title: Rat Model of Parkes Weber Syndrome.
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.
authors.affiliation: Department of Immunology, Biochemistry and Nutrition, Medical University of Warsaw, Warsaw, Poland; Department of Internal Medicine, Hypertension and Vascular Diseases, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Krzysztof
authors.initials: K
authors.lastname: Bojakowski
authors.affiliation: Department of Immunology, Biochemistry and Nutrition, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Gabriela
authors.initials: G
authors.lastname: Janusz
authors.affiliation: Department of Cytology, Faculty of Biology, University of Warsaw, Warsaw, Poland.
authors.forename: Iwona
authors.initials: I
authors.lastname: Grabowska
authors.affiliation: Department of Immunology, Biochemistry and Nutrition, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Oliwia
authors.initials: O
authors.lastname: Zegrocka-Stendel
authors.affiliation: Department of Immunology, Biochemistry and Nutrition, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Agnieszka
authors.initials: A
authors.lastname: Surowiecka-Pastewka
authors.affiliation: Department of Immunology, Biochemistry and Nutrition, Medical University of Warsaw, Warsaw, Poland; Department of Molecular and Translational Oncology, Maria Sklodowska-Curie Memorial Cancer Center and Institute of Oncology, Warsaw, Poland.
authors.forename: Magdalena
authors.initials: M
authors.lastname: Kowalewska
authors.affiliation: Department of Immunology, Biochemistry and Nutrition, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Dorota
authors.initials: D
authors.lastname: Maciejko
authors.affiliation: Department of Immunology, Biochemistry and Nutrition, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Koziak
mesh_terms: D000818:Animals; D001164:Arteriovenous Fistula / Q000473:pathology*; D001794:Blood Pressure; D004195:Disease Models, Animal; D005268:Femoral Vein / Q000473:pathology*; D006801:Humans; D007866:Leg / Q000503:physiopathology*; D008297:Male; D051381:Rats; D017208:Rats, Wistar; D013341:Sturge-Weber Syndrome / Q000473:pathology*; D014648:Varicose Veins / Q000473:pathology*