Q43.1 Hirschsprung's disease

✓ Billable ICD-10-CM 2026
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The ICD-10-CM code for Hirschsprung's disease is Q43.1 (FY2026). It is a billable, claim-ready diagnosis code.

Classification

Section
Q38-Q45: Other congenital malformations of the digestive system (Q38-Q45)
Category Q43
11 codes (10 billable)
FY2026 Status
Stable since FY2024

Also Known As

ICD-10-CM Alphabetic Index entries that lead to Q43.1:

  • Macrocolon
  • Aganglionosis (bowel) (colon)
  • Hirschsprung's disease or megacolon
  • Megacolon (acquired) (functional) (not Hirschsprung's disease) (in) › Hirschsprung's (disease)
  • Megacolon (acquired) (functional) (not Hirschsprung's disease) (in) › congenital, congenitum (aganglionic)
  • Dilatation › colon › congenital
  • Ileus (bowel) (colon) (inhibitory) (intestine) › neurogenic › Hirschsprung's disease or megacolon
  • Obstruction, obstructed, obstructive › intestine › neurogenic › Hirschsprung's disease or megacolon

Inclusion Terms

  • Aganglionosis
  • Congenital (aganglionic) megacolon

U.S. Hospital Utilization

  • An estimated 6,130 U.S. inpatient stays in 2023 included Q43.1 among the documented diagnoses.
  • 1,750 stays listed it as the principal diagnosis.

Source: National Inpatient Sample (NIS), Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality, 2016–2023. National survey-weighted estimates.

Official Coding Guidelines

POA indicator for congenital conditions/anomalies and Q00-Q99 exception

Congenital conditions and anomalies Assign “Y” for congenital conditions and anomalies except for categories Q00- Q99, Congenital anomalies, which are on the exempt list. Congenital conditions are always considered present on admission.

— ICD-10-CM Official Guidelines for Coding and Reporting, FY2026, Section III

Source: CMS — ICD-10-CM Official Guidelines for Coding and Reporting, FY2026

References

Related Codes

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Reviewed by Prajwal Shrestha, CPC, CRC
Certified Professional Coder (CPC) and Certified Risk Adjustment Coder (CRC) · AAPC Member ID 01997614 · About · Editorial policy · Content last reviewed: 2025-10-01

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