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Combined Vascular Malformation

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Vascular Malformations

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Abstract

Combined vascular malformation comprises two or more components in one lesion. It can involve any combination of capillary, lymphatic, venous, and arterial channels. Combined malformations may be classified as slow-flow and fast-flow lesion. Sometimes combined malformations are associated with skeletal or soft tissue overgrowth. Usually, it is confined to limbs but may involve any organ. Klippel-Trenaunay Syndrome is the commonest slow-flow combined vascular malformation having capillary, lymphatic, and venous components with limb deformity. Parkes–Weber syndrome is a fast-flow malformation associated with capillary malformation, multiple microarteriovenous fistulas, lymphatic component, skeletal, and soft tissue hypertrophy of limb. Diagnosis may be confirmed at birth clinically with diffuse enlargement of limb, mostly lower. Vascular malformation, truncal lipomatous masses, and musculoskeletal anomalies comprise another newly recognized syndrome named congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal anomalies (CLOVES) syndrome. Diagnosis of combined vascular malformation includes clinical evaluation, duplex scanning, radiography, MRI with contrast, lymphoscintigraphy, etc. Treatment may be planned according to complications like pain, malformation, mass effect, the progression of limb elongation, risk of pulmonary embolism. For slow-flow malformation surgery, sclerotherapy, and laser treatment are the best options. Surgical debulking, treatment of skeletal deformity, selective embolization, amputation are the options for fast-flow malformations. Regular monitoring is essential for all cases.

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Summary

Proper diagnosis is essential for patients affected by complex combined vascular malformations. These patients benefit from an interdisciplinary approach involving many medical and surgical specialists. Interventions must be tailored to the specific needs and symptoms of the patient. Outcomes are optimized with careful preoperative planning, identification of comorbidities, and realistic expectations on behalf of both the patient and surgeon.

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Authors and Affiliations

  1. National Institute of Cardiovascular Diseases and Hospital, Dhaka, Bangladesh

    Shantonu Kumar Ghosh

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  1. Shantonu Kumar Ghosh
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Editors and Affiliations

  1. Department of Surgery, Institute of Medical Science, Banaras Hindu University, Varanasi, India

    Ajay K. Khanna

  2. Department of Surgery, Institute of Medical Science, Banaras Hindu University, Varanasi, India

    Satyendra K. Tiwary

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Ghosh, S.K. (2021). Combined Vascular Malformation. In: Khanna, A.K., Tiwary, S.K. (eds) Vascular Malformations. Springer, Singapore. https://doi.org/10.1007/978-981-15-9762-6_10

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  • DOI: https://doi.org/10.1007/978-981-15-9762-6_10

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  • Publisher Name: Springer, Singapore

  • Print ISBN: 978-981-15-9761-9

  • Online ISBN: 978-981-15-9762-6

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