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LETTER TO EDITOR
Year : 2019  |  Volume : 18  |  Issue : 2  |  Page : 117-119  

High-flow type of peripheral arteriovenous malformation causing severe cosmetic deformity


1 Department of Radiology, Subharti Medical College, Meerut, Uttar Pradesh, India
2 Department of Pediatrics, Subharti Medical College, Meerut, Uttar Pradesh, India

Date of Web Publication8-May-2019

Correspondence Address:
Dr. Sonal Saran
Flat No 1, Ramdas Bhawan, Subharti University, Meerut, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aam.aam_31_18

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How to cite this article:
Saran S, Malik S, Sharma Y, Kharbanda A. High-flow type of peripheral arteriovenous malformation causing severe cosmetic deformity. Ann Afr Med 2019;18:117-9

How to cite this URL:
Saran S, Malik S, Sharma Y, Kharbanda A. High-flow type of peripheral arteriovenous malformation causing severe cosmetic deformity. Ann Afr Med [serial online] 2019 [cited 2019 Nov 17];18:117-9. Available from: http://www.annalsafrmed.org/text.asp?2019/18/2/117/257834



Sir,

A 45-year-old male presented with swelling in the left hand for 20 years. The swelling was multinodular and present in dorsal as well as palmer aspect of the hand. The swelling was initially small which gradually progressed in dimensions over years with gradual development of flexor contracture of the hand. Swelling was soft, compressible, and pulsatile with audible bruit. Dystrophic changes with ulceration and necrosis were present in the overlying skin [Figure 1]. The patient was subjected to ultrasound evaluation with high frequency transducer with the application of power Doppler. On ultrasound evaluation, multiple tortuous anechoic tubular channels were seen with dilatation at few sites. On power Doppler application, complete color filling of the tubular channels was seen with high velocity pulsatile arterial waveform and spectral broadening [Figure 2]a, [Figure 2]b, [Figure 2]c. This led to the provisional diagnosis of high-flow arteriovenous type of peripheral vascular malformation (PVM). Arterial supply of the PVM was from the ulnar and radial arteries with venous drainage into the cephalic and basilic veins. Doppler tracing in the basilic and cephalic veins showed arterialization of the venous channels [Figure 2]d. On magnetic resonance imaging (MRI) evaluation, the PVM was seen infiltrating into musculature of the hand with aneurysmal dilatation at few sites. Thenar/hypothenar and small muscles of the hand were poorly identified with evidence of atrophy [Figure 3].
Figure 1: (a) The multinodular swelling in the left hand of the patient with flexor contracture and skin changes. (b) The multinodular swelling in the left hand of the patient with flexor contracture and skin changes

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Figure 2: (a and b) Multiple tortuous anechoic tubular channels are seen with dilatation at few sites and complete color filling on power Doppler application. (c) Pulse wave Doppler evaluation identified pulsatile arterial wave form in the channels with a spectral broadening. (d) Doppler tracing in the cephalic vein showing pulsatile arterial waveform with arterialization of the venous channels

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Figure 3: Magnetic resonance imaging evaluation of the affected hand showing multiple tortuous tubular channels with flow void on spin-echo sequence and aneurysmal dilatation (a). The tortuous tubular channels are seen infiltrating into musculature of the hand with atrophy (b)

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PVMs include a wide array of lesions and can present with spectrum of clinical features ranging from asymptomatic incidental findings to life-threatening symptoms. PVMs are quite common in extremities and are generally confined to the skin, subcutaneous tissue, and muscles. Taking detailed clinical history and physical examination are prerequisite for making the diagnosis; however, to exactly characterize the lesion imaging is required. Radiologists are playing an increasingly important role not only in making the diagnosis of the PVMs but also for implementing interventional management. The management of complex PVMs should be done in dedicated vascular centers.[1]

Classifying the PVMs is the first step in radiological evaluation. The International Study of Vascular Anomalies expanded the radiological classification proposed by Jackson et al. and the combined classification is described in [Table 1].[2],[3]
Table 1: Combined classification system of peripheral vascular malformations by International Study of Vascular Anomalies and Jackson et al.

