Skip to main content

Advertisement

Log in

Arteriovenous Malformations: Syndrome Identification and Vascular Management

  • Vascular Disease (M Weinberg, Section Editor)
  • Published:
Current Treatment Options in Cardiovascular Medicine Aims and scope Submit manuscript

Abstract

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.

Recent findings

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.

Summary

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5

Similar content being viewed by others

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 management

    Google 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.

  4. Paltiel HJ, Burrows PE, Kozakewich HP, Zurakowski D, Mulliken JB. Soft-tissue vascular anomalies: utility of US for diagnosis. Radiology. 2000;214(3):747–54.

    Article  PubMed  CAS  Google Scholar 

  5. Greenspan A, et al. Imaging strategies in the evaluation of soft tissue hemangiomas of the extremities: correlation of the findings of plain radiography, CT, MRI and ultrasonography in 12 histologically proven cases. Skeletal Radiol. 1992;21:8–11.

    Article  Google Scholar 

  6. Jeon YH, Do YS, Shin SW, et al. Ethanol embolization of arteriovenous malformations: results and complications of 33 cases. J Korean Radiol Soc. 2003;49:263–70.

    Article  Google Scholar 

  7. Madani H, Farrant J, Chhaya N, Anwar I, Marmery H, Platts A, et al. Peripheral limb vascular malformations: an update of appropriate imaging and treatment options of a challenging condition. Br J Radiol. 2015;88(1047):20140406.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  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 

  9. •• Léauté-Labrèze C, Dumas de la Roque E, Hubiche T, Boralevi F, Thambo JB, Taïeb A. Propranolol for severe hemangiomas of infancy. N Engl J Med. 2008;358(24):2649–51.Early description of the use of propranolol to inhibit the growth of infantile hemangiomas.

    Article  PubMed  Google Scholar 

  10. Natali J, Merland JJ. Superselective arteriography and therapeutic embolization for vascular malformations (angiodysplasias). J Cardiovasc Surg. 1976;17:465.

    CAS  Google Scholar 

  11. Osuga K, Hori S, Kitayoshi H, et al. Embolization of high flow arteriovenous malformations: experience with use of superabsorbent polymer microspheres. J Vasc Interv Radiol. 2002;13(11):1125–33.

    Article  PubMed  Google Scholar 

  12. Sasaki GH, Pang CY, Wittliff JL. Pathogenesis and treatment of infant skin strawberry hemangiomas: clinical and in vitro studies of hormonal effects. Plast Reconstr Surg. 1984;73(3):359–70.

    Article  PubMed  CAS  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.

    Article  PubMed  CAS  Google Scholar 

  14. Kerber C. Intracranial cyoanacrylate: a new catheter therapy for arteriovenous malformation. Invest Radiol. 1975;10:536–8.

    Article  PubMed  CAS  Google Scholar 

  15. Vintner V, Galil KA, Lundie MJ, Kaufmann JCE. The histotoxicity of cyanoacrylates. Neuroradiology. 1985;27:279–91.

    Article  Google Scholar 

  16. Numan F, Omeroglu A, Kara B, Cantaşdemir M, Adaletli I, Kantarci F. Embolization of peripheral vascular malformations with ethylene vinyl alcohol copolymer (Onyx). J Vasc Interv Radiol. 2004;15(9):939–46.

    Article  PubMed  Google 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.

    Article  PubMed  Google Scholar 

  18. Cho SK, Do YS, Kim DI, Kim YW, Shin SW, Park KB, et al. Peripheral arteriovenous malformations with a dominant outflow vein: results of ethanol embolization. Korean J Radiol. 2008;9:258–67.

    Article  PubMed  PubMed Central  Google Scholar 

  19. Boxt LM, Levin DC, Fellows KE. Direct puncture angiography in congenital venous malformations. AJR. 1983;140:135.

    Article  PubMed  CAS  Google Scholar 

  20. Mitchell S, Shah A, Schwengel D. Pulmonary artery pressure changes during ethanol embolization procedures to treat vascular malformations: can cardiovascular collapse be predicted? JVIR. 2006;17:253–62.

