| Vascular malformation
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| Classification and external resources
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| MeSH
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D054079
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For information on vascular anomalies, click here
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hannan Javed, M.D.[2], Anmol Pitliya, M.B.B.S. M.D.[3]
Overview
Vascular malformation is a blood vessel abnormality. There are many types, but the most common is arteriovenous malformation. Clinical behavior and growth cycles vary widely and it may cause clinical and aesthetic problems. International Society for the Study of Vascular Anomalies (ISSVA) has classified vascular malformation into simple malformation, combined malformation, those of major named vessels, and those associated with other anomalies. Simple malformation have been divided further into capillary malformation, venous malformation and lymphatic malformation.
Classification
| | | | | | | | | | | | Vascular malformations | | | | | | | | | | | | | | |
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| | Simple vascular malformations | | | | Combined vascular malformations* | | | | | Vascular malformations of major named vessels | | | | Vascular malformations asscoiated with other anomalies | | | | | |
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| | Capillary malformations (CM) | | | Lymphatic malformations (LM) | | | Venous malformations (VM) | | | Arteriovenous malformations (AVM) | | | Arteriovenous fistula | | | | |
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| | | | Nevus simplex / salmon patch, “angel kiss”, “stork bite” | | | |
| Common (cystic) LM |
| • Macrocystic LM | |
| • Microcystic LM | |
| • Mixed cystic LM | |
| | | | Common VM | | | | Sporadic | | | | Sporadic | | | |
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| 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 | | | | | |
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| 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 | | | | | | |
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| | | | CM of CM-AVM | | | | Channel type LM | | | | Glomuvenous malformation (GVM) | | | | Others | | | | Others | | | | | | | |
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| | | | Cutis marmorata telangiectatica congenita (CMTC) | | | | “Acquired” progressive lymphatic anomaly (so called acquired progressive "lymphangioma") | | | | Cerebral cavernous malformation (CCM) | | | | | | | | | | | | | | | | | |
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| | | | Others | | | | Primary lymphedema | | | | Familial intraosseous vascular malformation (VMOS) | | | | | | | | | | | | | | | | | |
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| Telangiectasia |
| • Hereditary hemorrhagic telangiectasia (HHT) | |
| • Others | |
| | | | Others | | | | Verrucous venous malformation (formerly verrucous hemangioma) | | | | | | | | | | | | | | | | | |
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| | | | | | | | | | | | | | Others | | | | | | | | | | | | | | | | | |
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Simple Vascular Malformations
Simple Vascular Malformations are divided into:
- Capillary malformation
- Lymphatic malformation
- Venous malformation
- Arteriovenous malformation
- Arteriovenous fistula
Combined Vascular Malformations
| Combined vascular malformations*
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| CM + VM
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capillary-venous malformation
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CVM
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| CM + LM
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capillary-lymphatic malformation
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CLM
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| CM + AVM
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capillary-arteriovenous malformation
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CAVM
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| LM + VM
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lymphatic-venous malformation
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LVM
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| CM + LM + VM
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capillary-lymphatic-venous malformation
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CLVM
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| CM + LM + AVM
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capillary-lymphatic-arteriovenous malformation
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CLAVM
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| CM + VM + AVM
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capillary-venous-arteriovenous malformation
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CVAVM
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| CM + LM + VM + AVM
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capillary-lymphatic-venous-arteriovenous m.
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CLVAVM
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Vascular Malformations of Major Named Vessels
| Anomalies of major named vessels
(also known as "channel type" or "truncal" vascular malformations)
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Affect
- lymphatics
- veins
- arteries
Anomalies of
- origin
- course
- number
- length
- diameter (aplasia, hypoplasia, stenosis, ectasia / aneurysm)
- valves
- communication (AVF)
- persistence (of embryonal vessel)
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Vascular Malformations associated With other Anomalies
| Vascular malformations associated with other anomalies
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| Klippel-Trenaunay syndrome*
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CM + VM +/-LM + limb overgrowth
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| Parkes Weber syndrome
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CM + AVF + limb overgrowth
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| Servelle-Martorell syndrome
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limb VM + bone undergrowth
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| Sturge-Weber syndrome
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facial + leptomeningeal CM + eye anomalies
+/-bone and/or soft tissue overgrowth
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| Limb CM + congenital non-progressive limb overgrowth
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| Maffucci syndrome
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VM +/-spindle-cell hemangioma + enchondroma
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| Macrocephaly-CM (M-CM / MCAP)*
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| Microcephaly-CM (MICCAP)
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| CLOVES syndrome*
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LM + VM + CM +/-AVM+ lipomatous overgrowth
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| Proteus syndrome
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CM, VM and/or LM + asymmetrical somatic overgrowth
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| Bannayan-Riley-Ruvalcaba sd
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lower lip CM + face and neck LM + asymmetry and partial/generalized overgrowth
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See also
References