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High-flow vascular malformation includes arteriovenous malformation (AVM) and arteriovenous fistula (AVF) and constitutes approximately 10% of PVMs in the extremities. AVMs are generally congenital, whereas AVFs are frequently acquired. AVMs consist of multiple tortuous feeding arteries, nidus composed of tortuous vascular channels connecting arteries, and draining veins with the absence of capillary network. AVFs, on the other hand, consist of a single vascular channel connecting artery and vein.[4] The Schobinger classification outlining progressive clinical course of AVM is described in [Table 2].[5] Our patient fell into category III.
Table 2: The Schobinger classification outlining progressive clinical course of arteriovenous malformation

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Imaging evaluation should begin with ultrasound examination. This examination will allow immediate distinction between high- and slow-flow lesions without any doubt in most of the typical malformations. AVMs will reveal localized arterial and venous hypertrophies with high systolic flow, shunting, and spectral broadening. Radiography plays a limited role in classifying the lesion. Information which can be obtained from a plain radiograph includes the presence/absence of phlebolith and presence of any osseous deformity with or without joint involvement. MRI has become by far the most valuable modality in the confirmation, and characterization of PVMs. On spin-echo sequences, high-flow PVMs will show large flow voids with early enhancement of enlarged feeding arteries and nidus with shunting to draining veins. Infiltration of the tissue planes with muscular atrophy can also be seen.[6]

Computed tomography angiography (CTA) is advised in only selected cases with equivocal ultrasound and MRI. High spatial resolution of CTA allows extremely accurate assessment of the size of the PVM with the depiction of arterial feeders and draining veins. Its usefulness is limited by heavy radiation exposure and no additional therapeutic benefit. Catheter-based angiography, on the other hand, offers advantages of CTA with chance of on table interventional management. Direct-puncture of the nidus can help in estimating the volume and flow characteristics of the lesion. Catheter angiography is generally reserved for well worked up cases with definitive indication for radiological intervention. Radiological interventions are now widely accepted as first-line treatment for PVMs. With the exception of the capillary-based lesions which require conservative approach, rest all types of PVMs can be considered for interventional treatment.[6] Kawanabe et al. described the treatment approach toward PVMs based on the flow characteristics of the lesion. Most slow-flow malformations respond well to the intralesional percutaneous sclerotherapy, whereas transarterial embolotherapy is needed for fast-flow AVMs and AVFs.[7] Lee et al. described the absolute and relative indications for the treatment of PVMs.[8]

As our patient had high-flow type of AVM with progressive disabling discomfort, functional impairment, and severe cosmetic deformity, he was best suited for radiological interventional treatment by transarterial embolotherapy. He was referred to dedicated vascular center for the same. Our case outlines the need for radiological imaging workup of cases with PVMs as well as classification and proper interventional therapy for the same. Development in imaging and interventional treatment techniques has helped to improve the management of such patients.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Legiehn GM, Heran MK. Classification, diagnosis, and interventional radiologic management of vascular malformations. Orthop Clin North Am 2006;37:435-74, vii-viii.  Back to cited text no. 1
    
2.
Jackson IT, Carreño R, Potparic Z, Hussain K. Hemangiomas, vascular malformations, and lymphovenous malformations: Classification and methods of treatment. Plast Reconstr Surg 1993;91:1216-30.  Back to cited text no. 2
    
3.
Merrow AC, Gupta A, Patel MN, Adams DM 2014 revised classification of vascular lesions from the international society for the study of vascular anomalies: Radiologic-pathologic update. Radiographics 2016;36:1494-516.  Back to cited text no. 3
    
4.
Legiehn GM, Heran MK. A step-by-step practical approach to imaging diagnosis and interventional radiologic therapy in vascular malformations. Semin Intervent Radiol 2010;27:209-31.  Back to cited text no. 4
    
5.
Kohout MP, Hansen M, Pribaz JJ, Mulliken JB. Arteriovenous malformations of the head and neck: Natural history and management. Plast Reconstr Surg 1998;102:643-54.  Back to cited text no. 5
    
6.
Hyodoh H, Hori M, Akiba H, Tamakawa M, Hyodoh K, Hareyama M, et al. Peripheral vascular malformations: Imaging, treatment approaches, and therapeutic issues. Radiographics 2005;25 Suppl 1:S159-71.  Back to cited text no. 6
    
7.
Kawanabe T, Wakita S, Harii K, Hayashi N, Inoue Y. Sclerotherapy of hemangiomas and vascular malformations in lips. Jpn J Plast Reconstr Surg 1996;16:852-62.  Back to cited text no. 7
    
8.
Lee BB. New approaches to the treatment of congenital vascular malformations (CVMs) – A single centre experience. Eur J Vasc Endovasc Surg 2005;30:184-97.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1], [Table 2]



 

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