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| ===Classification of Vascular Malformations=== | | ===Classification of Vascular Malformations=== |
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| {{Family tree/start}}
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| {{Family tree | | | | | | | | | A01 | | | | | | | | | | |A01= Vascular malformations}}
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| {{Family tree | | | |,|-|-|-|v|-|^|-|-|v|-|-|.| | |}}
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| {{Family tree | | | B01 | | B02 | | B03 | | B04 | |B01=Simple vascular malformations|B02=Combined vascular malformations|B03=Vascular malformations of major named vessels|B04=Vascular malformations asscoiated with other anomalies}}
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| {{Family tree | | | |!| | | |!| | | |!| | | |!| | |}}
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| {{Family tree | | | |!| | | I02 | | I03 | | I04 | |I02=Combined vascular malformations*<table class="wikitable">
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| <tr><td>'''CM + VM'''</td><td>Capillary-venous malformation</td><td>CVM</td></tr>
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| <tr><td>'''CM + LM'''</td><td>Capillary-lymphatic malformation</td><td>CLM</td></tr>
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| <tr><td>'''CM + AVM'''</td><td>Capillary-arteriovenous malformation</td><td>CAVM</td></tr>
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| <tr><td>'''LM + VM'''</td><td>Lymphatic-venous malformation</td><td>LVM</td></tr>
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| <tr><td>'''CM + LM + VM'''</td><td>Capillary-lymphatic-venous malformation</td><td>CLVM</td></tr>
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| <tr><td>'''CM + LM + AVM'''</td><td>Capillary-lymphatic-arteriovenous malformation</td><td>CLVM</td></tr>
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| <tr><td>'''CM + VM + AVM'''</td><td>Capillary-venous-arteriovenous malformation</td><td>CVAVM</td></tr>
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| <tr><td>'''CM + LM + VM + AVM'''</td><td>Capillary-lymphatic-venous-arteriovenous malformation</td><td>CLVAVM</td></tr>
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| </table>|I03='''Anomalies of major named vessels'''<br>(also known as "channel type" or "truncal" vascular malformations)<br><table>
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| <tr><td>Affect</td></tr>
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| <tr><td>• Lymphatics<td></tr>
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| <tr><td>• Veins</tr>
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| <tr><td>• Arteries</tr>
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| <tr><td>Anomalies of</td></tr>
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| <tr><td>• Origin<td></tr>
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| <tr><td>• Course<td></tr>
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| <tr><td>• Number<td></tr>
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| <tr><td>• Diameter (aplasia,<br>hypoplasia, stenosis,<br>ectasia / aneurysm)<td></tr>
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| <tr><td>• Valves<td></tr>
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| <tr><td>• Communication (AVF)<td></tr>
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| <tr><td>• Persistence (of<br>embryonal vessel)<td></tr>
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| </table>|I04=Vascular malformations associated with other anomalies<table class="wikitable">
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| <tr><td>'''Klippel-Trenaunay syndrome'''</td><td>CM + VM +/-LM + limb overgrowth</td></tr>
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| <tr><td>'''Parke's Weber syndrome'''</td><td>CM + AVF + limb overgrowth</td></tr>
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| <tr><td>'''Servelle-Martorell syndrome'''</td><td>Limb VM + bone undergrowth</td></tr>
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| <tr><td>'''Sturge-Weber syndrome'''</td><td>Facial + leptomeningeal CM + eye anomalies +/-bone and/or soft tissue overgrowth</td></tr>
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| <tr><td>'''Maffucci syndrome'''</td><td>VM +/-spindle-cell hemangioma + enchondroma</td></tr>
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| <tr><td>'''CLOVES syndrome'''</td><td>LM + VM + CM +/-AVM+ lipomatous overgrowth</td></tr>
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| <tr><td>'''Proteus syndrome'''</td><td>CM, VM and/or LM + asymmetrical somatic overgrowth</td></tr>
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| <tr><td>'''Bannayan-Riley-Ruvalcaba sd'''</td><td>lower lip CM + face and neck LM + asymmetry and partial/generalized overgrowth</td></tr>
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| <tr><td colspan="2">'''Limb CM + congenital non-progressive limb overgrowth'''</td></tr>
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| <tr><td colspan="2">'''Macrocephaly-CM (M-CM / MCAP)'''</td></tr>
