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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}}
{{Family tree | | | C01 | | | C02 | | | C03 | | | C04 | | | C05 | | | | | | | | | | | | | | | | | | | | | | |C01=Capillary malformations|C02=Lymphatic malformations|C03=Venous malformations|C04=Arteriovenous malformations|C05=Arteriovenous fistula}}
{{Family tree | | |!| | | | |!| | | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |!| | | | |!| | | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |)| D01 | |)| E01 | |)| F01 | |)| G01 | |)| H01 | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |)| D01 | |)| E01 | |)| F01 | |)| G01 | |)| H01 | | | | | | | | | | | | | | | | | | | | | |D01=Nevus simplex / salmon patch, “angel kiss”, “stork bite”|}}
{{Family tree | | |!| | | | |!| | | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |!| | | | |!| | | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |)| D02 | |)| E02 | |)| F02 | |)| G02 | |)| H02 | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |)| D02 | |)| E02 | |)| F02 | |)| G02 | |)| H02 | | | | | | | | | | | | | | | | | | | | | |D02=Cutaneous and/or mucosal CM (also known as “port-wine” stain) <br>Nonsyndromic CM <br>CM with CNS and/or ocular anomalies (Sturge-Weber syndrome) <br>CM with bone and/or soft tissues overgrowth <br>Diffuse CM with overgrowth (DCMO)|}}
{{Family tree | | |!| | | | |!| | | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |!| | | | |!| | | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |)| D03 | |)| E03 | |)| F03 | |)| G03 | |)| H03 | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |)| D03 | |)| E03 | |)| F03 | |)| G03 | |)| H03 | | | | | | | | | | | | | | | | | | | | | |D03=Reticulate CM <br>CM of MIC-CAP (microcephaly-capillary malformation) <br>CM of MCAP (megalencephaly-capillary malformation-polymicrogyria)|}}
{{Family tree | | |!| | | | |!| | | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |!| | | | |!| | | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |)| D04 | |)| E04 | |)| F04 | |`| G04 | |`| H04 | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |)| D04 | |)| E04 | |)| F04 | |`| G04 | |`| H04 | | | | | | | | | | | | | | | | | | | | | |D04=CM of CM-AVM|}}
{{Family tree | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |)| D05 | |)| E05 | |)| F05 | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |)| D05 | |)| E05 | |)| F05 | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |D05=Cutis marmorata telangiectatica congenita (CMTC)|}}
{{Family tree | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |)| D06 | |)| E06 | |)| F06 | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |)| D06 | |)| E06 | |)| F06 | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |D06=Others|}}
{{Family tree | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |`| D07 | |`| E07 | |)| F07 | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |`| D07 | |`| E07 | |)| F07 | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |D07=Telangiectasia <br>Hereditary hemorrhagic telangiectasia (HHT) <br>Others|}}
{{Family tree | | | | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | | | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | | | | | | | | | | | |`| F08 | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | | | | | | | | | | | |`| F08 | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}

Revision as of 16:02, 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
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
Limb CM + congenital non-progressive limb overgrowth
Macrocephaly-CM (M-CM / MCAP)
Microcephaly-CM (MICCAP)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Capillary malformations
 
 
Lymphatic malformations
 
 
Venous malformations
 
 
Arteriovenous malformations
 
 
Arteriovenous fistula
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Nevus simplex / salmon patch, “angel kiss”, “stork bite”
 
 
 
{{{ E01 }}}
 
 
 
{{{ F01 }}}
 
 
 
{{{ G01 }}}
 
 
 
{{{ H01 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Cutaneous and/or mucosal CM (also known as “port-wine” stain)
Nonsyndromic CM
CM with CNS and/or ocular anomalies (Sturge-Weber syndrome)
CM with bone and/or soft tissues overgrowth
Diffuse CM with overgrowth (DCMO)
 
 
 
{{{ E02 }}}
 
 
 
{{{ F02 }}}
 
 
 
{{{ G02 }}}
 
 
 
{{{ H02 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Reticulate CM
CM of MIC-CAP (microcephaly-capillary malformation)
CM of MCAP (megalencephaly-capillary malformation-polymicrogyria)
 
 
 
{{{ E03 }}}
 
 
 
{{{ F03 }}}
 
 
 
{{{ G03 }}}
 
 
 
{{{ H03 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
CM of CM-AVM
 
 
 
{{{ E04 }}}
 
 
 
{{{ F04 }}}
 
 
 
{{{ G04 }}}
 
 
 
{{{ H04 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Cutis marmorata telangiectatica congenita (CMTC)
 
 
 
{{{ E05 }}}
 
 
 
{{{ F05 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Others
 
 
 
{{{ E06 }}}
 
 
 
{{{ F06 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Telangiectasia
Hereditary hemorrhagic telangiectasia (HHT)
Others
 
 
 
{{{ 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