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{{Short description|Blood vessel or lymph vessel abnormality}}

{{Infobox medical condition (new)
{{Infobox medical condition (new)
| name = Vascular malformation
| name = Vascular malformation
| image =
| image =
| caption =
| caption = |
| pronounce =
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|
| pronounce =
| field = cardiovascular
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| synonyms = '''Vascular giantism''' or '''Lymphangioma'''
| field = cardiovascular
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| symptoms =
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| synonyms = '''Vascular giantism''' or '''Lymphangiomas'''
| complications =
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| symptoms =
| complications =
| onset =
| onset =
| duration =
| duration =
| types =
| types =
| causes =
| causes =
| risks =
| risks =
| diagnosis =
| diagnosis =
| differential =
| differential =
| prevention =
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| treatment = In low-flow lesions, [[sclerotherapy]] can be extremely effective, either alone, in small lesions, or combined with surgical resection or embolization, in larger lesions.<ref>Jackson, Ian T., et al. "Hemangiomas, vascular malformations, and lymphovenous malformations: classification and methods of treatment." Plastic and reconstructive surgery 91.7 (1993): 1216-1230.</ref>
| prevention =
| medication =
⚫
| treatment = In low-flow lesions, sclerosant therapy can be extremely effective, either alone, in small lesions, or combined with surgical resection or embolization, in larger lesions. <ref>Jackson, Ian T., et al. "Hemangiomas, vascular malformations, and lymphovenous malformations: classification and methods of treatment." Plastic and reconstructive surgery 91.7 (1993): 1216-1230.</ref>
| medication =
| prognosis =
| prognosis =
| frequency =
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| deaths =
| deaths =
}}
}}
A '''vascular malformation''', is a [[blood vessel]] or [[lymph vessel]] [[vascular anomalies|abnormality]]. Vascular malformations are one of the classifications of [[vascular anomaly|vascular anomalies]], the other grouping is [[vascular tumor]]s.<ref name="Steiner">{{cite journal |last1=Steiner |first1=JE |last2=Drolet |first2=BA |title=Classification of Vascular Anomalies: An Update. |journal=Seminars in interventional radiology |date=September 2017 |volume=34 |issue=3 |pages=225-232 |doi=10.1055/s-0037-1604295 |pmid=28955111}}</ref> They may cause aesthetic problems as they have a growth cycle, and can continue to grow throughout life.
A '''vascular malformation''' is a type of [[vascular anomaly]].<ref name="Steiner">{{cite journal |last1=Steiner |first1=JE |last2=Drolet |first2=BA |title=Classification of Vascular Anomalies: An Update. |journal=Seminars in Interventional Radiology |date=September 2017 |volume=34 |issue=3 |pages=225–232 |doi=10.1055/s-0037-1604295 |pmid=28955111|pmc=5615389 }}</ref> They may cause aesthetic problems as they have a growth cycle, and can continue to grow throughout life.


Vascular malformations of the brain (VMBs) include those involving capillaries, and those involving the veins and arteries. Capillary malformations in the brain are known as [[Cavernous hemangioma#cerebral cavernous malformations|cerebral cavernous malformations]] or ''capillary cavernous malformations'' (CCMs). Those involving the mix of vessels are known as [[cerebral arteriovenous malformation]]s (AVMs or cAVMs). The arteriovenous type is the most common in the brain.<ref name="Leblanc">{{cite journal |last1=Leblanc |first1=GG |last2=Golanov |first2=E |last3=Awad |first3=IA |last4=Young |first4=WL |last5=Biology of Vascular Malformations of the Brain NINDS Workshop |first5=Collaborators. |title=Biology of vascular malformations of the brain. |journal=Stroke |date=December 2009 |volume=40 |issue=12 |pages=e694-702 |doi=10.1161/STROKEAHA.109.563692 |pmid=19834013}}</ref>
Vascular malformations of the brain include those involving [[capillaries]], and those involving the [[vein]]s and [[Artery|arteries]]. Capillary malformations in the brain are known as [[Cavernous hemangioma#cerebral cavernous malformations|cerebral cavernous malformations]] or ''capillary cavernous malformations''. Those involving the mix of vessels are known as [[cerebral arteriovenous malformation]]s (AVMs or cAVMs). The arteriovenous type is the most common in the brain.<ref name="Leblanc">{{cite journal |last1=Leblanc |first1=GG |last2=Golanov |first2=E |last3=Awad |first3=IA |last4=Young |first4=WL |title=Biology of vascular malformations of the brain. |journal=Stroke |date=December 2009 |volume=40 |issue=12 |pages=e694-702 |doi=10.1161/STROKEAHA.109.563692 |pmid=19834013|pmc=2810509 }}</ref>


