{"schema":"https://ver.cy/schemas/card/1.0.0","id":"vr.tr.congenital-cystic-adenomatoid-malformation","code":"thing-q246040","url":"https://ver.cy/models/thing/q246040/","name":"congenital cystic adenomatoid malformation","alternateNames":["congenital pulmonary airway malformation type 0","congenital pulmonary airway malformation type 1","congenital pulmonary airway malformation type 2","congenital pulmonary airway malformation type 3","congenital pulmonary airway malformation type 4"],"kind":"thing","status":"published","version":"0.1.0-research.1","language":"en","classifiers":{"family":"Thing Registry","category":"Cross-cutting context","entryKind":"thing","plane":"XCT","domain":["XCT.STA"],"industry":[],"navPath":"","tags":[],"facets":{}},"whatItIs":"Congenital cystic adenomatoid malformation (CCAM), now generally termed congenital pulmonary airway malformation (CPAM), is a developmental lung malformation characterized by abnormal airway structures that form cystic or solid-appearing lesions and replace normally developed lung tissue.","purpose":"Enable an agent to recognise congenital cystic adenomatoid malformation, assess lesion-specific evidence and clinical state, and support accountable specialist evaluation and follow-up.","scope":{"in":["Historical CCAM terminology, contemporary CPAM terminology and evidence supporting their mapping in individual records.","Suspected or confirmed lesions, including anatomical extent, cyst morphology and vascular relationships.","Prenatal lesion measurements, growth trajectory and associated fetal compromise.","Postnatal diagnostic confirmation, respiratory manifestations and lesion-associated complications.","Specialist decisions about observation, intervention and continuing surveillance."],"out":["General fetal development and normal pulmonary anatomy.","Independent models of bronchopulmonary sequestration, bronchogenic cyst and congenital diaphragmatic hernia.","Pleuropulmonary blastoma classification, staging and cancer treatment.","General management of neonatal respiratory failure or pneumonia.","Technical execution of fetal procedures, anaesthesia and lung resection."],"boundaries":[]},"distinguishingFeatures":["Assess whether imaging demonstrates malformed intrapulmonary cystic or microcystic tissue, rather than identifying every fetal thoracic mass as CCAM.","Document arterial supply and airway relationships when distinguishing an isolated airway malformation from sequestration or a hybrid lesion; retain uncertainty when vascular anatomy is incomplete.","Check diaphragmatic continuity and the identity of thoracic contents when distinguishing a pulmonary lesion from congenital diaphragmatic hernia.","Separate imaging morphology from tissue diagnosis: a cyst-size category alone must not establish a histopathological subtype.","Do not treat a cystic appearance as proof of benign developmental disease: type I pleuropulmonary blastoma can resemble developmental lung cysts and may require expert pathology review. [NCI diagnostic boundary](https://www.cancer.gov/types/lung/hp/child-pleuropulmonary-blastoma-treatment-pdq)"],"structure":{"bundles":[{"id":"diagnostic-identity","name":"Diagnostic identity","description":"Establish what the CCAM label means in this record and how strongly the lesion has been identified.","layers":[{"id":"terminology-and-evidence","name":"Terminology and evidence","description":"Connect the recorded name to the clinician's intended concept and supporting observations.","findings":[{"id":"ccam-cpam-mapping","name":"CCAM CPAM mapping","description":"Retain the original term and document whether its use corresponds to contemporary CPAM terminology, including any unresolved scope difference.","questions":[{"text":"What anatomical abnormality does the author mean by CCAM or CPAM in this record?","id":"ccam-cpam-mapping-q01","kind":"definition"},{"text":"Which dated imaging interpretation or pathology report supports the diagnosis, and who issued it?","id":"ccam-cpam-mapping-q02","kind":"provenance"}]}]},{"id":"competing-lesion-identities","name":"Competing lesion identities","description":"Make alternative diagnoses and reasons for retaining or excluding them explicit.","findings":[{"id":"differential-resolution","name":"Differential resolution","description":"Maintain a case-specific differential covering sequestration, hybrid lesions, other congenital thoracic abnormalities and relevant cystic neoplasms.","questions":[{"text":"Which observations favour CCAM over sequestration, a bronchogenic cyst, lobar overinflation or diaphragmatic hernia?","id":"differential-resolution-q01","kind":"boundary"},{"text":"What unresolved imaging, tissue or family-history findings require specialist review for pleuropulmonary blastoma?","id":"differential-resolution-q02","kind":"action"}]}]}]},{"id":"lesion-anatomy-and-classification","name":"Lesion anatomy and classification","description":"Describe the