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{{Family tree/start}}
{{Family tree | | | | | | | | | | | | | | | | | | | | | | | | A01 | | | | | | | | | | | | | | | | | | | | | |A01= Vascular malformations}}
{{Family tree | | | | | | | | | | | | | | | | | | | | | | | | A01 | | | | | | | | | | | | | | | | | | | | | |A01= Vascular malformations}}
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{{Family tree | | | | | | | | B01 | | | | | | | | B02 | | | | | | | | | | B03 | | | | | | | | B04 | | | | | |B01=Simple|B02=Combined|B03=of major named vessels|B04=asscoiated with other anomalies}}
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{{Family tree | | | | | | | | |!| | | | | | | | | |!| | | | | | | | | | | |!| | | | | | | | | |!| | | | | | |}}
{{Family tree | | | C01 | | | C02 | | | C03 | | | C04 | | | C05 | | | C06 | | | | C07 | | | | C08 | | | | | |}}
{{Family tree | | | | | | | | |!| | | | | | | | | I02 | | | | | | | | | | I03 | | | | | | | | I04 | | | | | | |I02=Combined vascular malformations*
<table class="wikitable">
<tr><td>'''CM + VM'''</td><td>Capillary-venous malformation</td><td>CVM</td></tr>
<tr><td>'''CM + LM'''</td><td>Capillary-lymphatic malformation</td><td>CLM</td></tr>
<tr><td>'''CM + AVM'''</td><td>Capillary-arteriovenous malformation</td><td>CAVM</td></tr>
<tr><td>'''LM + VM'''</td><td>Lymphatic-venous malformation</td><td>LVM</td></tr>
<tr><td>'''CM + LM + VM'''</td><td>Capillary-lymphatic-venous malformation</td><td>CLVM</td></tr>
<tr><td>'''CM + LM + AVM'''</td><td>Capillary-lymphatic-arteriovenous malformation</td><td>CLVM</td></tr>
<tr><td>'''CM + VM + AVM'''</td><td>Capillary-venous-arteriovenous malformation</td><td>CVAVM</td></tr>
<tr><td>'''CM + LM + VM + AVM'''</td><td>Capillary-lymphatic-venous-arteriovenous malformation</td><td>CLVAVM</td></tr>
</table>
|I03='''Anomalies of major named vessels'''<br>(also known as "channel type" or "truncal" vascular malformations)
|I04=Vascular malformations associated with other anomalies
|}}
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{{Family tree | | | C01 | | | C02 | | | C03 | | | C04 | | | C05 | | | | | | | | | | | | | | | | | | | | | | |C01=Capillary malformations|C02=Lymphatic malformations|C03=Venous malformations|C04=Arteriovenous malformations|C05=Arteriovenous fistula}}
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===Tables===
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Revision as of 14:33, 20 September 2018


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hannan Javed, M.D.[2]

Overview

Vascular Anomalies
Vascular Tumors Vascular Malformations
Benign

Locally aggressive or

Borderline

Malignant

Simple Combined° of major named vessels associated with other anomalies
Capillary malformations

Lymphatic malformations

Venous malformations

Arteriovenous malformations*

Arteriovenous fistula*

Capillary venous malformation , Capillary lymphatic malformation

Lymphatic venous malformation, Capillary lymphatic venous malformation

Capillary arteriovenous malformation

Capillary lymphatic arteriovenous malformation

others

See details See list

° defined as two or more vascular malformations found in one lesion

* high flow lesions

Classification

Classification of Vascular Malformations

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Vascular malformations
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Simple
 
 
 
 
 
 
 
Combined
 
 
 
 
 
 
 
 
 
of major named vessels
 
 
 
 
 