==Types==
==Types==
The International Society for the Study of Vascular Anomalies (ISSVA) classification has 5 types of Vascular Malformation.
A simple division of the vascular malformations is made into ''low-flow'' and ''high-flow'' types.<ref name="Steiner"/> Low-flow malformations involve a single type of blood or lymph vessel, and are known as ''simple vascular malformations''; high-flow malformations involve an artery. There are also malformations that are of mixed-flow involving more than one type of vessel, such as an [[arteriovenous malformation]].<ref name="Steiner"/>
{| class="wikitable"
|+ Types of Vascular Malformations
⚫
|-
! Simple !! Combined !! Of Major Named Vessels !! Associated with Other Anomalies
|-
| Capillary malformations || rowspan="5" | Defined as two or more vascular malformations found in one lesion.
| rowspan="5" |Abnormalities in the origin/course/number of major blood vessels that have anatomical names
| rowspan="5" |Syndromes in which vascular malformations are complicated by symptoms other than vascular anomalies
|-
| [[Lymphangioma|Lymphatic malformations]]
|-
|Venous malformations
|-
⚫
| [[Arteriovenous malformations]]*
|-
| [[Arteriovenous fistula]]*
|}
<nowiki>*</nowiki> denotes high-flow malformation


Low-flow vascular malformations include ''capillary malformations'', ''venous malformations'', and ''lymphatic malformations''.<ref name="Sadick">{{cite journal |last1=Sadick |first1=M |last2=Müller-Wille |first2=R |last3=Wildgruber |first3=M |last4=Wohlgemuth |first4=WA |title=Vascular Anomalies (Part I): Classification and Diagnostics of Vascular Anomalies. |journal=RoFo : Fortschritte auf dem Gebiete der Rontgenstrahlen und der Nuklearmedizin |date=September 2018 |volume=190 |issue=9 |pages=825-835 |doi=10.1055/a-0620-8925 |pmid=29874693}}</ref>
Vascular malformations can also be divided into ''low-flow'' and ''high-flow'' types.<ref name="Steiner" /> Low-flow malformations involve a single type of blood or lymph vessel, and are known as ''simple vascular malformations''; high-flow malformations involve an artery. There are also malformations that are of mixed-flow involving more than one type of vessel, such as an [[arteriovenous malformation]].<ref name="Steiner" /> Low-flow vascular malformations include ''capillary malformations'', ''venous malformations'', and ''lymphatic malformations''.<ref name="Sadick">{{cite journal |last1=Sadick |first1=M |last2=Müller-Wille |first2=R |last3=Wildgruber |first3=M |last4=Wohlgemuth |first4=WA |title=Vascular Anomalies (Part I): Classification and Diagnostics of Vascular Anomalies |journal= RöFo |date=September 2018 |volume=190 |issue=9 |pages=825–835 |doi=10.1055/a-0620-8925 |pmid=29874693|doi-access=free }}</ref>


