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Dr. Ahmed Mirza Al-Shammasi2031040009
Surgical Intern - KFU – Saudi Arabia
Arteriovenous Malformations
OutlinesDefinitionClassificationEpidemiologyPathophysiologyClinical PresentationDiagnosisManagement
Arteriovenous Malformations
Congenital Vascular anomaly in which there is abnormal connection between the arterial and venous systems.
Malformed vessel that results from developmental arrest during embryogenesis.
Vascular Anomalies
One of the most confusing and misunderstood conditions.
History of inconsistent terminology:Strawberry NevusCherry HemangiomaPort-wine stain
Confusion between Vascular malformations and Vascular tumors of childhood.
International Society for Vascular Anomalies in 1996
VascularAnomalies
Vascular Tumors of Childhood
Vascular Malformation
sCapillary
Hemangioma
Cavernous Hemangioma
Mixed Hemangioma
Venous
Arteriovenous
Arterial
Combined, Mixed
Lymphatic
Epidemiology
Records from the Heim Pal Hospital for Children in Budapest indicated an incidence of 1.2%.
Both sexes are affected equally.No racial predilection has been identified.Venous Malformation is the most common (50%)Followed by AVM, then Combined, lastly Arterial.AVM is more common in the intracranial
vasculature, most dangerous type.
Pathophysiology
Arrest in the development of the primitive vascular system.
Depending on the stage of arrest, divided into: Truncular and Extratruncular.
Arteries have deficient Mascularis layers, veins are dilated due to high flow.
The exact underlying eitiology is unknown
Clinical Presentation
Present as:Mass lesionBirthmarksAtypical varicositiesLimb enlargement
Most are evident at birth, except AVM which present at early childhood or adolescence.
Venous Malformations
Most common type, 50%.Pure venous or mixed.Diffused, soft, non-pulsatile,
compressible mass, bluish hue.Rapid refilling after release of pressure.Tend to enlarge over time, darkening and
superficial thickeningComplications: Thrombophlebitis,
Infection.
Arteriovenous Malformations
Cluster of AVFs that remained from early embryonic development.
Account for 1/3 of cases of VA.Potentially the most serious type.More common intracranially.Present commonly in later childhood,
adolescence or early adulthood.Complications: Steal syndrome (Ischemia,
Pain, Ulceration), Bleeding.
Staging of AVMsStage Manifestation
Stag IQuiescence
Cutaneous blush or warmth
Stage IIExpansion
Bruit, thrill or other signs of expansion
Stage IIIDestruction
Pain, bleeding, ulceration or infection
Stage IVDecompensati
onCardiac Failure
Arterial MalformationsLeast common type, 1-2% of total.Under-development of an arterial segment.Blood flows normally through an
undeveloped side channel or collateral.Enlarging bypassing segment is vulnerable
to:Compression/InjuryAneurysmal dilatationClot formation/obstruction
Ex. Persistent Sciatic Artery
DiagnosisEssentially by Imaging studies:
US (initial evaluation)CT scan, CT AngiographyMRI, MRA (leading imaging modality)Conventional Angiography
1.Diagnosis2.Determine the extent of the lesion3.Search for associated abnormalities
ManagementVenous Malformations:
Compressive stockings ± AspirinSclerotherapy, LASER surgery, Resection
Arteriovenous Malforamtions:Conservative treatment in absence of
symptomsSuper-selective embolization followed by
sclerotherapySelective embolization followed by surgical
resection
Management
Lymphatic MalformationsCompressive stockings are the
mainstay of ttt.Soft tissue debulking.LASER Surgery, Surgical resection
ReferencesRutherford Vascular Surgery, 6th edition.Terminology and classification of congenital
vascular malformations. Phlebology 2007; 22: 249–252
Evaluation and Treatment of Musculoskeletal Vascular Anomalies in Children: An Update and Summary for Orthopaedic Surgeons. UPOJ 2001; 14: 15-24
Arteriovenous Malformations. H Christian Schumacher, MD
Internet:www.emedicine.comwww.vdf.org
Thank You