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Vascular Surgery for Arteriovenous Malformations Workup

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

Imaging Studies

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

Plain radiography

Bony AVMs may demonstrate osteolysis.[5, 13]

Ultrasonography

Ultrasonography 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. AV 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.[5]

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[5] .

CT angiography (CTA; see the image below) provides three-dimensional (3D) reconstruction of the AVM. Cone-beam CTA (also referred to as rotational angiography) appears to be promising in the treatment of AVMs, both for facilitating preoperative surgical planning and for providing an intraoperative reference.[16, 17]

CT Angiogram of a pulmonary arteriovenous malforma CT Angiogram of a pulmonary arteriovenous malformation (AVM).

Magnetic resonance imaging

MRI of an AVM 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). (See the image below.) No contrast parenchymal enhancement (no tumor aspect) exists. If signal abnormalities are present, they may exist in relation to a fibrofatty matrix.[18, 19] Magnetic resonance angiography (MRA) also provides a 3D reconstruction of the AVM and its anomalous vascular network.[5]

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

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.[8]

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

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.[5, 1, 8]

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. 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.[8]

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.[1] Although preoperative embolization or sclerotherapy may minimize intraoperative bleeding, these techniques do not reduce the limits of resection.

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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.[3]

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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.[15] In addition, urinary high-molecular-weight (hMW) matrix metalloproteinases (MMPs) are elevated in vascular tumors and some vascular malformations; however, they can not distinguish between the two types of vascular lesions.[10]

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 (T+S). This will allow a rapid diagnosis of anemia, coagulopathy, or DIC, if present, and will allow resuscitation with the appropriate intravenous fluids or blood products.

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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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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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