===Capillary malformation===
=== Simple Types ===
⚫
[[File:Port-wine stain on leg.jpg|thumb|upright=0.6|[[Port-wine stain]] on leg.]]
Capillary malformations involve the [[capillary|capillaries]], and are the most common type. They used to refer only to [[port-wine stain]]s but now includes others.<ref name="Steiner"/>
⚫
It has been proposed that the category of capillary malformations, also called '''vascular stains''', be classified into seven major clinical types including [[nevus flammeus nuchae]] also known as nevus simplex, commonly known as ''stork bite'' or ''salmon patch''.<ref name="Proposal">{{cite journal |last1=Rozas-Muñoz |first1=E |last2=Frieden |first2=IJ |last3=Roé |first3=E |last4=Puig |first4=L |last5=Baselga |first5=E |title=Vascular Stains: Proposal for a Clinical Classification to Improve Diagnosis and Management. |journal=Pediatric dermatology |date=November 2016 |volume=33 |issue=6 |pages=570-584 |doi=10.1111/pde.12939 |pmid=27456075}}</ref>


===Venous malformation===
==== Capillary malformation ====
⚫
[[File:Port-wine stain on leg.jpg|thumb|upright=0.6|[[Port-wine stain]] on leg]]
Venous malformations are typically ill-defined masses, coloured from pale to dark blue. They can affect any tissue in the body.<ref name="Steiner"/> The mass is soft, and easily compressed, and their blue colouring is due to the dilated anomalous involved veins.<ref name="Chim">{{cite journal |last1=Chim |first1=H |last2=Drolet |first2=B |last3=Duffy |first3=K |last4=Koshima |first4=I |last5=Gosain |first5=AK |title=Vascular anomalies and lymphedema. |journal=Plastic and reconstructive surgery |date=August 2010 |volume=126 |issue=2 |pages=55e-69e |doi=10.1097/PRS.0b013e3181df803d |pmid=20679788}}</ref> They are most commonly found in the head and neck. Venous malformations can often extend deeper from their surface appearance, reaching underlying muscle or bone. In the neck they may extend into the [[oral mucosa|lining of the mouth cavity]] or into the [[salivary gland]]s.<ref name="Chim"/>
Capillary malformations involve the [[capillary|capillaries]], and are the most common type. They used to refer only to [[port-wine stain]]s but now include others.<ref name="Steiner"/> Capillary malformations are limited to the superficial layers of the skin but they can thicken, become nodular, and sometimes become disfiguring.<ref name="Puttgen">{{cite journal |last1=Puttgen |first1=KB |last2=Pearl |first2=M |last3=Tekes |first3=A |last4=Mitchell |first4=SE |title=Update on pediatric extracranial vascular anomalies of the head and neck. |journal=Child's Nervous System |date=October 2010 |volume=26 |issue=10 |pages=1417–33 |doi=10.1007/s00381-010-1202-2 |pmid=20697721|s2cid=12359248 }}</ref>
⚫
It has been proposed that the category of capillary malformations, also called '''vascular stains''', be classified into seven major clinical types including [[nevus flammeus nuchae]] also known as nevus simplex, commonly known as ''stork bite'' or ''salmon patch''.<ref name="Proposal">{{cite journal |last1=Rozas-Muñoz |first1=E |last2=Frieden |first2=IJ |last3=Roé |first3=E |last4=Puig |first4=L |last5=Baselga |first5=E |title=Vascular Stains: Proposal for a Clinical Classification to Improve Diagnosis and Management. |journal=Pediatric Dermatology |date=November 2016 |volume=33 |issue=6 |pages=570–584 |doi=10.1111/pde.12939 |pmid=27456075|s2cid=22474320 }}</ref>


A capillary malformation is also a feature of the disorder [[macrocephaly-capillary malformation]].<ref name="Gonzalez">{{cite journal|last1=Gonzalez|first1=ME|last2=Burk|first2=CJ|last3=Barbouth|first3=DS|last4=Connelly|first4=EA|date=May–June 2009|title=Macrocephaly-capillary malformation: a report of three cases and review of the literature.|journal=Pediatric Dermatology|volume=26|issue=3|pages=342–6|doi=10.1111/j.1525-1470.2009.00924.x|pmid=19706101|s2cid=27334360}}</ref> An example of capillary malformation is cerebral cavernous malformations. This disease is linked to the central nervous system (brain, eye, spinal cord). They are abnormal clusters of closely packed, thin-walled blood vessels that usually form caverns. The lesions contain slow-moving or clotted blood. Lesions in the brain and spinal cord are particularly fragile and likely to bleed.<ref>{{Cite web |title=Cerebral Cavernous Malformations {{!}} National Institute of Neurological Disorders and Stroke |url=https://www.ninds.nih.gov/health-information/disorders/cerebral-cavernous-malformations#:~:text=Cerebral%20cavernous%20malformations%20(CCMs)%E2%80%94,and%20can%20alter%20blood%20flow. |access-date=2024-09-12 |website=www.ninds.nih.gov |language=en}}</ref>[[File:Lymphangioma of the left side of the tongue Wellcome L0062760.jpg|thumb|left| Drawing of a microcystic lymphangioma on the left side of a boy's tongue]]
A severe venous malformation can involve the lymph vessels as a ''lymphaticovenous malformation''.<ref name="Chim/>


