A 45-year-old male patient presented with swelling in the left hand for 20 years. The swelling was initially small in size which gradually progressed in dimension over the years. The swelling was multi-nodular, soft, compressible and pulsatile with audible bruit. Dystrophic skin changes with ulceration and necrosis were present in the overlying skin.
On ultrasound evaluation, multiple tortuous anechoic tubular channels were seen with dilatation at few sites. On power Doppler application, complete colour filling of the tubular channels was seen with high velocity pulsatile arterial wave form and spectral broadening. This led to the provisional diagnosis of high flow arteriovenous type of peripheral vascular malformation (PVM). Arterial supply of the PVM was from ulnar and radial arteries with venous drainage into cephalic and basilic veins. Doppler tracing in the basilic and cephalic veins showed arterialisation of the venous channels. On MRI evaluation, the PVM was seen infiltrating into the 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.
• Background: PVMs include a wide array of lesions and can present with a spectrum of clinical features. PVMs are quite common in extremities and are generally confined to skin, subcutaneous tissue and muscles. Taking detailed clinical history and physical examination are a prerequisite for making the diagnosis, however, to exactly characterise the lesion imaging modalities are required. Radiologists are playing an increasingly important role not only in making the diagnosis of the PVMs but also for interventional management. The management of complex PVMs should be done in dedicated vascular centers. [1]
• Clinical perspective: High flow vascular malformation includes AVM and AVF and constitutes approximately 10 % of PVMs in the extremities. AVMs are generally congenital whereas AVFs are frequently acquired.
• Imaging perspective: Imaging evaluation should begin with ultrasound examination with color Doppler. This examination will allow immediate distinction between high and slow flow lesions without any doubt in most of the typical malformations. AVMs will reveal localised arterial and venous hypertrophy 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 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 characterisation 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 tissue planes with muscular atrophy can also be seen [2].
• Computed tomography angiography (CTA) is advised in only selected cases with equivocal ultrasound and MRI. Catheter-based angiography on the other hand offers advantages of CTA with the chance of on table interventional management. Radiological interventions are now widely accepted as first line treatment for PVMs. With exception of the capillary-based lesions which require a conservative approach, the rest of all types of PVMs can be considered for interventional treatment [2].
• Outcome: Most slow flow malformations respond well to the intralesional percutaneous sclerotherapy, whereas transarterial embolotherapy is needed for fast flow AVMs and AVFs [3].
• Take home message: Our case outlines the need for radiological imaging work up of cases with provisional diagnosis of PVMs as well as classification and proper interventional therapy for the same. Development in imaging and interventional treatment techniques has helped to improve management of such patients.
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High flow type of peripheral arteriovenous malformation of hand
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Based on the multiple imaging data of the left hand (ultrasound and color Doppler, MRI, etc.) and the clinical manifestations, the main findings are summarized as follows:
Considering the patient’s 20-year chronic course, palpable pulsation and audible bruit (murmur on auscultation with palpable thrill), and high-flow shunt features on imaging, the following conditions should be considered:
The differential diagnosis mainly depends on the clinical history (e.g., presence of trauma or surgery), hemodynamic parameters on imaging, and the extent of lesion involvement in surrounding tissues.
Taking into account the 20-year slowly progressive course, multiple nodular soft tissue swellings in the left hand, auscultated pulsatile murmur, and the high-flow shunt features observed on ultrasound and MRI, the most likely diagnosis is:
High-flow vascular malformation (Arteriovenous Malformation, AVM).
If further confirmation is needed, digital subtraction angiography (DSA) can be performed to delineate the feeding arteries and draining veins of the vascular malformation. Endovascular interventional therapy may be considered if necessary.
Rehabilitation should be based on the location of the lesion (left hand) and the characteristics of the vascular malformation, emphasizing functional recovery and safety. A phased approach is recommended as follows:
Throughout the rehabilitation process, close attention should be paid to the vascular status and the healing of skin ulcers. The exercise plan should be dynamically adjusted to ensure both safety and effectiveness.
Disclaimer: This report is based on the available imaging and clinical history for preliminary analysis. The recommendations provided herein are for reference only and do not replace in-person consultation or professional medical diagnosis and treatment. A definitive treatment plan should be made after considering the patient’s overall condition, laboratory tests, and specialist evaluations.
High flow type of peripheral arteriovenous malformation of hand
