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Vascular Surgery for Arteriovenous Malformations Treatment & Management

  • Author: Allison Leigh Speer, MD; Chief Editor: Vincent Lopez Rowe, MD  more...
 
Updated: Dec 09, 2014
 

Medical Care

The mainstays of arteriovenous malformation (AVM) management are not medical; rather, they involve interventional radiology procedures and surgery such as embolization, sclerotherapy, surgical resection, and reconstruction.[5, 1, 8]

Embolization/sclerosing agents

Absolute to 80% ethanol, N -butyl cyanoacrylate (NBCA) glue, various types of coils, and/or contour particles such as Ivalon can be used in various combinations, simultaneously or in stages, as primary or adjunctive embolization/sclerosing agents depending upon the location, severity, and extent of the arteriovenous malformation (AVM).

Ethanol is usually contraindicated and NBCA is relatively contraindicated for high-flow fistulous lesions due to the high risk of early wash into the systemic circulation. These fistulous AVMs can generally be treated through a staged approach. Coil embolization is used as a preliminary procedure to slow down the flow, resulting in decreased risk of subsequent distal thromboembolism. Then, agents such as ethanol or NBCA glue can be used to definitively treat the lesion.

Absolute to 80% ethanol can be given via transarterial, transvenous, or direct puncture injection. Ethanol has a high complication rate but results in fewest recurrences when used as a primary treatment for surgically inaccessible lesions. Major complications include deep vein thrombosis, transient nerve palsy, and ear cartilage necrosis. Minor complications are mainly skin changes.[20]

NBCA glue is predominately used for surgically excisable lesions as preoperative embolization therapy to reduce blood loss intraoperatively. It is not typically used as a permanent sclerosing agent because convincing evidence that it induces permanent damage to endothelial cells is lacking. Pulmonary embolism is rare but can occur.[20]

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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 be 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.

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).[5, 1] Indications for surgery are listed in Table 3 below.

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.[20]

 

Preoperative surgical planning should involve a thorough review of results from magnetic resonance imaging (MRI), magnetic resonance angiography (MRA), or angiography. 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 the involved skin must be excised widely, though if the overlying skin appears normal, it can be saved. (See the image below.)

Left thigh arteriovenous malformation (AVM). Panel Left thigh arteriovenous malformation (AVM). Panel A: intraoperative. Panel B: bisected.

To minimize recurrence, the goal of surgery for an AVM is complete resection, in contrast to the staged resection applicable to slow-flow vascular malformations. 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.[20] The possibility of recurrence is high, and experienced surgeons recognize that long-term follow-up is critical to ensure a cure.[5, 1]

A computed tomography (CT)-guided frameless robotic radiosurgical approach to intracranial AVMs has been described. Early results reported by Oermann et al in a retrospective study of 26 patients (median age, 41 years) at a median of 25 months' follow-up suggest that this approach can yield results comparable to those of frame-based methods.[21]

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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. With regard to AVMs in particular, possible consultations include the following:

  • Pediatric or general surgery
  • Plastic surgery
  • Vascular surgery
  • Neurosurgery
  • Otolaryngology
  • Orthopedic surgery
  • Radiology
  • Interventional radiology
  • Hematology
  • Gastroenterology
  • Physical therapy
  • Occupational therapy
  • Speech therapy
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Diet and Activity

No special diet is required or recommended.

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

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Long-Term Follow-up

Complications that may occur after embolization/sclerotherapy or surgical excision include the following:

  • Expansion
  • Recurrence
  • Poor cosmesis, disfigurement

In particular, the chance of recurrence after surgical resection of an AVM is high, and patients must be followed for years with regular physical examination, ultrasonography, magnetic resonance imaging, or some combination thereof.

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Contributor Information and Disclosures
Author

Allison Leigh Speer, MD Resident, General Surgery, University of Southern California; Former Research Fellow, Pediatric Surgery, Children's Hospital Los Angeles

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

Disclosure: Nothing to disclose.

Coauthor(s)

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, International Pediatric Endosurgery Group

Disclosure: Nothing to disclose.

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

Andre Panossian, MD, FACS is a member of the following medical societies: American Academy of Pediatrics, American Cleft Palate-Craniofacial Association, American College of Surgeons, American Society for Reconstructive Microsurgery, 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, 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, Society of Interventional Radiology

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

Vincent Lopez Rowe, MD Professor of Surgery, Program Director, Vascular Surgery Residency, Department of Surgery, Division of Vascular Surgery, Keck School of Medicine of the University of Southern California

Vincent Lopez Rowe, MD is a member of the following medical societies: American College of Surgeons, American Heart Association, Society for Vascular Surgery, Vascular and Endovascular Surgery Society, Society for Clinical Vascular Surgery, Pacific Coast Surgical Association, Western Vascular Society

Disclosure: Nothing to disclose.

Chief Editor

Vincent Lopez Rowe, MD Professor of Surgery, Program Director, Vascular Surgery Residency, Department of Surgery, Division of Vascular Surgery, Keck School of Medicine of the University of Southern California

Vincent Lopez Rowe, MD is a member of the following medical societies: American College of Surgeons, American Heart Association, Society for Vascular Surgery, Vascular and Endovascular Surgery Society, Society for Clinical Vascular Surgery, Pacific Coast Surgical Association, Western Vascular Society

Disclosure: Nothing to disclose.

References
  1. Mulliken JB, Fishman SJ, Burrows PE. Vascular anomalies. Curr Probl Surg. 2000 Aug. 37(8):517-84. [Medline].

