Vascular malformation

Vascular malformation
Classification and external resources
MeSH	D054079

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Vascular Malformation
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Overview
Classification Simple Vascular Malformations Capillary Malformation Lymphatic Malformation Venous Malformation Arteriovenous Malformation Arteriovenous Fistula Combined Vascular Malformations Vascular Malformations of Major Named Vessels Vascular Malformations associated With other Anomalies

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. It may cause aesthetic problems as it has a growth cycle and can continue to grow throughout life. This is also known as Vascular giantism or lymphangiomas.

Classification

Vascular malformations

Simple vascular malformations

Combined vascular malformations*

Vascular malformations of major named vessels

Vascular malformations asscoiated with other anomalies

Capillary malformations (CM)

Lymphatic malformations (LM)

Venous malformations (VM)

Arteriovenous malformations (AVM)

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

Simple Vascular Malformations

Simple Vascular Malformations are divided into:

• Capillary malformation
• Lymphatic malformation
• Venous malformation
• Arteriovenous malformation
• Arteriovenous fistula

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	CLVM
CM + VM + AVM	Capillary-venous-arteriovenous malformation	CVAVM
CM + LM + VM + AVM	Capillary-lymphatic-venous-arteriovenous malformation	CLVAVM

Vascular Malformations of Major Named Vessels

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

Affect
• Lymphatics
• Veins
• Arteries
Anomalies of
• Origin
• Course
• Number
• Diameter (aplasia, hypoplasia, stenosis, ectasia / aneurysm)
• Valves
• Communication (AVF)
• Persistence (of embryonal vessel)

Vascular Malformations associated With other Anomalies

Klippel-Trenaunay syndrome	CM + VM +/-LM + limb overgrowth
Parke's 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
Maffucci syndrome	VM +/-spindle-cell hemangioma + enchondroma
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
Limb CM + congenital non-progressive limb overgrowth
Macrocephaly-CM (M-CM / MCAP)
Microcephaly-CM (MICCAP)

See also

• Vascular disease
• Vascular anomaly

References

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