Sections

Vascular Surgery for Arteriovenous Malformations

Sections Vascular Surgery for Arteriovenous Malformations
Overview
Presentation
DDx
Workup
Treatment
Media Gallery
Tables
References

Presentation

History

Arteriovenous malformations (AVMs) are commonly misdiagnosed in infancy and childhood as an involuting hemangioma or capillary malformation because the lesion is not yet fast-flow, warm, or pulsatile.^[11]They may become more clinically evident during the second or third decade of life.^{[18, 14]}Enjolras et al observed a progression during childhood to grade II in 84% of patients.^[17]Puberty (32%), pregnancy (25% adult women), or trauma (20%) can trigger expansion.^{[6, 17, 2, 11]}These lesions grow proportionately with the child and never regress.

Physical Examination

The head and neck area is the most common location (70%) for AVMs, with a higher incidence of intracranial lesions than of extracranial lesions. Next in frequency are AVMs of the extremity, trunk, and viscera.^{[6, 2, 11]}

When fully developed, AVMs deepen in color with increased erythema, and local warmth, a palpable thrill, and a bruit. Patients with facial AVMs of the skin or facial bones may present with facial asymmetry, gingival hypertrophy, unstable teeth, periodontal bleeding, or skin or mucosal ulcers with secondary infection. Nasal AVMs may cause epistaxis. Bony AVMs create osteolysis (3/200 in the series by Enjolras et al).^[17]

Lower-limb skin changes resembling curious dry, brown-violaceous plaques may appear and are known histologically as pseudo-Kaposi sarcomas.^{[2, 11]}Distal-extremity AVMs may lead to ischemia of the tips of fingers or toes associated with arterial steal and venous hypertension. (See the image below.)

Ischemic contractures secondary to a right hand arteriovenous malformation (AVM). Panel A: ventral. Panel B: dorsal. Panel C: excellent outcome after surgical resection/amputation.

View Media Gallery

Later consequences of expanding AVMs with arteriovenous shunting include ischemic changes, indolent ulceration, intractable pain, and sudden life-threatening hemorrhage or recurrent intermittent bleeding.^[11]Increased cardiac output with subsequent congestive heart failure occurs in fewer than 2% of cases (5/200 in the series by Enjolras et al)^[6]and usually in newborns with a massive AVM or in young adults with a large rapidly worsening AVMs in the limb or trunk.^{[2, 11]}

Complications

Complications that may be apparent before embolization/sclerotherapy or surgical excision include the following:

Dystrophic skin changes
Ulceration
Tissue necrosis
Hemorrhage
Intractable pain
Congestive heart failure

