Venous malformation treatment options

  • Surgical treatment of venous malformations

    Venous malformations (VMs) are slow-flow vascular anomalies composed of dysplastic, dilated venous channels.  Present at birth and growing proportionally with the child, VMs often go unrecognized until they become symptomatic in later childhood or adulthood.  They may present as soft, compressible masses that enlarge with the Valsalva maneuver or dependency, commonly involving the head and neck, extremities, or trunk.

    While sclerotherapy remains the first-line treatment in many cases, surgery plays an essential role in select scenarios, either alone or in combination with other therapies.  Timely recognition of VMs and understanding when surgical referral is appropriate can significantly impact outcomes.

    Surgical excision of VMs is generally considered in the following situations:

    1.  Localized, Well-Demarcated Lesions

    Surgery is most effective for focal lesions with clear anatomical boundaries.  These are often amenable to complete excision, with low recurrence rates and excellent cosmetic and functional outcomes.

    2.  Symptomatic or Complicated Lesions

    Indications include:

    • Pain

    • Functional impairment (e.g., restricted joint mobility)

    • Recurrent thrombophlebitis

    • Bleeding or ulceration
      In these cases, surgery may provide symptom relief when other treatments have failed or are not feasible.

    3.  Lesions Resistant to Sclerotherapy

    VMs that are refractory to multiple sessions of sclerotherapy—particularly when symptoms persist or worsen—may benefit from surgical resection, especially if a distinct component remains poorly responsive.

    4.  Aesthetic Deformity or Mass Effect

    Prominent facial or extremity lesions may cause visible disfigurement or psychosocial distress.  Surgery may be considered in carefully selected patients, particularly when cosmetic goals are realistic and complications are minimized.

    5.  Residual or Recurrent Lesions

    Surgery can be useful for debulking or excising residual nodules following incomplete treatment with sclerotherapy or laser therapy.

    Surgical treatment of venous malformations is a complex and highly individualized process.  Complete resection is rarely feasible for extensive or infiltrative lesions, but partial excision or debulking can still yield meaningful improvements in symptoms, appearance, and quality of life.

    Surgical technique depends on the size, location, and involvement of the lesion, as well as the adjacent structures.

    a. Complete Excision

    • Reserved for well-circumscribed, localized lesions.

    • Goal is curative, with minimal recurrence.

    • Common in oral, buccal, or subcutaneous extremity lesions.

    b. Debulking Surgery

    • Used for extensive or infiltrative lesions where complete excision is not feasible or would cause functional/aesthetic morbidity.

    • Improves contour, reduces mass effect, and alleviates symptoms such as pain or bleeding.

    • Often staged, particularly in large or sensitive areas (e.g., face, neck).

    c. Combination with Sclerotherapy

    • Preoperative sclerotherapy can reduce lesion size and intraoperative bleeding.

    • Postoperative sclerotherapy may target residual components not accessible surgically.

    • This hybrid approach is commonly used in large, diffuse VMs.

    Intraoperative Considerations

    • Bleeding risk: VMs are highly vascular, with dilated, fragile channels.  Meticulous dissection and hemostasis are essential.  Tourniquets, topical hemostatic agents, vascular clips, electrocautery, and preoperative sclerotherapy, combined with the surgeon's training and experience, can help minimize potential risks.

    • Tissue preservation: Surgeons must balance lesion removal with preservation of critical nerves, muscles, and aesthetic units.

    • Defect management: In superficial lesions, incisions are planned to minimize visible scarring; skin grafting or flap reconstruction may be needed after extensive resections.

    Surgery aims to improve function, reduce symptoms, and enhance appearance, rather than completely eradicating the malformation in cases of diffuse lesions.

    Outcomes

    Surgical outcomes depend on the extent, depth, and location of the VM:

    • Well-circumscribed lesions (e.g., oral mucosa, localized subcutaneous VMs):

      • Excellent cosmetic and functional outcomes

      • Low recurrence rates when entirely excised

    • Extensive or infiltrative lesions (e.g., head and neck, extremities):

      • Surgery may offer partial symptom relief or volume reduction

      • Recurrence is common, especially when complete excision is not feasible

      • Combination with sclerotherapy often improves outcomes

    • Pediatric cases:

      • Often have better healing and adaptability

      • Long-term outcomes are improved when treatment is coordinated early and comprehensively

    Psychosocial Considerations

    Visible or symptomatic venous malformations—especially in the face, neck, or limbs—can have profound psychosocial impacts, which often improve significantly after successful surgery.

