Procedure Guides
Venous

Vascular Anomalies

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Procedure Guide

Indications

Symptomatic or cosmetically bothersome vascular malformations - See 2018 ISSVA Classifications

  • Simple vs Combined, Low flow (CM, LM, VM) vs High flow (AVM, AVF)

    • Telangiectasias: occur 29% M / 41% F, more with estrogen exposure, FHx, weight gain, or prolonged sitting

    • Venous malformations: 40% head and neck, 40% extremity, 20% trunk. If present at birth, they grow proportional to child particularly during puberty and pregnancy.

      • *Type 1: isolated nidus without discernible venous drainage

      • *Type 2: nidus drain into normal veins

      • Type 3: nidus drain into dysplastic vein

      • Type 4: no focal nidus with multiple ectatic veins and draining veins

      • *Respond best to sclerotherapy with less complications

    • MRI grades: (1) <5cm, well-defined margins; (2A) >5cm, well-defined margins; (2B) <5cm, ill-defined margins; (3) >5cm, ill-defined margins; smaller lesions with well-defined margins respond better to sclerotherapy

    • CM-AVM: RASA-1 gene, AD, salmon colored patch with underlying AVM

  • Vascular malformations of major named vessels

  • Vascular malformations associated with other anomalies - some of many examples below

    • Klippel-Trenaunay Syndrome (KTS): CM + VM, +/- LM + limb overgrowth

    • Parkes Weber Syndrome (PWS): CM + AVF + limb overgrowth

    • CLOVES syndrome: LM + VM + CM +/- AVM + lipomatous overgrowth

    • Proteus syndrome CM, VM, and/or LM + asymmetric somatic overgrowth

  • Vascular Tumors

    • Benign

      • Infantile hemangioma: very vascular, masslike

      • Congenital hemangioma

    • Locally Aggressive/Borderline

      • Kaposiform hemangioendothelioma: associated with Kasabach-Merritt syndrome (consumptive thrombocytopenia)

    • Malignant, e.g., angiosarcoma

Contraindications

  • No safe access

  • Pulmonary hypertension or atrial septal defect unless lesion in no way involves the systemic venous circulation

  • Systematic/local infection

  • Anaphylaxis to sclerosant

  • Uncorrected coagulopathy (generally, INR >2.0, Plts <50K for elective procedure)

  • Diffuse involvement of compartment due to risk of compartment syndrome

  • Lesion-related neuropathy (relative)

Efficacy and alternatives

  • Medications:

    • PIK3CA mutations (macro, micro, GLA): sirolimus, aprelisib

    • KRSA mutations (KLA, GSD): MEK inhibitors

    • EPHB4 mutations (CCLA): MEK inhibitor, sirolimus

  • Endovenous ablation and Percutaneous photocoagulation can also be helpful

  • Sclerotherapy: generally, 80-90% efficacy, fewer major complications (3-11 v. 20%) and 30d mortality (<1 v. 5.9%) than surgery

  • Surgery preferred for small (<2-4 cm), accessible malformations.

Pre-procedure care

  • Ideally evaluated by a multidisciplinary vascular anomalies clinic team, particularly for more complex lesions are those associated with syndromes.

  • Careful history and physical exam complemented with ultrasound.

  • Review imaging closely, often MRI and US, which are complementary.

  • Clarify GOC as large, complex lesions are rarely curable. Can maximize palliation with multiple modalities

    • Compression therapy for swelling and thrombophlebitis

    • NSAIDs and other anti-inflammatory drugs for superficial phlebitis

  • Labs: CBC, PT/INR, serum creatinine, d-dimer

  • Hold antiplatelet agents 5d pre-op if possible

  • Consider marking out the lesion in pre-op on the skin with patient standing and target most symptomatic areas.

procedure

Lots of variation pending specific lesion and anatomy. Below is a general approach for percutaneous sclerosis of a low-flow vascular malformation.

  • Dedicate some time to pre-procedure ultrasound (US) to recharacterize the lesion and plan approach. Good to identify potential critical structures to avoid, e.g., major arteries, nerves, confined compartments such as treating in the carpal tunnel.

  • Cannulate vascular component with needle (e.g. 21G micropuncture set) and inject dilute contrast to characterize, e.g., outflow, nidus, volume to fill the malformation.

    • Ideally work deep to superficial with needle placements to not shadow out deep elements with sclerosant superficially.

    • Approach peripheral aspect to avoid accessing portion closest to the skin and to not hold pressure as directly on the lesion at the end to minimal skin changes and risk of staining.

