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Q03.0 ICD-10-CM Code: Malformations of aqueduct of Sylvius

Compare with another codeCheck this code on a claim

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

Coding at a Glance

Tabular directives
4 inclusion terms · 4 Excludes1
Risk adjustment
CMS-HCC V28: 1 category · CMS-HCC V22 category 72 · RxHCC V08 category 155

Billable · FY2027A valid, specific ICD-10-CM code, reportable for dates of service in FY2027.

What you need to know

Source: CMS/NCHS Official ICD-10-CM tabular notes, quoted. From the CMS/NCHS tabular list for the release in force. A note the category or block publishes applies to this code too; the Instructions section marks which is which.

Excludes1Never report with this code
  • Arnold-Chiari syndrome, type II (Q07.0-)
  • acquired hydrocephalus (G91.-)
  • hydrocephalus due to congenital toxoplasmosis (P37.1)
  • hydrocephalus with spina bifida (Q05.0-Q05.4)
IncludesWhat this code covers
  • hydrocephalus in newborn
  • Anomaly of aqueduct of Sylvius
  • Obstruction of aqueduct of Sylvius, congenital
  • Stenosis of aqueduct of Sylvius

Most relevant related codes MedCoder-derived

Read off the official notes above and this code’s own position in the tabular list. Which to report is a documentation question; Compare shows the two side by side.

CompareCheck ClaimView Related Codes

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 v44, Appendix B.

  • MS-DRG 091 — OTHER DISORDERS OF NERVOUS SYSTEM WITH MCC (MDC 01)
  • MS-DRG 092 — OTHER DISORDERS OF NERVOUS SYSTEM WITH CC (MDC 01)
  • MS-DRG 093 — OTHER DISORDERS OF NERVOUS SYSTEM WITHOUT CC/MCC (MDC 01)
  • MS-DRG 791 — PREMATURITY WITH MAJOR PROBLEMS (MDC 15)
  • MS-DRG 793 — FULL TERM NEONATE WITH MAJOR PROBLEMS (MDC 15)

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 182 — Spinal Cord Disorders/Injuries

Other models: CMS-HCC V22 HCC 72 · RxHCC V08 HCC 155

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 Q03.0 in the official ICD-10-CM tabular list, quoted as published.

Source: CMS/CDC — ICD-10-CM Tabular ListRelease: FY2027Effective: October 1, 2026

Trace:FY2027 changesChange historyRelease, file and checksum

Notes without a marker are published on Q03.0 itself; “inherited from” names the category or block whose note applies here.

Includes

Conditions the official ICD-10-CM tabular list includes under this code.

  • hydrocephalus in newborn

Source: inherited from Q03

Inclusion Terms

Alternative terms the tabular list files under this code.

  • Anomaly of aqueduct of Sylvius
  • Obstruction of aqueduct of Sylvius, congenital
  • Stenosis of aqueduct of Sylvius

Excludes1 — Not Coded Here

Conditions generally not reported together with this code (Excludes1) -- an error unless the two conditions are documented as unrelated to each other (ICD-10-CM Official Guidelines, Section I.A.12.a).

Source: inherited from Q03

Coder workflow for Q03.0

MedCoder structured workflow — derived from this code’s own official record

Before you code Q03.0

  1. Check the Excludes1 note: if the documentation supports a condition named there, do not simply proceed with Q03.0. Excludes1 conditions are not reported together with this code unless the record shows they are unrelated (Guidelines I.A.12.a).

    See the official tabular notes

Choose the right path

  1. Does the documentation support a condition named in Q03.0’s Excludes1 note?
    Yes → Do not simply proceed: review the excluded code. Both are reported only when the record shows the two conditions are unrelated.
    No → Continue.

    ReviewQ07.0, G91, P37.1

Consider Q03.0. Then confirm the code is valid for the date of service in the Verify section.

Documentation check

The provider’s diagnostic statement
Codes are assigned from the provider’s documented diagnosis, not from clinical criteria, test values or a medication list (Guidelines I.A.19).