===Lymphatic malformation===
==== Lymphatic malformation ====
{{Main|Lymphangioma}}
{{Main|Lymphangioma}}
[[File:Lymphangioma of the left side of the tongue Wellcome L0062760.jpg|thumb| Drawing of a microcystic lymphangioma on the left side of a boy's tongue.]]
⚫
Lymphatic malformations are congenital, developing from badly-formed lymphatic vessels in early [[embryonic development]].<ref name="Elluru">{{cite journal |last1=Elluru |first1=RG |last2=Balakrishnan |first2=K |last3=Padua |first3=HM |title=Lymphatic malformations: diagnosis and management. |journal=Seminars in pediatric surgery |date=August 2014 |volume=23 |issue=4 |pages=178-85 |doi=10.1053/j.sempedsurg.2014.07.002 |pmid=25241095}}</ref> Abnormal development of the lymph vessels results in their failure to connect and drain into the venous system.<ref name="Elluru"/>


⚫
Lymphatic malformations are congenital, developing from badly-formed lymphatic vessels in early [[embryonic development]].<ref name="Elluru">{{cite journal |last1=Elluru |first1=RG |last2=Balakrishnan |first2=K |last3=Padua |first3=HM |date=August 2014 |title=Lymphatic malformations: diagnosis and management. |journal=Seminars in Pediatric Surgery |volume=23 |issue=4 |pages=178–85 |doi=10.1053/j.sempedsurg.2014.07.002 |pmid=25241095}}</ref> Abnormal development of the lymph vessels results in their failure to connect and drain into the venous system.<ref name="Elluru" />
⚫
These lymph vessels can become blocked due to the collection of lymph which forms a cyst as a mass, and are known as [[lymphangioma]]s. They can be macrocystic, microcystic, or a combination of the two.<ref name="Elluru"/> Macrocystic have cysts greater than 2 cm<sup>3</sup>, and microcystic lymphangiomas have cysts that are smaller than 2 cm<sup>3</sup>.<ref name="NIH">{{cite web |title=Microcystic lymphatic malformation {{!}} Genetic and Rare Diseases Information Center (GARD) – an NCATS Program |url=https://rarediseases.info.nih.gov/diseases/13020/microcystic-lymphatic-malformation |website=rarediseases.info.nih.gov}}</ref> A macrocystic lymphangioma is also known as a [[cystic hygroma]]. Cystic hygromas most often occur in the neck where they are known as ''nuchal hygromas''.<ref name="radiopaedia">{{cite web |last1=D'Souza |first1=Donna |title=Cystic hygroma {{!}} Radiology Reference Article {{!}} Radiopaedia.org |url=https://radiopaedia.org/articles/cystic-hygroma-1?lang=gb |website=Radiopaedia}}</ref>


⚫
These lymph vessels can become blocked due to the collection of lymph which forms a cyst as a mass, and are known as [[lymphangioma|lymphatic malformations]]s. They can be macrocystic, microcystic, or a combination of the two.<ref name="Elluru" /> Macrocystic have cysts greater than {{convert|2|cm3}}, and microcystic lymphatic malformation have cysts that are smaller than {{convert|2|cm3}}.<ref name="NIH">{{cite web |title=Microcystic lymphatic malformation {{!}} Genetic and Rare Diseases Information Center (GARD) – an NCATS Program |url=https://rarediseases.info.nih.gov/diseases/13020/microcystic-lymphatic-malformation |website=rarediseases.info.nih.gov}}</ref>
A severe venous malformation is known as a lymphaticovenous malformation that also involves the lymph vessels.<ref name="Chim"/>