    • Before surgery:

      • Children and adults may experience embarrassment, low self-esteem, or social withdrawal.

      • Pain, swelling, and functional issues can interfere with daily activities, such as school, work, or physical exercise.

    • After surgery:

      • Even partial improvement in appearance or symptoms can lead to significant improvement in confidence and social function.

      • Patients or families should be prepared for the possibility of staged treatments, residual disease, or scarring.

    • Psychological support:

      • Referral to appropriate health professionals or support groups may be beneficial, especially for adolescents or those with facial deformities.

      • Multidisciplinary care models often include psychological services for this reason.

  • Sclerotherapy

    It is the method of choice in the majority of venous malformations. It is a minimally invasive method with high rates of clinical response (75-90% of treated patients) and low complication rate.

    This method involves the percutaneous puncture of the vascular malformation and the injection of special pharmaceutical agents aiming for the permanent destruction of the abnormal vessels. The selection of the most suitable sclerotic agent is made by the medical specialist for optimal results.

    Sclerotherapy is ideally performed by specialized interventional radiologists with the aid of an angiography unit and ultrasound machine.


    Bleomycin Electrosclerotherapy (BEST)

    Bleomycin electrosclerotherapy is the latest development in the treatment of vascular malformations. This new treatment modality is being used since 2019 in a few specialized vascular malformation centers. Our multidisciplinary group of clinicians was one of the first worldwide to apply electrosclerotherapy for the treatment of vascular malformations and has already treated a large number of patients with this modality.
    This method is mainly indicated for the treatment of low-flow vascular malformations (venous and lymphatic malformations) as well as selected cases of arteriovenous malformations.
    Our clinical experience so far has shown that this method significantly reduces the number of treatment sessions required in order to achieve a good clinical result.
    With electrosclerotherapy, the effectiveness of sclerotherapy is increased while at the same time the dose of the administered sclerotic agent (Bleomycin) can be significantly reduced.

    The procedure is performed under general anesthesia. First, the sclerosing agent is administered either directly into the vascular malformation, or intravenously. Subsequently, thin needles are placed into the vascular malformation, usually under ultrasonographic and fluoroscopic guidance. These needles are connected to the electric pulse generator. By applying short electric pulses, the permeability of the cell membrane of the cells that form the wall of the vascular malformation is increased, resulting in a dramatic increase in the intracellular concentration of Bleomycin.

    Cryoablation

    It involves the use of extremely low temperatures to destroy abnormal tissue. Using imaging guidance a needle is advanced within the diseased tissue. Through the tip of the needle extremely cold gas is released into the targeted abnormal tissue.  It is a promising treatment for Fibroadipose vascular Anomaly(FAVA), a are type of low flow vascular malformation.

  • Laser therapy for venous malformations

    Rationale and Mechanism of Action

    Laser therapy in venous malformations is based on the principle of selective photothermolysis, where specific wavelengths of laser energy target hemoglobin or vascular structures, resulting in coagulation and collapse of ectatic vessels. However, due to the depth, size, and heterogeneity of VMs, laser therapy has a limited but focused role.

    Unlike capillary malformations or superficial hemangiomas, VMs often contain deep, compressible venous channels, making them less responsive to laser monotherapy.


    Types of Lasers Used in VM Treatment

    Laser Type: Nd:YAG Laser

    Wavelength: 1064 nm

    Target: Deep hemoglobin absorption

    Clinical Use in VMs: Used for deeper components of VMs, especially mucosal and oropharyngeal lesions

    Laser Type: Pulsed Dye Laser (PDL)

    Wavelength: 585–595 nm

    Target: Oxyhemoglobin (superficial vessels)

    Used for superficial components, telangiectasias, or residual vascular staining

    Laser Type: CO₂ Laser

    Wavelength: 10,600 nm

    Target: Water (ablation)

    Clinical Use in VMs: Used for debulking superficial or mucosal lesions, especially when combined with excision or to treat bleeding nodules


    Indications for Laser Therapy in VMs

    Laser therapy is not curative for most VMs but may be helpful in the following settings:

    • Superficial mucosal VMs (e.g., lip, tongue, buccal mucosa)

      • Nd:YAG and CO₂ lasers can coagulate venous channels and reduce bulk or bleeding.