 
 
asscoiated with other anomalies
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Combined vascular malformations*
CM + VMCapillary-venous malformationCVM
CM + LMCapillary-lymphatic malformationCLM
CM + AVMCapillary-arteriovenous malformationCAVM
LM + VMLymphatic-venous malformationLVM
CM + LM + VMCapillary-lymphatic-venous malformationCLVM
CM + LM + AVMCapillary-lymphatic-arteriovenous malformationCLVM
CM + VM + AVMCapillary-venous-arteriovenous malformationCVAVM
CM + LM + VM + AVMCapillary-lymphatic-venous-arteriovenous malformationCLVAVM
 
 
 
 
 
 
 
 
 
Anomalies of major named vessels
(also known as "channel type" or "truncal" vascular malformations)
 
 
 
 
 
 
 
Vascular malformations associated with other anomalies
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Capillary malformations
 
 
Lymphatic malformations
 
 
Venous malformations
 
 
Arteriovenous malformations
 
 
Arteriovenous fistula
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
{{{ D01 }}}
 
 
 
{{{ E01 }}}
 
 
 
{{{ F01 }}}
 
 
 
{{{ G01 }}}
 
 
 
{{{ H01 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
{{{ D02 }}}
 
 
 
{{{ E02 }}}
 
 
 
{{{ F02 }}}
 
 
 
{{{ G02 }}}
 
 
 
{{{ H02 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
{{{ D03 }}}
 
 
 
{{{ E03 }}}
 
 
 
{{{ F03 }}}
 
 
 
{{{ G03 }}}
 
 
 
{{{ H03 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
{{{ D04 }}}
 
 
 
{{{ E04 }}}
 
 
 
{{{ F04 }}}
 
 
 
{{{ G04 }}}
 
 
 
{{{ H04 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
{{{ D05 }}}
 
 
 
{{{ E05 }}}
 
 
 
{{{ F05 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
{{{ D06 }}}
 
 
 
{{{ E06 }}}
 
 
 
{{{ F06 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
{{{ D07 }}}
 
 
 
{{{ E07 }}}
 
 
 
{{{ F07 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
{{{ F08 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 


Tables

Anomalies of major named vessels

(also known as "channel type" or "truncal" vascular malformations)

Affect
  lymphatics
  veins
  arteries

Anomalies of

  origin
  course
  number
  length
  diameter (aplasia, hypoplasia, stenosis, ectasia / aneurysm)
  valves
  communication (AVF)
  persistence (of embryonal vessel)


Combined vascular malformations*
CM + VM capillary-venous malformation CVM
CM + LM capillary-lymphatic malformation CLM
CM + AVM capillary-arteriovenous malformation CAVM
LM + VM lymphatic-venous malformation LVM
CM + LM + VM capillary-lymphatic-venous malformation CLVM
CM + LM + AVM capillary-lymphatic-arteriovenous malformation CLAVM
CM + VM + AVM capillary-venous-arteriovenous malformation CVAVM
CM + LM + VM + AVM capillary-lymphatic-venous-arteriovenous m. CLVAVM


Vascular malformations associated with other anomalies
Klippel-Trenaunay syndrome * CM + VM +/-LM + limb overgrowth
Parkes Weber syndrome CM + AVF + limb overgrowth
Servelle-Martorell syndrome limb VM + bone undergrowth
Sturge-Weber syndrome facial + leptomeningeal CM + eye anomalies

+/-bone and/or soft tissue overgrowth

Limb CM + congenital non-progressive limb overgrowth
Maffucci syndrome VM +/-spindle-cell hemangioma + enchondroma
Macrocephaly-CM (M-CM / MCAP) *
Microcephaly-CM (MICCAP)
CLOVES syndrome * LM + VM + CM +/-AVM+ lipomatous overgrowth
Proteus syndrome CM, VM and/or LM + asymmetrical somatic overgrowth
Bannayan-Riley-Ruvalcaba sd lower lip CM + face and neck LM + asymmetry and partial/generalized overgrowth

Pathogenesis of xxx disease is due to:

  • yyy
  • zzz
    1. aaa
    2. vvv

Physiology

Medical Therapy

Naural History

Historical Perspective