Vascular Malformation

dr. Arry Missi Sawai Ghoai

Summary
Introduction
40.000 children are born with vascular anomalies each year in US

Divided into 2 groups : hemangioma and vascular malformation

Hemangiomas : distinct periods of proliferation, plateau, and in-volution.

Vascular malformations grow with the child and may not be found until early
childhood.

This review outline the classification, embryologic and histology of vascular

malformations, diagnosis, and treatment
Classification
 Endothelial Property :
 Hemangiomas -> Proliferative endothelium
 Vascular malformations -> stable endothelium
 Clinical, Histologic and Vascular flow Characteristic
 Hemangiomas
 Vascular malformations (low flow or high flow)
 Lymphatic malformations
Classification
 The International Society for the Study of Vascular Anomalies
classification system :
 Vascular tumors : hemangiomas, kaposiform hemangioendothelioma,
tufted angioma, pyogenic granuloma, and hemangiopericytoma.
 Vascular malformations
 Simple malformations -> capillary, venous, lymphatic, and arteriovenous
malformations
 Combined malformations -> capillary-lymphatic-venous, capillary-venous,
capillary-venous with arteriovenous shunting with or without fistulas, and
cutis marmorata telangiectasia congenita.
 Histology, Embryology, and Genetics
Vascular malformations result from morphologic errors in vascular
development, in utero, arising in the first trimester. Caused by
signaling dysregulations of vascular system formation.

Vascular malformations histology : normal endothelium, no cellular

hyperplasia, thin walled, large lumen, thin layer of pericytes and
smooth muscles

Lymphatic malformations histology : vascular space of variable

thickness, abnormal smooth and skeletal muscle.

The identification of genes responsible -> possibility of gene therapy

Evaluation

Fetal Diagnosis

Postnatal evaluation
Fetal Diagnosis

 Increasingly possible due to development of

sophisticated prenatal ultrasound evaluation.
 Vascular anomalies can be diagnosed at second and third
trimester of prenatal development
 Accurate diagnosis is still not reliable, with 41% incorrect
diagnosis.
 Postnatal diagnosis currently still remain as standard.
Imaging Modality
 Plain radiographs : skeletal growth disturbances and involvement and the
presence of phleboliths among venous malformations.
 Color Doppler ultrasound : real-time assessment of blood flow velocities and
patterns of superficial vascular lesions.
 CT Scan : limited role (lack of soft tissue detail and significant exposure of ionizing
radiation).
 MRI : detailed multiplanar images, with accurate depiction of soft tissue
relationships of the entire body.
 Angiography and venography : invasive, painful, but important diagnostic and
therapeutic role
 Venography : determination of lesion’s size, extend and venous drainage
patterns.
 Angiography : examine the degree of av-shunting, macrofistulae or
microfistulae, lesion’s nidus, and therapeutic embolization.
 Disadvantages : poor at determining anatomical relationship between lesion
and surrounding structures.
 Lymphography are rarely used in evaluation and treatment of lymphatic
malformations.
Capillary Malformations
 Intradermal vascular anomalies. Occur in 0.3 percent of newborns.
 Previously erroneously referred as port-wine stains.
 Must be differentiated from macular stains -> only dilated
capillaries without pathologic finding, fade in the first 2 years of
life.
 Present as benign pink / red cutaneous discoloration.
 Progressive, more nodular with age.
 Facial lesions -> soft tissue hypertrophy, associated with skeletal
change
Capillary Malformations
 Associated with several syndromes, e.g. Sturge-Weber
syndrome (facial capillary malformation in trigeminal nerve)
 In occipital region -> can represent underlying encephalocele.
 In upper back -> underlying spinal cord AVM (Cobb syndrome)
 Lumbosacral -> spina bivida occulta, meningocele, tethered
cord.
 Limb -> Klippel Trenaunay Syndrome
 Proteus syndrome -> cutaneous, vascular, skeletal and soft
tissue malformation
Capillary Malformations
 Imaging
 Spinal radiography
 CT Scan
 MRI
 Treatment
 585-nm pulsed dye laser -> intravascular thrombosis
 If resistant to pulsed dye laser -> use of intense pulsed light
 Lymphatic Malformations
Occur often in cervical
Slow flow anomaly of Macrocystic lesion ->
region, axilla, trunk, and
lymphatic channel Spontaneous involution
extremity

Cervicofacial lesion ->

Microcystic lesions -> Do present with skeletal
note involute distortion, malocclusion,
spontaneously mandibular overgrowth,
open bite
Lymphatic Malformations
 Symptomatic lymphatic malformations : Localized swelling, pain,
erythema
 Complications : Intralesional bleeding and infection -> rapid lesion
expansion
 Usually benign unless affect cervicofacial region ->airway obstruction
 MRI -> Determine extent and anatomical relationships between
surrounding structures
 CT Scan -> Additional diagnostic and therapeutic benefits (CT Guided
intervention)
 USG -> useful modality for sclerosant application.
Lymphatic Malformations
 Treatment : Surgical / Non Surgical
 Aspirations : high recurrence rates.
 Sclerotherapy : injection of sclerosing agent (hypertonic saline,
sodium tetradecyl sulfate, absolute ethanol, bleomycin) for
macrocystic lesion -> favorable outcome.
 OK-432 -> 86% success rate.
 Microcystic lesion -> do not respond well to sclerosant -> elastic
support and compression garments.
Lymphatic Malformations
 Surgical Therapy Indications : Functional limitations, intolerable
symptoms, altered aesthetics.
 Goal -> Completely resect the lesion -> seldom possible because
often involves normal anatomy
 Surgery routinely staged over months
 Cervical / Tongue lymphatic lesion : respiratory compromised ->
May require tracheostomy
 Tongue lesion -> Radiofrequency ablation and/or surgical debulking
 Axillary lesion -> axillary and brachial plexus dissection
Lymphatic Malformations
 Cervical lesions -> neck dissections
 Postoperatively need suction drainage with prophylactic
antibiotics to prevent seroma formation and infection.
 Lymphangioma circumscriptum : Wide local dissection and local
flaps or skin grafts
 Deeper component of lesion may lead to reccurence
 Venous Malformations
Histologically :
endothelia with variable
Most prevalent vascular Slow flow with gradual
dilated thick spongy
malformation venous dilation
channels without
smooth muscle

