Arteriovenous Malformations: Syndrome Identification and Vascular Management
Purpose of review
Vascular malformations represent a poorly understood set of conditions that create considerable diagnostic and therapeutic confusion. Historically, extensive surgical resections yielded poor results. More recently, the treatment paradigm has shifted to endovascular therapy such that embolization and sclerotherapy are now considered the first line of treatment. Although there is still a role for traditional surgical techniques, it is now most commonly integrated with endovascular therapies in a hybrid fashion. The goal of this review is to provide a greater understanding of the diagnosis and treatment of vascular malformations.
Vascular malformations can be high-flow (arteriovenous shunting) or low-flow (venous or lymphatic). Clinical presentation and treatment is different for each. Treatment of high-flow lesions must be directed at reducing or eliminating the nidus, usually via super-selective catheterization and embolization. Low-flow lesions are usually treated by direct injection of sclerosing agents under fluoroscopic or ultrasound guidance.
The cornerstone of managing patients with vascular malformations is making the proper diagnosis, which is often challenging. Even after a diagnosis is made, therapy itself may be challenging and generate frustration among patients and providers. Frequently, the treatment of vascular malformations is characterized by only slow and incremental improvements without complete lesion eradication. By combining a thorough understanding of the vascular malformation disease process with an array of endovascular techniques, vascular interventionalists may contribute greatly to the care of these patients.
KeywordsVascular disease Arteriovenous malformations Vascular management Arteriovenous shunting Venous or lymphatic
Compliance with Ethical Standards
Conflict of Interest
The authors declare that they have no conflicts of interest.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
References and Recommended Reading
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
- 1.Persaud TVN. Problems of birth defects. From Hippocrates to Thalidomide and after. Persaud TVN Part I Belief, mythology, magic and superstition. Commentary. Baltimore: University Park Press Maryland Publishers; 1977. p. 3–4.Google Scholar
- 2.• Belov S. Classification, terminology, and nosology of congenital vascular defects. In: Belov S, Loose DA, Weber J, editors. Vascular malformations. Reinbek: Einhorn-Presse; 1989. p. 25–30.Describing a new classification system for vascular anomalies has emerged that is based on advances in the study of these conditions and is useful in contemporary managementGoogle Scholar
- 3.•• International Society for the Study of Vascular Anomalies. Approved at the 20th ISSVA Workshop, Melbourne, April 2014. Retrieved from http://www.issva.org/UserFiles/file/Classifications-2014-Abbreviated.pdf.A current classification system of vascular anomalies as described by the ISSVA.
- 8.Blei F, Chianese J. Corticosteroid toxicity in infants treated for endangering hemangiomas: experience and guidelines for monitoring. Intl pediatrics. 1999;14:146–53.Google Scholar
- 10.Natali J, Merland JJ. Superselective arteriography and therapeutic embolization for vascular malformations (angiodysplasias). J Cardiovasc Surg. 1976;17:465.Google Scholar
- 13.•• Yakes WF, Haas DK, Parker SH, et al. Symptomatic vascular malformations: ethanol embolotherapy. Radiology. 1989;170:1059–66.Absolute ethanol was used to perform nine transcatheter embolizations and 21 direct percutaneous puncture embolizations in eight patients with unresectable vascular malformations. The authors showed how ethanol embolotherapy, performed according to strict techniques, can be efficacious in the management of vascular malformations.CrossRefPubMedGoogle Scholar
- 17.• Conway AM, Qato K, Drury J, Rosen RJ. Embolization techniques for high-flow arteriovenous malformations with a dominant outflow vein. J Vasc Surg Venous Lymphat Disord. 2015;3(2):178–83.The aim of this study was to assess the management and outcomes of arteriovenous malformations (AVMs) with a dominant outflow vein (DOV) treated with retrograde venous embolization. The authors showed how AVMs with a DOV can be successfully treated by a transvenous approach.CrossRefPubMedGoogle Scholar
- 22.• Yang Y, Sun M, Ma Q, Cheng X, Ao J, Tian L, et al. Bleomycin A5 sclerotherapy for cervicofacial lymphatic malformations. J Vasc Surgery. 2011;53:150–5.The purpose of this study was to document the results of bleomycin A5 sclerotherapy for cervicofacial lymphatic malformations (LM). These data suggest bleomycin A5 is a safe and effective intralesional agent for the treatment of macrocystic LMs, superficial oral mucosa LM, and localized deep microcystic lesions.CrossRefGoogle Scholar
- 25.Klippel M, Trenaunay P. Du naevus variqueux osterhypertrophique. Arch Gen Med (Paris). 1900;3:611.Google Scholar
- 26.Parkes Weber F. Haemangiectatic hypertrophy of the limbs: congenital phlebarterietasis and so-called congenital varicose veins. Br J Child Dis. 1918;15:13.Google Scholar
- 28.• Howlett DC, Roebuck DJ, Frazer CK, Ayers B. The use of ultrasound in the venous assessment of lower limb Klippel-Trenaunay syndrome. Eur J Radiol. 1994;18:224–6.Colour Doppler ultrasound was used to study the affected lower limbs of a group of patients with the Klippel-Trenaunay syndrome. From this study, the deep venous aplasia said to occur in the condition would appear to be less common than previously thought.CrossRefPubMedGoogle Scholar
- 32.Cronenwett JL, Johnston KW. Rutherford’s vascular surgery. 7th ed. Philadelphia: Saunders Elsevier; 2010. p. 1072–5.Google Scholar
- 37.Uller W, El-Sobky S, Alomari AI, Fishman SJ, Spencer SA, Taghinia AH, et al. Preoperative embolization of venous malformations using n-butyl cyanoacrylate. Vasc Endovascular Surg. 2018; https://doi.org/10.1177/1538574418762192.
- 38.Eivazi B, Werner JA. Extracranial vascular malformations (hemangiomas and vascular malformations) in children and adolescents—diagnosis, clinic, and therapy. GMS Curr Top Otorhinolaryngol Head Neck Surg. 2014;13 https://doi.org/10.3205/cto000105.