Abstract
The Parkes Weber syndrome is a congenital vascular malformation, characterized by varicose veins, arterio-venous fistulas and overgrown limbs. No broadly accepted animal model of Parkes Weber syndrome has been described. We created side-to-side arterio-venous fistula between common femoral vessels with proximal non-absorbable ligature on common femoral vein limiting the enlargement of the vein diameter in Wistar rats. Contralateral limb was sham operated. Invasive blood pressure measurements in both iliac and inferior cava veins were performed in rats 30 days after fistula creation. Tight circumference and femoral bone length were measured. Histopathology and morphology of soleus muscle, extensor digitorum longus muscle, and the common femoral vessel were analyzed. 30 days following arterio-venous fistula creation, a statistically significant elevation of blood pressure in common iliac vein and limb overgrowth was observed. Limb enlargement was caused by muscle overgrowth, varicose veins formation and bone elongation. Arterio-venous fistula with proximal outflow limitation led to significant increase of femoral vein circumference and venous wall thickness. Our study indicates that the described rat model mimics major clinical features characteristic for the human Parkes Weber syndrome: presence of arterio-venous fistula, venous hypertension and dilatation, varicose veins formation, and the limb hypertrophy. We reveal that limb overgrowth is caused by bone elongation, muscle hypertrophy, and venous dilatation. The newly established model will permit detailed studies on the mechanisms underlying the disease and on the efficacy of novel therapeutic strategies for the Parkes Weber syndrome treatment.
Highlights
The Parkes Weber syndrome (PWS), first described in 1907 [1], is characterized by a triad of arteriovenous fistulas (AVF), varicose veins and bone and soft tissue hypertrophy leading to limb enlargement
Our results indicate that the described rat model recapitulates major clinical features characteristic for the human PWS: presence of AVF, venous hypertension and dilatation, varicose veins formation, and the limb hypertrophy
Femoral vein circumference was significantly increased in limbs with AVF compared to sham-operated limbs (8.43±1.37 vs. 2.29±0.17 mm, p = 0.0022)
Summary
The Parkes Weber syndrome (PWS), first described in 1907 [1], is characterized by a triad of arteriovenous fistulas (AVF), varicose veins and bone and soft tissue hypertrophy leading to limb enlargement. The presence of AVF distinguishes PWS from the Klippel-Trenaunay syndrome, known as the capillary-lymphatic-venous malformation, CLVM [2]. Symptoms of PWS are congenital and present at birth. Vascular anomalies usually affect a limb, most commonly a leg, and less often a trunk. Capillary malformations, forming geographic patterns, are typically located on lateral side of the limb, buttocks or trunk. Arteriovenous shunt may lead to cardiac system failure or to limb ischemia. PWS, to other vascular malformations, significantly reduces the quality of life of the affected patients [6]
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