Vascular Surgery for Arteriovenous Malformations

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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]

Classification of vascular anomalies is based on the landmark 1982 paper by Mulliken and Glowacki, which proposed a simplified categorization of these anomalies that was based on biologic activity. [3] As a result, the following two main types of vascular anomalies are generally recognized:

Differentiating between these two types of anomalies 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.

During its 1996 workshop, the International Society for the Study of Vascular Anomalies (ISSVA) adopted a classification system designed to provide a common language for guiding treatment. It was based on Mulliken and Glowacki’s biologic study but also further distinguished vascular malformations according to hemodynamics and predominant anomalous channels. [4, 5, 6, 7]  At its 20th workshop in 2014, the ISSVA approved a revised classification, [8]  which was further revised at the ISSVA General Assembly in May 2018 (see Table 1 below). [9]

Table 1. ISSVA Classification of Vascular Anomalies (May 2018 Revision) (Open Table in a new window)

Benign vascular tumors

Infantile hemangioma/hemangioma of infancy

Congenital hemangioma

Tufted angioma

Spindle-cell angioma

Epithelioid angioma

Pyogenic granuloma (lobular capillary hemangioma)

Others

Related lesions

Simple vascular malformations

Capillary malformation (CM)

Lymphatic malformation (LM)

Venous malformation (VM)

Arteriovenous malformation (AVM)

Arteriovenous fistula (AVF) (congenital)

Locally aggressive or borderline vascular tumors

Kaposiform hemangioendothelioma

Retiform hemangioendothelioma

Papillary intralymphatic angioendothelioma (PILA), Dabska tumor

Composite hemangioendothelioma

Pseudomyogenic hemangioendothelioma

Polymorphous hemangioendothelioma

Hemangioendothelioma not otherwise specified

Kaposi sarcoma

Others

Combined vascular malformations

CM + VM = CVM

CM + LM = CLM

CM + AVM = CAVM

LM + VM = LVM

CM + LM + VM = CLVM

CM + LM + AVM = CLAVM

CM + VM + AVM = CVAVM

CM + LM + VM + AVM = CLVAVM

Malignant vascular tumors

Angiosarcoma

Epithelioid hemangioendothelioma

Others

Anomalies of major named vessels (channel-type or truncal vascular malformations)

Affecting

Anomalies of

Vascular malformations associated with other anomalies

Klippel-Trenaunay syndrome

Parkes Weber syndrome

Servelle-Martorell syndrome

Sturge-Weber syndrome

Limb CM + congenital nonprogressive limb overgrowth

Maffucci syndrome

Macrocephaly-CM

Microcephaly-CM

CLOVES syndrome

Proteus syndrome

Bannayan-Riley-Ruvalcaba syndrome

CLAPO syndrome

A few vascular anomalies remain provisionally unclassified. [9]  Additional information is available on the ISSVA Web site.

The pathogenesis of AVMs is not well understood [10, 11] but is thought to involve 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, whereas vascular malformations are due to errors in vessel remodeling. [12] 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 malformations [LMs], lymphaticovenous malformations [LVMs], and AVMs) and that the urinary increase in these proteins parallels the tissue remodeling seen in diffuse and expanding vascular malformations. [13] 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 offer 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). [14]

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.

Although vascular anomalies are among the most common pediatric abnormalities, occurring in approximately 1% of children, [15] AVMs are rare. [5]

Most AVMs are evident at birth (40% in a study of 200 AVMs by Enjolras et al), [16] 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 predominantly 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, 16]

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

The prognosis is excellent when AVMs are managed by an interdisciplinary team, and the best success is achieved in surgically accessible lesions treated with combined embolization and complete surgical resection. (See the image below.)

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].

ISSVA classification of vascular anomalies. International Society for the Study of Vascular Anomalies. Available at https://issva.clubexpress.com/docs.ashx?id=178348. April 2014; Accessed: August 26, 2016.

ISSVA classification for vascular anomalies. International Society for the Study of Vascular Anomalies. Available at http://www.issva.org/UserFiles/file/ISSVA-Classification-2018.pdf. May 2018; Accessed: January 18, 2019.

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

Zyck S, Sampath R. Arteriovenous Malformations. Treasure Island, FL: StatPearls; 2018. [Full Text].

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, Fang J, Upton J, Mulliken JB, 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, Zhuang X, Dong J, Qi Z, 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. 2016 Jan. 8 (1):69-74. [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].

Madsen HJ, Annam A, Harned R, Nakano TA, Larroque LO, Kulungowski AM. Symptom Resolution and Volumetric Reduction of Abdominal Lymphatic Malformations With Sclerotherapy. J Surg Res. 2019 Jan. 233:256-261. [Medline].

Bailey CR, Arun A, Towsley M, Choi WK, Betz JF, MacKenzie S, et al. MVP™ Micro Vascular Plug Systems for the Treatment of Pulmonary Arteriovenous Malformations. Cardiovasc Intervent Radiol. 2018 Nov 14. [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].

Spetzler RF, Martin NA. A proposed grading system for arteriovenous malformations. J Neurosurg. 1986 Oct. 65 (4):476-83. [Medline].

Lawton MT, Kim H, McCulloch CE, Mikhak B, Young WL. A supplementary grading scale for selecting patients with brain arteriovenous malformations for surgery. Neurosurgery. 2010 Apr. 66 (4):702-13; discussion 713. [Medline]. [Full Text].

Spetzler RF, Ponce FA. A 3-tier classification of cerebral arteriovenous malformations. Clinical article. J Neurosurg. 2011 Mar. 114 (3):842-9. [Medline].

Benign vascular tumors

Infantile hemangioma/hemangioma of infancy

Congenital hemangioma

Tufted angioma

Spindle-cell angioma

Epithelioid angioma

Pyogenic granuloma (lobular capillary hemangioma)

Others

Related lesions

Simple vascular malformations

Capillary malformation (CM)

Lymphatic malformation (LM)

Venous malformation (VM)

Arteriovenous malformation (AVM)

Arteriovenous fistula (AVF) (congenital)

Locally aggressive or borderline vascular tumors

Kaposiform hemangioendothelioma

Retiform hemangioendothelioma

Papillary intralymphatic angioendothelioma (PILA), Dabska tumor

Composite hemangioendothelioma

Pseudomyogenic hemangioendothelioma

Polymorphous hemangioendothelioma

Hemangioendothelioma not otherwise specified

Kaposi sarcoma

Others

Combined vascular malformations

CM + VM = CVM

CM + LM = CLM

CM + AVM = CAVM

LM + VM = LVM

CM + LM + VM = CLVM

CM + LM + AVM = CLAVM

CM + VM + AVM = CVAVM

CM + LM + VM + AVM = CLVAVM

Malignant vascular tumors

Angiosarcoma

Epithelioid hemangioendothelioma

Others

Anomalies of major named vessels (channel-type or truncal vascular malformations)

Affecting

Anomalies of

Vascular malformations associated with other anomalies

Klippel-Trenaunay syndrome

Parkes Weber syndrome

Servelle-Martorell syndrome

Sturge-Weber syndrome

Limb CM + congenital nonprogressive limb overgrowth

Maffucci syndrome

Macrocephaly-CM

Microcephaly-CM

CLOVES syndrome

Proteus syndrome

Bannayan-Riley-Ruvalcaba syndrome

CLAPO syndrome

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.

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. [25]

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.

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.

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.

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 Surgical Association, Pacific Coast Surgical Association, Society for Clinical Vascular Surgery, Society for Vascular Surgery, Western Vascular Society

Disclosure: Nothing to disclose.

Vascular Surgery for Arteriovenous Malformations

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