A severe venous malformation is known as a lymphaticovenous malformation that also involves the lymph vessels.<ref name="Chim">{{cite journal |last1=Chim |first1=H |last2=Drolet |first2=B |last3=Duffy |first3=K |last4=Koshima |first4=I |last5=Gosain |first5=AK |date=August 2010 |title=Vascular anomalies and lymphedema. |journal=Plastic and Reconstructive Surgery |volume=126 |issue=2 |pages=55e–69e |doi=10.1097/PRS.0b013e3181df803d |pmid=20679788 |s2cid=30865392}}</ref>
⚫
===Arteriovenous malformation===

[[File:3D Picture of Hand.jpg|thumb|3D image of an arteriovenous malformation shown in purple on the ring finger of a hand.]]
==== Venous malformations ====
Venous malformations are the type of vascular malformation that involves the veins. They can often extend deeper from their surface appearance, reaching underlying muscle or bone.<ref name="Chen">{{cite journal |last1=Chen |first1=RJ |last2=Vrazas |first2=JI |last3=Penington |first3=AJ |date=January 2021 |title=Surgical Management of Intramuscular Venous Malformations. |journal=Journal of Pediatric Orthopedics |volume=41 |issue=1 |pages=e67–e73 |doi=10.1097/BPO.0000000000001667 |pmid=32815867 |s2cid=221199574}}</ref> In the neck they may extend into the [[oral mucosa|lining of the mouth cavity]] or into the [[salivary gland]]s.<ref name="Chim" /> They are the most common of the [[vascular malformations]].<ref name="Markovic">{{cite journal |last1=Markovic |first1=JN |last2=Shortell |first2=CK |date=October 2021 |title=Venous malformations. |journal=The Journal of Cardiovascular Surgery |volume=62 |issue=5 |pages=456–466 |doi=10.23736/S0021-9509.21.11911-1 |pmid=34105926}}</ref> A severe venous malformation can involve the lymph vessels as a ''lymphaticovenous malformation''.<ref name="Chim" />[[File:3D Picture of Hand.jpg|thumb|3D image of an arteriovenous malformation shown in purple on the ring finger of a hand]]

==== Arteriovenous malformation ====
{{Main|Arteriovenous malformation}}
{{Main|Arteriovenous malformation}}
[[Arteriovenous malformation]]s occur between an artery and a vein.
[[Arteriovenous malformation]]s occur between an artery and a vein.


In the brain a [[cerebral arteriovenous malformation]] causes arterial blood to be directly shunted into the veins as there is an absence of a capillary bed. This carries a high risk of an [[intracranial hemorrhage]].<ref name="JCB">{{cite journal |last1=Mouchtouris |first1=N |last2=Jabbour |first2=PM |last3=Starke |first3=RM |last4=Hasan |first4=DM |last5=Zanaty |first5=M |last6=Theofanis |first6=T |last7=Ding |first7=D |last8=Tjoumakaris |first8=SI |last9=Dumont |first9=AS |last10=Ghobrial |first10=GM |last11=Kung |first11=D |last12=Rosenwasser |first12=RH |last13=Chalouhi |first13=N |title=Biology of cerebral arteriovenous malformations with a focus on inflammation. |journal=Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism |date=February 2015 |volume=35 |issue=2 |pages=167-75 |doi=10.1038/jcbfm.2014.179 |pmid=25407267}}</ref>
In the brain a [[cerebral arteriovenous malformation]] causes arterial blood to be directly shunted into the veins as there is an absence of a capillary bed. This carries a high risk of an [[intracranial hemorrhage]].<ref name="JCB">{{cite journal |last1=Mouchtouris |first1=N |last2=Jabbour |first2=PM |last3=Starke |first3=RM |last4=Hasan |first4=DM |last5=Zanaty |first5=M |last6=Theofanis |first6=T |last7=Ding |first7=D |last8=Tjoumakaris |first8=SI |last9=Dumont |first9=AS |last10=Ghobrial |first10=GM |last11=Kung |first11=D |last12=Rosenwasser |first12=RH |last13=Chalouhi |first13=N |date=February 2015 |title=Biology of cerebral arteriovenous malformations with a focus on inflammation. |journal=Journal of Cerebral Blood Flow and Metabolism |volume=35 |issue=2 |pages=167–75 |doi=10.1038/jcbfm.2014.179 |pmc=4426734 |pmid=25407267}}</ref>