Official instructions as workflow

  • Excludes1 — check before selecting Q03.0(4 notes)

    Coding workflow: If the documentation supports a condition named in this note, do not simply proceed with Q03.0: the two are not reported together. The one exception is when the record shows the two conditions are unrelated to each other.

    CompareQ07.0, G91, P37.1

    See the official tabular notes · Guidelines I.A.12.a

Coding decision scenarios

Pattern scenarios for this code’s structure — decision rules, not clinical cases

Documentation: Both the condition Q03.0 describes and a condition named in its Excludes1 note are documented for the same encounter.

Coding question: Can both codes be reported?

Path: Review the Excludes1 note and the excluded code, and look for a provider statement on whether the two conditions are related.

Reason: Excludes1 means the two are not coded together; the exception is when the record shows the conditions are unrelated to each other (Guidelines I.A.12.a).

ReviewQ07.0, G91, P37.1

Every row is derived from this code’s own record — its title, tabular notes, 7th-character family and same-category siblings — with fixed MedCoder wording; nothing is inferred about a patient. The official notes and guideline text are in the sections each row links to, and they control.

Don’t confuse Q03.0 with…

MedCoder analysis — pairs from this code’s official notes, distinctions written by MedCoder

The codes most likely to be picked instead of Q03.0 — named in its own official notes, or sharing its laterality or encounter family — with what separates them and which documentation moves the case. Each links to the full comparison.

  • Q07.0— Excludes1 conflict (Excludes1 — generally not reported together)

    Not coded here — the tabular list excludes this pair for the same encounter.

    Not reported together with this code, except where the two conditions are documented as unrelated.

    What moves you there: Documentation of Arnold-Chiari syndrome, type II moves the case to Q07.0; the two are not reported together unless documented as unrelated.

    Full comparison: Q03.0 vs Q07.0 →

  • G91— Excludes1 conflict (Excludes1 — generally not reported together)

    Not coded here — the tabular list excludes this pair for the same encounter.

    Not reported together with this code, except where the two conditions are documented as unrelated.

    What moves you there: Documentation of acquired hydrocephalus moves the case to G91; the two are not reported together unless documented as unrelated.

    Full comparison: Q03.0 vs G91 →

  • P37.1— Excludes1 conflict (Excludes1 — generally not reported together)

    Not coded here — the tabular list excludes this pair for the same encounter.

    Not reported together with this code, except where the two conditions are documented as unrelated.

    What moves you there: Documentation of hydrocephalus due to congenital toxoplasmosis moves the case to P37.1; the two are not reported together unless documented as unrelated.

    Full comparison: Q03.0 vs P37.1 →

  • Q05.0— Excludes1 conflict (Excludes1 — generally not reported together)

    Not coded here — the tabular list excludes this pair for the same encounter.

    Not reported together with this code, except where the two conditions are documented as unrelated.

    What moves you there: Documentation of hydrocephalus with spina bifida moves the case to Q05.0; the two are not reported together unless documented as unrelated.

    Full comparison: Q03.0 vs Q05.0 →

  • Q05.4— Excludes1 conflict (Excludes1 — generally not reported together)

    Not coded here — the tabular list excludes this pair for the same encounter.

    Not reported together with this code, except where the two conditions are documented as unrelated.

    What moves you there: Documentation of hydrocephalus with spina bifida moves the case to Q05.4; the two are not reported together unless documented as unrelated.

    Full comparison: Q03.0 vs Q05.4 →

  • Q03.9— Specificity relationship (Review)

    Overlapping diagnoses in the same category.

    Overlapping codes in the same category — the documentation decides which is the more specific fit.

    What moves you there: A record that documents no more than “Congenital hydrocephalus, unspecified” takes Q03.9; specificity in the record moves it back here.

    Full comparison: Q03.0 vs Q03.9 →

Relationships are read from this code's own official notes, its laterality siblings and its encounter family; the wording of each distinction is MedCoder editorial, and the documentation named in each “what moves you there” line is the official note phrase or sibling title itself. Source: CMS ICD-10-CM tabular instructional notes; ICD-10-CM Official Guidelines, Section I.A.12.a.

