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

Billing Status: YES. This is a valid, specific, and billable ICD-10-CM reference.

Coding at a Glance

Tabular directives
2 inclusion terms
Risk adjustment
CMS-HCC V28: 1 category

Inpatient Payment Groups (MS-DRG)

Potential MS-DRG participation — not a DRG assignment.

MS-DRGs this diagnosis helps define, as principal or secondary, per the CMS ICD-10-CM/PCS MS-DRG Definitions Manual v43, Appendix B.

  • MS-DRG 393 — OTHER DIGESTIVE SYSTEM DIAGNOSES WITH MCC (MDC 06)
  • MS-DRG 394 — OTHER DIGESTIVE SYSTEM DIAGNOSES WITH CC (MDC 06)
  • MS-DRG 395 — OTHER DIGESTIVE SYSTEM DIAGNOSES WITHOUT CC/MCC (MDC 06)

A diagnosis appearing in a group's logic does not by itself determine the DRG assigned to a stay; the grouper uses the full claim.

Risk Adjustment (CMS-HCC)

Payment categories this diagnosis maps to under CMS-HCC V28, payment year 2026.

  • HCC 78 — Intestinal Obstruction/Perforation

Risk scores depend on the enrollee's full accepted diagnosis set and segment; a category mapping alone does not determine payment.

Coding instructions

Official Tabular Instructional Notes: the inclusion, exclusion and sequencing notes published for Q43.1 in the official ICD-10-CM tabular list, quoted as published.

Source: CMS/CDC — ICD-10-CM Tabular ListRelease: FY2026Effective: October 1, 2025

Inclusion Terms

Alternative terms the tabular list files under this code.

  • Aganglionosis
  • Congenital (aganglionic) megacolon

Code Overview

Hirschsprung's disease is a billable ICD-10-CM diagnosis code (Q43.1).

MedCoder summary Summary composed by MedCoder from this code's official ICD-10-CM record. The tabular instructional notes themselves appear verbatim below.

Indexed Clinical Terms (8)

Official source data — entries quoted as published, in the Index’s own lookup phrasing

Clinical term phrases from the official ICD-10-CM Index to Diseases and Injuries that map to this code. These are alphabetic-index entries shown as the Index writes them — lookup phrasing, not necessarily the wording of a final diagnosis.

Verify Before Coding

  • CC as a secondary diagnosis (FY2026). Can raise the stay's MS-DRG severity tier.

From the code registry, the Medicare Code Editor, and the MS-DRG Definitions Manual. Check it against a full claim in Claim Check.

Relationships & Classification

MedCoder structured relationships — computed from published CMS and AHRQ datasets

Other codes that name Q43.1 or its code family, from the CMS ICD-10-CM tabular instructional notes. Tabular-note edges are stored at the code family level that carries each note.

Referenced by 2 Excludes1 notes across 2 chapters: K59.3 — Megacolon, not elsewhere classified, P76.1 — Transitory ileus of newborn.

These codes’ tabular lists mark this diagnosis as mutually exclusive — not reported together unless the conditions are documented as unrelated.

MS-DRG Grouper Relationships (FY2026)

Potential MS-DRG participation — not a DRG assignment.

FY2026 MS-DRG: CC — Complication or Comorbidity. Reported as a secondary diagnosis, this code raises the stay's MS-DRG severity tier — except when the principal diagnosis is one of 4 clinically related codes on its CMS exclusion list.

Named in the grouper logic of 3 MS-DRGs: DRG 393 (MDC 06), DRG 394 (MDC 06), DRG 395 (MDC 06).

From the CMS MS-DRG Definitions Manual (Appendices B and C). Actual DRG assignment depends on the complete claim.

Clinical classification (AHRQ CCSR):MAL002 — Digestive congenital anomalies (default).

Clinical Classifications Software Refined (CCSR) for ICD-10-CM Diagnoses. Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality.

Related Codes

Same clinical process (MS-DRG)

Acts as CC — raises the severity of other admissions. CMS groups these diagnoses into one MS-DRG principal-diagnosis exclusion process — a CC/MCC on this list never raises severity when the principal diagnosis is also on it.

Q43.2 — Other congenital functional disorders of colon, Q45.8 — Other specified congenital malformations of digestive system, Q45.9 — Congenital malformation of digestive system, unspecified

Same CMS-HCC risk category (V28)

CMS maps these diagnoses to the same Hierarchical Condition Category (Intestinal Obstruction/Perforation) for risk-adjusted payment.

