Vascular Surgery for Arteriovenous Malformations Treatment & Management

  • Author: Allison Leigh Speer, MD; Chief Editor: Vincent Lopez Rowe, MD   more...
 
Updated: Aug 9, 2010
 

Medical Care

The mainstays of arteriovenous malformation (AVM) management are not medical but rather involve interventional radiology procedures and surgery such as embolization, sclerotherapy, surgical resection, and reconstruction.[4, 1, 7]

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Surgical Care

Treatment is rare during infancy and early childhood for stage I AVM. Stage I AVMs can be followed with yearly examinations. Infrequently and after careful consideration, resection may be performed for a well-localized quiescent stage I AVM (ie, when complete resection is possible without poor cosmesis; however, this remains controversial). Usually, invasive treatment is delayed until local endangering signs (stage III) are present or cardiac complications (stage IV) develop. In the uncommon occurrence of congestive heart failure caused by an AVM, prompt embolization may be necessary.

As mentioned previously, proximal embolization of feeding arterial vessels should never by performed because this leads to rapid recruitment of new vessels from adjacent arteries to supply the nidus, with growth and progression of the lesion. Similarly, partial surgical excision leads to only temporary improvement followed by reexpansion of the AVM over time.

The management of AVMs is challenging because selecting the optimal therapy to minimize recurrence is often difficult. Sometimes complete resection is not possible or would result in severe disfigurement, as in the case of diffuse or infiltrating AVMs that invade pelvic tissues, permeate deep craniofacial structures, or penetrate all tissue planes of an extremity. In these cases, embolization or sclerotherapy is indicated and may be successful. Generally, the treatment of AVMs is either palliative to control a complication (intractable pain, skin ulceration, tissue necrosis, bleeding, or lytic bone lesion with risk of fracture) or aims to be curative (embolization followed by wide surgical resection and reconstruction).[4, 1] Indications for surgery are listed in Table 3.

Preoperative surgical planning should involve a thorough review of MRI/MRA and angiogram studies. Surgical resection is usually preceded by arterial embolization for temporary nidus occlusion. This minimizes intraoperative bleeding but does not reduce the limits of planned resection. Both the AVM nidus and involved skin must be excised widely, although if the overlying skin appears normal, it can be saved.

The goal of surgery is complete resection, unlike staged resection applicable to slow-flow vascular malformations, to minimize recurrence. The best wound coverage is primary closure with local or distant tissue flaps. Amputation is a viable option for the ischemic or nonfunctional extremity. Combined embolization and surgical resection is most successful for well-localized stage I-II AVMs. However, these patients must still be followed for years with regular physical examination, ultrasonography, and/or MRI.

In one series, all 16 patients with surgically accessible, localized, noninfiltrating AVMs who underwent preoperative angioembolization with subsequent surgical excision demonstrated no evidence of recurrence on angiography during a follow-up averaging 24.3 months.[17] The possibility of recurrence is high, and experienced surgeons recognize that long-term follow-up is critical to ensure a cure.[4, 1]

Table 3: Indications for Surgical Treatment of AVMs (Open Table in a new window)

Absolute Indications Relative Indications
  • Hemorrhage
  • Ischemia (arterial insufficiency or ulceration, gangrene)
  • Chronic venous insufficiency with venous hypertension
  • Lesions that compromise breathing, vision, hearing, or eating
  • High-output cardiac failure
  • Poor quality of life (disabling or intractable pain, functional impairment, severe cosmetic deformity)
  • Lesions with potentially high risk of complications (eg, hemarthrosis, fracture, or limb-threatening location)
  • Vascular-bone syndrome with limb length discrepancy
Table modified from Lee et al.[17]
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Consultations

As mentioned previously, the management of vascular anomalies requires interdisciplinary care and collaboration between many specialities. Specific consultations depend on the type of vascular anomaly and its location. Possible consultations specifically regarding AVMs include pediatric or general surgery, plastic surgery, vascular surgery, neurosurgery, otolaryngology, orthopedic surgery, radiology, interventional radiology, hematology, gastroenterology, physical therapy, occupational therapy, and speech therapy.

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Diet

No special diet is required or recommended.

