Vascular Surgery for Arteriovenous Malformations Treatment & Management
- Author: Allison Leigh Speer, MD; Chief Editor: Vincent Lopez Rowe, MD more...
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]
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.
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.
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|
|Table modified from Lee et al.|
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.)
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. 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.
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
Diet and Activity
No special diet is required or recommended.
Activity is not limited unless the patient is undergoing an interventional or surgical procedure.
Complications that may occur after embolization/sclerotherapy or surgical excision include the following:
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.
Mulliken JB, Fishman SJ, Burrows PE. Vascular anomalies. Curr Probl Surg. 2000 Aug. 37(8):517-84. [Medline].
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].
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].
Blei F. Basic science and clinical aspects of vascular anomalies. Curr Opin Pediatr. 2005 Aug. 17(4):501-9. [Medline].
Enjolras O, Wassef M, Chapot R. Color Atlas of Vascular Tumors and Vascular Malformations. New York: Cambridge University Press; 2007.
Chang MW. Updated classification of hemangiomas and other vascular anomalies. Lymphat Res Biol. 2003. 1(4):259-65. [Medline].
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].
Marler JJ, Mulliken JB. Current management of hemangiomas and vascular malformations. Clin Plast Surg. 2005 Jan. 32(1):99-116, ix. [Medline].
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].
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].
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].
Tasnadi G. Epidemiology and etiology of congenital vascular malformations. Semin Vasc Surg. 1993 Dec. 6(4):200-3. [Medline].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
Calva D, Howe JR. Hamartomatous polyposis syndromes. Surg Clin North Am. 2008 Aug. 88(4):779-817, vii. [Medline]. [Full Text].
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].
Turnbull MM, Humeniuk V, Stein B, Suthers GK. Arteriovenous malformations in Cowden syndrome. J Med Genet. 2005 Aug. 42(8):e50. [Medline]. [Full Text].
|Vascular Tumors||Vascular Malformations|
|Complex-combined vascular malformations
|C=capillary, V=venous, L=lymphatic, A=arterial, M=malformation, F=fistula|
|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.|
|Absolute Indications||Relative Indications|
|Table modified from Lee et al.|