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

Severe chronic constipation is an extremely rare disorder. Out of 39 370 patients seen in our hospital during the past two years only 309 children (=0,78%) were suffered from severe chronic constipation. Radiologic and electromanometrical examinations revealed as an organic cause anal sphincter achalasia or Hirschsprung's disease in 30,7% of these constipated children. In 54,9% however no pathomorphologic results could be found. Of all cases 14,4% examined were postoperative controls. Careful electromanometric and defecographic examination of every chronically constipated child is crucially important in determining the therapy as in most cases chronic constipation in childhood, is due to a functional asynchronism of the internal and external anal sphincter relaxation reflexes, it can most reliably be diagnosed by electromanometrical examinations. The accuracy and reliability of our electromanometrical method has been proved not only by comparison with radiologic and histologic techniques but also statistically by analysis of discriminance of the recorded parameters. The therapeutic approach depends on the underlying disease. Mechanical obstruction of the intestine, nervous dysregulation of colonic motility causing Hirschsprung's disease and organic anal sphincter achalasia require surgical treatment. In cases of achalasia myotomia of the internal anal sphincter gives excellent functional results. In 73 cases treated by myotomy no symptoms of anal incontinence were seen. Medical therapy includes administration of laxatives combined with high roughage diet, adjustment of living conditions including increased physical activity and bowel training in order to learn a conditioned defecation reflex. The matter can be more easily achieved by administering Dihydroergotamine and Lactulose initially.
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PMID:[Diagnosis and therapy of chronic constipation]. 96 5

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

The disturbance of defecation in the patient with Hirschsprung's disease and the pathophysiology of constipation in the constipated patient with simple megarectum were investigated. In Hirschsprung's disease, an existence of aganglionosis and the sphincter achalasia are two main factors which cause clinical symptoms such as chronic constipation and megacolon. As a surgical treatment, we have routinely performed our modified Duhamel's operation. Postoperative followup study indicated that the most cases in our series have gained a satisfactory defecatory function 1 to 3 years after operation. In chronically constipated patient with simple megarectum, on the other hand, it was found manometrically that the most of them had significantly high anal canal pressure and incomplete anal relaxation after rectal stimulation. These findings indicate that those patients have hypertonic and achalasic sphincter. Therefore, the authors propose "high anal pressure syndrome (HAPS)" for such constipated patient with simple megarectum. As to the surgical treatment, 6 patients with simple megarectum had a complete posterior internal sphincterotomy and the postoperative results were excellent or good in our series.
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PMID:[Pathophysiology of defecatory disturbance in the patient with Hirschsprung's disease and chronically constipated patients with simple megarectum]. 408 53

From January 1974 till the end of 1981, 105 children with chronic constipation not responding to any medical treatment, were submitted to anorectal myomectomy. This operation has both a diagnostic and a therapeutic value; the histological study of the muscle biopsy can confirm or not a Hirschsprung's disease. It is also an ideal treatment in cases of achalasia and idiopathic chronic constipation. If necessary a secondary rectosigmoidectomy can be performed. We did not have any important complication, none of our patients had soiling. Eighty-eight percent of our patients had regular bowel movements after the anorectal myomectomy with a better individual, social and family life.
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PMID:[The value of anorectal sphincteromyectomy for the diagnosis and treatment of chronic constipation in children]. 663 3

The basic pathophysiologic disturbance in Hirschsprung's disease is a functional obstruction caused by defective intramural nerve supply and by internal anal sphincter achalasia. Therapy consists in resecting the dysganglionic bowel segment. In each case however an aganglionic segment of different length and an internal analsphincter with a different degree of achalasia remain in situ. Therefore the postoperative results are dependant on an equilibrium between the proximal normal innervated colon and the length and function of the remaining aganglionic and achalic parts of the rectum and anal canal. In about one third of all patients with Hirschsprung's disease disturbances of this equilibrium postoperatively lead to enterocolitis, encopresis, or chronic constipation. Five years later however the authors could observe enterocolitis in only 7.3% chronic constipation in 9.5% and encopresis in 13.9% of their operated patients. With increasing time after operation there is a growing tendency towards the spontaneous regeneration. Therefore, the prognosis of Hirschsprung's disease is very good: about 90% of all cases can be cured.
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PMID:[Late study results following megacolon operations]. 674 49

