Presentation
Leg length discrepancy with right limb overgrowth since birth
Patient Data
lower limbs asymmetry with marked soft tissue hypertrophy on the right side along with leg length discrepancy
dilated vascular structures in the right lower limb mainly involving the subcutaneous compartment generating flow voids and pulsation artifact (best seen on the axial STIR images) that suggest a high flow component consistent with arteriovenous malformation
the MR angiography shows an early venous flow on the right side, as well as early opacification of the aforementioned vascular structures confirming the presence of arteriovenous shunts
subcutaneous edema involving the right leg
the VR reconstruction images clearly demonstrate the lower limbs asymmetry with marked soft tissue hypertrophy on the right side along with leg length discrepancy
dilated subcutaneous vascular structures are seen throughout the right lower limb
The ultrasound Doppler examination of the aforementioned vascular structures shows high-flow pulsatile waveforms with both systolic component and increased diastolic flow, suggesting arteriovenous shunts.
The image illustrates the asymmetric hypertrophy of the right leg subcutaneous soft tissue (blue) and muscles (red) compared to the left side.
Case Discussion
Imaging findings are most consistent with Parkes Weber syndrome.
Parkes Weber syndrome is an extremely rare congenital vascular anomaly. It is characterized by bony and soft tissue hyperplasia, capillary cutaneous malformations, and multiple arteriovenous fistulas throughout the affected upper or lower extremity (the lower limbs are more commonly affected). These abnormal shunts between the arteries and veins in the affected limb can cause high-output heart failure.
It should not be mistaken for the more common Klippel-Trénaunay syndrome, which has no arteriovenous shunt component and is treated in much the same way.
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