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Venous Malformation

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    Venous Malformation

    The circulatory system is made of veins, arteries, and lymphatic vessels that work together to move blood and fluids throughout the body. The major function of veins is to move blood back to the heart. Venous malformations (VMs) occur because of abnormalities in the formation of veins. VMs are a tangle of veins that have an abnormal communication with the body. Since these veins cannot drain like other normal veins, the blood gets trapped and over time these veins balloon out causing a visible deformity. These could be veins isolated from or draining into the circulatory (venous system).1,2

    Venous malformations are the most common form of malformation that is present at birth. They occur in 1-2/10,000 kids with a prevalence of 1%.3 Frequently, this condition is asymptomatic, but some patients can experience pain and discomfort, depending on the size and location of the VM.2

    Venous Malformations Diagnosis

    Various imagining modalities are used to diagnose VMs. These include:

    • MRI – this is the best imaging modality in diagnosing and identifying the extend of a malformation
    • Venography – this is where a small IV is placed and using a contrast dye the VM is visualized live
    • Ultrasound – this is the quickest and cheapest study in diagnosing a malformation1,2

    Symptoms of Venous Malformations

    Most commonly, VMs presents in the childhood or early adulthood. They can appear on any part of the body involving the head and neck (40%), trunk (20%), and extremities (40%).2,6,7 Moreover, VMs can appear on the internal organs too. Common symptoms include:

    • Pain
    • Swelling
    • Bluish or purplish lesion
    • Can be soft or compressible
    • May enlarge during puberty or pregnancy2,4

    The most common symptoms of VMs include swelling, pain, and disfigurement. The pain and swelling can come periodically, or they may exist simultaneously. Both symptoms may result from the formation of clots within the VM. It can be especially painful if the VM occurs close to a joint like the knee or elbow joint.

    Causes of Venous Malformations

    The underlying mechanism that causes VMs is not properly understood but it is caused by mutations in genes coding for various molecules. In most children, it occurs spontaneously but many patients have familial causes too. Venous malformations are always present at birth and are caused by genetic mutations.2,4

    One of the known mutations that occur on chromosome 9p is the gene coding for endothelial receptor. Many subtypes are transmitted from parents to their kids so family history becomes important. Moreover, patients with Turner syndrome may encounter VMs of the intestines and feet.5

    The presence of various risk factors during pregnancy can cause various malformations in a newborn. However, VMs are not known to be caused by any food, medication, or activity during pregnancy.

    Should venous malformations be treated?

    Like many other congenital conditions, venous malformations can cause disturbing problems and if untreated can lead to complications. Some of the complications include:

    • Thrombus or clot formation that causes pain
    • Pulmonary embolism and difficulty of respiration
    • Problems in vision
    • Chronic bleeding
    • Anemia due to bleeding (gastrointestinal malformations) 4

    Treatment Options for Venous Malformations

    Embolization with sclerotherapy is the least invasive and the gold-standard treatment indicated for venous malformations. It is a non-surgical intervention carried out by our skilled Vascular and Interventional Radiologist (IR). Different chemicals can be used for this procedure including 95% ethanol or sodium tetradecyl sulfate.8 The treatment is performed in an outpatient setting and takes about 30-45 minutes.

    During the procedure, our specialist will inject the above-mentioned solution into the vein. The abnormal veins then collapsed due to the solution’s effect causing it to fade away over time.

    After the treatment, you can experience some mild discomfort at the treatment site while the veins close. Patients are typically prescribed a 5-to-7-day course of anti-inflammatory medication.

    Some of the side effects of sclerotherapy include:

    • Allergic reactions like flares or blushing may occur
    • Bruising

    Other treatment options for VMs include:

    • Band ligations for Gastrointestinal VMs
    • Compression band (garments) on extremities VMs
    • Laser treatment
    • Surgical resection 2

    Treatment Outcomes

    For most patients, sclerotherapy is an effective treatment as it stops the venous malformation from growing and disrupting healthy tissues. The treatment with ethanol was reported to be effective in 95% of the people. However, venous malformations are reported to reoccur in several cases.2 Reoccurrence of VMs may warrant repeat treatment.

    Reference List:

    1. Venous Malformation | Johns Hopkins Medicine. Accessed March 4, 2022. https://www.hopkinsmedicine.org/health/conditions-and-diseases/venous-malformation
    2. Behravesh S, Yakes W, Gupta N, et al. Venous malformations: clinical diagnosis and treatment. Cardiovascular Diagnosis and Therapy. 2016;6(6):557. doi:10.21037/CDT.2016.11.10
    3. Eifert S, Villavicencio JL, Kao TC, Taute BM, Rich NM. Prevalence of deep venous anomalies in congenital vascular malformations of venous predominance. Journal of Vascular Surgery. 2000;31(3):462-471. doi:10.1067/MVA.2000.101464
    4. Venous Malformation | Boston Children’s Hospital. Accessed March 4, 2022. https://www.childrenshospital.org/conditions-and-treatments/conditions/v/venous-malformation
    5. Venous Malformations: Practice Essentials, History of the Procedure, Epidemiology. Accessed March 4, 2022. https://emedicine.medscape.com/article/1296303-overview#a8
    6. Dubois J, Garel L. Imaging and therapeutic approach of hemangiomas and vascular malformations in the pediatric age group. Pediatric Radiology 1999 29:12. 1999;29(12):879-893. doi:10.1007/S002470050718
    7. Yakes WF, Luethke J, Stavros AT, Slater D. MR Imaging of Symptomatic Peripheral Vascular Malformations Staging breast ca withUS View project The Yakes AVM Classification System for Therapeutic Endovascular Management of AVMs. View project. Article in American Journal of Roentgenology. Published online 1992. doi:10.2214/ajr.159.1.1609682
    8. Sclerotherapy: Background, Etiology, Indications. Accessed March 4, 2022. https://emedicine.medscape.com/article/1271091-overview
    9. Sclerotherapy. Accessed March 4, 2022. https://my.clevelandclinic.org/health/treatments/6763-sclerotherapy#recovery-and-outlook

    The above information explains what is involved and the possible risks. It is not meant to be a substitute for informed discussion between you and your doctor, but can act as a starting point for such a discussion.

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