Hirschsprung's disease and other congenital functional disorders of colon

This code signifies Hirschsprung's disease, a congenital aganglionic megacolon, characterized by the absence of ganglion cells in a segment of the bowel, leading to functional obstruction. It also encompasses other congenital functional disorders affecting the colon's motility and function from birth.

Apply this code when documentation confirms a diagnosis of Hirschsprung's disease, including short-segment, long-segment, or total colonic aganglionosis. It is also appropriate for other congenital conditions causing functional colonic obstruction or dysmotility, such as congenital intestinal pseudo-obstruction, when explicitly stated.

• Ensure documentation specifies "congenital" for functional disorders of the colon to differentiate from acquired conditions.
• Do not use for acquired megacolon or other non-congenital causes of colonic obstruction.
• Consider sequencing with codes for associated complications, such as enterocolitis or perforation, if present.