eMedicine Specialties > Vascular Surgery > Medical Topics

Arteriovenous Malformations: Differential Diagnoses & Workup

Author: Steven L Lee, MD, Chief, Pediatric Surgery, Department of Surgery, Kaiser-Permanente, Los Angeles Medical Center
Coauthor(s): Devin P Puapong, MD, Fellow, Division of Pediatric Surgery, University of Oklahoma Health Sciences Center; Jeffrey J DuBois, MD, Consulting Staff, Division of Pediatric Surgery, Kaiser Permanente, North Sacramento Medical Center
Contributor Information and Disclosures

Updated: May 18, 2009

Differential Diagnoses

Arteriovenous Fistulas
Glomus Tumors
Hemangioblastoma
Hemangiomas, Hepatic
Osler-Weber-Rendu Disease
Varicose Veins

Other Problems to Be Considered

Hemangioendothelioma
Hemangiopericytoma
Klippel-Trenaunay-Weber syndrome
Lymphangioma
Skeletal muscle hemangioma
Sturge-Weber syndrome

Workup

Laboratory Studies

  • No lab test reliably distinguishes hemangiomas from vascular malformations; however, identification of elevated angiogenic growth factors, such as basic fibroblastic growth factor, secreted in the urine of patients with hemangiomas may be useful in distinguishing proliferative vascular tumors from nonproliferating lesions.
  • Perform a CBC count to rule out platelet trapping if the patient has spontaneous bleeding.
  • Perform clotting studies if the patient has evidence of hemorrhage or hematoma formation.

Imaging Studies

  • Plain films
    • Hemangiomas demonstrate soft tissue fullness and are not usually associated with bone involvement; however, bony distortion due to mass effect may be observed.
    • Vascular malformations may be associated with skeletal overgrowth or bony destruction due to high-flow lesions. Phleboliths also may be observed.
  • Ultrasound
    • Both early hemangiomas and vascular malformations are high-flow lesions and are difficult to differentiate.
    • Venous malformations demonstrate slow flow.
  • Angiography
    • Angiography is not often necessary unless embolization is used prior to surgical excision.
    • Hemangiomas demonstrate peripheral feeding arteries and draining veins.
    • Whereas hemangiomas demonstrate intense parenchymal staining, vascular malformations typically demonstrate ectatic vessels without parenchyma.
    • Arteriovenous malformations are characterized by rapid venous shunting.
  • Magnetic resonance imaging and magnetic resonance angiography
    • MRI and magnetic resonance angiography accurately distinguish hemangiomas from vascular malformations, including arteriovenous malformations.
    • These studies are the most useful of all imaging studies.
  • Nuclear medicine is useful for screening the entire body for vascular anomalies.

Histologic Findings

  • Hemangioma: Proliferating hemangiomas are plump and have actively dividing endothelial cells. The basement membrane is multilaminated (due to a high proliferation rate), and mast cells are abundant (up to 40 times normal). Involuting hemangiomas have flat endothelial cells, vascular thrombosis, and collagen and fat deposition.
  • Vascular malformations (ectatic capillaries, veins, or lymphatics): These anomalies show no evidence of rapid endothelial proliferation. Capillary vascular malformation has dilated capillaries and venules of variable density in the dermis.

More on Arteriovenous Malformations

Overview: Arteriovenous Malformations
Differential Diagnoses & Workup: Arteriovenous Malformations
Treatment & Medication: Arteriovenous Malformations
Follow-up: Arteriovenous Malformations
Multimedia: Arteriovenous Malformations
References
Further Reading
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References

  1. Castori M, Majore S, Binni F, Grammatico P. Paradoxical association of extensive nevus flammeus together with unilateral lower limb and breast hypoplasia. Am J Med Genet A. Feb 2009;149A(2):266-7. [Medline].

  2. Gao XH, Wang LL, Zhang L, Hong YX, Wei H, Chen HD. Familial nevus flammeus associated with early onset cherry angiomas. Int J Dermatol. Dec 2008;47(12):1284-6. [Medline].

  3. Alster TS, Tanzi EL. Combined 595-nm and 1,064-nm Laser Irradiation of Recalcitrant and Hypertrophic Port-Wine Stains in Children and Adults. Dermatol Surg. Apr 9 2009;[Medline].

  4. Tierney EP, Hanke CW. Treatment of nodules associated with port wine stains with CO2 laser: case series and review of the literature. J Drugs Dermatol. Feb 2009;8(2):157-61. [Medline].

  5. Matsuda D, Iwamura M, Baba S. Cavernous hemangioma of the adrenal gland. Int J Urol. Apr 2009;16(4):424. [Medline].

  6. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. Mar 1982;69(3):412-22. [Medline].

  7. Qing Y, Cen Y, Xu X, Duan W, Liu Y. [Surgical treatment of hemangioma and vascular malformation in body surface]. Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi. Mar 2009;23(3):325-7. [Medline].

  8. Tongers J, Widera C, Kempf T, Drexler H, Westhoff-Bleck M. Two vascular arteriovenous malformations with left-to-right shunting and right-heart failure in a single patient. Int J Cardiol. Apr 30 2009;[Medline].

  9. Abe S. Local steroid therapy of adnexal strawberry hemangiomas in infants-long term follow-up. Eur J Plast Surg. 1986;9:29.

