Hirschsprung's disease

Abstract

Hirschsprung's disease (HD) or congenital aganglionic megacolon is characterized by an absence of ganglion cells in the myenteric Auerbach's plexus, the deep submucosal Henle's plexus, and the submucosal Meissner's plexus in the distal colon. The usual presentation of Hirschsprung's disease can be demonstrated by the following clinical scenario. A 3-day-old male presented with failure to pass meconium within 48 h of birth and progressive abdominal distension. Barium study showed dilated colon with no specific zone of transition. The newborn was delivered at 40 weeks gestation after an otherwise normal pregnancy. Transanal rectal biopsy demonstrated the absence of ganglion cells on multiple sections and special staining. The patient underwent an exploratory laparotomy, upon which he was found to have markedly dilated small bowel and colon up to the area of sigmoid. A transverse loop colostomy was performed. The patient recovered well from the initial surgery and underwent definitive surgical therapy of the HD at a later time. The following core competencies are addressed in this article: Medical knowledge, Patient care. Republished with permission from: Prabhakaran S. Absite corner: Hirschsprung's disease. OPUS 12 Scientist 2009; 3(3):44-46.