What is the name of the condition characterized by congenital absence of the right colon and rectum?

The condition characterized by congenital absence of the right colon and rectum is known as right congenital aganglionic megacolon. This condition results from a failure of neural crest cells to migrate completely along the colon, leading to a lack of ganglion cells in that segment of the intestine. The absence of nerve cells affects peristalsis and normal bowel function, leading to severe constipation and potential bowel obstruction.

In contrast, Hirschsprung disease, while related to the absence of ganglion cells, specifically involves the aganglionosis of the distal colon rather than the right colon. Intestinal malrotation pertains to abnormal positioning of the intestines during development, which can lead to complications but does not involve the absence of the colon. Meckel's diverticulum is an outpouching of the intestines and is not associated with the congenital absence of any part of the bowel.

Understanding these distinctions highlights why right congenital aganglionic megacolon accurately describes the condition in question.