Prevalence and inventory of venous anatomical abnormalities in the arms of patients with combined capillary, venous and lymphatic malformations (Klippel-Trénaunay syndrome).

OBJECTIVES: To investigate the prevalence of arm-involvement in Klippel-Trénaunay syndrome (KTS)-patients and to describe the venous anatomy and/or venous aberrations present in the arm, and if possible, their relationship to complaints (pain, congestion and thromboembolic events). METHODS: A retrospective cohort-study was performed with data from medical records of a large KTS-cohort (n = 173) from a tertiary referral center. Within this cohort, a descriptive study (n = 12) was performed on the KTS-patients with arm involvement and who had been examined with Colour Duplex Ultrasonography (CDU). RESULTS: Our KTS-cohort (n = 173) comprised 43 patients (24.9%) with arm-involvement; in nineteen patients (11.0%) the arm was the only affected limb. Of those KTS patients investigated with CDU, 9 out of 12 (75%) had an aberrant venous anatomy. CONCLUSION: Future research needs to clarity whether the complaints of KTS-patients in general are caused by an aberrant venous anatomy, coagulation alterations and/or other factors. Herein, the KTS-arms may play an important role.

Vascular replacement using a layered elastin-collagen vascular graft in a porcine model: one week patency versus one month occlusion.

A persistent clinical demand exists for a suitable arterial prosthesis. In this study, a vascular conduit mimicking the native 3-layered artery, and constructed from the extracellular matrix proteins type I collagen and elastin, was evaluated for its performance as a blood vessel equivalent. A tubular 3-layered graft (elastin-collagen-collagen) was prepared using highly purified type I collagen fibrils and elastin fibers, resembling the 3-layered native blood vessel architecture. The vascular graft was crosslinked and heparinised (37 ± 4 µg heparin/mg graft), and evaluated as a vascular graft using a porcine bilateral iliac artery model. An intra-animal comparison with clinically-used heparinised ePTFE (Propaten®) was made. Analyses included biochemical characterization, duplex scanning, (immuno)histochemistry and scanning electron microscopy. The tubular graft was easy to handle with adequate suturability. Implantation resulted in pulsating grafts without leakage. One week after implantation, both ePTFE and the natural acellular graft had 100% patencies on duplex scanning. Grafts were partially endothelialised (Von Willebrand-positive endothelium with a laminin-positive basal membrane layer). After one month, layered thrombi were found in the natural (4/4) and ePTFE graft (1/4), resulting in occlusion which in case of the natural graft is likely due to the porosity of the inner elastin layer. In vivo application of a molecularly-defined tubular graft, based on nature's matrix proteins, for vascular surgery is feasible.