pulmonary lesion in terms that remain traceable across imaging, surgery and pathology.","layers":[{"id":"pulmonary-and-vascular-map","name":"Pulmonary and vascular map","description":"Locate the lesion and establish its relationships to functioning lung, vessels and adjacent structures.","findings":[{"id":"extent-and-connections","name":"Extent and connections","description":"Record affected lobes, lesion boundaries, arterial supply, venous drainage and any demonstrated airway relationship.","questions":[{"text":"Which lobes are involved, and what are the lesion dimensions on the latest adequately documented study?","id":"extent-and-connections-q01","kind":"measurement"},{"text":"Does demonstrated systemic arterial supply support a sequestration component, and what vascular anatomy remains unresolved?","id":"extent-and-connections-q02","kind":"boundary"}]}]},{"id":"imaging-and-tissue-phenotype","name":"Imaging and tissue phenotype","description":"Keep visible cyst morphology distinct from classification based on sampled tissue.","findings":[{"id":"phenotype-classification-concordance","name":"Phenotype classification concordance","description":"Record the imaging category and any histological subtype independently, with their methods and disagreements.","questions":[{"text":"What cyst-size convention and measurements support the reported macrocystic, microcystic or mixed appearance?","id":"phenotype-classification-concordance-q01","kind":"measurement"},{"text":"Which specimen and pathology report establish the subtype, and does that interpretation agree with the imaging diagnosis?","id":"phenotype-classification-concordance-q02","kind":"provenance"}]}]}]},{"id":"prenatal-course-and-birth-transition","name":"Prenatal course and birth transition","description":"Track fetal lesion behaviour and the evidence needed to plan assessment around delivery.","layers":[{"id":"serial-fetal-assessment","name":"Serial fetal assessment","description":"Compare dated lesion measurements alongside documented effects on the fetus.","findings":[{"id":"growth-and-fetal-compromise","name":"Growth and fetal compromise","description":"Record lesion growth, reported CVR, dominant cyst behaviour and assessments of hydrops or thoracic compression without assigning an unsupported universal intervention threshold.","questions":[{"text":"How have lesion dimensions and CVR changed with gestational age, and are the measurement methods comparable?","id":"growth-and-fetal-compromise-q01","kind":"measurement"},{"text":"What evidence of hydrops or worsening compression has the fetal team documented, and what review does its protocol require?","id":"growth-and-fetal-compromise-q02","kind":"action"}]}]},{"id":"delivery-and-neonatal-reassessment","name":"Delivery and neonatal reassessment","description":"Carry prenatal uncertainty and lesion-specific concerns into neonatal evaluation.","findings":[{"id":"birth-handover-and-confirmation","name":"Birth handover and confirmation","description":"Connect the delivery plan, initial respiratory assessment and planned postnatal lesion characterisation.","questions":[{"text":"What delivery setting and neonatal assessment have the responsible teams selected from the fetal findings?","id":"birth-handover-and-confirmation-q01","kind":"action"},{"text":"If the lesion becomes less visible prenatally, what postnatal evidence is required before recording anatomical resolution?","id":"birth-handover-and-confirmation-q02","kind":"boundary"}]}]}]},{"id":"postnatal-decisions-and-outcomes","name":"Postnatal decisions and outcomes","description":"Relate symptoms and diagnostic confidence to specialist decisions and subsequent lesion status.","layers":[{"id":"clinical-burden-and-treatment-choice","name":"Clinical burden and treatment choice","description":"Separate symptoms attributed to the lesion from coincidental illness and document the management rationale.","findings":[{"id":"observation-or-intervention","name":"Observation or intervention","description":"Record respiratory burden, infection history, pneumothorax and diagnostic concerns alongside the specialist's assessment of observation and intervention.","questions":[{"text":"Which respiratory symptoms or complications are attributed to this lesion, and what evidence supports that attribution?","id":"observation-or-intervention-q01","kind":"boundary"},{"text":"Why was surveillance or intervention selected, and which changes would trigger reconsideration?","id":"observation-or-intervention-q02","kind":"action"}]}]},{"id":"residual-lesion-and-follow-up","name":"Residual lesion and follow-up","description":"Reconcile treatment results, final diagnosis and the continuing need for assessment.","findings":[{"id":"anatomical-and-diagnostic-outcome","name":"Anatomical and diagnostic outcome","description":"Track residual abnormalities, pathology-driven reclassification and follow-up responsibility after either observation or