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

The term ultrashort Hirschsprung's disease has been used to define a spectrum of conditions with clinical presentation similar to Hirschsprung's disease but with presence of ganglion cells on rectal biopsy. In contrast to Hirschsprung's disease, there is no transition zone on barium enema. However, as in classical Hirschsprung's disease, there is no reflex internal sphincter relaxation on rectal manometry. We reviewed the presentation of five patients with chronic constipation who fulfilled the criteria for ultrashort Hirschsprung's disease. After positive anorectal manometry, despite the presence of ganglion cells on suction rectal biopsy, internal sphincter myomectomy was performed in four patients. Resolution of symptoms was noted in all operated patients. Normal ganglion cells were present throughout the entire length of all myomectomy specimens. There is controversy in the literature concerning the diagnosis of ultrashort Hirschsprung's disease. Most of the confusion concerns whether a short segment of aganglionosis proximal to the dentate line is permissible for the diagnosis of ultrashort Hirschsprung's disease and to what extent such aganglionosis is physiological. Perhaps it would be more accurate to define this entity by the presence of ganglion cells on rectal biopsy as well as the failure of the internal sphincter to relax on rectal manometry and to describe it as anorectal achalasia in severely constipated patients.
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PMID:Ultrashort Hirschsprung's disease: myth or reality. 227 26

A case report of an adolescent female with esophageal achalasia and behavioral features of anorexia nervosa is presented. This unusual combination of problems may cause similar physical symptoms and thus create diagnostic confusion.
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PMID:Achalasia in an adolescent with behavioral features compatible with anorexia nervosa. 649 Apr 84

Achalasia is an incurable neuromuscular disorder of the esophagus, resulting from destruction of the esophageal myenteric plexus. This leads to aperistalsis and failure of the lower esophageal sphincter to relax after swallowing. Symptoms of achalasia are gradual in onset and include dysphagia, regurgitation, and weight loss. Severe malnutrition can ensue. Wernicke's encephalopathy (WE) is a serious, potentially fatal, neurologic disorder caused by thiamine deficiency (vitamin B(1)), classically described as presenting with a triad of ocular abnormalities, ataxia, and confusion. The incidence is uncertain, and many cases likely go unrecognized. It is usually diagnosed in the alcoholic population. We describe its onset after the successful surgical treatment of achalasia.
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PMID:Wernicke's encephalopathy after laparoscopic cardiomyotomy for achalasia. 1759 43

Wernicke's encephalopathy is a neurological disorder caused by thiamine (vitamin B1) deficiency characterized by vertigo, ataxia, and mental confusion. Wernicke's encephalopathy has a causative association with alcoholism but recently there has been an increased prevalence also in other clinical conditions. In literature potentially fatal Wernicke's encephalopathy onset in an advanced achalasia has been previously reported only once. We describe for the first time an improvement of achalasic symptoms in a young patient affected by end-stage achalasia and anorexia nervosa (coming from ineffective Heller-Dor myotomy) after vitamin B1 supplementation. This case report suggest a potential positive impact of B1 supplementation on end-stage achalasic patients and requires systematic studies to confirm this observation.
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PMID:A case of vomiting in an anorexic achalasic patient. 2309 Aug 6