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Q33.0 ICD-10-CM Code: Congenital cystic lung

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

Coding at a Glance

Tabular directives
3 inclusion terms · 2 Excludes1

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 205 — OTHER RESPIRATORY SYSTEM DIAGNOSES WITH MCC (MDC 04)
  • MS-DRG 206 — OTHER RESPIRATORY SYSTEM DIAGNOSES WITHOUT MCC (MDC 04)

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.

Coding instructions

Official Tabular Instructional Notes: the inclusion, exclusion and sequencing notes published for Q33.0 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

Inclusion Terms

Alternative terms the tabular list files under this code.

  • Congenital cystic lung disease
  • Congenital honeycomb lung
  • Congenital polycystic lung disease

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).

Coder workflow for Q33.0

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

Before you code Q33.0

  1. Check the Excludes1 note: if the documentation supports a condition named there, do not simply proceed with Q33.0. 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 Q33.0’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.

    ReviewE84.0, J98.4

Consider Q33.0. 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 Q33.0(2 notes)

    Coding workflow: If the documentation supports a condition named in this note, do not simply proceed with Q33.0: the two are not reported together. The one exception is when the record shows the two conditions are unrelated to each other.

    CompareE84.0, J98.4

    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 Q33.0 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).

ReviewE84.0, J98.4

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 Q33.0 with…

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

The codes most likely to be picked instead of Q33.0 — 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.

  • E84.0— 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 cystic fibrosis moves the case to E84.0; the two are not reported together unless documented as unrelated.

    Full comparison: Q33.0 vs E84.0 →

  • J98.4— 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 cystic lung disease, acquired or unspecified moves the case to J98.4; the two are not reported together unless documented as unrelated.

    Full comparison: Q33.0 vs J98.4 →

  • Q33.9— 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 malformation of lung, unspecified” takes Q33.9; specificity in the record moves it back here.

    Full comparison: Q33.0 vs Q33.9 →

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.

Code Overview

Congenital cystic lung is a billable ICD-10-CM diagnosis code (Q33.0).

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 (10)

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 (FY2027). 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 Q33.0 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 1 Code Also instruction: P28 — Other respiratory conditions originating in the perinatal period (via Q33.-).

These codes suggest coding this condition alongside when both are present.

MS-DRG Grouper Relationships (FY2027)

Potential MS-DRG participation — not a DRG assignment.

FY2027 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 13 clinically related codes on its CMS exclusion list.

Named in the grouper logic of 2 MS-DRGs: DRG 205 (MDC 04), DRG 206 (MDC 04).

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

Clinical classification (AHRQ CCSR):MAL007 — Respiratory congenital malformations (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.

Q33.1 — Accessory lobe of lung, Q33.2 — Sequestration of lung, Q33.3 — Agenesis of lung, Q33.4 — Congenital bronchiectasis, Q33.5 — Ectopic tissue in lung, Q33.6 — Congenital hypoplasia and dysplasia of lung, Q33.8 — Other congenital malformations of lung, Q33.9 — Congenital malformation of lung, unspecified, Q34.0 — Anomaly of pleura, Q34.1 — Congenital cyst of mediastinum, Q34.8 — Other specified congenital malformations of respiratory system, Q34.9 — Congenital malformation of respiratory system, unspecified

Same clinical category (CCSR)

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

Q31.2 — Laryngeal hypoplasia, Q31.3 — Laryngocele, Q31.5 — Congenital laryngomalacia, Q31.8 — Other congenital malformations of larynx, Q31.9 — Congenital malformation of larynx, unspecified, Q32.0 — Congenital tracheomalacia, Q32.1 — Other congenital malformations of trachea, Q32.2 — Congenital bronchomalacia, Q32.3 — Congenital stenosis of bronchus, Q32.4 — Other congenital malformations of bronchus, Q33.1 — Accessory lobe of lung, Q33.2 — Sequestration of lung, Q33.3 — Agenesis of lung, Q33.4 — Congenital bronchiectasis, Q33.5 — Ectopic tissue in lung, Q33.6 — Congenital hypoplasia and dysplasia of lung, Q33.8 — Other congenital malformations of lung, Q33.9 — Congenital malformation of lung, unspecified, Q34.0 — Anomaly of pleura, Q34.1 — Congenital cyst of mediastinum, +10 more

Same Index main term, other category

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

Q04.6 — Congenital cerebral cysts (porencephalic), Q06.8 — Other specified congenital malformations of spinal cord (neuroenteric), Q11.0 — Cystic eyeball (eyeball), Q12.8 — Other congenital lens malformations (lens, congenital), Q15.8 — Other specified congenital malformations of eye (eye NEC, congenital), Q18.0 — Sinus, fistula and cyst of branchial cleft (branchial), Q18.1 — Preauricular sinus and cyst (ear), Q18.8 — Other specified congenital malformations of face and neck (medial, face and neck), Q24.8 — Other specified congenital malformations of heart (celomic), Q31.8 — Other congenital malformations of larynx (congenital NEC, larynx), Q34.1 — Congenital cyst of mediastinum (mediastinum, congenital), Q38.3 — Other congenital malformations of tongue (congenital NEC, tongue), Q38.4 — Congenital malformations of salivary glands and ducts (congenital NEC, salivary gland), Q38.6 — Other congenital malformations of mouth (congenital NEC, sublingual), Q39.8 — Other congenital malformations of esophagus (congenital NEC, esophagus), Q43.8 — Other specified congenital malformations of intestine (enterogenous), Q44.4 — Choledochal cyst (choledochus, congenital), Q44.6 — Cystic disease of liver (liver), Q45.2 — Congenital pancreatic cyst (pancreas, pancreatic, congenital), Q45.8 — Other specified congenital malformations of digestive system (omentum, congenital), +201 more

Contextual Map

Every relationship of Q33.0 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 Q33.0 with these 3 related codes in Claim Check

Hierarchy

Excludes1

Referenced by Code Also instructions

Clinical classification (CCSR)

MS-DRG Grouper

MDC crossing

  • MDC 04 — Diseases and Disorders of the Respiratory System[MDC crossing]: “Diseases and Disorders of the Respiratory 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. 4,000 same-MDC procedures group here; browse them on the MDC page.”— CMS MS-DRG Definitions Manual · FY2027

Index entries (10)

  • Cyst (colloid) (mucous) (simple) (retention), bronchogenic (mediastinal) (sequestration), congenital[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Cyst (colloid) (mucous) (simple) (retention), congenital NEC, lung[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Cyst (colloid) (mucous) (simple) (retention), lung, congenital[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Cystic, lung disease, congenital[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Disease, diseased, cystic, lung, congenital[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Disease, diseased, lung, cystic, congenital[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Disease, diseased, lung, polycystic, congenital[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Disease, diseased, polycystic, lung or pulmonary, congenital[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • and 2 more

Nearest codes (9)

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 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
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. "Q33.0 — Congenital cystic lung." ICD-10-CM FY2027. https://medcoder.ai/icd10/code/q33.0-congenital-cystic-lung

Change history

  • FY2016 — October 1, 2015
    In the code set at ICD-10-CM adoption
    Congenital cystic lung

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 Q33.0 in its code family, with their registry titles.

View all codes in the Q33 family