Vascular Surgery for Arteriovenous Malformations Clinical Presentation

  • Author: Allison Leigh Speer, MD; Chief Editor: Vincent Lopez Rowe, MD   more...
 
Updated: Aug 9, 2010
 

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.[7] AVMs may become more clinically evident during the second or third decade of life.[13, 10] Enjolras et al observed a progression during childhood to grade II in 84% of patients.[12] Puberty (32%), pregnancy (25% adult women), or trauma (20%) can trigger expansion.[4, 12, 1, 7] These lesions grow proportionately with the child and never regress.

Next

Physical

The head and neck is the most common location (70%) for AVMs, with a higher incidence of intracranial lesions compared to extracranial lesions. This is followed in frequency by AVMs of the extremity, trunk, and viscera.[4, 1, 7] 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 and/or facial bones may present with facial asymmetry, gingival hypertrophy, unstable teeth, periodontal bleeding, or skin/mucosal ulcers with secondary infection. Nasal AVMs may cause epistaxis. Bony AVMs create osteolysis (3/200 in the series by Enjolras et al).[12] Lower limb skin changes resembling curious dry, brown-violaceous plaques may appear and are known histologically as pseudo-Kaposi sarcomas.[1, 7] Distal extremity AVMs may lead to ischemia of the tips of fingers or toes associated with arterial steal and venous hypertension.

Later consequences of expanding AVMs with arteriovenous shunting include ischemic changes, indolent ulceration, intractable pain, and sudden life-threatening hemorrhage or recurrent, intermittent bleeding.[7] Increased cardiac output with subsequent congestive heart failure occurs in less than 2% of cases (5/200 in the series by Enjolras et al)[4] and usually in newborns with a massive AVM or in young adults with a large rapidly worsening AVMs in the limb or trunk.[1, 7]

Previous
Proceed to Differential Diagnoses
 
 
Contributor Information and Disclosures
Author

Allison Leigh Speer, MD  Research Fellow, Pediatric Surgery, Childrens Hospital Los Angeles

Allison Leigh Speer, MD is a member of the following medical societies: American College of Surgeons and Association for Academic Surgery

Disclosure: Nothing to disclose.

Coauthor(s)

Andre Panossian, MD  Assistant Professor of Surgery, Division of Plastic Surgery, University of Southern California Keck School of Medicine, Childrens Hospital Los Angeles

Andre Panossian, MD is a member of the following medical societies: American Cleft Palate/Craniofacial Association, American College of Surgeons, and 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, and 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, and Society of Cardiovascular and 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 and International Pediatric Endosurgery Group

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard M Stillman†, MD, FACS  Honorary Medical Staff, Northwest Medical Center; Former Chief of Staff and Medical Director, Wound Healing Center, Department of Surgery, Northwest Medical Center

Richard M Stillman†, MD, FACS is a member of the following medical societies: American College of Angiology, American College of Surgeons, Association for Academic Surgery, and Society of University Surgeons

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Vincent Lopez Rowe, MD  Associate Professor of Surgery, Department of Surgery, Division of Vascular Surgery, University of Southern California Medical Center

Vincent Lopez Rowe, MD is a member of the following medical societies: American College of Surgeons, American Heart Association, Pacific Coast Surgical Association, Peripheral Vascular Surgery Society, Society for Clinical Vascular Surgery, Society for Vascular Surgery, and Western Vascular Surgical Society

Disclosure: Nothing to disclose.

Paolo Zamboni, MD  Professor of Surgery, Chief of Day Surgery Unit, Chair of Vascular Diseases Center, University of Ferrara, Italy

Paolo Zamboni, MD is a member of the following medical societies: American Venous Forum and New York Academy of Sciences

Disclosure: Nothing to disclose.

Chief Editor

Vincent Lopez Rowe, MD  Associate Professor of Surgery, Department of Surgery, Division of Vascular Surgery, University of Southern California Medical Center

Vincent Lopez Rowe, MD is a member of the following medical societies: American College of Surgeons, American Heart Association, Pacific Coast Surgical Association, Peripheral Vascular Surgery Society, Society for Clinical Vascular Surgery, Society for Vascular Surgery, and Western Vascular Surgical Society

Disclosure: Nothing to disclose.

References

  1. Mulliken JB, Fishman SJ, Burrows PE. Vascular anomalies. Curr Probl Surg. Aug 2000;37(8):517-84. [Medline].

  2. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. Mar 1982;69(3):412-22. [Medline].

  3. Blei F. Basic science and clinical aspects of vascular anomalies. Curr Opin Pediatr. Aug 2005;17(4):501-9. [Medline].

  4. Enjolras O, Wassef M, Chapot R. Color Atlas of Vascular Tumors and Vascular Malformations. New York: Cambridge University Press; 2007.

