Rosai-Dorfman Disease Presenting as Peripheral Vascular Insufficiency.

Rosai-Dorfman (-Destombes) disease (RDDD) is a rare idiopathic disorder of histiocyte proliferation, usually involving lymph node stations. The most common clinical finding is a bilateral cervical lymphadenopathy, fever, and weight loss. Arterial or venous structures are notably not involved. We hereby present a case of a 78-year-old Caucasian man, presenting with symptoms of progressive arterial insufficiency and right lower-limb edema, along with a nonpulsatile mass at the middle third of the thigh. Initial diagnostic hypothesis was a superficial femoral artery aneurysm thrombosis with a secondary postcompressive superficial femoral vein thrombosis. Duplex examination showed right superficial femoral arterial and venous thrombosis, along with a hypoechogenic mass causing compression of the neurovascular bundle. Suspecting a connective tissue sarcoma, computed tomography scan was performed after combined en bloc removal of the mass along with femoral artery and vein and prosthetic reconstruction of vascular continuity. Histopathology diagnosis was connective tissue RDDD. The atypical presentation of this rare syndrome induces us to include in differential diagnosis, among other more common forms of external compression of the neurovascular bundles, even rare conditions such as these, which generally only involve lymphatic stations.

A rare case of internal jugular vein aneurysmal degeneration in a type 1 neurofibromatosis complicated by potentially life-threatening thrombosis.

Type 1 neurofibromatosis (NF1) is an autosomal dominant disorder characterized by multiple forms of neural crest cell proliferation. Associated venous malformations are extremely rare. We present a case of a giant thrombosed internal jugular aneurysm with brachiocephalic vein thrombosis in an NF1 patient. Surgical correction required prophylactic left brachiocephalic vein ligation, aneurysm evacuation, and proximal jugular vein ligation. Extreme vein friability caused severe intraoperative bleeding. Vein wall histology confirmed neurofibromatosis infiltration. Jugular vein aneurysm and infiltration in NF1, although exceedingly rare, can pose a serious threat to the patient during surgical correction, with major vessel thrombosis enhancing morbidity and mortality.

The mid-distal deep femoral artery: few important centimeters in vascular surgery.

The deep femoral artery is an important artery in lower-limb revascularization. Employing its initial centimeters as either an inflow or an outflow site has been advocated by many authors. The purpose of this work was to highlight the importance of the mid-distal section of the deep femoral artery, underlining its indications and advantages. From January 1998 to December 2004, we performed, at the Misericordia Hospital Vascular Surgery Unit in Grosseto, Italy, 45 bypasses employing the mid-distal deep femoral artery as an inflow or outflow site. Twenty patients (44.4%) had nonhealing ulcers and/or gangrene, while the remainder (25 patients, 55.6%) presented with rest pain or severe claudication. In 41 cases (91.1%), the mid-distal deep femoral artery was used as the inflow site for peripheral bypasses. In four cases (8.9%), the mid-distal deep femoral artery was employed as the outflow site, twice (4.4%) after aortobifemoral branch thrombosis and twice (4.4%), in the same patient, after inguinal prosthetic infection healing. When the mid-distal deep femoral artery was employed as the inflow site, primary and secondary patency at 1 and 5 years were 92.72% vs. 95.20% and 57.39% vs. 72.81%, respectively. We had two early (<30 days) failures (4.4%) in patients with posterior tibial distal anastomosis, which required above-knee amputation. In two cases (4.4%), we had to perform a transmetatarsal amputation. In one patient, after healing of the inguinal prosthetic infection of an aortobifemoral bypass, we employed the mid-distal deep femoral artery both as inflow and as outflow site. This patient required a monolateral above-knee amputation after 5 months. The mid-distal deep femoral artery is a good outflow and inflow site in patients who have previously undergone surgical interventions in Scarpa's triangle, in those having inadequate vein segment, in those with local inguinal healed infection, and in obese patients. The surgical technique is a practical, easy, elegant, and fast procedure, along with being an optimal alternative to reexploration of scarred inguinal tissue.

Effects of simvastatin on walking performance and symptoms of intermittent claudication in hypercholesterolemic patients with peripheral vascular disease.

PURPOSE: To analyze the effects of short-term therapy with simvastatin on walking performance in hypercholesterolemic patients with peripheral vascular disease. METHODS: Eighty-six patients with peripheral arterial disease (Fontaine stage II), intermittent claudication, and total cholesterol levels >220 mg/dL were enrolled in a randomized, placebo-controlled, double-blind study. Forty-three patients were assigned to simvastatin (40 mg/d); the remaining 43 patients were assigned to placebo treatment. All patients underwent an exercise test and clinical examination, and completed a self-assessment questionnaire at 0, 3, and 6 months. Pain-free and total walking distance, resting and postexercise ankle-brachial indexes, and questionnaire scores were determined at each follow-up. RESULTS: At 6 months, the mean pain-free walking distance had increased 90 meters (95% confidence interval [CI]: 64 to 116 meters; P <0.005) more in the simvastatin group than in the placebo group. Similar results were seen for the total walking distance (mean between-group difference in the change, 126 meters; 95% CI: 101 to 151 meters; P <0.001), and for the ankle-brachial index at rest (mean, 0.09; 95% CI: 0.06 to 0.12; P <0.01) and after exercise (mean, 0.19; 95% CI: 0.14 to 0.24; P <0.005). There was also a greater improvement in claudication symptoms among patients treated with simvastatin. The effects on walking performance, ankle-brachial indexes, and questionnaire scores had also been significant at 3 months. CONCLUSION: High-dose short-term therapy with simvastatin may improve walking performance, ankle-brachial pressure indexes, and symptoms of claudication in hypercholesterolemic patients with peripheral vascular disease.