NURUL AZIZAH BUSTAM

BACKGROUNDAffects only 0.8% to 1% of the populationoften associated with significant lifelong

morbidityVascular anomalies may be classified as

either vascular tumors (not the subject of this study) or vascular malformations, depending on whether they are proliferative or nonproliferative.

Vascular tumors

• present at birth, are• proliferative lesions• typically involute

spontaneously during childhood

Vascular malformations

• present at birth and grow at a rate similar to the patient, although they may not become clinically evident until later in life

• have normal endothelial cells and are not proliferative lesions

• do not regress but rather tend to progress over the patient’s lifetime

Vascular anomalie

s

Lack of consensus regarding how to approach vascular malformations

The diagnosis and treatment of such lesions is best accomplished when they are classified based on :vascular composition (arterial, venous, lymphatic, or

combined)flow dynamics (high or low flow)

Both prognosis and treatment of vascular malformations are primarily dependent upon the velocity of flow within the lesion low-flow lesions of any type faring far better than high-flow

lesions in all regardsLow-flow vascular malformations (LFVM) are usually treated

with transcutaneous sclerotherapy and/or surgical resectionHigh-flow lesions (HFVM) usually require trans-arterial

catheter-based interventions and/or surgical resection.

PURPOSEThe purpose of this study was to evaluate the

efficacy and safety of our previously described imaging protocol and therapeutic algorithm in the treatment of LFVM and HFVM in a large series of patients implemented by our multidisciplinary team.

METHODSPatient selectionthe approval of the Institutional Review BoardMedical records of patients treated by the

multidisciplinary vascular malformation team at our institution directed by the senior author from January 2006 to June 2011 were analyzed.

The database is prospectively maintained and included 136 malformations in 135 patients at the time of review.

Multidisciplinary approachMeets monthly to review all vascular

malformation patients. This team consists of vascular surgeons,

pediatric surgeons, adult and pediatric orthopedic surgeons, adult and pediatric plastic surgeons, adult and pediatric hematologists, adult and pediatric dermatologists, adult and pediatric ophthalmologists, and diagnostic and interventional radiologists.

Classification of lesions

Vascular malformations

high-flow

include an arterial

component

low-flow

venous lymphatic combined

vascular tumors

excluded from

consideration in this

study

Radiographic workupdceMRI was obtained for every patient

deemed a candidate for intervention in order to determine flow characteristics (high flow vs low flow) and extent of the lesion.

TREATMENT ALGORITHM

Only patients who are symptomatic or have complications of their vascular malformation are considered candidates for therapeutic intervention, given the potential for additional morbidity related to any intervention.

Such indications include hemorrhage, debilitating pain, or functional disability

For patients without a clear indication to intervene, conservative management is employed, which may entail observation alone, or compression stockings.

Evaluation of outcomesPatients were assessed at multiple time

points beginning with their initial evaluation.An outcomes grading system based on

patient-and physician-derived treatment goals emphasis on changes in symptoms and functional status (1 worse; 2 unchanged; 3 significantly improved; 4 completely resolved).

Patients were evaluated postprocedurally in the clinic between procedures as necessary and within 4 weeks of the final procedure.

RESULTSDuring the 5½-year period, 135 patients with

136 vascular malformations were evaluated and treated by the multidisciplinary vascular malformation team at our institution.

The 59 (43.7%) males and 76 (56.3%) females ranged in age from 1 day to 68 years (mean, 25.3 17.0 years) at the time of initial evaluation.

DISCUSSIONIt is important to note that only those patients meeting

criteria for intervention are considered candidates for therapeutic intervention.

Indications for intervention may include the following conditions or complications of VMs: bleeding;signs and symptoms of chronic venous insufficiency (painful

varicosity, edema, skin changes, ulcers, recurrent superficial thrombophlebitis);

lesions located at a life threatening region involving or close to vital structures (e.g., proximity to the airway), or located in an area threatening vital functions (e.g., sight, eating, hearing, or breathing);

disabling pain:

functional impairment (e.g., genital region);cosmetically severe deformity;lesions located at regions with high risk of

complications (e.g., hemarthrosis, thromboembolism);lesions combined producing the vascularbone

syndrome (length discrepancy of the lower extremities, affecting the bone itself) or the destructive angiodysplastic arthritis(Hauert disease);

lesions obstructing the outflow and drainage of vital organ (i.e., liver, brain);

persistent lymph leak due to a combined lymphatic malformation lesion with/without infection;

recurrent sepsis, local and/or general, due to a combined lymphatic malformation lesion.

The most agreed upon factor leading to successful management of patients with vascular malformations is the multidisciplinary approach.

Utilizing the multidisciplinary approach to individualize treatment algorithms, conservative management is appropriate for patients with tolerable symptoms and uncomplicated lesions, as these patients are unlikely to progress clinically and would not benefit from therapeutic intervention.

Further, in our cohort, patients with low-flow lesions are treated with sclerotherapy (foam and/or ethanol), primary resection, or a combination of modalities with improvement in symptoms and attainment of patient and physician directed goals of therapy in 87.5% of patients.

High flow lesions are also managed successfully, with symptomatic improvement in 89.5%.

Due to the lower efficacy and higher complication rate of ethanol compared to foam, we advocate the use of foam as the initial treatment of low-flow lesions, reserving ethanol primarily for patients who do not experience an adequate response to foam.

CONCLUSIONSThis study provides strong evidence that experienced

providers working in the context of a coordinated and structured multidisciplinary team can treat these complex patients successfully.

Once hemodynamic physiology is discerned, focused, and individualized treatment strategies can be applied.

Patients with both LFVM and HFVM can be managed to attain treatment goals set by both the patient and provider.

For low-flow lesions in particular, we recommend consideration of foam as the initial treatment, reserving ethanol for patients who do not achieve their goals of therapy with foam-based agents.

Thank you