BACKGROUND/AIMS Rats with a spontaneous null mutation in endothelin receptor type B or Ednrb (sl/sl; spotting lethal) lack enteric neurons in the distal bowel and usually die within the first week after birth. This early postnatal lethality limits their use for examining the potential of cell therapy to treat Hirschsprung disease, and for studies of the influence of EDNRB on the mature CNS and ...

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...

In the management of chronic constipation Hirschsprung's disease may be diagnosed on the basis of radiological investigation, anorectal manometry, and rectal biopsy. Though x ray films and manometric studies may be highly suggestive, the definitive diagnosis requires rectal biopsy.' 2 The classical method of rectal biopsy involves taking a full thickness of rectal mucosa and underlying muscle, ...