Q43.1Hirschsprung's disease

Q43.1 is the ICD-10-CM diagnosis code for Hirschsprung's disease. It belongs to category Q43 (other congenital malformations of intestine), block Q38-Q45 (other congenital malformations of the digestive system) and chapter 17 (congenital malformations, deformations, chromosomal abnormalities, and genetic disorders). It is billable, so it can be reported on its own for dates of service from October 1, 2026 through September 30, 2027.

Within Q43 (other congenital malformations of intestine), Q43.1 is specifically for Hirschsprung's disease. Related codes cover Meckel's diverticulum (displaced) (hypertrophic) (Q43.0), other congenital functional disorders of colon (Q43.2), congenital malformations of intestinal fixation (Q43.3), duplication of intestine (Q43.4), ectopic anus (Q43.5), congenital fistula of rectum and anus (Q43.6), persistent cloaca (Q43.7), other specified congenital malformations of intestine (Q43.8) and 1 more.

As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.

In MS-DRG v44.0, it is part of the grouping logic for DRG 393 (Other Digestive System Diagnoses with MCC, relative weight 1.5881), DRG 394 (Other Digestive System Diagnoses with CC, relative weight 0.9228) and DRG 395 (Other Digestive System Diagnoses without CC/MCC, relative weight 0.6382), in MDC 06 (Diseases and Disorders of the Digestive System), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.

It is exempt from present-on-admission (POA) reporting, so no POA indicator is required.

It has been part of ICD-10-CM since the code set took effect on October 1, 2015 and hasn't changed since.

Before ICD-10, this condition was coded in ICD-9-CM as 751.3 (Hirschsprung's disease and other congenital functional disorders of colon).