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Query: UMLS:C0021843 (bowel obstruction)
 9,927 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The radiological findings in 13 patients with total aganglionosis coli were reviewed. There was a male to female ratio of 7:6. Fifty-four percent of patients presented in the first week of life, but a significant number (31%) did not present until after 1 month of age. All patients had plain film evidence of bowel obstruction when referred for a barium enema. There were no pathognomonic barium enema findings, and barium enema results covered the entire spectrum of findings which can be encountered in the neonate and young infant with bowel obstruction. Seventy-seven percent had normal calibre colon, 23% had micro colon, 23% had a shortened colon, 46% had colonic wall irregularity, 33% had significant ileal reflux. Delayed evacuation of barium from colon occurred in the two patients who had delayed films. Total colonic aganglionosis should be considered in any infant or young child with plain film evidence of bowel obstruction, whatever the barium enema findings. Hirschsprung's disease and the level of transition can only be definitively diagnosed by biopsy.
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PMID:Radiological findings in total aganglionosis coli. 672 46

A case of colonic atresia (CA) and aganglionosis is presented, which is probably the tenth in the English literature. The boy presented on day 3 of life with delayed passage of meconium, milk intolerance, and progressive abdominal distension. A barium enema study was complicated by barium peritonitis. Emergency peritoneal lavage was done and the intestinal obstruction was relieved by a proximal defunctioning ileostomy; type III CA (Grosfeld classification) was identified. The proximal atretic end was the blind-ending caecum and the distal atretic end commenced in the splenic area as a microcolon extending to the pelvis and replacing the normal colon. Total colonic aganglionosis (TCA) was confirmed by biopsies. This is the first case in the literature of TCA with CA documented before any attempts at colonic resection and anastomosis were made. This association highlights the recommendation for biopsy of the micro-colon and rectum when Type III CA (Grosfeld classification) is encountered in a newborn. The infant underwent a cholecystectomy and Duhamel-Martin operation at 14 months that was complicated by abdominal wound dehiscence; the ileostomy was closed at 22 months of age.
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PMID:A male newborn with colonic atresia and total colonic aganglionosis. 1007 52

Total colonic aganglionosis (TCA) is an unusual form of Hirschsprung's disease, and most of these present within the first few weeks and nearly all by the end of the first year of life. Very few cases presenting in older childhood or adulthood have been reported. Without a high index of suspicion these cases can easily be missed and, therefore, patients undergo repeated laparotomies for subacute intestinal obstruction without relief of symptoms. The diagnosis is suspected on operative findings and confirmed by histological evaluation of seromuscular biopsy specimens. Proper diagnosis will prevent loss of bowel length caused by inadvertent resection and will help in performing an optimal procedure to treat these patients. Discussed herein is another case of TCA in a 12-year-old boy with review of relevant literature.
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PMID:Total colonic aganglionosis: diagnosis and management in a 12-year-old boy. 1050 41

Total colonic aganglionosis is a rare phenotype of Hirschsprung disease (HD). While the diagnosis is generally established within the neonatal period there are reports of delayed presentation. In this case, we describe a 9-month old girl with no previous medical or surgical history who presented with a small bowel obstruction. A contrast enema performed as part of the work up demonstrated right sided colonic diverticula with no other anatomical abnormalities. Surgical intervention was required to manage the obstruction and colonic biopsies were consistent with HD. This case highlights the association of the unusual finding of colonic diverticula with total colonic HD in infants.
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PMID:Colonic diverticulosis in an infant: A clue to total colonic Hirschsprung disease. 2968 Feb 78