Coding context

Guidelines, coding notes, decision aids, relationships (with MS-DRG and CCSR classification), hierarchy, HCC, coverage and the context map: what a coder reaches for after the core. Each section names whether it is official source data, a MedCoder-derived relationship or MedCoder editorial.

Code Overview

Malformations of aqueduct of Sylvius is a billable ICD-10-CM diagnosis code (Q03.0).

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

Verify Before Coding

  • No Medicare Code Editor or MS-DRG Definitions Manual restrictions apply to this code.

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-derived relationships — computed from published CMS and AHRQ datasets

Other codes that name Q03.0 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: G91 — Hydrocephalus (via Q03.-), Q75 — Other congenital malformations of skull and face bones (via Q03.-).

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

MS-DRG Grouper Relationships (FY2027)

Potential MS-DRG participation — not a DRG assignment.

FY2027 MS-DRG: not on the CMS CC/MCC list — as a secondary diagnosis this code does not change MS-DRG severity for that release.

Named in the grouper logic of 5 MS-DRGs: DRG 091 (MDC 01), DRG 092 (MDC 01), DRG 093 (MDC 01), DRG 791 (MDC 15), DRG 793 (MDC 15).

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

Clinical classification (AHRQ CCSR):MAL004 — Nervous system 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 CMS-HCC risk category (V28)

CMS maps these diagnoses to the same Hierarchical Condition Category (Spinal Cord Disorders/Injuries) for risk-adjusted payment.

P11.5 — Birth injury to spine and spinal cord, Q00.0 — Anencephaly, Q00.1 — Craniorachischisis, Q00.2 — Iniencephaly, Q01.0 — Frontal encephalocele, Q01.1 — Nasofrontal encephalocele, Q01.2 — Occipital encephalocele, Q01.8 — Encephalocele of other sites, Q01.9 — Encephalocele, unspecified, Q02 — Microcephaly, Q03.1 — Atresia of foramina of Magendie and Luschka, Q03.8 — Other congenital hydrocephalus, Q03.9 — Congenital hydrocephalus, unspecified, Q04.0 — Congenital malformations of corpus callosum, Q04.1 — Arhinencephaly, Q04.2 — Holoprosencephaly, Q04.3 — Other reduction deformities of brain, Q04.4 — Septo-optic dysplasia of brain, Q04.5 — Megalencephaly, Q04.6 — Congenital cerebral cysts, +230 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.

Quadriplegia, Paraplegia

Same clinical category (CCSR)

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

Q00.0 — Anencephaly, Q00.1 — Craniorachischisis, Q00.2 — Iniencephaly, Q01.0 — Frontal encephalocele, Q01.1 — Nasofrontal encephalocele, Q01.2 — Occipital encephalocele, Q01.8 — Encephalocele of other sites, Q01.9 — Encephalocele, unspecified, Q02 — Microcephaly, Q03.1 — Atresia of foramina of Magendie and Luschka, Q03.8 — Other congenital hydrocephalus, Q03.9 — Congenital hydrocephalus, unspecified, Q04.0 — Congenital malformations of corpus callosum, Q04.1 — Arhinencephaly, Q04.2 — Holoprosencephaly, Q04.3 — Other reduction deformities of brain, Q04.4 — Septo-optic dysplasia of brain, Q04.5 — Megalencephaly, Q04.6 — Congenital cerebral cysts, Q04.8 — Other specified congenital malformations of brain, +29 more

Same Index main term, other category

The ICD-10-CM Index to Diseases and Injuries files this code under the main terms “Anomaly, anomalous”, “Hydrocephalus”, “Atresia, atretic”, …; these codes share that main term but sit in a different category of the Tabular List.