treatment.","questions":[{"text":"What imaging, operative or pathology evidence establishes persistent, removed or reclassified lesion status?","id":"anatomical-and-diagnostic-outcome-q01","kind":"provenance"},{"text":"Who owns the next review, what will it assess, and what criteria justify continuing or ending surveillance?","id":"anatomical-and-diagnostic-outcome-q02","kind":"action"}]}]}]},{"id":"evidence-and-external-alignment","name":"Evidence and external alignment","description":"What the world already says about this thing, gathered so the model can be checked against it.","layers":[{"id":"reported-evidence","name":"Reported evidence","description":"Findings from the breadth pass, kept separate from the structural claims.","findings":[{"id":"evidence-confidence-notes","name":"Check these first","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these check these first hold for the sense of congenital cystic adenomatoid malformation this model covers, and on what evidence?","id":"evidence-confidence-notes-q01","kind":"provenance"}]},{"id":"evidence-kinds","name":"Kinds and varieties","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these kinds and varieties hold for the sense of congenital cystic adenomatoid malformation this model covers, and on what evidence?","id":"evidence-kinds-q01","kind":"provenance"}]},{"id":"evidence-identifiers","name":"Identifiers and schemes","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these identifiers and schemes hold for the sense of congenital cystic adenomatoid malformation this model covers, and on what evidence?","id":"evidence-identifiers-q01","kind":"provenance"}]},{"id":"evidence-real-world-use","name":"Real-world use","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these real-world use hold for the sense of congenital cystic adenomatoid malformation this model covers, and on what evidence?","id":"evidence-real-world-use-q01","kind":"provenance"}]},{"id":"evidence-measurements","name":"Typical measurements","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these typical measurements hold for the sense of congenital cystic adenomatoid malformation this model covers, and on what evidence?","id":"evidence-measurements-q01","kind":"provenance"}]},{"id":"evidence-failure-modes-and-hazards","name":"Failure modes and hazards","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these failure modes and hazards hold for the sense of congenital cystic adenomatoid malformation this model covers, and on what evidence?","id":"evidence-failure-modes-and-hazards-q01","kind":"provenance"}]},{"id":"evidence-neighbours","name":"Neighbouring kinds and how to tell them apart","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these neighbouring kinds and how to tell them apart hold for the sense of congenital cystic adenomatoid malformation this model covers, and on what evidence?","id":"evidence-neighbours-q01","kind":"provenance"}]}]}]}]},"agentConduct":{"may":["Explain the condition in general terms, citing medical sources.","Help clinicians find records and guidance they are authorized to access.","Point families to support and specialist services."],"mustNot":["Diagnose or rule out the condition in a specific fetus or child.","Advise on pregnancy decisions or surgery.","Share a family's prenatal or medical records without consent.","Present prognosis figures as certain for an individual case."],"requiresHuman":["Telling families about a diagnosis.","Any decision about delivery, surgery or follow-up."]},"ethics":{"considerations":["Prenatal findings create hard choices for families who need accurate, compassionate information.","Many cases have good outcomes; overstating risk causes needless distress."],"affectedParties":["Children and fetuses","Parents","Clinicians"]},"owners":{"steward":"The treating clinicians; the family holds the right to the child's health information.","roles":[],"masterSystems":["Patient health records","Congenital anomaly registers"]},"relations":[{"target":"Q18975820","type":"parent","note":"registry parent class"},{"target":"Q3392853","type":"parent","note":"registry parent class"},{"target":"Q55785339","type":"parent","note":"registry parent class"},{"target":"Q55785522","type":"parent","note":"registry parent class"}],"interaction":{"identity":{"applicability":"required","items":["Vercy registry: vr.tr.congenital-cystic-adenomatoid-malformation","Wikidata: Q246040 (https://www.wikidata.org/wiki/Q246040)"]},"properties":{"applicability":"not-applicable","items":["Diagnostic certainty: suspected; imaging-supported; histologically confirmed; disputed; reclassified","Observation age: gestational weeks and days, or postnatal age, with observation date","Pulmonary distribution: affected lung, lobe or lobes and relation to adjacent structures","Cyst morphology: macrocystic; microcystic; mixed; indeterminate, with the reporting convention","Lesion and dominant cyst dimensions: