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Arteriovenous Malformations: Follow-up
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 |
| « Previous Page | Next Page » |
References
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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.
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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
Follow-up: Arteriovenous Malformations