    • Can require multiple needle placements. *NEVER remove a needle until done with the procedure to avoid extravasation, which would require terminating the case.*

  • Take off contrast syringe and allow blood/fluid to bleed back and confirm adequate needle positioning.

    • If no bleed back, some will place a new needle and leave that one alone. Others use bleomycin for treatment of more solid components but do so carefully.

  • (Optional) Tourniquet proximal to draining veins if in an extremity.

  • Inject sclerosant.

    • US + fluoroscopy are good for monitoring as can see many sclerosants well under US and fluoroscopy can show displacement of contrast to stop when contrast starts pushing into outflow. *Avoid over pressurizing*

  • Can use more potent agent like ethanol for deeper lesions while bleomycin or STS for more superficial lesions.

    • Absolute ethanol: 

      • Most effective and most toxic (21-23% complication rate over all, major include skin ulceration, blistering, nerve injury, cardiac arrest from systemic toxicity)

        • Pulmonary artery hypertension: pre-capillary arterial constriction, most during first bolus. Should monitor for this if giving >0.5 mL/kg. If pulmonary artery pressure >25 mmHg, can give NTG 0.5-3.0 mcg/kg/min. Prevent by controlling outflow as this is a systemic toxicity of alcohol.

        • Hemoglobinuria: prevent/treat with hydration. Can place Foley and give D5W with NaHCO3 plus Lasix.

      • Dose limit: 0.2 mL/kg (VM) vs 1.0 mL/kg (AVM)

    • Sodium tetradecyl sulfate (STS): 1% or 3%, 4-10 mL per session, max of 30 mL

      • Low complication rate: skin pigmentation, allergic rxn, soft tissue necrosis, DVT/PE, TIA, hemolytic anemia

      Ethanolamine oleate: good for H&N, low complication rate, can cause skin ulceration/necrosis like ethanol

    • Bleomycin: similar efficacy to alcohol with fewer complications for superficial lesions

      • Causes skin hyperpigmentation if adhesive used within 48 hrs

      • Single session dose limit: (ped) 0.5-1 U/kg, (adult) 10-15 U

      • Cumulative dose limit: (peds) 300 U, (adult) 400 U

    • Doxycycline: works very well for lymphatic malformations

      • 100 mg reconstituted in 1 cc Isovue 300 and 3 cc saline (25mg/cc) with max of 150 mg (neonate/infant) vs 1000 mg (adults)

      • Low complication rate but severe discomfort on injection, rarer complications hypoglycemia, acidosis, hemolytic anemia, enamel dysplasia, skin ulceration, nerve injury

    • Sodium morrhuate: widely available w/ low complication rate, can cause pigmentation

    • Pingyangmycin: similar to bleomycin but more cost-effective

    • Polidocanol: low complication rate, hyperpigmentation

    • OK-432 (Picibanil): minimal systemic effects, can cause fever, pain

    • n-Butyl Cyanoacrylate: often used with sclerosant. Good if planning resection to limit bleeding.

    • Ethylcellulose: like ethanol gel with embolic properties

  • Tricks/Troubleshooting

    • Place gauze soaked in chilled saline on skin and leave angiocath sheath or access needle for 20 min

    • If extraluminal injection, stop and inject some saline to flush out.

    • Watch for skin changes during injections.

    • If persistent bleeding at access site, use Afrin which anesthesia carts often carry

  • Wait 3-10 min and confirm adequate sclerosis -> release tourniquet (*slowly*) if used

  • Some allow sclerosant to drain (microcytic) or aspirate out (macrocystic) prior to removing each needle.

  • Ensure careful post-op positioning to not dislodge sclerosant. Wrap or apply pressure garment if using.

Complications

  • See sclerosant-specific risks above.

  • Overlying skin breakdown: reported 8-33% but likely driven by historic use of primarily alcohol, sotradecol minor complications 0-14% and major complications 2%

  • Localized intravascular coagulopathy: thrombosis of intralesional blood causing severe coagulopathy due to consumption of coagulation factors, plt counts often around 100-150

  • Infection, bleeding, local blistering, full-thickness cutaneous necrosis, nerve injury, PE

Post-procedure Care and Follow Up

  • Be sure to consent and dictate as “venous embolization/sclerotherapy” for billing purposes

  • Observe 4-24 hrs dependent upon risk for compartment syndrome or anaphylaxis

  • PO pain control, minimize local swelling/bruising via elevation and ice. Steroids can also help but reduce the efficacy so some only use for patients with severe swelling or pain after.

  • Optional wrapping or compression garment (30-40 mmHg) placed intra-operatively. Avoid removing/showering for 2 days. Then remove for shower and replace after.