==== Arteriovenous fistula ====
{{Main|Arteriovenous fistula}}

=== Combined Types ===
Combined types are defined as two or more vascular malformations found in one lesion. Examples of combined types include lymphatic-venous malformation (LVM) or capillary-venous-arteriovenous malformation (VAVM).

== Terminology ==
The International Society for the Study of Vascular Anomalies (ISSVA) classification is a basic and systematic classification of vascular anomalies with international acceptance.
As such terms such as "Lymphangioma" and "Cystic Hygroma", which were used widely in the past, are outdated. Newer research may only reference ISSVA terminology and, as a consequence, sources of information can be missed by doctors and patients unaware of the ISSVA convention.
{| class="wikitable"
|+Terminology
!ISSVA name
!Outdated names
|-
|Lymphatic malformation
|Lymphangioma, Cystic hygroma, Lymphangioma circumscriptum, Cavernous lymphangioma, lymphangiomatosis
|-
|Venous malformation
|Caveronous Hemangioma
|-
|Capillary Malformation
|Port-wine strain, Capillary hemangioma
|}


==See also==
== See also ==
* [[Arteriovenous fistula]]
* [[Arteriovenous fistula]]
* [[Lymphangioma|Lymphatic malformations]]
* [[Lymphohemangioma]]
* [[Lymphohemangioma]]
* [[Telangiectasia]]
* [[Telangiectasia]]
* [[Vascular disease]]
* [[Vascular disease]]
{{clear}}


==References==
==References==
{{reflist}}
{{reflist}}
== External links ==
{{Medical resources
{{Medical resources
| DiseasesDB =
| DiseasesDB =
| ICD10 =
| ICD10 =
| ICD9 =
| ICD9 =
| ICDO =
| ICDO =
| OMIM =
| OMIM =
| MedlinePlus =
| MedlinePlus =
| eMedicineSubj =
| eMedicineSubj =
| eMedicineTopic =
| eMedicineTopic =
| MeshID = D054079
| MeshID = D054079
}}
}}
{{Vascular diseases}}
{{Vascular anomalies}}
{{Congenital vascular defects}}


[[Category:Vascular diseases]]
[[Category:Angiogenesis]]
[[Category:Vascular anomalies]]

Latest revision as of 19:42, 3 October 2024

Vascular malformation
Other namesVascular giantism or Lymphangioma
SpecialtyCardiovascular
TreatmentIn low-flow lesions, sclerotherapy can be extremely effective, either alone, in small lesions, or combined with surgical resection or embolization, in larger lesions.[1]

A vascular malformation is a type of vascular anomaly.[2] They may cause aesthetic problems as they have a growth cycle, and can continue to grow throughout life.

Vascular malformations of the brain include those involving capillaries, and those involving the veins and arteries. Capillary malformations in the brain are known as cerebral cavernous malformations or capillary cavernous malformations. Those involving the mix of vessels are known as cerebral arteriovenous malformations (AVMs or cAVMs). The arteriovenous type is the most common in the brain.[3]

Types

[edit]

The International Society for the Study of Vascular Anomalies (ISSVA) classification has 5 types of Vascular Malformation.

