Q87.82 ICD-10-CM Code: Arterial tortuosity syndrome

Coding instructions

Official Tabular Instructional Notes: the inclusion, exclusion and sequencing notes published for Q87.82 in the official ICD-10-CM tabular list, quoted as published.

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

Notes without a marker are published on Q87.82 itself; “inherited from” names the category or block whose note applies here.

Coder workflow for Q87.82

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

Before you code Q87.82

1. Check the Excludes1 note: if the documentation supports a condition named there, do not simply proceed with Q87.82. 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 Q87.82’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.

   ReviewE71.510

Consider Q87.82. Then work the Use Additional Code note, and 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).

The conditions the Use Additional Code note names

Reported with this code when documented; a conditional instruction (“if applicable”, “if known”) applies only when the record supports it.

Official instructions as workflow

• Excludes1 — check before selecting Q87.82(1 note)

  Coding workflow: If the documentation supports a condition named in this note, do not simply proceed with Q87.82: the two are not reported together. The one exception is when the record shows the two conditions are unrelated to each other.

  CompareE71.510

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

• Use Additional Code — after identifying Q87.82(1 note)

  Coding workflow: Check whether the documentation supports the additional code(s) the note names, and report them with Q87.82 when it does. Where the instruction is conditional (“if applicable”, “if known”), it applies only when the record documents the condition.

  See the official tabular notes · Guidelines I.A.13

Coding decision scenarios

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

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.

Indexed Clinical Terms (1)

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.

• Syndrome, arterial tortuosity

Don’t confuse Q87.82 with…

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

The codes most likely to be picked instead of Q87.82 — 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.

• E71.510— 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 Zellweger syndrome moves the case to E71.510; the two are not reported together unless documented as unrelated.

  Full comparison: Q87.82 vs E71.510 →

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.

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 Q87.82 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: Q87.A — Loeys-Dietz syndrome, Q89.7 — Multiple congenital malformations, not elsewhere classified (via Q87.-).

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 39 clinically related codes on its CMS exclusion list.

Named in the grouper logic of 3 MS-DRGs: DRG 564 (MDC 08), DRG 565 (MDC 08), DRG 566 (MDC 08).

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

Clinical classification (AHRQ CCSR):MAL010 — Other specified and unspecified 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.

E78.72 — Smith-Lemli-Opitz syndrome, Q87.2 — Congenital malformation syndromes predominantly involving limbs, Q87.3 — Congenital malformation syndromes involving early overgrowth, Q87.40 — Marfan syndrome, unspecified, Q87.410 — Marfan syndrome with aortic dilation, Q87.418 — Marfan syndrome with other cardiovascular manifestations, Q87.42 — Marfan syndrome with ocular manifestations, Q87.43 — Marfan syndrome with skeletal manifestation, Q87.5 — Other congenital malformation syndromes with other skeletal changes, Q87.81 — Alport syndrome, Q87.83 — Bardet-Biedl syndrome, Q87.84 — Laurence-Moon syndrome, Q87.85 — MED13L syndrome, Q87.86 — Kleefstra syndrome, Q87.87 — Hao-Fountain Syndrome, Q87.89 — Other specified congenital malformation syndromes, not elsewhere classified, Q89.81 — Kabuki syndrome, Q89.89 — Other specified congenital malformations, Q99.2 — Fragile X chromosome, Q99.811 — Usher syndrome, type 1, +18 more

Same clinical category (CCSR)

AHRQ's Clinical Classifications Software groups these diagnoses under the same clinical category (Other specified and unspecified congenital anomalies).

Q87.19 — Other congenital malformation syndromes predominantly associated with short stature, Q87.2 — Congenital malformation syndromes predominantly involving limbs, Q87.3 — Congenital malformation syndromes involving early overgrowth, Q87.40 — Marfan syndrome, unspecified, Q87.410 — Marfan syndrome with aortic dilation, Q87.418 — Marfan syndrome with other cardiovascular manifestations, Q87.42 — Marfan syndrome with ocular manifestations, Q87.43 — Marfan syndrome with skeletal manifestation, Q87.5 — Other congenital malformation syndromes with other skeletal changes, Q87.81 — Alport syndrome, Q87.83 — Bardet-Biedl syndrome, Q87.84 — Laurence-Moon syndrome, Q87.85 — MED13L syndrome, Q87.87 — Hao-Fountain Syndrome, Q87.88 — CTNNB1 syndrome, Q87.89 — Other specified congenital malformation syndromes, not elsewhere classified, Q89.01 — Asplenia (congenital), Q89.09 — Congenital malformations of spleen, Q89.1 — Congenital malformations of adrenal gland, Q89.2 — Congenital malformations of other endocrine glands, +59 more

