself. If endoscopy has to be performed, open-tube esophagoscopy should be performed by an experienced endoscopist. 2) In functional diseases of the esophageal body or Phase II dysfunction, endoscopy is frequently valuable. In spastic disorders, it helps to differentiate between primary spasm of neuromuscular origin and spasm secondary to esophagitis or an obstructive process. In scleroderma and pulsion diverticulum, endoscopy helps to identify such unsuspected complications as esophagitis, hiatal hernia, and carcinoma. 3) In Phase III or however esophageal sphincter dysfunctions, endoscopic examination is essential both to rule out organic lesions that stimulate functional disorders, and to determine the presence and extent of esophagitis.
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PMID:Functional diseases of the esophagus: role of endoscopy. 68 97

Anal fissures belong to the autonomic diseases. A raised sympathicotonus (spasm dystrophy) is responsible for the origin, the poor healing tendency and the burning pain. This excessive effect of the sympathetic leads first to functional and later to organic disorders of the sphincter in the form of an achalasia. The empirically developed methods of operation have intervention in the autonomic nervous system in common, and only differ in the extent of this "invasion of the autonomic". Functional changes must be treated with local anesthetics or by stretching the sphincter, organic changes by sphincterotomy.
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PMID:[Sphincter surgery. Etiology and therapy of the anal fissure (author's transl)]. 82 52

The characteristic esophageal pressure tracings and radiological appearances of diffuse spasm and achalasia are reported. In two patients of the series spastic activity involves the gastroesophageal junction. The findings indicate that differentiation of so-called cardiospasm and achalasia can only be obtained by simultaneous electromanometric pressure measurement and serial film technique. The resulting surgical methods are briefly reviewed.
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PMID:[Functional studies on the differential diagnosis of so-called cardiospasm and esophageal achalasia. Simultaneous electromanometric and serial radiographic studies]. 115 11

Nine cases of congenital achalasia of the cardia in children are reported, of which four were below 1 year old and recording the youngest age of 3 months. A hereditary element is present in four children. Association with Hirschsprung's disease of the colon in one case and with hiatal hernia in another case, favours a more generalized neuromuscular pathology than a simple spasm of the cardia.
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PMID:Congenital achalasia of the oesophagus in children. 117 20

Twenty-four patients underwent combined Collis-Belsey reconstruction of the esophagogastric junction. The primary indication for operation in 19 patients was gastroesophageal reflux. Three patients had achalasia, one diffuse spasm, and one an incarcerated combined sliding and paraesophageal hernia. Postoperatively, symptoms were relieved in all 19 patiients undergoing repair for gastroesophgeal reflux with or without peptic strictures of the esophagus, and barium swallows showed no gastroesophageal reflux. Preoperative average mean and peak pressures in the distal esophageal high pressure zone (HPZ) were 1.38 and 2.72 mm. Hg, respectively; two thirds had no measurable HPZ. Postoperative mean and peak pressures were 6 and 12.36 mm. Hg, respectively; average HPZ length was 2.81 cm. Of 19 patients with massive reflux preoperatively, postoperative acid reflux testing demonstrated no reflux in 14 and minimal to moderate reflux in five. Collis-Belsey reconstruction ot the esophagogastric junction effectively relieves symptoms and controls the complications of gastroesophageal reflux.
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PMID:Collis-Belsey reconstruction of the esophagogastric junction. Indications, physiology, and technical considerations. 124 55


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