47 patients with functional chronic constipation associated with an achalasia of the anal sphincter were treated with the alpha blocking agent Phenoxybenzamin. Dosages were used according to the manufacturers' instructions. 29 of the children were completely cured. There was significant improvement in a further 13 children and only 5 patients showed no improvement. Treatment failures were associated with additional severe psychological disturbances for which psycho-therapeutic management was required.
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PMID:[Effect of alpha-blocking agents on the internal anal sphincter muscle]. 741 57

Anorectal sphincteromyectomy is a suitable answer to the problem of the chronic constipation in childhood. It took a long time to be recognized beside others methods as biofeedack. This surgical procedure has been described for the first time by B. Duhamel in 1965; we still remained faithful and proud of this idea. We wanted to understand and to explain the basis of the conception of anorectal achalasia. Several works about the fetal development of the anorectal sphincter tried to explain it. A good exposition makes the technique less difficult and the dissection of the internal sphincter easier. We have analysed our 22 recent children. In 21 cases, we noticed a good result. The histological study of the fragment of the myomectomy showed a normal cytology of the smooth muscular tissue, a normal intrinsic innervation and arterial vascularisation. The surgical procedure is efficient because it relieves the functional disorder of the internal anal sphincter. It is a mechanical action; the external sphincter finds again its normal function, or is allowed to be reeducated.
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PMID:[Constipation in children. Value of anorectal sphincteromyectomy]. 773 45

A physiological sphincter exists at the rectosigmoid junction; this rectosigmoid sphincter (RSS) relaxes on sigmoid colon contraction and contracts on rectal contraction. Eight cases of RSS hypertonia are presented herein. Six women and two men (mean age, 44.2 +/- 10.3 years) complained of chronic constipation of 9.6 +/- 6.2 years' duration. They had had normal bowel habits before that time. Anorectal physiologic studies were performed. Intestinal transit was delayed by the accumulation of pellets in the sigmoid colon. Defecography and electromyography of the external anal sphincter and levator ani muscle were normal. The resting pressure was normal in the sigmoid colon, rectum, and rectal neck (p > 0.05) but elevated in the RSS (p < 0.01). The sigmoidorectal inhibitory and excitatory reflexes were absent. Biopsies from the sigmoid colon and rectum showed normal histologic findings, but those from the RSS were aganglionic. A diagnosis of RSS "achalasia" was made. Five of the eight patients had improvement as a result of RSS dilatation. The remaining three patients underwent sigmoidomyotomy. The eight patients are now 9 to 38 months without recurrence of constipation. Rectosigmoid junction achalasia constitutes a clinicopathologic entity that should be considered in the etiology of constipation.
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PMID:The hypertonic rectosigmoid junction: description of a new clinicopathologic entity causing constipation. 910 39

Gastroesophageal reflux disease, achalasia and esophageal spasms are the most frequent esophageal motility disorders and are associated with dysphagia and non-cardiac chest pain. The diagnosis of achalasia is based on manometric criteria. Pneumatic dilatation, laparoscopic myotomy, and the minimal invasive injection of botulinum toxin are therapeutic options. Long-term-pH-metry is the gold standard to diagnose gastroesophageal reflux disease. Proton pump inhibitors (PPI) are the first-line therapy in reflux disease. Esophageal manometry and pH-metry are essential investigations prior to an antireflux operation. The evaluation of chronic constipation refractory to medical treatment should include anal manometry, and MR-defecography for the diagnosis of anorectal outlet obstruction such as anismus which could be treated successfully by biofeedback therapy.
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PMID:[Gastrointestinal motility disorders relevant to general practice]. 1242 42

Gastrointestinal motility disorders encompass a wide array of signs and symptoms that can occur anywhere throughout the luminal gastrointestinal tract. Motility disorders are often chronic in nature and dramatically affect patients' quality of life. These prevalent disorders cause a tremendous impact both to the individual patient and to society as a whole. Significant progress has been made over the last 5 years in understanding the etiology and pathophysiology of gastrointestinal motility disorders. This clinical update will focus on seven of the most common gastrointestinal motility disorders (achalasia, non-achalasia esophageal motility disorders, dyspepsia, gastroparesis, chronic intestinal pseudo-obstruction, irritable bowel syndrome, and chronic constipation) with an emphasis on current treatment options and new therapeutic modalities.
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PMID:Gastrointestinal motility disorders: an update. 1684 50


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