Types of Vascular Malformations
Simple Combined Of Major Named Vessels Associated with Other Anomalies
Capillary malformations Defined as two or more vascular malformations found in one lesion. Abnormalities in the origin/course/number of major blood vessels that have anatomical names Syndromes in which vascular malformations are complicated by symptoms other than vascular anomalies
Lymphatic malformations
Venous malformations
Arteriovenous malformations*
Arteriovenous fistula*

* denotes high-flow malformation

Vascular malformations can also be divided into low-flow and high-flow types.[2] Low-flow malformations involve a single type of blood or lymph vessel, and are known as simple vascular malformations; high-flow malformations involve an artery. There are also malformations that are of mixed-flow involving more than one type of vessel, such as an arteriovenous malformation.[2] Low-flow vascular malformations include capillary malformations, venous malformations, and lymphatic malformations.[4]

Simple Types

[edit]

Capillary malformation

[edit]
Port-wine stain on leg

Capillary malformations involve the capillaries, and are the most common type. They used to refer only to port-wine stains but now include others.[2] Capillary malformations are limited to the superficial layers of the skin but they can thicken, become nodular, and sometimes become disfiguring.[5] It has been proposed that the category of capillary malformations, also called vascular stains, be classified into seven major clinical types including nevus flammeus nuchae also known as nevus simplex, commonly known as stork bite or salmon patch.[6]

A capillary malformation is also a feature of the disorder macrocephaly-capillary malformation.[7] An example of capillary malformation is cerebral cavernous malformations. This disease is linked to the central nervous system (brain, eye, spinal cord). They are abnormal clusters of closely packed, thin-walled blood vessels that usually form caverns. The lesions contain slow-moving or clotted blood. Lesions in the brain and spinal cord are particularly fragile and likely to bleed.[8]

Drawing of a microcystic lymphangioma on the left side of a boy's tongue

Lymphatic malformation

[edit]
Main article: Lymphangioma

Lymphatic malformations are congenital, developing from badly-formed lymphatic vessels in early embryonic development.[9] Abnormal development of the lymph vessels results in their failure to connect and drain into the venous system.[9]

These lymph vessels can become blocked due to the collection of lymph which forms a cyst as a mass, and are known as lymphatic malformationss. They can be macrocystic, microcystic, or a combination of the two.[9] Macrocystic have cysts greater than 2 cubic centimetres (0.12 cu in), and microcystic lymphatic malformation have cysts that are smaller than 2 cubic centimetres (0.12 cu in).[10]

A severe venous malformation is known as a lymphaticovenous malformation that also involves the lymph vessels.[11]

Venous malformations

[edit]

Venous malformations are the type of vascular malformation that involves the veins. They can often extend deeper from their surface appearance, reaching underlying muscle or bone.[12] In the neck they may extend into the lining of the mouth cavity or into the salivary glands.[11] They are the most common of the vascular malformations.[13] A severe venous malformation can involve the lymph vessels as a lymphaticovenous malformation.[11]

3D image of an arteriovenous malformation shown in purple on the ring finger of a hand

Arteriovenous malformation

[edit]
Main article: Arteriovenous malformation

Arteriovenous malformations occur between an artery and a vein.

In the brain a cerebral arteriovenous malformation causes arterial blood to be directly shunted into the veins as there is an absence of a capillary bed. This carries a high risk of an intracranial hemorrhage.[14]

Arteriovenous fistula

[edit]
Main article: Arteriovenous fistula

Combined Types

[edit]

Combined types are defined as two or more vascular malformations found in one lesion. Examples of combined types include lymphatic-venous malformation (LVM) or capillary-venous-arteriovenous malformation (VAVM).

Terminology

[edit]

The International Society for the Study of Vascular Anomalies (ISSVA) classification is a basic and systematic classification of vascular anomalies with international acceptance. As such terms such as "Lymphangioma" and "Cystic Hygroma", which were used widely in the past, are outdated. Newer research may only reference ISSVA terminology and, as a consequence, sources of information can be missed by doctors and patients unaware of the ISSVA convention.

