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ARTERIOVENOUS MALFORMATION (AVM)

Introduction

Vascular malformations are developmental anomalies that are present at birth but may not be visually apparent.  These malformations can  be slow-flow (ie, capillary, lymphatic, venous) or fast-flow (ie, arterial). Arteriovenous malformations (AVMs) represent a combination of these elements.  Arteriovenous malformations (AVMs) occur equally in males and females.  Low flow malformation are much more common than lhigh flow malformations. 3

Incidence and Related Conditions3,3,4

  • CLOVES Syndrome - This is an acronym: (CLO)=congenital lipomatous overgrowth; (V)vascular malformation; (E) epidermal nevi; (s) scoliosis and spinal deformities. CLOVES patients have PIK3CA mutations in affected tissues.4
  • Ksaabch-Meritt Syndrome - This syndrome includes bleeding diathesis with purpura, thrombocytopenia and secondary consumptive coagulopathy.
  • Klippel-Trénaunay Syndrome - This syndrome includes skeletal hyperetrophy or hypoplasia; in cases with hypertrophy there is frequently massive extremity size and caillary, venous and/or lymphatic malformations.
  • Parkes Weber Syndrome - This syndrome includes extremity hypertrophy with lymphatic hyperplasia, lypomatosis, and arterial malformations with A-V fistulas and venous varicosities.
  • Proteus Syndrome - This syndrome includes highly variable asymmetric overgrowth with vascular malformations and abnormal adipose tissue and nevi.
  • Malfucci’s Syndrome - This syndrome includes multiple congenital enchondromas with hemangioms.

Differential Diagnosis

  • Capillary malformation
  • Hemangioma
ICD-10 Codes

  • ARTERIOVENOUS MALFORMATION (AVM)

    Diagnostic Guide Name

    ARTERIOVENOUS MALFORMATION (AVM)

    ICD 10 Diagnosis, Single Code, Left Code, Right Code and Bilateral Code

    DIAGNOSISSINGLE CODE ONLYLEFTRIGHTBILATERAL (If Available)
    ARTERIOVENOUS MALFORMATION (AVM), PERIPHERAL, UPPER LIMBQ27.31   

    ICD-10 Reference

    Reproduced from the International statistical classification of diseases and related health problems, 10th revision, Fifth edition, 2016. Geneva, World Health Organization, 2016 https://apps.who.int/iris/handle/10665/246208

Clinical Presentation Photos and Related Diagrams
A-V Malformation left hand and upper extremity
  • Arteriovenous Malformation (AVM)
    Arteriovenous Malformation (AVM)
  • Arteriovenous Malformation (AVM)
    Arteriovenous Malformation (AVM)
  • The AVM involves the entire left upper extremity. Note previous vascular surgery (1) and (2) with vascular swelling on radial wrist at (2).
    The AVM involves the entire left upper extremity. Note previous vascular surgery (1) and (2) with vascular swelling on radial wrist at (2).
Symptoms
Abnormal enlarged numerous blood vessels
Spontaneous bleeding
Pain and swelling of involved parts
Infections
Painful paresthesias caused by secondary nerve entrapments
History of recent trauma
History of puberty or pregnancy
Typical History

A typical patient is a young adult female with a vascular anomaly is her left hand present since early childhood.  After the birth of her son 18 months ago and a recent auto accident where her hand hit the steering wheel, she developed pain and swelling in her hand. The pain is associated with enlarged pulsing vessels in her hand.  The skin over these vessels feels warm to the touch.  There is a ulcer on the tip of her index finger which bleed spontaneously once and was difficult to stop.

Positive Tests, Exams or Signs
Work-up Options
Images (X-Ray, MRI, etc.)
Cervical Spine Infection Secondary To A-V Malformation Bacterema
  • Cervical fusion for multiple level cervical disk space infection secondary to bacteremia from infected A-V malformation finger ulcers.
    Cervical fusion for multiple level cervical disk space infection secondary to bacteremia from infected A-V malformation finger ulcers.
Treatment Options
Conservative
  • Compression garments for asymptomatic AVMs
Operative
  • Proximal arterial ligation has been tried but often worsens symptoms
  • Excision with revascularization
  • Embolization 4
  • Thermoablation
  • Sclerotherapy
  • Amputation
Treatment Photos and Diagrams
  • AV Malformation Amputation 1
    AV Malformation Amputation 1
  • AV Malformation Amputation 2
    AV Malformation Amputation 2
Complications
  • Chronic ulcerations
  • Uncontrolled bleeding, incomplete resection, necrosis, distal ischemia recurrence (rapid collateralization of adjacent arteries)
  • Bacterimia with distant infections
  • Osteomyelitis
  • Consumption coagulopathies
  • Congestive Heart Failure
Key Educational Points
  • Symptoms get worse with growth, puberty, pregnancy and trauma
  • Surgery is pallative and proximal ligation is frequently unsuccessful
  • High flow lesions especially in the proximal upper extremity can cause cardiac problems and coagulopathies
  • Patients with a high flow lesion may have a positive Braham-Nicoladoni Sign. A positive B-N sign occurs when a tourniquet is temporarily applied to the proximal aspect of the involved extremity and the occlusion of flowd causes a decrease in the cardiac pulse rate. 3,3
References

New Articles

  1. Bowman J, et al. Outcomes of sclerotherapy and embolization for arteriovenous and venous malformations. Semin Vasc Surg 2013;26(1):48-54. PMID: 23932562
  2. Jabłecki J, Elsaftawy A, Kaczmarzyk J, Kaczmarzyk L. Surgical treatment of hemangiomas and arteriovenous malformations in upper extremity. Pol Przegl Chir 2013;85(3):107-13. PMID: 23612616
  3. Jacobs BJ, Anzarut A, Imbriglia JE. Vascular anomalies of upper extremity. J Hand Surg Am 2010; 35A: 1703-1709. PMID: PMID: 20888510 
  4. Labow BI, Pike CM, Upton J. Overgrowth of the hand and upper extremity and associated syndromes. J Hand Surg Am 2016; 41A: 473-482. PMID: 26783065

Reviews

  1. Ek ET, Suh N, Carlson MG. Vascular anomalies of the hand and wrist. J Am Acad Orthop Surg 2014;22(6):352-60. PMID: 24860131
  2. Willard KJ, Cappel MA, Kozin SH, Abzug JM. Congenital and infantile skin lesions affecting the hand and upper extremity, part 1: vascular neoplasms and malformations. J Hand Surg Am 2013;38(11):2271-83. PMID: 2707594

Classics

  1. Griffin JM, Vasconez LO, Schatten WE. Congenital arteriovenous malformations of the upper extremity. Plast Reconstr Surg 1978;62(1):49-58. PMID: 662964
  2. Erikson U, Hemmingsson A. Congenital vascular malformations of the hand. Acta Radiol Diagn (Stockh) 1973;14(6):753-60.  PMID: 4775006
  3. Upton J, Mulliken JB, Murray JE. Classification and rationale for management of vascular anomalies in the upper extremity. J Hand Surg Am 1985; 10A(6): 970-975. PMID: 4078289
  4. Moore JR, Weiland AJ. Embolotherapy in treatment of congenital arteriovenous malformations of the hand: A case report. J Hand Surg Am 1985; 10A: 135-139. PMID: 3968395
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