Total Aganglionic Colon in an Adult

Myers, Maurice; Bradburn, D. M.; R, Vela; Payzant, A; Karlin, Sophie

doi:10.1097/00000658-196601000-00015

Cited by 27 publications

(9 citation statements)

References 8 publications

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“…The first issue relates to the mode of presentation that is often much later than expected considering the length of the aganglionic segment. The later than expected presentation of TCA (N6 months) in 14% of cases reported in this study is in keeping with other studies [13][14][15]21,22]. We previously reported 9 (27%) of those with TCA presenting outside the NN period (8 presenting N6 months and 2 [2%] N12 months) [22].…”

Section: Discussionsupporting

confidence: 91%

“…We previously reported 9 (27%) of those with TCA presenting outside the NN period (8 presenting N6 months and 2 [2%] N12 months) [22]. In keeping with this, there are a number of reports of TCA presenting as late as adolescence and early adulthood [13][14][15].…”

Section: Discussionmentioning

confidence: 67%

“…Although extensive disease would suggest a severe clinical picture, with early presentation, and although most present within the first few weeks of life [12], a number of late presenting TCA cases have been reported [13][14][15], even as adolescents and adults suggesting an ongoing pathophysiologic process.…”

mentioning

confidence: 99%

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Clinical and genetic differences in total colonic aganglionosis in Hirschsprung's disease

Moore

Zaahl

2009

Journal of Pediatric Surgery

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Section: Discussionsupporting

confidence: 91%

Section: Discussionmentioning

confidence: 67%

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Clinical and genetic differences in total colonic aganglionosis in Hirschsprung's disease

Moore

Zaahl

2009

Journal of Pediatric Surgery

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“…It has also been reported that symptoms have a progressive development with worsening of constipation and a diagnosis made after the first year of life or in rare cases in adolescence or adulthood [111][112][113][114][115].…”

Section: Total Colonic Aganglionosismentioning

confidence: 99%

Interstitial cells of Cajal in the normal gut and in intestinal motility disorders of childhood

2007

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Interstitial cells of Cajal (ICCs) are pacemaker cells which are densely distributed throughout the whole gastrointestinal tract. ICCs have important functions in neurotransmission, generation of slow waves and regulation of mechanical activities in the gastrointestinal tract, especially for the coordinated gastrointestinal peristalsis. Therefore, a loss of ICCs could result in gastrointestinal motor dysfunction. In recent years c-kit labeling has been widely used to study pathological changes of ICCs in gastrointestinal motility disorders. Paediatric gastrointestinal motility disorders such as hypertrophic pyloric stenosis, Hirschsprung's disease, total colonic aganglionosis, hypoganglionosis, intestinal neuronal dysplasia, internal anal sphincter achalasia, megacystis microcolon intestinal hypoperistalsis syndrome have been reported to be associated with loss or deficiency of ICCs networks. This review describes the distribution of ICCs in the normal gastrointestinal tract and its altered distribution in intestinal motility disorders of childhood.

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“…Patients with adult Hirschsprung's disease are usually laxative-dependent and have a lifelong history of constipation [7][8][9]14,15]. With few exceptions [16,17], these patients have a short or an ultra-short segment of aganglionosis [6,7,9].…”

Section: Introductionmentioning

confidence: 99%