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===Classification of Vascular Malformations===
===Classification of Vascular Malformations===
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|I03='''Anomalies of major named vessels'''<br>(also known as "channel type" or "truncal" vascular malformations)
|I03='''Anomalies of major named vessels'''<br>(also known as "channel type" or "truncal" vascular malformations)
|I04=Vascular malformations associated with other anomalies
|I04=Vascular malformations associated with other anomalies
<table class="wikitable">
<tr><td>'''Klippel-Trenaunay syndrome'''</td><td>CM + VM +/-LM + limb overgrowth</td></tr>
<tr><td>'''Parke's Weber syndrome'''</td><td>CM + AVF + limb overgrowth</td></tr>
<tr><td>'''Servelle-Martorell syndrome'''</td><td>Limb VM + bone undergrowth</td></tr>
<tr><td>'''Sturge-Weber syndrome'''</td><td>Facial + leptomeningeal CM + eye anomalies
+/-bone and/or soft tissue overgrowth</td></tr>
<tr><td>'''Maffucci syndrome'''</td><td>VM +/-spindle-cell hemangioma + enchondroma</td></tr>
<tr><td>'''Limb CM + congenital non-progressive limb overgrowth'''</td><td></td></tr>
<tr><td>'''Macrocephaly-CM (M-CM / MCAP)'''</td><td></td></tr>
<tr><td>'''Microcephaly-CM (MICCAP)'''</td><td></td></tr>
<tr><td>'''CLOVES syndrome'''</td><td>LM + VM + CM +/-AVM+ lipomatous overgrowth</td></tr>
<tr><td>'''Proteus syndrome'''</td><td>CM, VM and/or LM + asymmetrical somatic overgrowth</td></tr>
<tr><td>'''Bannayan-Riley-Ruvalcaba sd'''</td><td>lower lip CM + face and neck LM + asymmetry and partial/generalized overgrowth</td></tr>
</table>
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===Tables===
===Tables===

Revision as of 15:21, 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
Klippel-Trenaunay syndromeCM + VM +/-LM + limb overgrowth
Parke's Weber syndromeCM + AVF + limb overgrowth
Servelle-Martorell syndromeLimb VM + bone undergrowth
Sturge-Weber syndromeFacial + leptomeningeal CM + eye anomalies +/-bone and/or soft tissue overgrowth
Maffucci syndromeVM +/-spindle-cell hemangioma + enchondroma
Limb CM + congenital non-progressive limb overgrowth
Macrocephaly-CM (M-CM / MCAP)
Microcephaly-CM (MICCAP)
CLOVES syndromeLM + VM + CM +/-AVM+ lipomatous overgrowth
Proteus syndromeCM, VM and/or LM + asymmetrical somatic overgrowth
Bannayan-Riley-Ruvalcaba sdlower lip CM + face and neck LM + asymmetry and partial/generalized overgrowth
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Capillary malformations
 
 
Lymphatic malformations
 
 
Venous malformations
 
 
Arteriovenous malformations
 
 
Arteriovenous fistula
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
{{{ D01 }}}
 
 
 
{{{ E01 }}}
 
 
 
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{{{ D02 }}}
 
 
 
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{{{ G02 }}}
 
 
 
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{{{ 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