Arteriovenous Malformations

Outlines
Definisi
Klasifikasi
Epidemiologi
Patofisiologi
Gejala klinis
Diagnosis
Management

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 Nevus
Cherry Hemangioma
Port-wine stain

Confusion between Vascular

malformations and Vascular tumors of

childhood.

International Society for Vascular

Anomalies in 1996
Vascular
Anomalies
Vascular
Malformation
s
Venous

Vascular
Tumors of
Childhood
Capillary
Hemangioma

Arteriovenous
Arterial
Lymphatic
Combined, Mixed

Cavernous
Hemangioma
Mixed
Hemangioma

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.

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 lesion
Birthmarks
Atypical varicosities
Limb 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 thickening
Complications: 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 AVMs
Stage

Manifestation

Stag I
Quiescence

Cutaneous blush or warmth

Stage II
Expansion

Bruit, thrill or other signs of expansion

Stage III
Destruction

Pain, bleeding, ulceration or infection

Stage IV
Decompensati
on

Cardiac Failure

Arterial Malformations
Least 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/Injury
Aneurysmal dilatation
Clot formation/obstruction
Ex. Persistent Sciatic Artery

Diagnosis
Essentially by Imaging studies:
US (initial evaluation)
CT scan, CT Angiography
MRI, MRA (leading imaging modality)
Conventional Angiography
1. Diagnosis
2. Determine the extent of the lesion
3. Search for associated abnormalities

Management
Venous Malformations:
Compressive stockings Aspirin
Sclerotherapy, LASER surgery,

Resection
Arteriovenous Malforamtions:
Conservative treatment in absence of

symptoms
Super-selective embolization followed
by sclerotherapy
Selective embolization followed by
surgical resection

Management
Lymphatic Malformations
Compressive stockings are the

mainstay of ttt.
Soft tissue debulking.
LASER Surgery, Surgical resection

References
Rutherford Vascular Surgery, 6th edition.
Terminology and classification of congenital

vascular malformations. Phlebology 2007;

22: 249252
Evaluation and Treatment of
Musculoskeletal Vascular Anomalies in
Children: An Update and Summary for
Orthopaedic Surgeons. UPOJ 2001; 14: 1524
Arteriovenous Malformations. H Christian
Schumacher, MD
Internet:

Thank You