three lesion dimensions and maximum dominant cyst diameter in mm, with modality and date","Prenatal lesion volume ratio: reported CVR with calculation convention, input units, head circumference and gestational age","Vascular anatomy: documented arterial origin and venous drainage; unresolved vessels explicitly identified","Histopathological classification: reported classification system and subtype; unclassified; tissue unavailable","Lesion-associated compromise: dated assessment of hydrops, mediastinal displacement, respiratory symptoms, infection and pneumothorax; absent, present or unknown for each","Management status: under evaluation; surveillance; intervention planned; intervention completed; residual lesion; follow-up incomplete"]},"recognition":{"applicability":"optional","items":["Imaging may show multiple air-filled or fluid-filled cystic spaces grouped within a lung region.","Some lesions appear predominantly solid on prenatal ultrasound because their cysts are too small to resolve individually.","Larger lesions may compress adjacent lung and displace central chest structures, although these features are not specific to this condition.","Overlapping appearances with other congenital lung lesions mean visual findings alone do not establish the diagnosis."]},"capabilities":{"applicability":"required","items":["Reconcile CCAM and CPAM labels while preserving the original diagnosis and its evidential basis.","Compare serial prenatal measurements using compatible methods and identify changes needing specialist review.","Prepare a lesion-specific differential review from imaging, vascular anatomy and pathology.","Assemble a fetal-to-neonatal handover covering lesion location, diagnostic uncertainty and anticipated care needs.","Record the evidence, alternatives and clinician responsibility behind observation or intervention.","Track postnatal confirmation, specimen results and surveillance obligations through changes in diagnosis."]},"hazards":{"applicability":"required","items":["Breathing difficulty in newborns with large lesions.","Repeated chest infections if untreated."]},"interfaces":{"applicability":"required","items":["The International Classification of Diseases, Tenth Revision, issued by the World Health Organization, includes congenital cystic lung under category Q33.0."]},"context":{"applicability":"required","items":["This condition is encountered in prenatal imaging, neonatal assessment and investigations of respiratory symptoms or recurrent lung infection.","The term supports communication among imaging, paediatric, surgical and pathology specialists about abnormal lung development and lesion-specific findings."]}},"sources":[{"title":"Wikidata item Q246040: congenital cystic adenomatoid malformation","url":"https://www.wikidata.org/wiki/Q246040","note":"identity and sense of the item"},{"title":"Wikipedia: Congenital pulmonary airway malformation","url":"https://en.wikipedia.org/wiki/Congenital_pulmonary_airway_malformation","note":"general description of the item"}],"openQuestions":["Does the registry intend historical CCAM to encompass the full contemporary CPAM category, and does an existing world model already own that concept?","Which imaging morphology and histopathological classification conventions should this model adopt, including how it should represent disputed subtype boundaries?","Which prenatal measurements, trajectories and fetal findings support intervention decisions across gestational ages and treatment centres?","For asymptomatic postnatal lesions, what comparative evidence supports surveillance versus elective resection, and what follow-up duration is justified?","Which findings should prompt expert pathology review or DICER1 evaluation, and how should the model distinguish initial misclassification as CPAM from evidence of subsequent neoplastic change?"],"resources":{"spec":"/models/things/publications/thing-q246040/spec.json"},"provenance":{"origin":"thing registry research (pass 2)","builtFrom":["models/things/publications/thing-q246040/spec.json"],"providers":["Codex"],"researchStatus":"unreviewed","generatedAt":"2026-09-12T08:49:51Z","builder":"tools/build_cards.py@1.0.0"},"completeness":{"sections":{"classifiers":"filled","whatItIs":"filled","purpose":"filled","distinguishingFeatures":"filled","structure":"filled","agentConduct":"filled","ethics":"filled","owners":"filled","relations":"filled","interaction.identity":"filled","interaction.properties":"not-applicable","interaction.recognition":"filled","interaction.capabilities":"filled","interaction.hazards":"filled","interaction.interfaces":"filled","interaction.context":"filled","sources":"filled"},"notes":{"_gate":"Published through the thing publication gate 1.0 on 2026-10-07T10:10:29+00:00: completeness 1.00; sense: Wikidata description; 2 live sources; review by codex: pass","interaction.properties":"Plane XCT: no invented physical properties."},"score":1.0}}