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Parkes Weber Sydrome: a rare case of vascular malformation.


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Department of Adult Cardiology - Vascular Medicine - Philippine Heart Center


Background: Vascular malformations are developmental abnormalities of the vascular system, which may involve the capillaries, arteries or veins. Parkes-Weber Syndrome is a congenital vascular malformation characterized by cutaneous flush with underlying multiple microarteriovenous fistulas (AVF) with enlarged arteries, veins and capillary-venous malformations
in association with soft tissue and skeletal hypertrophy of a limb.

Case: A 25 year-old male presented with chronic ulcer and progressive swelling of his left limb of two years. A hyperpigmented, raised lesion was noted on his left medial malleolus at age 8, which remained unchanged until age fourteen, after which an increase in the size of the lesion was noted associated with lower limb asymmetry, hypertrophy and increased limb length. At age 23, his left lower limb became progressively swollen with darkening of skin and ulceration noted particularly at the left gaiter's area. He was treated for cellulitis and was given several antibiotics but to no avail. Over the past 2 years, his condition worsened prompting consultation at our institution. Examination revealed a limb length discrepancy of 5.5 cm and limb-girth discrepancy of 7.5 cm and the presence of an 8x5 cm oval-shaped active ulcer is present over the left medial malleolar area with multiple dilated and tortuous veins on the left calf. Arterial and venous ultrasound showed multiple fast flowing arterio‐venous malformations in the left lower limb (as shown in Figure 2) that was later confirmed by peripheral computed tomographic angiogram (CTA). Images on CTA revealed the presence of arteriovenous malformation (AVM) with intramuscular and intra-osseous components in the posterior and medial aspect of the left distal femur and proximal tibia (as shown in Figure 3-A) arising from the distal superficial femoral artery and popliteal arteries and draining to the superficial femoral vein (as shown in Figure 3-AC). AVM with intramuscular and subcutaneous components are also seen in the left calf more at its medial aspect with feeding arteries arising from the mid and distal aspect of posterior tibial artery and distal anterior tibial arteries with intramuscular and subcutaneous varices draining this AVM (as shown in Figure 3-C). Multiple subcutaneous thickening at the medial malleolar region of the left lower leg was also noted secondary to the AVM (as shown in Figure 3-BC). 3-dimensional reconstruction of the CTA revealed extensive varicosities of the superficial and deep veins of the left leg.

Conclusion: We report a case of a rare form of vascular malformation. To our knowledge, this is the first local report on Parkes-Weber Syndrome (PKWS). On the basis of the clinical characteristics and imaging features, the diagnosis of PKWS was made. The patient was managed conservatively with wound care and compression stockings.

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Philippine Heart Center Medical Library EP.R.005.17 Fulltext Print Format

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