Sections

Vascular Surgery for Arteriovenous Malformations

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

DDx

Differential Diagnoses

Workup

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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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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. 2021 Apr. 22 (4):568-576. [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].
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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.