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| <tr><td colspan="2">'''Microcephaly-CM (MICCAP)'''</td></tr>
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| </table>|}}
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| {{Family tree | | | |)|-|-|-|v|-|-|-|v|-|-|-|v|-|-|-|.| | |}}
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| {{Family tree | | | C01 | | C02 | | C03 | | C04 | | C05 | |C01=Capillary malformations (CM)|C02=Lymphatic malformations (LM)|C03=Venous malformations (VM)|C04=Arteriovenous<br>malformations (AVM)|C05=Arteriovenous fistula}}
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| {{Family tree | | |!| | | |!| | | |!| | | |!| | | |!| | | | |}}
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| {{Family tree | | |)| D01 |)| E01 |)| F01 |)| G01 |)| H01 | |D01=Nevus simplex / salmon patch, “angel kiss”, “stork bite”|E01=<table>
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| <tr><td>Common (cystic) LM </td></tr>
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| <tr><td>• Macrocystic LM<td></tr>
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| <tr><td>• Microcystic LM<td></tr>
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| <tr><td>• Mixed cystic LM<td></tr>
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| </table>|F01=Common VM|G01=Sporadic|H01=Sporadic}}
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| {{Family tree | | |!| | | |!| | | |!| | | |!| | | |!| | | | |}}
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| {{Family tree | | |)| D02 |)| E02 |)| F02 |)| G02 |)| H02 | |D02=<table>
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| <tr><td>Cutaneous and/or mucosal CM (also known as “port-wine” stain) </td></tr>
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| <tr><td>• Nonsyndromic CM<td></tr>
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| <tr><td>• CM with CNS and/or ocular anomalies (Sturge-Weber syndrome)<td></tr>
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| <tr><td>• CM with bone and/or soft tissues overgrowth <td></tr>
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| <tr><td>• Diffuse CM with overgrowth (DCMO) <td></tr>
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| </table>|E02=Generalized lymphatic anomaly (GLA)<br>Kaposiform lymphangiomatosis (KLA)|F02=Familial VM cutaneo-mucosal (VMCM)|G02=In HHT|H02=In HHT|}}
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| {{Family tree | | |!| | | |!| | | |!| | | |!| | | |!| | | | |}}
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| {{Family tree | | |)| D03 |)| E03 |)| F03 |)| G03 |)| H03 | |D03=<table>
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| <tr><td>Reticulate CM </td></tr>
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| <tr><td>• CM of MIC-CAP (microcephaly-capillary malformation)<td></tr>
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| <tr><td>• CM of MCAP (megalencephaly-capillary malformation-polymicrogyria)<td></tr>
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| </table>
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| |E03=LM in Gorham-Stout disease|F03=Blue rubber bleb nevus (Bean) syndrome VM|G03=In CM-AVM|H03=In CM-AVM|}}
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| {{Family tree | | |!| | | |!| | | |!| | | |!| | | |!| | | | | |}}
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| {{Family tree | | |)| D04 |)| E04 |)| F04 |`| G04 |`| H04 | | |D04=CM of CM-AVM|E04=Channel type LM|F04=Glomuvenous malformation (GVM)|G04=Others|H04=Others|}}
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| {{Family tree | | |!| | | |!| | | |!| | | | | | | | | | | | | |}}
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| {{Family tree | | |)| D05 |)| E05 |)| F05 | | | | | | | | | | | |D05=Cutis marmorata telangiectatica congenita (CMTC)|E05=“Acquired” progressive lymphatic anomaly (so called acquired progressive "lymphangioma")|F05=Cerebral cavernous malformation (CCM) |}}
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| {{Family tree | | |!| | | |!| | | |!| | | | | | | | | | | | | |}}
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| {{Family tree | | |)| D06 |)| E06 |)| F06 | | | | | | | | | | |D06=Others|E06=Primary lymphedema |F06=Familial intraosseous vascular malformation (VMOS)|}}
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| {{Family tree | | |`| D07 |`| E07 |)| F07 | | | | | | | | | | |D07=<table>
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| <tr><td>Telangiectasia </td></tr>
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| <tr><td>• Hereditary hemorrhagic telangiectasia (HHT) <td></tr>
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| <tr><td>• Others<td></tr>
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| </table>|E07=Others|F07=Verrucous venous malformation (formerly verrucous hemangioma)|}}
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| {{Family tree | | | | | | | | | | |`| F08 | | | | | | | | | | |F08=Others|}}
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| {{Family tree/end}}
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