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Vascular Surgery for Arteriovenous Malformations

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

Background

Vascular anomalies are among the most common congenital abnormalities in infants and children. Historically, their treatment has been impeded by confusing terminology and imprecise classification. Lesions were named with descriptive terms (eg, strawberry hemangioma or port-wine stain) or histopathologic terms (eg, capillary hemangioma, cavernous hemangioma, or lymphangioma). Although some vascular anomalies may appear similar, their biologic behavior differs markedly; therefore, treatment should be based on a proper classification system.[1, 2]

The current classification system is based on the landmark 1982 paper by Mulliken and Glowacki, which proposed a simplified categorization of vascular anomalies based on biologic activity.[3] As a result, we now recognize the following two main types of vascular anomalies:

  • Vascular tumors
  • Vascular malformations

Differentiating between these two types is essential because their treatment is quite different. The management of vascular anomalies is a dynamic and rapidly developing subspecialty, which requires interdisciplinary care and collaboration.

The International Society for the Study of Vascular Anomalies (ISSVA) was started in 1992 after a series of biennial international workshops begun in 1976 by Drs John Mulliken and Anthony Young.[4] The primary goal of the ISSVA is to improve the understanding and management of vascular anomalies by promoting interdisciplinary and international collaboration.

The classification system adopted by the ISSVA during its 1996 workshop provides a common language and guides treatment. It is based on Mulliken and Glowacki’s biologic study but also further distinguishes vascular malformations according to hemodynamics and predominant anomalous channels (see Table 1 below).[4, 5, 6, 7]

Table 1: ISSVA 1996 Classification of Vascular Anomalies (Open Table in a new window)

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

 

The goal of this article is to review one type of fast-flow vascular malformation in particular: arteriovenous malformations (AVMs).

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Pathophysiology

The pathogenesis of AVMs is not well understood[8] but is thought to result from abnormal vasculogenesis. Multiple biologic studies since 1982 have demonstrated clear differences between vascular tumors (hemangiomas) and vascular malformations. Some have hypothesized that infantile hemangiomas result from excess angiogenesis, while vascular malformations are due to errors in vessel remodeling.[9] Although some vascular malformations thicken, expand, or multiply with time, whether true angiogenesis occurs is unclear.

Marler et al suggested that vascular malformations may be angiogenesis-dependent disorders. They found that urinary high-molecular-weight matrix metalloproteinases (hMW MMPs) and basic fibroblast growth factor (bFGF) levels are elevated in vascular tumors and some vascular malformations (eg, lymphatic malformation [LM], lymphaticovenous malformation [LVM], and AVMs) and that the urinary increase in these proteins parallels the tissue remodeling seen in diffuse and expanding vascular malformations.[10] They suggested that drugs targeting bFGF or MMPs may be an adequate therapeutic strategy for patients suffering from these vascular anomalies.

Inherited forms of vascular malformations are rare[5] but may allow insight into the molecular mechanisms and signaling pathways involved in the pathogenesis of these lesions. This may in turn identify potential novel therapeutic targets, though at present, it is unclear whether the more common sporadic vascular malformations share similar biologic mechanisms with the infrequent inherited vascular malformations.

An AVM is a hemodynamically active, fast-flow vascular malformation. Arterial feeders and enlarged draining veins directly connect through micro- and macroarteriovenous fistulas that create the nidus or epicenter of the AVM. AVMs may occur both superficially and viscerally. They are usually present at birth and rarely regress.[5] They have a normal endothelial cell cycle and grow commensurately with the child.[3, 1]

The natural history of AVMs is organized into a clinical staging system proposed by Schobinger at the 1990 ISSVA meeting in Amsterdam (see Table 2 below).[11]

Table 2: Schobinger Staging for AVMs (Open Table in a new window)

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.
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Epidemiology

Although vascular anomalies are among the most common pediatric abnormalities, occurring in approximately 1% of children,[12] AVMs are rare.[5]

Most AVMs are evident at birth (40% in a study of 200 AVMs by Enjolras et al),[13] though they may not be clinically relevant. Mulliken and Glowacki noted that 90% of vascular malformations were present at birth in a series of 23 patients; however, these lesions were predominately venous in type and may not be representative of AVMs per se.[3]

The female-to-male ratio for vascular malformations is 1:1.[3, 13]

Mortality/Morbidity

AVMs never regress and usually follow the stages outlined by Schobinger. Morbidity and mortality are dependent on several factors, as follows:

  • Location and size of the AVM
  • Whether the AVM is surgically accessible or amenable to palliative embolization/sclerotherapy
  • Presence or absence of congestive heart failure
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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
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  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].

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  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].

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  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].

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  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].

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  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].

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  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].

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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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