Evaluation and management of persistent problems after surgery for Hirschsprung disease in a child

Abstract

Hirschsprung disease occurs approximately once in every 5000 live-born infants. It is characterized by the absence of ganglion cells in the myenteric and submucosal plexuses of the intestine. Once the diagnosis of Hirschsprung disease has been made, most patients are now treated with a transanal approach to resection. We review the early and late postoperative complications. Late complications include persistent mechanical obstruction, recurrent or acquired aganglionosis, disordered motility in the proximal colon or small bowel, internal sphincter achalasia, or functional megacolon caused by stool-holding behavior. These children require complex interdisciplinary care to ensure an adequate quality of life.