Hirschsprung's disease is associated with a deficiency in which type of cells?

Hirschsprung's disease, also known as congenital megacolon, is primarily associated with a deficiency of ganglion cells, specifically the enteric ganglion cells in the bowel wall. In this condition, the absence of these cells, known as neural crest-derived cells, leads to a lack of ganglionic plexuses in a segment of the colon. As a result, the affected segment cannot properly relax and expand, hindering peristalsis and causing functional obstruction.

The ganglion cells are crucial for the enteric nervous system, which controls intestinal motility. Their absence results in impaired bowel function, leading to severe constipation and abdominal distension. Understanding the role of these ganglion cells helps clarify why the absence of them is central to the pathology of Hirschsprung's disease.