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Hirschsprung Disease
Pronunciation: HERSH-sproong
Congenital absence of nerve cells in the colon causing functional bowel obstruction.
Full Definition
Hirschsprung disease is a congenital condition characterized by the absence of ganglion cells (aganglionosis) in the distal colon and rectum, preventing normal peristalsis and causing functional obstruction. The condition ranges from short-segment disease affecting only the rectosigmoid to long-segment disease extending to the transverse or even small bowel. Diagnosis is confirmed by rectal biopsy, and treatment involves surgical removal of the aganglionic segment with pull-through procedures.
Usage
Usage note: Named after Harald Hirschsprung; do not use possessive form.
In Context
- "Rectal biopsy confirmed the diagnosis of Hirschsprung disease with short-segment aganglionosis." — Pathology report
- "The patient with Hirschsprung disease underwent successful Soave pull-through procedure." — Surgical case notes
Also known as
congenital megacolon
aganglionosis
Don't confuse with
functional constipation
meconium plug syndrome