Giant Symptomatic Aneurysm Secondary to Hereditary Hemorrhagic Telangiectasia of a Main Hepatic Artery with Aberrant Origin in Superior Mesenteric Artery.

BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is a rare disease consistent in vascular dysplasias affecting different organs. Liver involvement includes telangiectases, arteriovenous shunting, and ischemic cholangitis, and its prevalence ranges from 8% to 31%. Spontaneous pseudoaneurysms have never been reported associated to HHT. Several variations in the origin of the main hepatic artery (MHA) have been described in large radiological series and can be found in around 4% of general population. METHODS: A 64-year-old woman with history of HHT was diagnosed of acute symptomatic saccular aneurysm involving the MHA. The associated portovenous fistulae suggested that liver perfusion could be impaired if embolization of the MHA without revascularization was performed. Because of an aberrant origin on the superior mesenteric artery, a hybrid procedure was designed, consistent on endovascular exclusion of the MHA associated with a surgical aortic-hepatic bypass graft. RESULTS: Angio computed tomography (Angio CT) was performed at discharge, 1 month, and 6 months after intervention, revealed complete exclusion of the aneurysm, which reduced 50% in diameter after 6 months. Aneurysms of the MHA can be associated with HHT when intrahepatic arteriovenous fistulae are present. This complication is rare, and the reported cases have been treated by surgical and totally endovascular procedures. The confluence of this very rare condition with an uncommon vascular anatomy makes this a unique case that required the design of a customized intervention. CONCLUSIONS: Hybrid procedures are useful to treat conditions that cannot be solved with standard interventions. Longer series of cases with long-term surveillance are needed to asses the results of these techniques in these complex pathology.

Comparison of Bypass with Endoscopically Harvested Internal Saphenous Vein versus Bypass with Surgically Harvested Internal Saphenous Vein for Lower Limb Arterial Disease.

BACKGROUND: Patients with lower limb arterial disease have a high risk for complications related with surgical wounds. The endoscopic extraction of the great saphenous vein (GSV) is a less invasive alternative to the conventional surgical extraction. METHODS: A clinical and ultrasonographic follow-up was carried out on the lower limb bypass with GSV performed in our institution between years 2007 and 2012. Patients were selected for open or endoscopic harvesting depending on the surgeon assigned (endoscopic or open surgeon). Follow-up was performed at 1, 3, 6, and 12 months after surgery and annually thereafter. All the GSV endoscopic harvestings (GSVEH) were performed by the same surgeon. Data for primary, assisted, and secondary patency and amputation-free survival were analyzed. Anatomopathalogic analysis were performed on pares of samples of the same vein dissected surgically and endoscopically from the same patient. RESULTS: Sixty bypass surgery has been performed on 60 patients (54 men and 6 women), 30 with GSVEH (50%), and 30 with GSV open harvesting (GSVOH). All patients were intervened for critical limb ischemia (Rutherford cathegory 4, 5, and 6). Significant differences were found between both groups for suture dehiscence (GSVEH 0%, GSVOH 20%, P = 0.01) and infection (GSVEH 3%, GSVOH 30% P, 0.006). No significant differences were found between both groups regarding to primary patency, assisted primary patency, or amputation-free survival. An anatomopathologic comparison of segments of veins extracted surgically and endoscopically of the same patients did not show any significant differences. CONCLUSIONS: Although no statistically significant differences were found between GSVOH and GSVEH bypass for lower limb revascularization, there is a trend toward poorer patency rates for the endoscopic technique. GSVEH lowers the risks for infection and dehiscence of surgical wounds.

Retroperitoneal fibrosis: single-centre experience from 1992 to 2010, current status of knowledge and review of the international literature.

OBJECTIVE: The aims of this study were to investigate retroperitoneal fibrosis in a Spanish hospital and present a review of the international literature to attempt to elucidate a diagnostic and therapeutic approach to this unusual pathology. MATERIAL AND METHODS: A database search was performed in the pathology department and in the documentation service using the key words "retroperitoneal fibrosis" and "Ormond's disease", limiting the search to the years 1990-2010. Cases in which secondary retroperitoneal fibrosis was considered were excluded. In addition, a PubMed literature search was performed using the terms "retroperitoneal fibrosis" and "Ormond's", limiting the search to 1985-2011. RESULTS: Twenty-two patients were diagnosed with idiopathic retroperitoneal fibrosis (IRF) or Ormond's disease. The most common symptom at the time of diagnosis was flank pain. With regard to laboratory findings, five patients (22.7%) had anaemia and eight (36.3%) had renal failure. Computed tomography (CT) was performed in 20 patients (90.9%) and the most common finding observed was retroperitoneal mass. Eighteen patients were started on corticosteroids, in six cases in association with azathioprine. Three patients had recurrence at 12, 24 and 72 months, respectively, and 15 patients required emergency surgery. Nine open surgical procedures were performed. CONCLUSIONS: At present, IRF is considered an autoimmune disease that presents with local and systemic signs and symptoms. CT and magnetic resonance imaging are the two tests of choice in IRF diagnosis and follow-up. [(18)F]Fluorodeoxyglucose positron emission tomography is starting to be used for assessment and treatment response. A combination of medical and surgical treatment is usually applied. It is essential to administer corticosteroids alone or in association with other immunosuppressive drugs such as azathioprine. Laparoscopic ureterolysis, or robotic ureterolysis, if available, is the technique of choice.

Fiabilidad de los tests diagnósticos no invasivos en la recurrencia de la trombosis venosa profunda / The reliability of non-invasive diagnostic tests in recurring deep vein thrombosis

Introducción. Los síntomas y signos clínicos de la trombosis venosa profunda recurrente (TVPR) pueden ser muy anodinos, confundiéndose con la sintomatología producida por un síndrome postrombótico. Los errores diagnósticos tienen gran trascendencia, ya que los falsos positivos suponen realizar un tratamiento posiblemente indefinido con anticoagulantes, y los falsos negativos exponen al paciente a nuevos episodios de TVPR y/o a un embolismo pulmonar. Desarrollo. En esta revisión se discutirán los mecanismos fisiopatológicos de la TVPR y se realizará una valoración de los diferentes tests diagnósticos y su fiabilidad

Introduction. The clinical signs and symptoms of recurring deep vein thrombosis (RDVT) can be very anodyne, and may easily be confused with the symptomatology produced by a post-thrombotic syndrome. Diagnostic errors have very important effects because false positives mean carrying out a possibly indefinite treatment with anticoagulant drugs and false negatives expose the patient to new episodes of RDVT and/or a pulmonary embolism. Development. In this review the pathophysiological mechanisms of RDVT will be discussed and we will also evaluate the different diagnostic tests and their reliability