  2. Vaišnyte B, Vajauskas D, Palionis D, Misonis N, Kurminas M, Nevidomskyte D, et al. Diagnostic Methods, Treatment Modalities, and Follow-up of Extracranial Arteriovenous Malformations. Medicina (Kaunas). 2012. 48(8):388-98. [Medline].

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

  4. Blei F. Basic science and clinical aspects of vascular anomalies. Curr Opin Pediatr. 2005 Aug. 17(4):501-9. [Medline].

  5. Enjolras O, Wassef M, Chapot R. Color Atlas of Vascular Tumors and Vascular Malformations. New York: Cambridge University Press; 2007.

  6. Chang MW. Updated classification of hemangiomas and other vascular anomalies. Lymphat Res Biol. 2003. 1(4):259-65. [Medline].

  7. Al-Adnani M, Williams S, Rampling D, Ashworth M, Malone M, Sebire NJ. Histopathological reporting of paediatric cutaneous vascular anomalies in relation to proposed multidisciplinary classification system. J Clin Pathol. 2006 Dec. 59(12):1278-82. [Medline].

  8. Marler JJ, Mulliken JB. Current management of hemangiomas and vascular malformations. Clin Plast Surg. 2005 Jan. 32(1):99-116, ix. [Medline].

  9. Chiller KG, Frieden IJ, Arbiser JL. Molecular pathogenesis of vascular anomalies: classification into three categories based upon clinical and biochemical characteristics. Lymphat Res Biol. 2003. 1(4):267-81. [Medline].

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

  11. Kohout MP, Hansen M, Pribaz JJ, Mulliken JB. Arteriovenous malformations of the head and neck: natural history and management. Plast Reconstr Surg. 1998 Sep. 102(3):643-54. [Medline].

  12. Tasnadi G. Epidemiology and etiology of congenital vascular malformations. Semin Vasc Surg. 1993 Dec. 6(4):200-3. [Medline].

  13. Enjolras O, Logeart I, Gelbert F, et al. [Arteriovenous malformations: a study of 200 cases]. Ann Dermatol Venereol. 2000 Jan. 127(1):17-22. [Medline].

  14. Khong PL, Burrows PE, Kozakewich HP, Mulliken JB. Fast-flow lingual vascular anomalies in the young patient: is imaging diagnostic?. Pediatr Radiol. 2003 Feb. 33(2):118-22. [Medline].

  15. Zhang L, Lin X, Wang W, et al. Circulating level of vascular endothelial growth factor in differentiating hemangioma from vascular malformation patients. Plast Reconstr Surg. 2005 Jul. 116(1):200-4. [Medline].

  16. Srinivasan VM, Schafer S, Ghali MG, Arthur A, Duckworth EA. Cone-beam CT angiography (Dyna CT) for intraoperative localization of cerebral arteriovenous malformations. J Neurointerv Surg. 2014 Dec 5. [Medline].

  17. Li ZF, Hong B, Xv Y, Huang QH, Zhao WY, Liu JM. Using DynaCT rotational angiography for angioarchitecture evaluation and complication detection in spinal vascular diseases. Clin Neurol Neurosurg. 2014 Nov 10. 128C:56-59. [Medline].

  18. Burrows PE, Laor T, Paltiel H, Robertson RL. Diagnostic imaging in the evaluation of vascular birthmarks. Dermatol Clin. 1998 Jul. 16(3):455-88. [Medline].

  19. Robertson RL, Robson CD, Barnes PD, Burrows PE. Head and neck vascular anomalies of childhood. Neuroimaging Clin N Am. 1999 Feb. 9(1):115-32. [Medline].

  20. Lee BB, Do YS, Yakes W, Kim DI, Mattassi R, Hyon WS. Management of arteriovenous malformations: a multidisciplinary approach. J Vasc Surg. 2004 Mar. 39(3):590-600. [Medline].

  21. Oermann EK, Murthy N, Chen V, Baimeedi A, Sasaki-Adams D, McGrail K, et al. A Multicenter Retrospective Study of Frameless Robotic Radiosurgery for Intracranial Arteriovenous Malformation. Front Oncol. 2014. 4:298. [Medline]. [Full Text].

  22. 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. 2003 Dec. 73(6):1240-9. [Medline]. [Full Text].

  23. Schirmer CM, Hwang SW, Riesenburger RI, Choi IS, David CA. Obliteration of a metameric spinal arteriovenous malformation (Cobb syndrome) using combined endovascular embolization and surgical excision. J Neurosurg Pediatr. 2012 Jul. 10(1):44-9. [Medline].

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

  25. 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. 1999 Aug. 8(8):1461-72. [Medline].

  26. 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. 1998 Mar. 7(3):507-15. [Medline].

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

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

  29. Tan WH, Baris HN, Burrows PE, Robson CD, Alomari AI, Mulliken JB. The spectrum of vascular anomalies in patients with PTEN mutations: implications for diagnosis and management. J Med Genet. 2007 Sep. 44(9):594-602. [Medline].

  30. Turnbull MM, Humeniuk V, Stein B, Suthers GK. Arteriovenous malformations in Cowden syndrome. J Med Genet. 2005 Aug. 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 - Quiescence Pink-bluish stain, warmth, and arteriovenous shunting are revealed by Doppler scanning. The arteriovenous malformation mimics a capillary malformation or involuting hemangioma.
II - Expansion The description is the same as stage I, plus enlargement, pulsations, thrill, and bruit and tortuous/tense veins.
III - Destruction The description is the same as stage II, plus dystrophic skin changes, ulceration, bleeding, persistent pain, or tissue necrosis. Bony lytic lesions may occur.
IV - Decompensation The 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.[20]
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