Contextual Map

Every relationship of Q87.82 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 Q87.82 with these 2 related codes in Claim Check

Hierarchy

• Q00-QA1 — Chapter 17: Congenital Malformations, Deformations and Chromosomal Abnormalities (Q00-QA1) (Q00-QA1)[Hierarchy]— CMS ICD-10-CM tabular structure · icd10cm-fy2026

Referenced by Excludes1 notes

• Q87.A — Loeys-Dietz syndrome[Excludes1]: “arterial tortuosity syndrome (Q87.82)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026
• Q89.7 — Multiple congenital malformations, not elsewhere classified[Excludes1](via Q87.-): “congenital malformation syndromes affecting multiple systems (Q87.-)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026

Clinical classification (CCSR)

• MAL010 — Other specified and unspecified congenital anomalies[CCSR]— AHRQ CCSR for ICD-10-CM Diagnoses (HCUP)

MS-DRG Grouper

• CC — Complication or Comorbidity [MS-DRG severity]: “As a secondary diagnosis this code can raise the stay's MS-DRG severity tier (CC).”— CMS MS-DRG Definitions Manual (Appendix C) · FY2026
• DRG 564 — OTHER MUSCULOSKELETAL SYSTEM AND CONNECTIVE TISSUE DIAGNOSES WITH MCC[MS-DRG]: “OTHER MUSCULOSKELETAL SYSTEM AND CONNECTIVE TISSUE DIAGNOSES WITH MCC (MDC 08)”— CMS MS-DRG Definitions Manual (Appendix B) · FY2026
• DRG 565 — OTHER MUSCULOSKELETAL SYSTEM AND CONNECTIVE TISSUE DIAGNOSES WITH CC[MS-DRG]: “OTHER MUSCULOSKELETAL SYSTEM AND CONNECTIVE TISSUE DIAGNOSES WITH CC (MDC 08)”— CMS MS-DRG Definitions Manual (Appendix B) · FY2026
• DRG 566 — OTHER MUSCULOSKELETAL SYSTEM AND CONNECTIVE TISSUE DIAGNOSES WITHOUT CC/MCC[MS-DRG]: “OTHER MUSCULOSKELETAL SYSTEM AND CONNECTIVE TISSUE DIAGNOSES WITHOUT CC/MCC (MDC 08)”— CMS MS-DRG Definitions Manual (Appendix B) · FY2026

MDC crossing

• MDC 08 — Diseases and Disorders of the Musculoskeletal System and Connective Tissue[MDC crossing]: “Diseases and Disorders of the Musculoskeletal System and Connective Tissue — 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. 18,508 same-MDC procedures group here; browse them on the MDC page.”— CMS MS-DRG Definitions Manual · FY2026

Index entries

• Syndrome, arterial tortuosity[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026

Nearest codes (25)

• Q87 — Other specified congenital malformation syndromes affecting multiple systems[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• Q87.0 — Congenital malformation syndromes predominantly affecting facial appearance[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• Q87.1 — Congenital malformation syndromes predominantly associated with short stature[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• Q87.11 — Prader-Willi syndrome[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• Q87.19 — Other congenital malformation syndromes predominantly associated with short stature[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• Q87.2 — Congenital malformation syndromes predominantly involving limbs[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• Q87.3 — Congenital malformation syndromes involving early overgrowth[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• Q87.4 — Marfan syndrome[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• and 17 more

Change history

• FY2017 — Added to the code set[Change history]— CMS release files (code change ledger) · icd10cm-fy2017

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

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

Nearest Codes in This Family

Official ICD-10-CM classifications closest to Q87.82 in its code family, with their registry titles.

• Q87.42 — Marfan syndrome with ocular manifestations
• Q87.43 — Marfan syndrome with skeletal manifestation
• Q87.5 — Other congenital malformation syndromes with other skeletal changes
• Q87.8 — Other specified congenital malformation syndromes, not elsewhere classified
• Q87.81 — Alport syndrome
• Q87.83 — Bardet-Biedl syndrome
• Q87.84 — Laurence-Moon syndrome
• Q87.85 — MED13L syndrome
• Q87.86 — Kleefstra syndrome
• Q87.87 — Hao-Fountain Syndrome

View all codes in the Q87 family