eMedicine Specialties > Vascular Surgery > Medical Topics

Arteriovenous Malformations: Follow-up

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

Follow-up

Complications

  • Hemangioma
    • Residual scars, excess fibrofatty tissue, or severe disfigurement in 10-30% of patients
    • Airway obstruction (subglottic hemangioma, nasal hemangioma)
    • Visual axis obstruction (preorbital or orbital hemangioma)
    • Oral/digestive tract obstruction
    • Bilateral auditory canal obstruction (bilateral parotid hemangiomas)
    • Kasabach-Merritt syndrome (platelet-trapping thrombocytopenia)
    • Congestive heart failure (especially with visceral hemangiomas)
  • Vascular malformations
    • Severe disfigurement
    • Extremity overgrowth (Klippel-Trenaunay syndrome)
    • Seizures, hemiparesis/plegia cerebral atrophy from leptomeninges involvement (Sturge-Weber syndrome)
    • Congestive heart failure
    • Other steal phenomenon with distal ischemia
    • Platelet trapping
    • Massive bleeding

Prognosis

  • Hemangioma
    • Complete involution in 70-90%
    • Risk of developing complications (listed above) in 10-30%
  • Vascular malformations
    • Do not involute or fade
    • Satisfactory cosmetic results accomplished with surgical treatment modalities (see Surgical Care)

Miscellaneous

Medicolegal Pitfalls

  • Occasionally, Klippel-Trenaunay syndrome, an unusual venous malformation, is encountered.
    • In this condition, the major venous outflow tracts of the lower extremity have an anomalous deficiency.
    • Patients typically present with a triad of lower extremity varicosities, port-wine stains, and limb hypertrophy.
    • Patients may be referred for vein stripping of the obvious varicose veins; however, Klippel-Trenaunay syndrome is a contraindication for vein stripping because these varicosities may represent the predominant venous collaterals around the absent deep vein.

Special Concerns

  • The majority of hemangiomas occur as isolated lesions, but a few syndromes are recognized.
    • Sternal clefting and midline abdominal effects: This is when the sternum and upper abdominal wall fail to fuse; it may be associated with extensive hemangiomas.
    • Neurological defects: Hemangiomas over the lumbosacral region may be associated with an underlying tethered cord.
    • Dandy-Walker syndrome: This is when posterior fossa abnormalities are associated with large plaquelike facial hemangiomas.
    • Cardiovascular defects: This is associated with infant hemangiomas and associated right-sided aortic arch coarctation.
  • Associated vascular malformation syndromes are as follows:
    • Sturge-Weber syndrome: This is associated with facial capillary malformations. The ophthalmic distribution is associated with underlying leptomeninges involvement.
    • Klippel-Trenaunay syndrome: This is when capillary malformations are associated with underlying lymphatic and venous malformations with overgrowth of the effected extremity.
    • Parkes Weber syndrome: This is associated with capillary malformations overlying a high-flow vascular malformation.
    • Osler-Weber-Rendu syndrome (hereditary hemorrhagic telangiectasia): This autosomal dominant syndrome is characterized by small cutaneous vascular lesions on the lips and mucous membranes. It usually develops in adolescence. It may be associated with significant GI bleeding, hematuria, and CNS bleeding.
    • von Hippel-Lindau syndrome: This is associated with cerebellar cysts and vascular malformations of the retina and cerebellum.
  • Other malformations are considered separately because they cannot be easily or accurately categorized into the hemangioma/vascular malformation classification system.
    • Macular stains (stork bites, angel's kiss, salmon patch): These usually develop on the forehead or nuchal area. Fading occurs during the first year; if the stain persists, then the patient has a true capillary vascular malformation.
    • Telangiectasias: These can be classified as capillary vascular malformations and are commonly observed as spider angiomas manifesting in childhood and adulthood. Laser photocoagulation is the treatment of choice.
 


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
[ CLOSE WINDOW ]

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].

[ CLOSE WINDOW ]

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

[ CLOSE WINDOW ]

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

[ CLOSE WINDOW ]

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.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.