Hirschsprung's disease

This code represents Hirschsprung's disease, a congenital anomaly characterized by the absence of ganglion cells in a segment of the bowel, typically the distal colon. This aganglionosis results in a functional obstruction due to the inability of the affected bowel segment to relax and propel stool.

Use this code for patients diagnosed with Hirschsprung's disease, whether newly diagnosed or for ongoing management of the condition. Documentation supporting this diagnosis typically includes clinical presentation (e.g., chronic constipation, abdominal distension), imaging studies (e.g., barium enema showing a transition zone), and definitive diagnosis via rectal biopsy demonstrating the absence of ganglion cells.

• Do not confuse with acquired megacolon or other causes of intestinal obstruction; ensure documentation specifies congenital aganglionosis.
• Consider additional codes for any associated complications, such as enterocolitis (K55.0-), intestinal perforation (K63.1), or malnutrition (E40-E46).
• If a surgical procedure (e.g., pull-through procedure) is performed, ensure appropriate CPT codes are also reported.