aganglionosis
Pronunciation: ah-gang-glee-oh-NO-sis
Congenital absence of ganglion cells in the bowel wall, the pathological hallmark of Hirschsprung disease.
Full Definition
Aganglionosis refers to the congenital absence of ganglion cells in the enteric nervous system of the bowel wall, representing the fundamental pathological feature of Hirschsprung disease. This condition results from failure of neural crest cell migration during embryonic development, leading to a functional obstruction due to inability of the affected bowel segment to relax normally. Aganglionosis can involve variable lengths of bowel, from short-segment disease affecting only the rectosigmoid to total colonic involvement. The term is essential in pediatric gastroenterology documentation and requires precise usage to distinguish from other motility disorders.
Usage
Usage note: Distinguish from hypoganglionosis or other neural abnormalities.
In Context
- "Histological examination revealed aganglionosis extending to the splenic flexure." — Pathology report
- "The level of aganglionosis was determined by multiple biopsies." — Operative planning note