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:
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