Q43.1

Billable

Hirschsprung's disease

HCC Category Mapping

V28HCC 78 — Intestinal Obstruction/Perforation

0.000

What This Code Means

A birth defect where nerve cells are missing from part of the large intestine, preventing normal bowel movements and causing severe constipation or blockage.

Coding Tips

•Hirschsprung's disease requires specification of the extent (short segment, long segment, or total colonic involvement) if documented
•This is typically diagnosed in infancy and may require surgical intervention

Clinical Significance

Hirschsprung's disease is a serious congenital condition requiring surgical intervention and lifelong monitoring for complications including enterocolitis and bowel obstruction. Early diagnosis and treatment are critical to prevent life-threatening complications, making accurate coding essential for tracking outcomes and ensuring appropriate specialist care.

Documentation Requirements

✓Confirmation of absent ganglion cells in intestinal wall
✓Length of aganglionic segment (short-segment vs. long-segment)
✓Histopathological confirmation from biopsy
✓Associated complications (enterocolitis, perforation, obstruction)
✓Surgical procedures performed (pull-through operations)
✓Current bowel function status
✓Any genetic testing results or family history

Commonly Confused Codes

K59.00 — Functional constipation (acquired, not congenital aganglionic)Q42.9 — Structural atresia/stenosis rather than functional nerve absence K56.60 — Mechanical intestinal obstruction (complication, not primary diagnosis)Q43.2 — Other functional disorders without specific ganglion cell absence

Code Hierarchy

└Q43Other congenital malformations of intestine └Q43.1Hirschsprung's disease

└Q43.1Hirschsprung's disease