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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 | | | | | | | | | | | | | | | | | | | | | |D01=Nevus simplex / salmon patch, “angel kiss”, “stork bite”|}}
{{Family tree | | |)| D01 | |)| E01 | |)| F01 | |)| G01 | |)| H01 | | | | | | | | | | | | | | | | | | | | | |D01=Nevus simplex / salmon patch, “angel kiss”, “stork bite”|E01=Common (cystic) LM <br>Macrocystic  LM <br>Microcystic  LM <br>Mixed cystic LM|F01=Common VM|G01=Sporadic|H01=Sporadic}}
{{Family tree | | |!| | | | |!| | | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |!| | | | |!| | | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | |}}
{{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 | | |)| 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)|E02=Generalized lymphatic anomaly (GLA) <br>Kaposiform lymphangiomatosis (KLA)|F02=Familial VM cutaneo-mucosal (VMCM)|G02=In HHT|H02=In HHT|}}
{{Family tree | | |!| | | | |!| | | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |!| | | | |!| | | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | |}}
{{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 | | |)| D03 | |)| E03 | |)| F03 | |)| G03 | |)| H03 | | | | | | | | | | | | | | | | | | | | | |D03=Reticulate CM <br>CM of MIC-CAP (microcephaly-capillary malformation) <br>CM of MCAP (megalencephaly-capillary malformation-polymicrogyria)|E03=LM in Gorham-Stout disease|F03=Blue rubber bleb nevus (Bean) syndrome VM|G03=In CM-AVM|H03=In CM-AVM|}}
{{Family tree | | |!| | | | |!| | | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |!| | | | |!| | | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |)| D04 | |)| E04 | |)| F04 | |`| G04 | |`| H04 | | | | | | | | | | | | | | | | | | | | | |D04=CM of CM-AVM|}}
{{Family tree | | |)| D04 | |)| E04 | |)| F04 | |`| G04 | |`| H04 | | | | | | | | | | | | | | | | | | | | | |D04=CM of CM-AVM|E04=Channel type LM|F04=Glomuvenous malformation (GVM)|G04=Others|H04=Others|}}
{{Family tree | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |)| D05 | |)| E05 | |)| F05 | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |D05=Cutis marmorata telangiectatica congenita (CMTC)|}}
{{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) |}}
{{Family tree | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |)| D06 | |)| E06 | |)| F06 | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |D06=Others|}}
{{Family tree | | |)| D06 | |)| E06 | |)| F06 | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |D06=Others|E06=Primary lymphedema |F06=Familial intraosseous vascular malformation (VMOS)|}}
{{Family tree | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |!| | | | |!| | | | |!| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | |`| D07 | |`| E07 | |)| F07 | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |D07=Telangiectasia <br>Hereditary hemorrhagic telangiectasia (HHT) <br>Others|}}
{{Family tree | | |`| D07 | |`| E07 | |)| F07 | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |D07=Telangiectasia <br>Hereditary hemorrhagic telangiectasia (HHT) <br>Others|E07=Others|F07=Verrucous venous malformation (formerly verrucous hemangioma)|}}
{{Family tree | | | | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | | | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | | | | | | | | | | | |`| F08 | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | | | | | | | | | | | |`| F08 | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |F08=Others|}}
{{Family tree | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}

Revision as of 17:35, 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”
 
 
 
Common (cystic) LM
Macrocystic LM
Microcystic LM
Mixed cystic LM
 
 
 
Common VM
 
 
 
Sporadic
 
 
 
Sporadic
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
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)
 
 
 
Generalized lymphatic anomaly (GLA)
Kaposiform lymphangiomatosis (KLA)
 
 
 
Familial VM cutaneo-mucosal (VMCM)
 
 
 
In HHT
 
 
 
In HHT
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Reticulate CM
CM of MIC-CAP (microcephaly-capillary malformation)
CM of MCAP (megalencephaly-capillary malformation-polymicrogyria)
 
 
 
LM in Gorham-Stout disease
 
 
 
Blue rubber bleb nevus (Bean) syndrome VM
 
 
 
In CM-AVM
 
 
 
In CM-AVM
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
CM of CM-AVM
 
 
 
Channel type LM
 
 
 
Glomuvenous malformation (GVM)
 
 
 
Others
 
 
 
Others
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Cutis marmorata telangiectatica congenita (CMTC)
 
 
 
“Acquired” progressive lymphatic anomaly (so called acquired progressive "lymphangioma")
 
 
 
Cerebral cavernous malformation (CCM)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Others
 
 
 
Primary lymphedema
 
 
 
Familial intraosseous vascular malformation (VMOS)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Telangiectasia
Hereditary hemorrhagic telangiectasia (HHT)
Others
 
 
 
Others
 
 
 
Verrucous venous malformation (formerly verrucous hemangioma)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Others
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

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