Journal of pediatric surgery
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The term ultrashort Hirschsprung's disease has been used to define a spectrum of conditions with clinical presentation similar to Hirschsprung's disease but with presence of ganglion cells on rectal biopsy. In contrast to Hirschsprung's disease, there is no transition zone on barium enema. However, as in classical Hirschsprung's disease, there is no reflex internal sphincter relaxation on rectal manometry. ⋯ There is controversy in the literature concerning the diagnosis of ultrashort Hirschsprung's disease. Most of the confusion concerns whether a short segment of aganglionosis proximal to the dentate line is permissible for the diagnosis of ultrashort Hirschsprung's disease and to what extent such aganglionosis is physiological. Perhaps it would be more accurate to define this entity by the presence of ganglion cells on rectal biopsy as well as the failure of the internal sphincter to relax on rectal manometry and to describe it as anorectal achalasia in severely constipated patients.
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There is no consensus regarding the most appropriate management of pediatric blunt liver injury. This study addresses this issue by reviewing our experience with blunt liver trauma in relationship to the grade of injury. Forty-one pediatric patients with blunt abdominal trauma and documented liver injury were managed from 1979 to 1989. ⋯ The mean (+/- SEM) transfusion volume for the remaining nine children was 14.8 +/- 2.5 mL/kg. Blunt liver injury represents a spectrum from a minimal parenchymal hematoma to massive liver disruption. We conclude that celiotomy is necessary for hepatic injury hemodynamically stable injured children with transfusion requirements less than 40 mL/kg can be managed nonoperatively in an appropriate setting.