2027 ICD-10-CM Diagnosis Code Q43.1Hirschsprung's disease
ICD-10-CM Codes›Q00-Q99›Q38-Q45›Q43
- Billable — Valid for Submission
- CC — Complication or Comorbidity
- Risk Adjusts — HCC 78
- POA Exempt
- Chronic Condition
Q43.1 is a billable ICD-10-CM diagnosis code for Hirschsprung's disease. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 393 through 395. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 4 closely related codes. The code is exempt from POA reporting. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Digestive congenital anomalies.
For Medicare Advantage risk adjustment, Q43.1 maps to CMS-HCC Category 78 (Intestinal Obstruction/Perforation) under the V28 model, adding a risk factor of about 0.326 for a community, non-dual, aged beneficiary in payment year 2027.
Code Identity
Code Classification
Present on Admission (POA)Billing
Q43.1 is exempt from POA reporting on inpatient claims to general acute care hospitals. Review the other POA exempt codes in Other congenital malformations of the digestive system (Q38-Q45).
Medicare Risk Adjustment (HCC)Billing
Q43.1 maps to a payment category in the CMS-HCC model used to risk-adjust Medicare Advantage payments. Weights are the published community factors for payment year 2027.
Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.
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
- Structural abnormality of Auerbach plexus
- Total colonic aganglionosis
- Total intestinal aganglionosis
- Waardenburg Shah syndrome
- Waardenburg syndrome
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to Q43.1: its own notes plus those printed at Chapter 17. A note printed at a category, block or chapter applies to every code under it.
Applicable To
Conditions this code is used for: synonyms of the title or, for "other specified" codes, the conditions assigned to it. The list is not exhaustive.
- Aganglionosis
- Congenital (aganglionic) megacolon
Excludes2
Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.
Notes
General instructions on how these codes are used.
- Codes from this chapter are not for use on maternal records
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name Q43.1, its category, or a range that includes it.
Excludes1 3
These codes carry an Excludes1 note naming Q43.1: they are not reported together with it, unless the two conditions are unrelated.
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
congenital Q43.1
Macrocolon See Also: Megacolon; Q43.1
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
Hirschsprung Disease
congenital megacolon resulting from the absence of ganglion cells (aganglionosis) in a distal segment of the large intestine. the aganglionic segment is permanently contracted thus causing dilatation proximal to it. in most cases, the aganglionic segment is within the rectum and sigmoid colon.
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.
Code HistoryHistory
Questions About Q43.1Overview
What is the ICD-10 code for Hirschsprung's disease?
The ICD-10-CM code for Hirschsprung's disease is Q43.1 (sometimes written as Q431). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
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, 2026 through September 30, 2027.
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 394, 395, 393, with relative weights from 0.6379 to 1.5877 depending on complications. Higher weights mean higher Medicare reimbursement.
Is Q43.1 a CC or MCC?
CMS lists Q43.1 as a CC (complication or comorbidity) for FY 2027. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 4 closely related codes in its exclusion list.
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. The code appears in the Other congenital malformations of the digestive system (Q38-Q45) range of the CMS exempt list.
What HCC is Q43.1?
Q43.1 (Hirschsprung's disease) maps to CMS-HCC Category 78 (Intestinal Obstruction/Perforation), commonly written as HCC 78, in the CMS-HCC V28 model used for Medicare Advantage risk adjustment in payment year 2027. It does not map to any RxHCC in the Part D prescription drug model.
Does Q43.1 risk-adjust for Medicare Advantage payment?
Yes. When documented and reported on a Medicare Advantage encounter, Q43.1 adds a risk adjustment factor of about 0.326 to the beneficiary's RAF score for a community, non-dual, aged enrollee (published V28 weights range from 0.326 to 0.688 depending on the payment segment). A more severe related category (HCC 77) supersedes it when both are reported. See the full factor table on the HCC 78 category page.