K68.19 — Other retroperitoneal abscess, P77.1 — Stage 1 necrotizing enterocolitis in newborn, P77.2 — Stage 2 necrotizing enterocolitis in newborn, P77.3 — Stage 3 necrotizing enterocolitis in newborn, P77.9 — Necrotizing enterocolitis in newborn, unspecified, P78.0 — Perinatal intestinal perforation, Q40.0 — Congenital hypertrophic pyloric stenosis, Q41.0 — Congenital absence, atresia and stenosis of duodenum, Q41.1 — Congenital absence, atresia and stenosis of jejunum, Q41.2 — Congenital absence, atresia and stenosis of ileum, Q41.8 — Congenital absence, atresia and stenosis of other specified parts of small intestine, Q41.9 — Congenital absence, atresia and stenosis of small intestine, part unspecified, Q42.0 — Congenital absence, atresia and stenosis of rectum with fistula, Q42.1 — Congenital absence, atresia and stenosis of rectum without fistula, Q42.2 — Congenital absence, atresia and stenosis of anus with fistula, Q42.3 — Congenital absence, atresia and stenosis of anus without fistula, Q42.8 — Congenital absence, atresia and stenosis of other parts of large intestine, Q42.9 — Congenital absence, atresia and stenosis of large intestine, part unspecified, Q43.2 — Other congenital functional disorders of colon, Q43.3 — Congenital malformations of intestinal fixation, +55 more

Related risk categories

These categories interact through CMS's HCC hierarchy — one can suppress the other's risk-adjustment weight when both are present on a claim.

Intestine Transplant Status/Complications

Same clinical category (CCSR)

AHRQ's Clinical Classifications Software groups these diagnoses under the same clinical category (Digestive congenital anomalies).

Q41.2 — Congenital absence, atresia and stenosis of ileum, Q41.8 — Congenital absence, atresia and stenosis of other specified parts of small intestine, Q41.9 — Congenital absence, atresia and stenosis of small intestine, part unspecified, Q42.0 — Congenital absence, atresia and stenosis of rectum with fistula, Q42.1 — Congenital absence, atresia and stenosis of rectum without fistula, Q42.2 — Congenital absence, atresia and stenosis of anus with fistula, Q42.3 — Congenital absence, atresia and stenosis of anus without fistula, Q42.8 — Congenital absence, atresia and stenosis of other parts of large intestine, Q42.9 — Congenital absence, atresia and stenosis of large intestine, part unspecified, Q43.0 — Meckel's diverticulum (displaced) (hypertrophic), Q43.2 — Other congenital functional disorders of colon, Q43.3 — Congenital malformations of intestinal fixation, Q43.4 — Duplication of intestine, Q43.5 — Ectopic anus, Q43.6 — Congenital fistula of rectum and anus, Q43.7 — Persistent cloaca, Q43.8 — Other specified congenital malformations of intestine, Q43.9 — Congenital malformation of intestine, unspecified, Q44.0 — Agenesis, aplasia and hypoplasia of gallbladder, Q44.1 — Other congenital malformations of gallbladder, +41 more

Same Index main term, other category

The ICD-10-CM Index to Diseases and Injuries files this code under the main terms “Obstruction, obstructed, obstructive”, “Dilatation”; these codes share that main term but sit in a different category of the Tabular List.

Q25.44 — Congenital dilation of aorta (aorta, congenital), Q31.8 — Other congenital malformations of larynx (larynx NEC, congenital), Q32.1 — Other congenital malformations of trachea (trachea, congenital), Q34.8 — Other specified congenital malformations of respiratory system (upper respiratory, congenital), Q39.5 — Congenital dilatation of esophagus (esophagus, congenital), Q40.0 — Congenital hypertrophic pyloric stenosis (pylorus, congenital), Q40.2 — Other specified congenital malformations of stomach (stomach NEC, congenital), Q41.9 — Congenital absence, atresia and stenosis of small intestine, part unspecified (intestine, congenital), Q42.8 — Congenital absence, atresia and stenosis of other parts of large intestine (intestine, congenital, large, specified part NEC), Q42.9 — Congenital absence, atresia and stenosis of large intestine, part unspecified (intestine, congenital, large), Q44.1 — Other congenital malformations of gallbladder (gallbladder, congenital), Q44.3 — Congenital stenosis and stricture of bile ducts (bile duct or passage, congenital), Q44.5 — Other congenital malformations of bile ducts (cystic duct, congenital), Q62.2 — Congenital megaureter (ureter, congenital), Q62.39 — Other obstructive defects of renal pelvis and ureter (ureter NEC, congenital), Q64.31 — Congenital bladder neck obstruction (bladder-neck, congenital), Q64.39 — Other atresia and stenosis of urethra and bladder neck (urethra NEC, congenital), Q64.79 — Other congenital malformations of bladder and urethra (bladder, congenital), T81.529 — Obstruction due to foreign body accidentally left in body following unspecified procedure (due to foreign body accidentally left in operative wound), T82.09 — Other mechanical complication of heart valve prosthesis (intracardiac ball valve prosthesis), +138 more

Contextual Map

Every relationship of Q43.1 in one view: hierarchy, official tabular instructions in both directions, clinical classification, risk adjustment, MS-DRG participation, index terms and change history — each edge carrying the CMS source it derives from.