  10. Coffin CM, Dehner LP, O'Shea PA. Vascular tumors. In: Pediatric Soft Tissue Tumors: A Clinical, Pathological, and Therapeutic Approach. Baltimore, Md: Lippincott, Williams & Wilkins; 1997:. 40-79.

  11. Kim EJ, Halim AX, Dowd CF. The relationship of coexisting extranidal aneurysms to intracranial hemorrhage in patients harboring brain arteriovenous malformations. Neurosurgery. Jun 2004;54(6):1349-57; discussion 1357-8.

  12. Low DW. Hemangiomas and vascular malformations. Semin Pediatr Surg. May 1994;3(2):40-61. [Medline].

  13. Steinmetz OK, Palerme LP. Images in clinical medicine. Acquired arteriovenous fistula. N Engl J Med. May 20 2004;350(21):2180. [Medline].

  14. White CW, Wolf SJ, Korones DN, et al. Treatment of childhood angiomatous diseases with recombinant interferon alfa-2a. J Pediatr. Jan 1991;118(1):59-66. [Medline].

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Further Reading

Clinical guidelines
Skin cancer.
Finnish Medical Society Duodecim - Professional Association.  2001 Apr 30 (revised 2005 May 25).  Various pagings.  NGC:004600

ACR Appropriateness Criteria® soft tissue masses.
American College of Radiology - Medical Specialty Society.  1995 (revised 2005).  6 pages.  NGC:004784


Clinical trials

 Genetic Basis of Hemangiomas

Personalized Interactive Laser Therapy of Port Wine Stain

Arteriovenous Fistula Tissue Bank

Propranolol in Capillary Hemangiomas

Related eMedicine topics

 Arteriovenous Malformations   (Neurology)

Arteriovenous Fistulas

Hemangioblastoma

Hemangiomas, Hepatic

Renal Arteriovenous Malformation

Osler-Weber-Rendu Disease

Dandy-Walker Malformation

Klippel-Trenaunay-Weber Syndrome

Sturge-Weber Syndrome

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Keywords

arteriovenous malformations, hemangiomas, vascular malformations, capillary hemangioma, cavernous hemangioma, strawberry nevus, port-wine stain, portwine stain, port wine stain, arteriovenous fistula, nevus flammeus, vascular anomaly, cherry hemangioma, Klippel-Trenaunay syndrome, KTS, Dandy-Walker syndrome, Sturge-Weber syndrome, Parkes Weber syndrome, Osler-Weber-Rendu syndrome, von Hippel-Lindau syndrome, macular stains, stork bites, angel's kiss, angels kisses, salmon patch, telangiectasias, spider veins, birthmark, birth mark

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Contributor Information and Disclosures

Author

Steven L Lee, MD, Chief, Pediatric Surgery, Department of Surgery, Kaiser-Permanente, Los Angeles Medical Center
Steven L Lee, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Association for Academic Surgery, Society of American Gastrointestinal and Endoscopic Surgeons, and Society of Laparoendoscopic Surgeons
Disclosure: Nothing to disclose.

Coauthor(s)

Devin P Puapong, MD, Fellow, Division of Pediatric Surgery, University of Oklahoma Health Sciences Center
Disclosure: Nothing to disclose.

Jeffrey J DuBois, MD, Consulting Staff, Division of Pediatric Surgery, Kaiser Permanente, North Sacramento Medical Center
Jeffrey J DuBois, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Association for Academic Surgery, California Medical Association, Society for Surgery of the Alimentary Tract, Society of American Gastrointestinal and Endoscopic Surgeons, and Society of Critical Care Medicine
Disclosure: Nothing to disclose.

Medical Editor

Richard M Stillman, MD, FACS, Honorary Medical Staff, Northwest Medical Center; Former Chief of Staff and Medical Director, Wound Healing Center, Department of Surgery, Northwest Medical Center
Richard M Stillman, MD, FACS is a member of the following medical societies: American College of Angiology, American College of Surgeons, Association for Academic Surgery, and Society of University Surgeons
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Vincent Lopez Rowe, MD, Assistant Professor of Surgery, Department of Surgery, Division of Vascular Surgery, University of Southern California Medical Center
Vincent Lopez Rowe, MD is a member of the following medical societies: American College of Surgeons, Association for Academic Surgery, Peripheral Vascular Surgery Society, Society for Clinical Vascular Surgery, and Society for Vascular Surgery
Disclosure: Nothing to disclose.

CME Editor

Paolo Zamboni, MD, Professor of Surgery, Chief of Day Surgery Unit, Chair of Vascular Diseases Center, University of Ferrara, Italy
Paolo Zamboni, MD is a member of the following medical societies: American Venous Forum and New York Academy of Sciences
Disclosure: Nothing to disclose.

Chief Editor

William H Pearce, MD, Chief, Division of Vascular Surgery, Violet and Charles Baldwin Professor of Vascular Surgery, Department of Surgery, Northwestern University School of Medicine
William H Pearce, MD is a member of the following medical societies: American College of Surgeons, American Heart Association, American Surgical Association, Association for Academic Surgery, Association of VA Surgeons, Central Surgical Association, New York Academy of Sciences, Society for Vascular Surgery, Society of Critical Care Medicine, Society of University Surgeons, and Western Surgical Association
Disclosure: Nothing to disclose.

 
 
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