Terminology
ISSVA name Outdated names
Lymphatic malformation Lymphangioma, Cystic hygroma, Lymphangioma circumscriptum, Cavernous lymphangioma, lymphangiomatosis
Venous malformation Caveronous Hemangioma
Capillary Malformation Port-wine strain, Capillary hemangioma

See also

[edit]

References

[edit]
  1. ^ Jackson, Ian T., et al. "Hemangiomas, vascular malformations, and lymphovenous malformations: classification and methods of treatment." Plastic and reconstructive surgery 91.7 (1993): 1216-1230.
  2. ^ a b c d Steiner, JE; Drolet, BA (September 2017). "Classification of Vascular Anomalies: An Update". Seminars in Interventional Radiology. 34 (3): 225–232. doi:10.1055/s-0037-1604295. PMC 5615389. PMID 28955111.
  3. ^ Leblanc, GG; Golanov, E; Awad, IA; Young, WL (December 2009). "Biology of vascular malformations of the brain". Stroke. 40 (12): e694-702. doi:10.1161/STROKEAHA.109.563692. PMC 2810509. PMID 19834013.
  4. ^ Sadick, M; Müller-Wille, R; Wildgruber, M; Wohlgemuth, WA (September 2018). "Vascular Anomalies (Part I): Classification and Diagnostics of Vascular Anomalies". RöFo. 190 (9): 825–835. doi:10.1055/a-0620-8925. PMID 29874693.
  5. ^ Puttgen, KB; Pearl, M; Tekes, A; Mitchell, SE (October 2010). "Update on pediatric extracranial vascular anomalies of the head and neck". Child's Nervous System. 26 (10): 1417–33. doi:10.1007/s00381-010-1202-2. PMID 20697721. S2CID 12359248.
  6. ^ Rozas-Muñoz, E; Frieden, IJ; Roé, E; Puig, L; Baselga, E (November 2016). "Vascular Stains: Proposal for a Clinical Classification to Improve Diagnosis and Management". Pediatric Dermatology. 33 (6): 570–584. doi:10.1111/pde.12939. PMID 27456075. S2CID 22474320.
  7. ^ Gonzalez, ME; Burk, CJ; Barbouth, DS; Connelly, EA (May–June 2009). "Macrocephaly-capillary malformation: a report of three cases and review of the literature". Pediatric Dermatology. 26 (3): 342–6. doi:10.1111/j.1525-1470.2009.00924.x. PMID 19706101. S2CID 27334360.
  8. ^ "Cerebral Cavernous Malformations | National Institute of Neurological Disorders and Stroke". www.ninds.nih.gov. Retrieved 2024-09-12.
  9. ^ a b c Elluru, RG; Balakrishnan, K; Padua, HM (August 2014). "Lymphatic malformations: diagnosis and management". Seminars in Pediatric Surgery. 23 (4): 178–85. doi:10.1053/j.sempedsurg.2014.07.002. PMID 25241095.
  10. ^ "Microcystic lymphatic malformation | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov.
  11. ^ a b c Chim, H; Drolet, B; Duffy, K; Koshima, I; Gosain, AK (August 2010). "Vascular anomalies and lymphedema". Plastic and Reconstructive Surgery. 126 (2): 55e–69e. doi:10.1097/PRS.0b013e3181df803d. PMID 20679788. S2CID 30865392.
  12. ^ Chen, RJ; Vrazas, JI; Penington, AJ (January 2021). "Surgical Management of Intramuscular Venous Malformations". Journal of Pediatric Orthopedics. 41 (1): e67–e73. doi:10.1097/BPO.0000000000001667. PMID 32815867. S2CID 221199574.
  13. ^ Markovic, JN; Shortell, CK (October 2021). "Venous malformations". The Journal of Cardiovascular Surgery. 62 (5): 456–466. doi:10.23736/S0021-9509.21.11911-1. PMID 34105926.
  14. ^ Mouchtouris, N; Jabbour, PM; Starke, RM; Hasan, DM; Zanaty, M; Theofanis, T; Ding, D; Tjoumakaris, SI; Dumont, AS; Ghobrial, GM; Kung, D; Rosenwasser, RH; Chalouhi, N (February 2015). "Biology of cerebral arteriovenous malformations with a focus on inflammation". Journal of Cerebral Blood Flow and Metabolism. 35 (2): 167–75. doi:10.1038/jcbfm.2014.179. PMC 4426734. PMID 25407267.
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