Total colonic aganglionosis initially diagnosed in an adolescent

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Total Colonic Aganglionosis in Siblings

Hirschsprung’s disease (HD) is attributed to failure of craniocaudal neural crest cell migration in the hindgut. Histopathology is absent of ganglion cells in the myenteric and submucosal plexuses. Most patients present with intestinal obstruction, in which delayed meconium passage is the most important history when suspecting HD. In eighty percent of cases, the pathology is limited limited to ...

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Total colonic aganglionosis in Hirschsprung disease.

Total colonic aganglionosis (TCA) is a relatively uncommon form of Hirschsprung disease (HSCR), occurring in approximately 2%-13% of cases. It can probably be classified as TCA (defined as aganglionosis extending from the anus to at least the ileocecal valve, but not >50 cm proximal to the ileocecal valve) and total colonic and small bowel aganglionosis, which may involve a very long segment of...

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Total Colonic Aganglionosis: a Rare Entity in Children

ABSTACT Total colonic aganglionosis being an unusual form of Hirschsprung’s disease is described in two children. Both the patients were male, one 8-year old and the other 12-year old. They presented with features of sub acute intestinal obstruction preceded by a past history of chronic constipation since birth and operative intervention for similar complaints few years back. A high index of su...

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Ten-year experience in the management of total colonic aganglionosis.

PURPOSE The aim of this study was to review the 10 years' experience in the management of patients with total colonic aganglionosis (TCA) and follow-up of their health condition. METHODS Cases of 25 patients with TCA in the Children's Hospital of Fudan University from 1996 to 2005 were reviewed and analyzed. The confirmed diagnosis was established by an intraoperative frozen-section biopsy of...

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Perils of Total Colonic Aganglionosis Presenting in Neonatal Age

BACKGROUND The purpose of this study is to review the cases of total colonic aganglionosis seen in the span of ten years at a pediatric surgery unit of a tertiary care public hospital in New Delhi. METHODS Medical records of twelve patients with total colonic aganglionosis were retrieved. RESULTS Ten out of the twelve patients were males; seven were of the Muslim community. Average recorded...

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ژورنال

عنوان ژورنال: Gastroenterology

سال: 1984

ISSN: 0016-5085

DOI: 10.1016/0016-5085(84)90205-1