    • Cosmetic refinement after other treatments

      • PDL or Nd:YAG can be used to treat residual surface vessels or staining after surgery or sclerotherapy.

    • Bleeding control

      • Ablative lasers (e.g., CO₂) can effectively control mucosal bleeding or ulceration, particularly in vascular-rich areas such as the oral cavity.

    • Patients unfit for surgery or sclerotherapy

      • Lasers may offer symptom relief for bleeding or visible lesions in patients with comorbidities or who are on anticoagulation.

    Efficacy and Limitations

    Efficacy

    • Best suited for superficial, compressible, well-visualized lesions.

    • May reduce color, bulk, and bleeding in mucosal VMs.

    • Can improve cosmesis in select facial lesions, especially when combined with other modalities.

    Limitations

    • Limited penetration depth: Cannot reach deep venous channels, so ineffective for most subcutaneous or intramuscular VMs.

    • Partial results are often achieved, requiring multiple sessions, and complete resolution is uncommon.

    • Risk of complications:

      • Scarring, ulceration, hypo/hyperpigmentation (especially with Nd:YAG in darker skin types)

      • Pain during and after treatment, particularly on the mucosa

    • Operator-dependent: Efficacy and safety are closely tied to the laser type, parameters, and the clinician's experience.

    Role in Multimodal Therapy

    Laser therapy is not a first-line monotherapy for most VMs, but can be a valuable adjunct:

    • Combined with sclerotherapy: After sclerotherapy reduces lesion volume, lasers may help target residual surface components.

    • Post-surgical refinement: After debulking, lasers can address telangiectatic patches or venous staining.

    • Bridging therapy: For patients awaiting surgery or sclerotherapy, lasers may provide temporary symptom relief.

    Laser therapy has a limited but strategic role in the treatment of venous malformations. While it does not replace sclerotherapy or surgery, it may provide valuable cosmetic or symptomatic benefits in carefully selected cases, particularly for superficial, mucosal, or residual lesions. General clinicians should be aware of its uses, limitations, and the need for referral to laser-trained specialists in vascular anomalies when considering this modality.

  • Vascular Malformations

    Science has made great leaps and has led to many discoveries of the pathways that affect the growth and development of vascular tumors and vascular anomalies.  One of the important discoveries is that some of these pathways are the same as the ones used by some cancers.  In recent years, specialists have been repurposing the use of medications that target the abnormal pathway to treat patients with vascular anomalies.  Since most of these medications are new and are still actively studied, they are mostly used “off-label”, meaning not officially approved for this use yet.

    The two main pathways that have been explored are the PI3K/AKT/mTOR and RAS/RAF/ERK/MEK, both of which lead to cell growth.  Mutations (or genetic changes to the genetic make-up of the abnormal cells) of the PI3K/AKT/mTOR pathway tend to be seen in “slow blood flow” anomalies, such as venous or lymphatic malformations.  Sirolimus (Rapamune) targets (or inhibits) mTOR and was the first medication tried.  It is not unusual for the pathways to “crosstalk,” and it is always helpful to obtain a sample of the affected tissue (biopsy) to identify a mutation.  This information helps reinforce the diagnosis and may offer us an additional treatment option.

    The main advantage of targeted medicinal treatment is that the entire vascular anomaly is treated at the microscopic level, without affecting normal tissue.  Most medications are taken by mouth or applied as creams and many patients notice rapid improvement to pain, bleeding, and loss of function.  These medicines also tend to allow for less bleeding during surgery.  As with any medicine, side effects may occur.  In the case of Sirolimus, it can include effects on the immune system and a mild increase in risk of infection, elevated triglycerides, which are managed and not permanent.  We will discuss with you in detail the possible side effects. 

    The decision on which medication, and the best timing of treatment remains individualized.  There are treatment and patient factors that influence whether someone undergoes surgery only, sclerotherapy/ embolization, medication use or a combination of the above.  Your treatment team will review all relevant factors and make a recommendation on the best path.           

    Coagulation Disorders

    Slow blood flow malformations, such as venous malformations, may also carry blood clotting risks.  This is due to consumption/ break down of platelets (blood cells that make clots) and clotting proteins (fibrinogen) inside the abnormal vessels (a term known as “localized intravascular coagulation/ LIC”). These changes can lead to bleeding or blood clot formation, which causes pain and sometimes serious complications.  Blood thinners (heparin or direct oral anticoagulants) are often used, especially around surgeries and interventional procedures.