Hirschprung Disease

Other Known Aliasescongenital aganglionic megacolon, congenital intestinal aganglionosis

Definitionmotor disorder of the intestines due to failure of the neural crest cells, which are precursors of ganglion cells) to fully migrate during embryonic development of the colon.

Clinical SignificanceAs a result of this aganglionosis of the colon, the distal intestines are unable to relax and cause a functional obstruction. Children affected by this condition fail to pass their meconium stool in the first 48 hours of life and may have abdominal distention, bilous emesis, and/or enterocolitis. Diagnosis is made with contrast enema and suction rectal biopsy.

HistoryNamed after Harald Hirschprung (1830-1916), who was a Danish physician and received his medical doctorate from the University of Copenhagen in 1855. In 1870, he became the first Danish pediatrician and was appointed chief physician of Queen Louisa Hospital for Children in 1879. He presented his eponymous findings at the Berlin Congress for Children’s Diseases in 1886 where he described two infants who had died from “constipation associated with dilation and hypertrophy of the colon”. He published his findings a year later in an article entitled “Stuhlträgheit Neugeborener in Folge von Dilatation und Hypertrophie des Colons”. Other notable contributions of Dr. Hirschprung include being one of the first physicians to successfully reduce intussuception by pneumatic means and being an advocate for free health care for all children.


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