Sections

Vascular Surgery for Arteriovenous Malformations

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

Workup

Laboratory Studies

No laboratory tests reliably diagnose arteriovenous malformations (AVMs). Serum levels of vascular endothelial growth factor (VEGF) are significantly higher in proliferating hemangiomas than in involuting hemangiomas and vascular malformations.^[20]In addition, urinary high-molecular-weight (hMW) matrix metalloproteinases (MMPs) are elevated in vascular tumors and some vascular malformations; however, they cannot distinguish between the two types of vascular lesions.^[13]

If a patient has spontaneous bleeding, perform a complete blood count (CBC), coagulation studies such as prothrombin time (PT) and partial thromboplastin time (PTT), a disseminated intravascular coagulation (DIC) panel, and a type and screen. This will allow a rapid diagnosis of anemia, coagulopathy, or DIC, if present, and will allow resuscitation with the appropriate intravenous (IV) fluids or blood products.

Imaging Studies

Clinical diagnosis is confirmed by means of ultrasonography (US) with color Doppler examination. Magnetic resonance imaging (MRI) is best for evaluating the extent of the AVM.

Plain radiography

Bony AVMs may demonstrate osteolysis.^{[6, 18]}

Ultrasonography

US with color Doppler of an AVM usually demonstrates low-resistance high-velocity arterial flow above the baseline, with high diastolic flux, and pulsatile venous flow below the baseline. Vessels are tortuous. Arteriovenous shunting is seen. Pulsed Doppler measures the arterial output on the affected side compared with the normal side (eg, carotid, humeral, femoral arteries). This noninvasive technique is an excellent and reliable way to follow the course of an AVM or to monitor response to treatment.^[6]

Computed tomography

Computed tomography (CT) does not easily distinguish between hemangiomas and vascular malformations. CT with iodinated contrast identifies AVMs as a highly enhancing lesion and can demonstrate soft-tissue involvement, as well as dilated feeding and draining vessels^[6].

CT angiography (CTA; see the image below) provides three-dimensional (3D) reconstruction of the AVM. Cone-beam CTA (also referred to as rotational angiography) has shown promise in the treatment of AVMs, both for facilitating preoperative surgical planning and for providing an intraoperative reference.^{[21, 22]}

CT Angiogram of a pulmonary arteriovenous malformation (AVM).

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Magnetic resonance imaging

MRI of an AVM (see the image below) demonstrates a collection of vascular flow voids (black tubular structures) corresponding to fast-flow vessels, in all sequences (spin-echo T1- and T2-weighted sequences). No contrast parenchymal enhancement (no tumor aspect) exists. If signal abnormalities are present, they may exist in relation to a fibrofatty matrix.^{[23, 24]}Magnetic resonance angiography (MRA) also provides a 3D reconstruction of the AVM and its anomalous vascular network.^[6]

MRI of a rectal arteriovenous malformation (AVM). Panel A: Axial, intraperitoneal rectum. Panel B: Axial, extraperitoneal rectum. Panel C: Coronal, posterior to lumbosacral prominence.

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Angiography

Angiography (see the image below) is not solely diagnostic but can be therapeutic with embolization. Angiography demonstrates variably dilated or tortuous feeding arteries, arterial venous shunting (occasionally with visualization of discrete fistulae), and dilated draining veins. Feeding arteries may be aneurysmal in older patients.^[11]

Angiogram of a rectal arteriovenous malformation (AVM). Panel A: arterial phase. Panel B: venous phase.

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Proximal embolization of feeding vessels is contraindicated and should never be performed. After a period of improvement, a vascular recruitment phenomenon occurs with rapid recruitment of flow from nearby arteries, which allows new collaterals to supply the nidus. This allows the lesions to recur and progress. In addition, proximal arterial embolization denies access for subsequent embolization.^{[6, 2, 11]}

Angiography typically precedes interventional therapy or surgical resection. Patients with AVMs that are unresectable may undergo palliative superselective arterial or retrograde venous embolization for control of pain, hemorrhage, or congestive heart failure (CHF). Typically, palliative embolization provides only transient improvement. Resectable AVMs may be surgically removed 24-72 hours after arterial embolization for temporary nidus occlusion. Embolization can be with coils or glue.^[11]

Sclerotherapy is another radiologic option that uses angiography and involves the injection of ethanol into the nidus. The risk of soft-tissue and neurologic damage is high; therefore, this technique should be performed only by an experienced endovascular specialist in carefully selected patients.^[2]Although preoperative embolization or sclerotherapy may minimize intraoperative bleeding, these techniques do not reduce the limits of resection.

Histologic Findings

Vascular malformations are composed of vascular channels lined by flat “mature” epithelium and are not hypercellular, in contrast to hemangiomas. The endothelium is not proliferative. AVMs have predominately arterial and venous anomalous channels.^[4]

Treatment & Management

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Jia H, Chen Y, Yang X, Lee Y, Zou Y, Zhou J, et al. Treatment of Challenging Extracranial Arteriovenous Malformations: A Single-Center Experience and Literature Review. Ann Plast Surg. 2023 Jan 18. [QxMD MEDLINE Link].
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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].
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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].
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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. 2021 Apr. 22 (4):568-576. [QxMD MEDLINE Link].
Li XY, Wang DM, Wen MZ, Zheng LZ, Wang ZF, Ren-Cai, et al. Ethanol Embolization of Chest Wall Arteriovenous Malformations: Four-Year Findings. J Endovasc Ther. 2023 Jan 21. 15266028221149908. [QxMD MEDLINE Link].
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Lee SY, Kang DH, Kim YS, Lee J, Lee SJ, Huh S, et al. Efficacy and safety of transarterial bleomycin sclerotherapy of early-stage facial arteriovenous malformation: Single-center multidisciplinary team experience. J Plast Reconstr Aesthet Surg. 2023 Feb. 77:379-387. [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].
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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.^[29]

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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, FACS Professor and Chief, Division of Vascular and Endovascular Surgery, Gonda (Goldschmied) Vascular Center, University of California, Los Angeles, David Geffen School of Medicine

Vincent Lopez Rowe, MD, FACS is a member of the following medical societies: American College of Surgeons, American Heart Association, American Surgical Association, Association of Program Directors in Vascular Surgery, Los Angeles Surgical Society, Pacific Coast Surgical Association, Society for Clinical Vascular Surgery, Society for Vascular Surgery, Society of Black Academic Surgeons, Society of Black Vascular Surgeons, Southern California Vascular Surgical Society, Western Vascular Society

Disclosure: Nothing to disclose.