eMedicine Specialties > Vascular Surgery > Medical Topics
Arteriovenous Malformations: Treatment & Medication
Updated: May 18, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
- Hemangioma
- Of hemangiomas, 70-90% resolve completely.
- The key is to determine which lesions require early intervention to minimize long-term complications or disfigurement.
- Steroids, both systemic and intralesional, have been used to treat rapidly growing hemangiomas. The mechanism by which steroids induce regression is not entirely clear. Both local and systemic administration of steroids may lead to adverse cushingoid effects.
- Another antiangiogenic agent being investigated is interferon alfa-2a. This agent has shown promise in the treatment of pulmonary hemangiomatosis, life-threatening hemangiomas, and diffuse neonatal hemangiomatosis. Adverse effects include hemodynamic changes, viral symptoms, neutropenia, alopecia, anemia, and depression.
- Chemotherapy is reserved for life-threatening hemangiomas.
- Cyclophosphamide may reverse the platelet-trapping coagulopathy associated with Kasabach-Merritt syndrome.
- Because of its long-term risks, radiation therapy plays a role only in the treatment of drug-resistant congestive heart failure.
- Vascular malformations
- Capillary vascular malformations are treated surgically. See Surgical Care.
- Treatment for venous malformations is compression garment therapy. Early use may prevent long-term morbidity and the need for surgery.
- Sclerotherapy may be used for spider veins and true varicose veins.
Surgical Care
- Hemangioma
- Surgical care is most commonly used when involution is incomplete or leaves excess fibrofatty tissue or redundant skin. Complex reconstruction may be required for significant distortion of facial features. Excision prior to full involution should be considered for lesions that are pedunculated, bulky, occurring on the nasal tip, or ulcerated. Debulking is required if these lesions are obstructing vital structures (eg, visual-field obstruction).
- Techniques using cryotherapy (ablation with liquid nitrogen) and sclerotherapy have essentially been abandoned because of unfavorable scarring and inconsistent results. Embolization is used only for selective cases such as lesions associated with life-threatening platelet trapping.
- Laser photocoagulation (eg, yellow dye, argon) remains a controversial treatment modality for hemangiomas. This technique appears to be more effective for early and superficial lesions than for thick, mature hemangiomas.
- Vascular malformations
- Excisional treatment is warranted for venous malformations because spontaneous involution is not anticipated. Occasionally, the specific nature of the lesion is uncertain and excision is indicated for diagnosis.
- Laser photocoagulation is the treatment of choice for superficial capillary vascular malformations. Multiple treatments are required, and 100% clearance is not always possible. Laser therapy can also be used to coagulate superficial venous malformations of the face.
- Venous stripping may be required to treat varicose veins associated with certain vascular malformations. One notable exception to the use of this procedure is in persons with Klippel-Trenaunay syndrome in whom the varicose veins may serve as the principal venous return from the lower extremity.
- Arteriovenous malformations
- Complete excision of the malformation is required because subtotal resections result in recurrence. Preoperative embolization may be used, followed shortly with operative resection, preferably within 24 hours.
- Simple ligation or embolization is contraindicated for arteriovascular anomalies because rapid collateral arterial flow develops.
Consultations
Consultations are required frequently, depending on the location of the lesions, rapidity of growth, associated symptoms or deformities, or simply to assure spontaneous regression or benignity.
- Head and neck
- Consultations may be warranted for vascular lesions that may impinge on the airways, visual sight, oral structures, or potentially involve the neural axis.
- Consider consulting a plastic surgeon, pediatric surgeon, ophthalmologist, otolaryngologist, neurologist, and/or neurosurgeon, as indicated by the lesion.
- Intrathoracic/intra-abdominal lesions
- For example, lesions involving the liver may result in platelet trapping or high-output heart failure, particularly in infants and children.
- As such, early involvement of a pediatric surgeon, interventional radiologist, and/or thoracic or general surgeon may be indicated.
- Extremity lesions
- In addition to visible lesions, deep-seated intramuscular vascular malformations may require surgical excision.
- Laser therapy may dictate referral to a dermatologist or plastic surgeon.
- An orthopedist, pediatric surgeon, interventional radiologist, and/or vascular surgeon may lend valuable expertise for more extensive or deep-seated lesions.
Diet
No special diet is required.
Activity
No activity limitation is required unless the patient is undergoing laser therapy or operative intervention.
Medication
Medical therapy is generally reserved only for proliferative lesions (eg, rapidly expanding hemangiomas), particularly in infants younger than 6 months. Hemangiomas in children older than 5 months or those lesions that are no longer rapidly proliferating respond less predictably to medical intervention. Venous malformations rarely respond to medical therapy.