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Activity

Activity is not limited unless the patient is undergoing an interventional or surgical procedure.

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Contributor Information and Disclosures
Author

Allison Leigh Speer, MD  Research Fellow, Pediatric Surgery, Childrens Hospital Los Angeles

Allison Leigh Speer, MD is a member of the following medical societies: American College of Surgeons and Association for Academic Surgery

Disclosure: Nothing to disclose.

Coauthor(s)

Andre Panossian, MD  Assistant Professor of Surgery, Division of Plastic Surgery, University of Southern California Keck School of Medicine, Childrens Hospital Los Angeles

Andre Panossian, MD is a member of the following medical societies: American Cleft Palate/Craniofacial Association, American College of Surgeons, and American Society of Reconstructive Transplantation

Disclosure: Nothing to disclose.

Alexandre Arkader, MD  Assistant Professor of Orthopaedic Surgery, University of Southern California Keck School of Medicine; Director, Orthopaedic Oncology Program, Childrens Orthopaedic Center, Childrens Hospital Los Angeles

Alexandre Arkader, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, Connective Tissue Oncology Society, and Pediatric Orthopaedic Society of North America

Disclosure: Nothing to disclose.

Philip Stanley, MBBS, MRCP  Attending Radiologist, Childrens Hospital Los Angeles

Philip Stanley, MBBS, MRCP is a member of the following medical societies: American Roentgen Ray Society, Radiological Society of North America, and Society of Cardiovascular and Interventional Radiology

Disclosure: Nothing to disclose.

Dean M Anselmo, MD  Attending Surgeon, Division of Pediatric Surgery, Childrens Hospital Los Angeles

Dean M Anselmo, MD is a member of the following medical societies: American Pediatric Surgical Association and International Pediatric Endosurgery Group

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Vincent Lopez Rowe, MD  Associate 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, American Heart Association, Pacific Coast Surgical Association, Peripheral Vascular Surgery Society, Society for Clinical Vascular Surgery, Society for Vascular Surgery, and Western Vascular Surgical Society

Disclosure: Nothing to disclose.

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

Vincent Lopez Rowe, MD  Associate 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, American Heart Association, Pacific Coast Surgical Association, Peripheral Vascular Surgery Society, Society for Clinical Vascular Surgery, Society for Vascular Surgery, and Western Vascular Surgical Society

Disclosure: Nothing to disclose.

References

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  9. Marler JJ, Fishman SJ, Kilroy SM, et al. Increased expression of urinary matrix metalloproteinases parallels the extent and activity of vascular anomalies. Pediatrics. Jul 2005;116(1):38-45. [Medline].

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  18. Eerola I, Boon LM, Mulliken JB, et al. Capillary malformation-arteriovenous malformation, a new clinical and genetic disorder caused by RASA1 mutations. Am J Hum Genet. Dec 2003;73(6):1240-9. [Medline]. [Full Text].

  19. Enjolras O, Chapot R, Merland JJ. Vascular anomalies and the growth of limbs: a review. J Pediatr Orthop B. Nov 2004;13(6):349-57. [Medline].

  20. Marsh DJ, Kum JB, Lunetta KL, et al. PTEN mutation spectrum and genotype-phenotype correlations in Bannayan-Riley-Ruvalcaba syndrome suggest a single entity with Cowden syndrome. Hum Mol Genet. Aug 1999;8(8):1461-72. [Medline].

  21. Marsh DJ, Coulon V, Lunetta KL, et al. Mutation spectrum and genotype-phenotype analyses in Cowden disease and Bannayan-Zonana syndrome, two hamartoma syndromes with germline PTEN mutation. Hum Mol Genet. Mar 1998;7(3):507-15. [Medline].

  22. Takaya N, Iwase T, Maehara A, et al. Transcatheter embolization of arteriovenous malformations in Cowden disease. Jpn Circ J. Apr 1999;63(4):326-9. [Medline].

  23. Calva D, Howe JR. Hamartomatous polyposis syndromes. Surg Clin North Am. Aug 2008;88(4):779-817, vii. [Medline]. [Full Text].