  5. Chang MW. Updated classification of hemangiomas and other vascular anomalies. Lymphat Res Biol. 2003;1(4):259-65. [Medline].

  6. 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. Dec 2006;59(12):1278-82. [Medline].

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

  8. 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].

  9. Marler JJ, Fishman SJ, Kilroy SM, et al. Increased expression of urinary matrix metalloproteinases parallels the extent and activity of vascular anomalies. Pediatrics. Jul 2005;116(1):38-45. [Medline].

  10. Kohout MP, Hansen M, Pribaz JJ, Mulliken JB. Arteriovenous malformations of the head and neck: natural history and management. Plast Reconstr Surg. Sep 1998;102(3):643-54. [Medline].

  11. Tasnadi G. Epidemiology and etiology of congenital vascular malformations. Semin Vasc Surg. Dec 1993;6(4):200-3. [Medline].

  12. Enjolras O, Logeart I, Gelbert F, et al. [Arteriovenous malformations: a study of 200 cases]. Ann Dermatol Venereol. Jan 2000;127(1):17-22. [Medline].

  13. Khong PL, Burrows PE, Kozakewich HP, Mulliken JB. Fast-flow lingual vascular anomalies in the young patient: is imaging diagnostic?. Pediatr Radiol. Feb 2003;33(2):118-22. [Medline].

  14. 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. Jul 2005;116(1):200-4. [Medline].

  15. Burrows PE, Laor T, Paltiel H, Robertson RL. Diagnostic imaging in the evaluation of vascular birthmarks. Dermatol Clin. Jul 1998;16(3):455-88. [Medline].

  16. Robertson RL, Robson CD, Barnes PD, Burrows PE. Head and neck vascular anomalies of childhood. Neuroimaging Clin N Am. Feb 1999;9(1):115-32. [Medline].

  17. Lee BB, Do YS, Yakes W, Kim DI, Mattassi R, Hyon WS. Management of arteriovenous malformations: a multidisciplinary approach. J Vasc Surg. Mar 2004;39(3):590-600. [Medline].

  18. Eerola I, Boon LM, Mulliken JB, et al. Capillary malformation-arteriovenous malformation, a new clinical and genetic disorder caused by RASA1 mutations. Am J Hum Genet. Dec 2003;73(6):1240-9. [Medline]. [Full Text].

  19. Enjolras O, Chapot R, Merland JJ. Vascular anomalies and the growth of limbs: a review. J Pediatr Orthop B. Nov 2004;13(6):349-57. [Medline].

  20. Marsh DJ, Kum JB, Lunetta KL, et al. PTEN mutation spectrum and genotype-phenotype correlations in Bannayan-Riley-Ruvalcaba syndrome suggest a single entity with Cowden syndrome. Hum Mol Genet. Aug 1999;8(8):1461-72. [Medline].

  21. Marsh DJ, Coulon V, Lunetta KL, et al. Mutation spectrum and genotype-phenotype analyses in Cowden disease and Bannayan-Zonana syndrome, two hamartoma syndromes with germline PTEN mutation. Hum Mol Genet. Mar 1998;7(3):507-15. [Medline].

  22. Takaya N, Iwase T, Maehara A, et al. Transcatheter embolization of arteriovenous malformations in Cowden disease. Jpn Circ J. Apr 1999;63(4):326-9. [Medline].

  23. Calva D, Howe JR. Hamartomatous polyposis syndromes. Surg Clin North Am. Aug 2008;88(4):779-817, vii. [Medline]. [Full Text].

  24. Tan WH, Baris HN, Burrows PE, Robson CD, Alomari AI, Mulliken JB. The spectrum of vascular anomalies in patients with PTEN mutations: implications for diagnosis and management. J Med Genet. Sep 2007;44(9):594-602. [Medline].

  25. Turnbull MM, Humeniuk V, Stein B, Suthers GK. Arteriovenous malformations in Cowden syndrome. J Med Genet. Aug 2005;42(8):e50. [Medline]. [Full Text].

 
Previous
Next
 
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 - QuiescencePink-bluish stain, warmth, and arteriovenous shunting are revealed by Doppler scanning. The arteriovenous malformation mimics a capillary malformation or involuting hemangioma.
II - ExpansionThe description is the same as stage I, plus enlargement, pulsations, thrill, and bruit and tortuous/tense veins.
III - DestructionThe description is the same as stage II, plus dystrophic skin changes, ulceration, bleeding, persistent pain, or tissue necrosis. Bony lytic lesions may occur.
IV - DecompensationThe 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.[17]
Previous
Next
 
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.