Obstructive jaundice secondary to multiple hepatic artery aneurysms in a 14-year-old boy with neurofibromatosis type 1.

Neurofibromatosis type 1 is the most common inherited disorder of the nervous system, affecting approximately 1 in 3,000 people. A small but significant subset of these patients develop vasculopathies. We present the first reported case of neurofibromatosis type 1 presenting with obstructive jaundice secondary to multiple hepatic artery aneurysms. Therapy included staged coil embolization of the hepatic artery aneurysms and resection of a large retroperitoneal neurofibroma.

Varying presentations in patients with symptomatic type IV vascular Ehlers-Danlos syndrome.

Ehlers-Danlos syndrome (EDS) represents a group of inheritable connective tissue disorders. Patients with type IV or vascular EDS, autosomal dominant pattern of inheritance, may present with aneurysm formation or arterial dissection. Due to vessel fragility, operative therapy for such disorders has been reserved for compelling indications in which benefit clearly warrants risk, yet assessment of risk is largely clinical with operative decisions guided by factors such as response to previous operations and age at onset of index vascular complications. We present 2 patients with differences in their clinical presentations and outcomes and review the literature with emphasis on operative decision making.

Late outcomes of carotid artery stenting: a critical review.

The role of carotid artery stenting (CAS) as an alternative to carotid endarterectomy for the treatment of extracranial carotid occlusive disease for stroke prevention continues to evolve. Although technical and device refinements aimed at making CAS safer continue to this day, safety as measured by 30-day and 1-year outcomes has been the primary recipient of regulatory and practice attention. Relatively less emphasis has been placed on the incidence of recurrent stenosis after CAS and the efficacy of CAS in late stroke prevention. Data on late outcomes of CAS, including factors of potential influence, have been emerging and are addressed in this review.

Peripheral atherectomy in TransAtlantic InterSociety Consensus type C femoropopliteal lesions for limb salvage.

BACKGROUND: The optimal therapy for TransAtlantic Societal Consensus (TASC) type C femoropopliteal lesions remains a critical issue in the treatment of infrainguinal occlusive disease. The purpose of this study was to evaluate the outcome of limbs with TASC C femoropopliteal lesions and critical limb ischemia treated with the FoxHollow SilverHawk atherectomy catheter. METHODS: From September 2004 to September 2005, 18 consecutive femoropopliteal procedures performed in 17 limbs in 16 patients were reviewed. Demographic data, baseline angiographic findings, and indications for the procedures were recorded. Clinical outcomes including symptom resolution and limb salvage were determined for the 17 primary procedures. Hemodynamic improvement was compared by using the paired Student t test. Stenosis-free patency was determined by the Kaplan-Meier method. RESULTS: The mean age was 72.5 years (range, 47-88 years). Fifty percent of the patients had four or more of the following risk factors: hypertension, diabetes, tobacco use, hyperlipidemia, renal insufficiency, and coronary artery disease. The indication was tissue loss in 13 limbs and rest pain in 4. All patients had a second level of disease, either inflow or tibial/pedal, which was treated concurrently when appropriate. Initial resolution of symptoms was achieved in 12 limbs, and partial healing was achieved in 2 others. Early amputation was necessary in the remaining three patients, but this was likely due to severe inframalleolar disease and advanced forefoot ischemia at the time of presentation. Five patients have remained symptom-free without restenosis at a mean follow-up of 6 months. Two patients have required late amputation for hemodynamic failure. The ankle-brachial index improved from 0.39 +/- 0.08 (mean +/- SEM) before surgery to 0.75 +/- 0.08 in the immediate postoperative period (P = .02). However, it returned toward baseline at 6 months after surgery, with a mean of 0.48 +/- 0.07. Stenosis-free patency of the femoropopliteal segment was 22% at 12 months. CONCLUSIONS: Peripheral atherectomy can achieve good early clinical and hemodynamic success in patients with TASC C lesions and critical limb ischemia. However, mid-term restenosis rates are high in this challenging cohort of patients.