Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
2
pubmed:dateCreated
2002-7-29
pubmed:abstractText
Total colonic aganglionosis (TCA) is a rare, hybrid form of Hirschsprung's disease. It is a functional rather than mechanical obstruction, characterized by the absence of intrinsic ganglion cells in the myenteric and submucosal plexuses of the bowel wall. Ganglion cells regulate normal colonic peristaltic activity. Paucity of ganglion cells results in an aganglionic segment of bowel that is functionally abnormal and does not propagate the normal peristaltic wave that moves to it from the proximal ganglionic bowel. The lesion originates in the rectum and extends proximally over a variable distance of the bowel. The further the lesion extends, the more difficult the management becomes. Clinical and radiologic findings can be useful in diagnosis, but they are not pathognomonic. The definitive diagnosis is made following suction biopsy of the rectum, colon, and ileum. Ultimate treatment for TCA is surgical, although no single surgical procedure has been proven superior. Total parenteral nutrition during the postoperative period is essential to ensure appropriate fluid and electrolyte status. Improvements in supportive care and earlier recognition and diagnosis of TCA in infants have led to a significantly increased rate of survival since the lesion was first recognized. The embryology, pathogenesis, clinical presentation, diagnosis, management prognosis, and outcome of TCA are discussed. A case study is presented.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
N
pubmed:status
MEDLINE
pubmed:month
Mar
pubmed:issn
0730-0832
pubmed:author
pubmed:issnType
Print
pubmed:volume
20
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
23-9
pubmed:dateRevised
2005-11-16
pubmed:meshHeading
pubmed:year
2001
pubmed:articleTitle
Total colonic aganglionosis: a case study.
pubmed:affiliation
Baylor University Medical Center, Dallas, Texas 75246, USA. lamarkham@baylordallas.edu
pubmed:publicationType
Journal Article, Review, Case Reports