    Article  PubMed  Google Scholar 

  21. Hsu TS, Weiss RA. Foam sclerotherapy: a new era. Arch Dermatol. 2003;139(11):1494–6.

    Article  PubMed  Google 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.

    Article  Google Scholar 

  23. Muir T, Kirsten M, Fourie P, Dippenaar N, Ionescu GO. Intralesional bleomycin injection (IBI) treatment for haemangiomas and congenital vascular malformations. Pediatr Surg Int. 2004;19:766–73.

    Article  PubMed  CAS  Google Scholar 

  24. Khmamouche MR, Debbagh A, Mahfoud T, Aassab R, Lkhoyaali S, Ichou M, et al. Flagellate erythema secondary to bleomycin: a new case report and review of the literature. J Drugs Dermatol. 2014;13(8):983–4.

    PubMed  Google 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 

  27. Huang Y, Jiang M, Li W. Endovenous laser treatment combined with a surgical strategy for treatment of venous insufficiency in lower extremity: report of 208 cases. J Vasc Surg. 2005;42(3):494–501.

    Article  PubMed  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.

    Article  PubMed  CAS  Google Scholar 

  29. Greinwald JH Jr, Burke DK, Sato Y, Poust RI, Kimura K, Bauman NM, et al. Treatment of lymphangiomas in children: an update of picibanil (OK-432) sclerotherapy. Otolaryngol Head Neck Surg. 1999;121(4):381–7.

    Article  PubMed  Google Scholar 

  30. Kinmonth JB, Young AE, Edwards JM, O'Donnell TF, Thomas ML. Mixed vascular deformities of the lower limbs, with particular reference to lymphography and surgical treatment. Br J Surg. 1976;63(12):899–906.

    Article  PubMed  CAS  Google Scholar 

  31. Mendel T, Louis DS. Major vascular malformations of the upper extremity: long-term observation. J Hand Surg. 1997;22A:302.

    Article  Google Scholar 

  32. Cronenwett JL, Johnston KW. Rutherford’s vascular surgery. 7th ed. Philadelphia: Saunders Elsevier; 2010. p. 1072–5.

    Google Scholar 

  33. Pireau N, Boon LM, Poilvache P, Docquier PL. Surgical treatment of intra-articular knee venous malformations: when and how? J Pediatr Orthop. 2016;36(3):316–22.

    Article  PubMed  Google Scholar 

  34. Johnson JN, Shaughnessy WJ, Stans AA. Management of knee arthropathy in patients with vascular malformations. J Pediatr Orthop. 2009;29(4):380–4.

    Article  PubMed  Google Scholar 

  35. Cil BE, Vargel I, Geyik S, Peynircioglu B, Cavusoglu T. Venous vascular malformations of the craniofacial region: pre-operative embolisation with direct percutaneous puncture and N-butyl cyanoacrylate. Br J Radiol. 2008;81(972):935–9.

    Article  PubMed  CAS  Google Scholar 

  36. James CA, Braswell LE, Wright LB. Preoperative sclerotherapy of facial venous malformations: impact on surgical parameters and long-term follow-up. J Vasc Intervent Radiol. 2011;22(7):953–60.

    Article  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.

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Allan M. Conway MD.

Ethics declarations

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.

Additional information

This article is part of the Topical Collection on Vascular Disease

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Conway, A.M., Rosen, R.J. Arteriovenous Malformations: Syndrome Identification and Vascular Management. Curr Treat Options Cardio Med 20, 67 (2018). https://doi.org/10.1007/s11936-018-0662-7

Download citation

  • Published:

  • DOI: https://doi.org/10.1007/s11936-018-0662-7

Keywords

Navigation