Aganglionosis of the Small Intestine: A Rare Form of Hirschsprung's Disease

Absence of ganglion cells in the small intestine, a rare form of Hirschsprung's disease, is a condition found in newborns and associated with increased morbidity and mortality. Families are faced with numerous challenges, including maintenance of nutrition, the need for surgical interventions, and the potential for complications associated with the medical technologies necessary to maintain children with aganglionosis. Home care needs are not unlike those of other children with severe intestinal dysfunction such as short bowel syndrome. This article gives an overview of Hirschsprung's disease and presents three case studies of small bowel Hirschsprung's. One center's experience with Hirschsprung's disease supports the need for a multidisciplinary approach to long-term management and home care.