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  25. Turnbull MM, Humeniuk V, Stein B, Suthers GK. Arteriovenous malformations in Cowden syndrome. J Med Genet. Aug 2005;42(8):e50. [Medline]. [Full Text].

 
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Panel A: 12-year-old female with right facial arteriovenous malformation (AVM) s/p sclerotherapy. Panel B: 12.5-year-old female 4 months after resection of right facial AVM with preoperative embolization, complex closure, and lip reconstruction with rotational advancement flaps. Panel C: 13-year-old female with good recovery and no residual palpable or pulsatile AVM. Panel D: 14-year-old female with regrowth of AVM after the onset of puberty.
MRI of a rectal arteriovenous malformation (AVM). Panel A: Axial, intraperitoneal rectum. Panel B: Axial, extraperitoneal rectum. Panel C: Coronal, posterior to lumbosacral prominence.
Angiogram of a rectal arteriovenous malformation (AVM). Panel A: arterial phase. Panel B: venous phase.
Ischemic contractures secondary to a right hand arteriovenous malformation (AVM). Panel A: ventral. Panel B: dorsal. Panel C: excellent outcome after surgical resection/amputation.
Left thigh arteriovenous malformation (AVM). Panel A: intraoperative. Panel B: bisected.
CT Angiogram of a pulmonary arteriovenous malformation (AVM).
Table 1: ISSVA 1996 Classification of Vascular Anomalies
Vascular Tumors Vascular Malformations
  • Infantile hemangiomas
  • Congenital hemangiomas
  • Rapidly involuting congenital hemangioma (RICH)
  • Noninvoluting congenital hemangioma (NICH)
  • Tufted angioma (+/- Kasabach-Merritt syndrome)
  • Kaposiform hemangioendothelioma
  • (+/- Kasabach-Merritt syndrome)
  • Spindle cell hemangioendothelioma
  • Other, rare hemangioendotheliomas (eg, epithelioid, composite, retiform, polymorphous, Dabska tumor, lymphangioendotheliomatosis)
  • Dermatologic acquired vascular tumors (pyogenic granuloma, targetoid hemangioma, glomeruloid hemangioma, microvenular hemangioma, etc.)
  • Slow-flow
  • Capillary malformation (CM)
    • Port-wine stain
    • Telangiectasia
    • Angiokeratoma
  • Venous malformation (VM)
    • Common sporadic VM
    • Bean syndrome
    • Familial cutaneous and mucosal venous malformation (VMCM)
    • Glomuvenous malformation (GVM)
    • Maffucci syndrome
  • Lymphatic malformation (LM)
Fast-flow
  • Arterial malformation (AM)
  • Arteriovenous fistula (AVF)
  • Arteriovenous malformation (AVM)
Complex-combined vascular malformations
  • CVM, CLM, LVM, CLVM, AVM-LM, CM-AVM
C=capillary, V=venous, L=lymphatic, A=arterial, M=malformation, F=fistula
Table 2: Schobinger Staging for AVMs
Stage Description
I - QuiescencePink-bluish stain, warmth, and arteriovenous shunting are revealed by Doppler scanning. The arteriovenous malformation mimics a capillary malformation or involuting hemangioma.
II - ExpansionThe description is the same as stage I, plus enlargement, pulsations, thrill, and bruit and tortuous/tense veins.
III - DestructionThe description is the same as stage II, plus dystrophic skin changes, ulceration, bleeding, persistent pain, or tissue necrosis. Bony lytic lesions may occur.
IV - DecompensationThe description is the same as stage III, plus congestive cardiac failure with increased cardiac output and left ventricle hypertrophy.
Table 3: Indications for Surgical Treatment of AVMs
Absolute Indications Relative Indications
  • Hemorrhage
  • Ischemia (arterial insufficiency or ulceration, gangrene)
  • Chronic venous insufficiency with venous hypertension
  • Lesions that compromise breathing, vision, hearing, or eating
  • High-output cardiac failure
  • Poor quality of life (disabling or intractable pain, functional impairment, severe cosmetic deformity)
  • Lesions with potentially high risk of complications (eg, hemarthrosis, fracture, or limb-threatening location)
  • Vascular-bone syndrome with limb length discrepancy
Table modified from Lee et al.[17]
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