Corticosteroids
Certain metabolites of cortisol exhibit antiangiogenic effects. In vitro, cortisol has also been shown to reduce estrogen-binding capacity in proliferating hemangiomas.
Prednisone (Deltasone, Orasone, Sterapred)
Results in decreased circulating estradiol levels in treated infants.
Adult
Pediatric
2-3 mg/kg/d PO for 2-3 wk; taper to 1-1.5 mg/kg/d
Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI disease
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Abrupt discontinuation may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use
Methylprednisolone (Medrol, Solu-Medrol)
Occasional use of high-dose steroid therapy required if no response to standard dose.
Adult
Pediatric
30-100 mg/kg/d PO/IM/IV as single dose
Coadministration with digoxin may increase digitalis toxicity secondary to hypokalemia; estrogens may increase levels; phenobarbital, phenytoin, and rifampin may decrease levels (adjust dose); monitor patients for hypokalemia when taking medication concurrently with diuretics
Documented hypersensitivity; viral, fungal, or tubercular skin infections
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Hyperglycemia, edema, osteonecrosis, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, growth suppression, myopathy, and infections are possible complications
Triamcinolone (Aristospan Intra-articular)
Intralesional injection may reduce systemic effects of drug.
Adult
Pediatric
0.2 mg/kg intralesional injection; 1-2 injections/wk; may require general anesthesia
Coadministration with barbiturates, phenytoin, and rifampin decreases effects
Documented hypersensitivity; fungal, viral, and bacterial skin infections
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Multiple complications (eg, severe infections, hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression) may occur; abrupt discontinuation may cause adrenal crisis; major complications have been reported from periocular use
Biological response modifiers
Interferons may induce release of 2',5'-oligoadenylate synthetase, which inhibits proliferation of endothelial cells.
Interferon alfa-2a (Roferon-A)
Protein product manufactured by recombinant DNA technology. Mechanism of antitumor activity is not clearly understood; however, direct antiproliferative effects against malignant cells and modulation of host immune response may play important roles.
Adult
30 million U/m2 SC qd/qod for 4 mo or longer
Pediatric
Administer as in adults
Theophylline may increase toxicity; cimetidine may increase antitumor effects; zidovudine and vinblastine may increase toxicity
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in severe hepatic or renal insufficiencies, seizure disorders, multiple sclerosis, or compromised CNS; significant hemodynamic alterations require ICU observation
Sclerosing agents
Effect produced by irritation and inflammation of venous endothelium and thrombus formation. Injected vein is occluded and fibrous tissue develops, resulting in vein obliteration.
Sodium tetradecyl sulfate (Sotradecol)
May be useful in sclerosing residual venous lesions.
Adult
3% solution injected into lesion; amount depends on size of lesion (5-90 mL); repeat prn
Pediatric
Administer as in adults
None reported
Documented hypersensitivity; arterial disease, thrombophlebitis, valvular or deep vein incompetence, phlebitis; migraines; cellulitis; acute infections
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
For IV use only; inadvertent intra-arterial injection may result in severe ischemic damage
Antiplatelet therapies
Occasionally, lesions respond to no other therapy.
Pentoxifylline (Trental)
May alter rheology of red blood cells, which, in turn, reduces blood viscosity.
Adult
400 mg PO tid
Pediatric
Not recommended
Coadministration with cimetidine or theophylline increases effect/toxic potential; pentoxifylline increases effect of antihypertensives
Documented hypersensitivity; cerebral and/or retinal hemorrhage
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 |
| « Previous Page | Next Page » |
References
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].
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].
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].
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].
Matsuda D, Iwamura M, Baba S. Cavernous hemangioma of the adrenal gland. Int J Urol. Apr 2009;16(4):424. [Medline].
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].
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].
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].
Abe S. Local steroid therapy of adnexal strawberry hemangiomas in infants-long term follow-up. Eur J Plast Surg. 1986;9:29.
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.
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.
Low DW. Hemangiomas and vascular malformations. Semin Pediatr Surg. May 1994;3(2):40-61. [Medline].
Steinmetz OK, Palerme LP. Images in clinical medicine. Acquired arteriovenous fistula. N Engl J Med. May 20 2004;350(21):2180. [Medline].
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].
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)
Renal Arteriovenous Malformation
Osler-Weber-Rendu Disease
Dandy-Walker Malformation
Klippel-Trenaunay-Weber Syndrome
Sturge-Weber Syndrome
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
