Vascular malformation

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

* denotes high-flow malformation

Vascular malformations can also be divided into low-flow and high-flow types. 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. Low-flow vascular malformations include capillary malformations, venous malformations, and lymphatic malformations.{{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 }}

File:Port-wine stain on leg.jpg 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. Capillary malformations are limited to the superficial layers of the skin but they can thicken, become nodular, and sometimes become disfiguring.{{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 }}

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.{{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 }}

A capillary malformation is also a feature of the disorder macrocephaly-capillary malformation.{{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}} 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.{{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}}File:Lymphangioma of the left side of the tongue Wellcome L0062760.jpg

{{Main|Lymphangioma}}

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

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. Macrocystic have cysts greater than {{convert|2|cm3}}, and microcystic lymphatic malformation have cysts that are smaller than {{convert|2|cm3}}.{{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}}

A severe venous malformation is known as a lymphaticovenous malformation that also involves the lymph vessels.{{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}}

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.{{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}} In the neck they may extend into the lining of the mouth cavity or into the salivary glands. They are the most common of the vascular malformations.{{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}} A severe venous malformation can involve the lymph vessels as a lymphaticovenous malformation.File:3D Picture of Hand.jpg

{{Main|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.{{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}}

{{Main|Arteriovenous fistula}}

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).

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.

• Arteriovenous fistula
• Lymphatic malformations
• Lymphohemangioma
• Telangiectasia
• Vascular disease

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{{Vascular anomalies}}

Category:Angiogenesis

Category:Vascular anomalies