Run Q43.1 with these 2 related codes in Claim Check

Hierarchy

Referenced by Excludes1 notes

Clinical classification (CCSR)

Risk adjustment (CMS-HCC)

  • HCC 78 — Intestinal Obstruction/Perforation [CMS-HCC]— CMS-HCC V28 · 2026

MS-DRG Grouper

MDC crossing

  • MDC 06 — Diseases and Disorders of the Digestive System[MDC crossing]: “Diseases and Disorders of the Digestive System — the grouper's crossing between diagnoses and procedures: a principal diagnosis sets the MDC, and same-MDC procedures move the stay to its surgical DRGs. 5,744 same-MDC procedures group here; browse them on the MDC page.”— CMS MS-DRG Definitions Manual · FY2026

Index entries

  • Aganglionosis (bowel) (colon)[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Dilatation, colon, congenital[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Hirschsprung's disease or megacolon[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Ileus (bowel) (colon) (inhibitory) (intestine), neurogenic, Hirschsprung's disease or megacolon[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Macrocolon[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Megacolon (acquired) (functional) (not Hirschsprung's disease) (in), congenital, congenitum (aganglionic)[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Megacolon (acquired) (functional) (not Hirschsprung's disease) (in), Hirschsprung's (disease)[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Obstruction, obstructed, obstructive, intestine, neurogenic, Hirschsprung's disease or megacolon[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026

Nearest codes (10)

Change history

  • FY2016 — In the code set at ICD-10-CM adoption [Change history]— CMS release files (code change ledger) · icd10cm-fy2016

Sources for this page

Codes, titles, notes, index terms and mappings on this page are transcribed from the datasets below. Relationships MedCoder computed and text MedCoder wrote are labelled where they appear.

Code, title, tabular notes and index terms Official source data
CMS/CDC ICD-10-CM FY2026 tabular list, index and tables, effective October 1, 2025 Release, file and checksum · Publisher’s page
Claim edits Official source data
CMS Definitions of Medicare Code Edits — v43.1 (April 2026) Release, file and checksum · Publisher’s page
Inpatient payment groups Official source data
CMS MS-DRG Definitions Manual (incl. Appendix B diagnosis index, Appendix C CC/MCC list, Appendix E procedure index) and IPPS Final Rule tables — v43 Release, file and checksum · Publisher’s page
Risk adjustment Official source data
2026 Mid-Year Final ICD-10 Mappings + Model Software (cms.gov/files/zip/2026-midyear-final-icd-10-mappings.zip, 2026-midyear-final-model-software.zip) — CMS-HCC V28, PY2026 mid-year final release Release, file and checksum · Publisher’s page
Change history and date-of-service validity Official source data
CMS ICD-10-CM release addenda, ingested release by release into the change ledger Release, file and checksum · Publisher’s page
Comparisons, relationships and the contextual map MedCoder structured relationship
Computed by MedCoder from the tabular notes and tables above; every derived relationship is marked as derived where it appears
Summary and FAQ answers MedCoder explanation
Written by MedCoder to explain the sources above: drafted with AI assistance, checked by a person against the release files, and labelled as MedCoder text where it appears. Not official text.

Labels on this page: Official source data · MedCoder structured relationship · MedCoder explanation. How to read the labels · All data sources and release dates

Cite this page

Reference this page in a research guide, syllabus or article. The release is included because code content changes each year.

MedCoder.ai. "Q43.1 — Hirschsprung's disease." ICD-10-CM FY2026. https://medcoder.ai/icd10/code/q43.1-hirschsprungs-disease

Change history

  • FY2016 — October 1, 2015
    In the code set at ICD-10-CM adoption
    Hirschsprung's disease

No changes since FY2016 — additions, deletions, description changes and billable-status changes are tracked through FY2027 (effective October 1, 2026), and none are recorded for this code. Note changes are tracked from FY2027 only.

Nearest Codes in This Family

Official ICD-10-CM classifications closest to Q43.1 in its code family, with their registry titles.

View all codes in the Q43 family