O66.3 — Obstructed labor due to other abnormalities of fetus (causing disproportion, with obstructed labor), O88.23 — Thromboembolism in the puerperium (artery, precerebral, puerperal), P37.1 — Congenital toxoplasmosis (due to toxoplasmosis), P76.0 — Meconium plug syndrome (meconium, newborn), P76.2 — Intestinal obstruction due to inspissated milk (intestine, newborn, due to, inspissated milk), P76.8 — Other specified intestinal obstruction of newborn (intestine, newborn, specified NEC), P76.9 — Intestinal obstruction of newborn, unspecified (intestine, newborn), P83.5 — Congenital hydrocele (genitalia, genital organor system, male, hydrocele), Q00.0 — Anencephaly (hemicrania), Q02 — Microcephaly (skull, with, microcephaly), Q04.8 — Other specified congenital malformations of brain (meninges, cerebral), Q04.9 — Congenital malformation of brain, unspecified (dura), Q05.0 — Cervical spina bifida with hydrocephalus (congenital, with spina bifida, cervical), Q05.1 — Thoracic spina bifida with hydrocephalus (congenital, with spina bifida, dorsal), Q05.2 — Lumbar spina bifida with hydrocephalus (congenital, with spina bifida, lumbar), Q05.3 — Sacral spina bifida with hydrocephalus (congenital, with spina bifida, sacral), Q05.4 — Unspecified spina bifida with hydrocephalus (congenital, with spina bifida), Q05.9 — Spina bifida, unspecified (meningocele), Q06.3 — Other congenital cauda equina malformations (cauda equina), Q06.9 — Congenital malformation of spinal cord, unspecified (meninges, spinal), +666 more

Contextual Map

Every relationship of Q03.0 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 Q03.0 with these 2 related codes in Claim Check

Hierarchy

Referenced by Excludes1 notes

Clinical classification (CCSR)

Risk adjustment (CMS-HCC)

  • HCC 182 — Spinal Cord Disorders/Injuries [CMS-HCC]— CMS-HCC V28 · 2026

MS-DRG Grouper

MDC crossing

  • MDC 01 — Diseases and Disorders of the Nervous System[MDC crossing]: “Diseases and Disorders of the Nervous 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. 8,919 same-MDC procedures group here; browse them on the MDC page.”— CMS MS-DRG Definitions Manual · FY2027
  • MDC 15 — Newborns and Other Neonates with Conditions Originating in Perinatal Period[MDC crossing]: “Newborns and Other Neonates with Conditions Originating in Perinatal Period — 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.”— CMS MS-DRG Definitions Manual · FY2027

Index entries

  • Anomaly, anomalous (congenital) (unspecified type), aqueduct of Sylvius[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Atresia, atretic, aqueduct of Sylvius[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Hydrocephalus (acquired) (external) (internal) (malignant) (recurrent), aqueduct Sylvius stricture[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Obstruction, obstructed, obstructive, aqueduct of Sylvius, congenital[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Occlusion, occluded, aqueduct of Sylvius, congenital[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Septum, septate (congenital), aqueduct of Sylvius[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Stenosis, stenotic (cicatricial), aqueduct of Sylvius (congenital)[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Stricture, aqueduct of Sylvius (congenital)[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027

Nearest codes

Change history

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

Reference

Index terms and tables, published questions and FAQ, every source behind this page with its release and checksum, the date-of-service check and the complete change history.

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.

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 FY2027 tabular list, index and tables, effective October 1, 2026 Release, file and checksum · Publisher’s page
Claim edits Official source data
CMS Definitions of Medicare Code Edits — v44.0 (October 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 — v44 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-derived 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 editorial 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.

Current data releases:ICD-10-CM FY2027 · ICD-10-PCS FY2027 · HCPCS October 2026 · MS-DRG v44 · Medicare Code Editor v44.0 · NCCI PTP Q4 2026 · MUE Q4 2026 · NCD code lists 2026-01 · LCD export September 28, 2026 · All releases and sources

Labels on this page: Official source data · MedCoder-derived relationship · MedCoder editorial explanation. How to read the labels · All data sources and release dates · CMS coding rules

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. "Q03.0 — Malformations of aqueduct of Sylvius." ICD-10-CM FY2027. https://medcoder.ai/icd10/code/q03.0-malformations-of-aqueduct-of-sylvius

Change history

  • FY2016 — October 1, 2015
    In the code set at ICD-10-CM adoption
    Malformations of aqueduct of Sylvius

No changes since FY2016 — additions, deletions, description changes and billable-status changes are tracked through FY2027, 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 Q03.0 in its code family, with their registry titles.

View all codes in the Q03 family