Vascular Surgery for Arteriovenous Malformations Differential Diagnoses

Sections

Vascular Surgery for Arteriovenous Malformations

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

DDx

Differential Diagnoses

Arterial Vascular Malformations Including Hemangiomas and Lymphangiomas
Arteriovenous Fistulas
Capillary Hemangioma
Complex-combined vascular malformation (eg, CM-AVM)
Congenital hemangioma (RICH or NICH)
Hepatic Hemangiomas
Infantile Hemangioma
Orthopedic Surgery for Hemangioma
Plastic Surgery for Capillary Malformations
Plastic Surgery for Hemangioma
Surgery for Congenital Arterial, Venous, and Lymphatic Anomalies

Workup

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.
Marler JJ, Mulliken JB. Current management of hemangiomas and vascular malformations. Clin Plast Surg. 2005 Jan. 32(1):99-116, ix. [Medline].
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, 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, 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. 2014 Dec 5. [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].
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].

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).

of 6

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 (eg, pyogenic granuloma, targetoid hemangioma, glomeruloid hemangioma, microvenular hemangioma)	Slow-flow vascular malformations 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 vascular malformations 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. ISSVA 2014 Classification of Vascular Anomalies

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 Others	Simple vascular malformations Capillary malformation (CM) Cutaneous/mucosal CM (port-wine stain) Telangiectasia Cutis marmorata telangiectatica congenita (CMTC) Nevus simplex/Salmon patch Others Lymphatic malformation (LM) Common/cystic LM Generalized lymphatic anomaly (GLA) LM in Gorham-Stout disease Channel-type LM 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) Others Arteriovenous malformation (AVM) Sporadic In hereditary hemorrhagic telangiectasia (HHT) In CM-AVM Others Arteriovenous fistula (AVF) Sporadic In HHT In CM-AVM Others
Locally aggressive or borderline vascular tumors Kaposiform hemangioendothelioma Retiform hemangioendothelioma Papillary intralymphatic angioendothelioma, Dabska tumor Composite hemangioendothelioma Kaposi sarcoma Others	Combined vascular malformations CM + VM = CVM CM + LM = CLM CM + AVM = CAVM LM + VM = LVM CM + LM + VM = CLVM 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 e,bryonal vessel)
	Vascular malformations associated with other anomalies Klippel-Trenaunay syndrome Parkes Weber syndrome Servelle-Martorell syndrome Sturge-Weber syndrome Limb CM + congenital nonprogressive limb hypertrophy Maffucci syndrome Macrocephaly-CM Microcephaly-CM CLOVES syndrome Proteus syndrome Bannayan-Riley-Ruvalcaba syndrome
C=capillary, V=venous, L=lymphatic, A=arterial, M=malformation, F=fistula.

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

Others

Simple vascular malformations

Capillary malformation (CM)

Cutaneous/mucosal CM (port-wine stain)
Telangiectasia
Cutis marmorata telangiectatica congenita (CMTC)
Nevus simplex/Salmon patch
Others

Lymphatic malformation (LM)

Common/cystic LM
Generalized lymphatic anomaly (GLA)
LM in Gorham-Stout disease
Channel-type LM
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)
Others

Arteriovenous malformation (AVM)

Sporadic
In hereditary hemorrhagic telangiectasia (HHT)
In CM-AVM
Others

Arteriovenous fistula (AVF)

Sporadic
In HHT
In CM-AVM
Others

Locally aggressive or borderline vascular tumors

Kaposiform hemangioendothelioma

Retiform hemangioendothelioma

Papillary intralymphatic angioendothelioma, Dabska tumor

Composite hemangioendothelioma

Kaposi sarcoma

Others

Combined vascular malformations

CM + VM = CVM

CM + LM = CLM

CM + AVM = CAVM

LM + VM = LVM

CM + LM + VM = CLVM

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 e,bryonal vessel)

Vascular malformations associated with other anomalies

Klippel-Trenaunay syndrome

Parkes Weber syndrome

Servelle-Martorell syndrome

Sturge-Weber syndrome

Limb CM + congenital nonprogressive limb hypertrophy

Maffucci syndrome

Macrocephaly-CM

Microcephaly-CM

CLOVES syndrome

Proteus syndrome

Bannayan-Riley-Ruvalcaba syndrome

C=capillary, V=venous, L=lymphatic, A=arterial, M=malformation, F=fistula.

Table 3. 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 4. 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.^[21]

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.^[21]

Back to List

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.

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.

Chief Editor

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

Vascular Surgery for Arteriovenous Malformations Differential Diagnoses

Differential Diagnoses

About

Membership

Apps

WebMD Network

Editions

What to Read Next on Medscape

Vascular Surgery for Arteriovenous Malformations Differential Diagnoses

Differential Diagnoses

References

Tables

Contributor Information and Disclosures

What would you like to print?

Find Us On

About

Membership

Apps

WebMD Network

Editions

What to Read Next on Medscape

Related Conditions and Diseases

Medscape Consult

News & Perspective

Tools

Most Popular Articles

Recommended