Total colonic aganglionosis and Hirschsprung's disease: Shades of the same or different?

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dc.contributor.authorMoore S.W.
dc.date.accessioned2011-05-15T16:03:45Z
dc.date.available2011-05-15T16:03:45Z
dc.date.issued2009
dc.description.abstractTotal colonic aganglionosis is a relatively uncommon form of Hirschsprung's disease (HSCR) occurring in approximately 2-13% of cases. It can probably be divided into total colonic aganglionosis (TCA; defined as aganglionosis extending from the anus to at least the ileocaecal valve, but no more than 50 cm proximal to the ileocaecal valve) and total colonic and small bowel aganglionosis, which may involve a very long segment of aganglionosis. Clinically, they appear to represent a different spectrum of disease in terms of presentation and difficulties in diagnosis which may be experienced, suggesting a different pathophysiology from the more common forms of HSCR. It is not yet clear whether TCA merely represents a long form of HSCR or a different expression of the disease. There are a number of differences between TCA and other forms of HSCR, which require an explanation if its ubiquitous clinical features are to be understood. There is some evidence suggesting that instead of being purely congenital, it may represent certain different pathophysiologic mechanisms, some of which may continue to be active after birth. This study reviews all that is known about the clinical, radiological and histopathologic differences between TCA and the more frequently encountered recto-sigmoid (or short-segment) and correlates them with what is currently known about the genetic and molecular biologic background to find possible pathogenetic mechanisms. © 2009 Springer-Verlag.
dc.description.versionReview
dc.identifier.citationPediatric Surgery International
dc.identifier.citation25
dc.identifier.citation8
dc.identifier.issn1790358
dc.identifier.other10.1007/s00383-009-2408-9
dc.identifier.urihttp://hdl.handle.net/10019.1/12765
dc.subjectclinical feature
dc.subjectcolon aganglionosis
dc.subjectdifferential diagnosis
dc.subjectHirschsprung disease
dc.subjecthistopathology
dc.subjecthuman
dc.subjecthuman cell
dc.subjecthuman tissue
dc.subjectileocecal valve
dc.subjectmolecular biology
dc.subjectpathophysiology
dc.subjectpriority journal
dc.subjectreview
dc.subjectsensitivity and specificity
dc.subjecttotal colonic aganglionosis
dc.subjectAnimals
dc.subjectDisease Models, Animal
dc.subjectHirschsprung Disease
dc.subjectHumans
dc.subjectMice
dc.subjectRats
dc.titleTotal colonic aganglionosis and Hirschsprung's disease: Shades of the same or different?
dc.typeReview
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