Differential Diagnoses

Zyck S, Sampath R. Arteriovenous Malformations. Treasure Island, FL: StatPearls; 2020. [Full Text].
Mulliken JB, Fishman SJ, Burrows PE. Vascular anomalies. Curr Probl Surg. 2000 Aug. 37 (8):517-84. [QxMD MEDLINE Link].
Vaišnytė B, Vajauskas D, Palionis D, Misonis N, Kurminas M, Nevidomskytė D, et al. Diagnostic methods, treatment modalities, and follow-up of extracranial arteriovenous malformations. Medicina (Kaunas). 2012. 48 (8):388-98. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Blei F. Basic science and clinical aspects of vascular anomalies. Curr Opin Pediatr. 2005 Aug. 17 (4):501-9. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Dasgupta R, Fishman SJ. ISSVA classification. Semin Pediatr Surg. 2014 Aug. 23 (4):158-61. [QxMD MEDLINE Link]. [Full Text].
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: March 2, 2021.
Marler JJ, Mulliken JB. Current management of hemangiomas and vascular malformations. Clin Plast Surg. 2005 Jan. 32 (1):99-116, ix. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Florian IA, Beni L, Moisoiu V, Timis TL, Florian TS, Balașa A, et al. 'De Novo' Brain AVMs-Hypotheses for Development and a Systematic Review of Reported Cases. Medicina (Kaunas). 2021 Feb 26. 57 (3):201. [QxMD MEDLINE Link].
Tasnádi G. Epidemiology and etiology of congenital vascular malformations. Semin Vasc Surg. 1993 Dec. 6 (4):200-3. [QxMD MEDLINE Link].
Enjolras O, Logeart I, Gelbert F, Lemarchand-Venencie F, Reizine D, Guichard JP, et al. [Arteriovenous malformations: a study of 200 cases]. Ann Dermatol Venereol. 2000 Jan. 127 (1):17-22. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. 2015 Jan. 128:56-9. [QxMD MEDLINE Link].
Burrows PE, Laor T, Paltiel H, Robertson RL. Diagnostic imaging in the evaluation of vascular birthmarks. Dermatol Clin. 1998 Jul. 16 (3):455-88. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. 2019 Mar. 42 (3):389-395. [QxMD MEDLINE Link].
Kim R, Do YS, Park KB. How to Treat Peripheral Arteriovenous Malformations. Korean J Radiol. 2020 Dec 21. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link]. [Full Text].
Spetzler RF, Martin NA. A proposed grading system for arteriovenous malformations. J Neurosurg. 1986 Oct. 65 (4):476-83. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link]. [Full Text].
Spetzler RF, Ponce FA. A 3-tier classification of cerebral arteriovenous malformations. Clinical article. J Neurosurg. 2011 Mar. 114 (3):842-9. [QxMD MEDLINE Link].
Rojas-Villabona A, Sokolska M, Solbach T, Grieve J, Rega M, Torrealdea F, et al. Planning of gamma knife radiosurgery (GKR) for brain arteriovenous malformations using triple magnetic resonance angiography (triple-MRA). Br J Neurosurg. 2021 Feb 28. 1-11. [QxMD MEDLINE Link].

Media Gallery

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).

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Table 1. ISSVA Classification of Vascular Anomalies (May 2018 Revision)

Vascular Tumors

Vascular Malformations

Benign vascular tumors

Infantile hemangioma/hemangioma of infancy

Congenital hemangioma

Rapidly involuting (RICH)
Noninvoluting (NICH)
Partially involuting (PICH)

Tufted angioma

Spindle-cell angioma

Epithelioid angioma

Pyogenic granuloma (lobular capillary hemangioma)

Others

Related lesions

Simple vascular malformations

Capillary malformation (CM)

Nevus simplex/salmon patch, "angel kiss," "stork bite"
Cutaneous/mucosal CM (port-wine stain)
Reticulate CM
CM of CM-AVM (arteriovenous malformation)
Cutis marmorata telangiectatica congenita (CMTC)
Others
Telangiectasia (hereditary hemorrhagic telangiectasia [HHT], others)

Lymphatic malformation (LM)

Common (cystic) LM (macrocystic, microcystic, mixed cystic)
Generalized lymphatic anomaly (GLA) (kaposiform lymphangiomatosis [KLA])
LM in Gorham-Stout disease
Channel-type LM
"Acquired" progressive lymphatic anomaly (acquired progressive "lymphangioma")
Primary lymphedema
Others

Venous malformation (VM)

Common VM
Familial VM cutaneomucosal (VMCM)
Blue rubber bleb nevus (Bean) syndrome VM
Glomuvenous malformation (GVM)
Cerebral cavernous malformation (CCM)
Familial intraosseous vascular malformation (VMOS)
Verrucous venous malformation (formerly verrucous hemangioma)
Others

Arteriovenous malformation (AVM)

Sporadic
In HHT
In CM-AVM
Others

Arteriovenous fistula (AVF) (congenital)

Sporadic
In HHT
In CM-AVM
Others

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

Lymphatics
Veins
Arteries

Anomalies of

Origin
Course
Number
Length
Diameter
Valves
Communication (AVF)
Persistence (of embryonal vessel)

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

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.^[27]

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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)

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.

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.

Chief Editor

Vincent Lopez Rowe, MD Professor of Surgery, Program Director, Integrated Vascular Surgery Residency and Fellowship, Department of Surgery, Division of Vascular Surgery and Endovascular Therapy, 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.