Clinically : Compressible
Generally grow with the
subcutaneous masses
child, but can expand
with an associated
with trauma and
cutaneous deep blue
hormonal change
discoloration
 Venous Malformations
Head and neck lesion : unilateral and can cause facial asymmetry,
malocclusion, and open-bite deformity.
Tongue and oropharyngeal lesions : cause respirations, obstruction,
sleep apnea

Extremity lesions : limb hypertrophy and length discrepancy ->

pathological fracture

Multiple cutaneous lesions on trunk, palms, soles -> Marker for blue-
rubber bleb nevus syndrome -> has gastrointestinal lesion : GI
Hemorrhage, intussusception and volvulus.
 Venous Malformations

MRI : best use to assesses the extent of the venous malformation

Venography : preoperative planning and sclerosant infiltration

Plain radiography : calcific mass of phleboliths

 Venous Malformations
 Treatment : for lesion with functional limitations, pain, or aesthetic deformity
 Therapeutic modalities include elastic compression garments, sclerosing
agents, and surgery.
 Compression garments : reduction of swelling and pain
 Prophylactic aspirin (80 mg daily) prevent painful thrombotic events
 Sclerosant : damage endothelial cell -> intravascular thrombosis and fibrosis
(usually need repeated treatments).
 Surgical indications : lesions with severe symptoms, functional limitations,
aesthetic distortions
 Arteriovenous Malformations
High flow lesion with artery and vein connection

Diagnosed at birth, often misdiagnosed for capillary malformation or

hemangioma

Pathogenesis : fetal error development -> arteriovenous channels of

primitive retiform flexus fail to regress

Expansion of lesion -> increased blood flow from adjacent vessel.

Ischemia -> lesion’s proliferations
 Arteriovenous Malformations
Schobinger Clinical Staging
 Stage I : Quiescent warm pink blue macules lesion
 Stage II : Expand with pulsations, thrills, and bruits
 Stage III : Become destructive with pain, bleeding, or
ulceration
 Stage IV : Decompensate – Congestive heart failure
Periods of Rapid Growth usually found after trauma or
hormonal change (puberty / pregnancy)
 Arteriovenous Malformations
 Color Doppler examination -> determination of flow rates,
volumes, and reversal of flow
 MRI -> extent of the lesion and the relationships with its
surrounding anatomy
 Angiography -> ideal modality for identifying vessel anatomy,
shunting, fistulas, and vessel tortuosity, also for therapeutic
embolization of AVM
 Arteriovenous Malformations
 Treatment : Based on the clinical stage and presentation
 Smaller well localized stage I : excision and reconstruction
 Larger stage I lesion : difficult to predict progression
 Lesion with intolerable symptoms : pain, bleeding, ulceration
(stage II or II) -> more urgent treatment.
 Larger and diffuse AVM -> best manage with arterial
embolization followed by surgical resection in the next 24-48
hours
 Arteriovenous Malformations
 Embolization -> ablative agent (ethanol or sodium tetradecyl
sulfate) close to the nidus to destroy the endothelium.
 Particle embolization use coil or particles to occlude flow
temporarily to minimize intraoperative blood loss.
 More complete resection -> decrease chance of recurrence
 Ligation of feeding vessel should be AVOIDED -> cause rapid
collateralization from adjacent arteries and limits access to
nidus
 Arteriovenous Malformations
 Large lesion in extremity -> potentially require amputation as definitive
treatment.
 Wide excision and reconstruction of the defect (primary closure, skin
grafts, local flaps or free tissue transfer) is often enough.
 Very large AVM -> may require cardiopulmonary bypass with hypothermic
cardiac arrest to limit hemorrhage during excision (extensive facial AVM
successfully remedied using cardiopulmonary bypass)
 Stage I and well-circumscribed stage II – most favorable outcome.
 Extensive lesion -> Best treated with aggressive combined therapy
Conclusions
 Successful treatment of vascular anomalies requires a team based management
approach.
 Specialized teams can appropriately diagnose and treat these lesions and
advance the knowledge currently in place through continued education and
research.
 Future advances in basic science research and molecular genetic will herald
breakthrough in the etiopathogenesis and therapy of these challenging problems
Thank You
References : Arneja, J. S., & Gosain, A. K. (2008).
Vascular malformations. In Plastic and Reconstructive
Surgery (Vol. 121, Issue 4).
https://doi.org/10.1097/01.prs.0000304607.29622.3c