2026 ICD-10-CM Diagnosis Code Q43.1Hirschsprung's disease

ICD-10-CM Codes›Q00-Q99›Q38-Q45›Q43

ICD-10-CM Q43.1
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

Q43.1 is a billable ICD-10-CM diagnosis code for Hirschsprung's disease. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 393 through 395. The code is exempt from POA reporting. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Digestive congenital anomalies.

Code Identity

ICD-10-CM Code
Q43.1
Billable Status
Yes — Valid for Submission
Code Describes
Hirschsprung's disease
Short Description
Hirschsprung's disease
Same as the full description in the CMS dataset.
Parent Code
Q43
Other congenital malformations of intestine

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ38-Q45Other congenital malformations of the digestive system
CategoryQ43Other congenital malformations of intestine
This CodeQ43.1Hirschsprung's disease

Present on Admission (POA)Billing

Q43.1 is exempt from POA reporting on inpatient claims to general acute care hospitals. Review other POA exempt codes.

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Aganglionosis of Auerbach's plexus
  • Aganglionosis of colon
  • Aganglionosis of large intestine
  • Central alveolar hypoventilation syndrome
  • Congenital aganglionic megacolon
  • Congenital aganglionosis of small intestine
  • Congenital central hypoventilation
  • Congenital dilatation of colon
  • Congenital hypoplasia of nail unit
  • Extensive aganglionosis Hirschsprung disease
  • Goldberg Shprintzen megacolon syndrome
  • Haddad syndrome
  • Hirschsprung disease of rectosigmoid region
  • Hirschsprung disease with deafness and polydactyly syndrome
  • Hirschsprung disease with nail hypoplasia and dysmorphism
  • Hirschsprung disease with type D brachydactyly syndrome
  • Hirschsprung disease, ganglioneuroblastoma syndrome
  • Long segment Hirschsprung's disease
  • Mowat-Wilson syndrome
  • Mowat-Wilson syndrome due to monosomy 2q22
  • Peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, Waardenburg syndrome, Hirschsprung disease
  • Secondary megacolon - congenital
  • Short segment Hirschsprung's disease
  • Total colonic aganglionosis
  • Total intestinal aganglionosis
  • Waardenburg Shah syndrome
  • Waardenburg syndrome

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Aganglionosis
  • Congenital (aganglionic) megacolon

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

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

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR MAL002
Digestive congenital anomalies
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Waardenburg Syndrome

    rare, autosomal dominant disease with variable penetrance and several known clinical types. characteristics may include depigmentation of the hair and skin, congenital deafness, heterochromia iridis, medial eyebrow hyperplasia, hypertrophy of the nasal root, and especially dystopia canthorum. the underlying cause may be defective development of the neural crest (neurocristopathy). waardenburg's syndrome may be closely related to piebaldism. klein-waardenburg syndrome refers to a disorder that also includes upper limb abnormalities.

  • Total Intestinal Aganglionosis

    a complete lack of ganglia in the intestine. this is an extremely severe form of hirschsprung disease.

Patient EducationClinical

Birth Defects

A birth defect is a problem that happens while a baby is developing in the mother's body. Most birth defects happen during the first 3 months of pregnancy. One out of every 33 babies in the United States is born with a birth defect.

The full article covers:

  • What are birth defects?
  • What causes birth defects?
  • Who is at risk of having a baby with birth defects?
  • How are birth defects diagnosed?
  • What are the treatments for birth defects?
  • Can birth defects be prevented?

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert Q43.1 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
751.3 Hirschsprung's disease
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About Q43.1Overview

Is Q43.1 (Other congenital malformations of intestine) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report Hirschsprung's disease on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does Q43.1 group to?

When Hirschsprung's disease is the principal diagnosis on an inpatient stay, it groups to MS-DRG 393, 394, 395, with relative weights from 0.6490 to 1.5993 depending on complications. Higher weights mean higher Medicare reimbursement.

Is Q43.1 exempt from POA reporting?

Yes. CMS lists this code among those exempt from present on admission reporting, so hospitals do not assign a POA indicator for Hirschsprung's disease on inpatient claims.

What is the ICD-9 equivalent of Q43.1?

Under the General Equivalence Mappings, Hirschsprung's disease converts to ICD-9-CM 751.3 (Hirschsprung's disease). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.