Orthotopic liver transplantation is currently the treatment of choice in patients with end-stage liver disease for which no other therapy is available. In children, segmental liver transplantation with living donor, reduced-size cadaveric, and split cadaveric allografts has become an important therapeutic option. However, the resulting expansion of the donor pool has increased the risk for postoperative vascular and biliary complications, which affect children more frequently than adults. Early recognition of these complications requires radiologic evaluation because their clinical manifestations are frequently nonspecific and vary widely. Doppler ultrasonography (US) plays the leading role in the postoperative evaluation of pediatric patients. Current magnetic resonance (MR) imaging techniques, including MR angiography and MR cholangiography, may provide a wealth of pertinent information and should be used when findings at US are inconclusive. Computed tomography is a valuable complement to US in the evaluation of complications involving the hepatic parenchyma as well as extrahepatic sites and is commonly used to guide percutaneous aspiration and fluid collection drainage. Familiarity with and early recognition of the imaging appearances of the various postoperative complications of pediatric liver transplantation are crucial for graft and patient survival.
1 November 2016 in European Journal of Obstetrics & Gynecology and Reproductive Biology
Uterine Arteriovenous Malformation (UAVM) is a rare but life-threating cause of uterine bleeding. The clinical management of this condition is challenging, and there is a need to describe the most adequate approach for these patients. Uterine artery embolization (UAE) is the most widely-published treatment in the literature in recent years, although there is a need to update the evidence on this treatment and to compare it with other available therapies. Thus, the objective of this systematic review is to quantify the efficacy of UAE of UAVM. In addition, we evaluated the clinical context of the patients included, the treatment complications, and the pregnancy outcomes after UAE. With this goal in mind, we finally included 371 patients spread over all continents who were included in 95 studies. Our results show that, similar to other medical therapies, the global success rate after embolization treatment was 88.4%, presenting a low risk of adverse outcomes (1.8%), even in women with later pregnancy (77% had no complications). To date, this is the largest systematic review conducted in this field, although there are still some points to address in future studies. The results obtained in our study should be outlined in UAE protocols and guidelines to aid in clinical decision-making in patients with UAVM.
28 February 2019 in Intractable & Rare Diseases Research
Behçet's Disease (BD) is a rare, chronic and recurrent inflammatory multisystemic condition of unknown origin that can affect any tissue. The vascular system is involved in 5-40% of cases of BD, including venous and arterial beds and it has a relapsing course. Budd-Chiari syndrome (BCS) is a rare complication of BD with a frequency of < 5% among patients with vascular involvement and is more frequent in men (89.5%). Two clinical presentation groups of BCS related to BD have been described: the "symptomatic" form and the "silent" form. We present a case of BD in a young woman presented as symptomatic severe BCS with rapid progression of coagulopathy reaching a spontaneous INR of 1.74 and increased ascites by ultrasound control. BD was confirmed through clinical history. The patient was treated with a high-dose pulse of corticosteroids and cyclophosphamide with a strikingly favorable response in the first forty-eight hours. Although several studies have demonstrated a survival improvement with the use of transjugular intrahepatic portosystemic shunt in patients with severe BCS, it was discarded due to the lack of evidence of this procedure in patients with BD and the fact that it could trigger a vascular pathergy phenomenon. Vascular BD should be suspected in recurrent venous and/or arterial thrombosis since it is associated with high morbidity and mortality. Immunosuppressive treatment is critical for the management of vascular involvement in BD. However, the role of anticoagulation is debatable. We suggest an algorithm for the management of BCS associated with BD.
1 February 2012 in Revista Portuguesa de Cardiologia
Transcatheter aortic valve implantation (TAVI) is an alternative to surgical aortic valve replacement in patients with severe aortic stenosis (AS) and unacceptably high surgical risk. We present our first two years' experience with TAVI. A total of 76 AS patients were evaluated for TAVI and 23 of them underwent a TAVI procedure. These patients had a mean EuroSCORE of 22.4% and a mean age of 81.5 years, and were prospectively followed for a mean of 12.9 ± 11 months. The percutaneous aortic valve was successfully implanted in 100% of the patients. Mortality at 30 days was 4%. The most common complications were access site-related bleeding and transfusion (22%), followed by new permanent pacemaker implantation (9%). After a mean follow-up of 12.9 months, survival was 87%. In a maximum follow-up of 30 months there were no cases of prosthesis dysfunction or cardiovascular death. Two years after the introduction of a TAVI program in our center, the procedure has established itself as a safe and effective alternative for patients with severe AS and unacceptably high surgical risk.