Stereotactic MRI Based Image Guidance for the Treatment of Vascular Malformations - a Pilot Study...
Vascular MalformationsTreatment of vascular malformations requires the placement of a needle within anatomically non well defined, pathological vessels, which may be as small as 1 mm, with the current state of the art relying exclusively on two dimensional fluoroscopy images for guidance. The investigators hypothesize that the combination of stereotactic image guidance with existing targeting methods will result in faster and more reproducible needle placements.
AVAST Anomalies Vasculaires Associées au Syndrome de Turner (Vascular Abnormalities Associated With...
Turner SyndromePeripheral Angiopathy2 moreTurner syndrome is a genetic condition, rare, due to the total or partial absence of one X chromosome, affecting 1/2500 newborn female. It combines almost constantly short stature and ovarian failure with infertility. Other anomalies are inconstant: morphological characteristics of varying intensity, associated malformations, and increased risk of acquired diseases ... The prognosis of patients reaching the Turner Syndrome is linked to cardiovascular complications (congenital heart disease, dilatation of the ascending aorta with risk of dissection or rupture of aneurysm), causing early mortality with reduction of life expectancy of at least 10 years. For these reasons, screening for heart disease and dilatation of the ascending aorta is established and is intended to prevent the complications associated with medical treatment and / or surgery to increase life expectancy and reduce the co-morbidities. On the vascular level, the recommendations other than those relating to the monitoring of the diameter of the ascending aorta include research of renal artery stenosis by doppler ultrasound if the patient is hypertensive and looking for lymphedema. However, other arterial lesions were described in the literature, outside of the aneurysm of the ascending aorta. These peripheral arterial lesions can also be life and / or functional prognosis of the patient. Ascending aorta dilation seems not to be exclusive in Turner syndrome. In addition, specific vascular lesions outside the affected artery are described: hepatic cirrhosis by vascular depletion, lymphedema and varicose veins. The prevalence of venous or lymphatic disease is unknown. A single-center review of 9 cases of patients followed at the University Hospital of Strasbourg showed the presence of vascular lesions discovered incidentally during assessments performed for reasons other than cardiovascular screening: cystic lymphangioma, internal carotid aneurysm, agenesis of the inferior vena cava, early varicose veins, embryonic cerebral artery, etc ... None of these patients showed any dilatation of the ascending aorta or heart disease. Peripheral vascular abnormalities in this patient group are exclusive. In this study, we seek to demonstrate that arterial disease in Turner syndrome involve the entire arterial territory and is not confined to the ascending aorta. Screening for arterial lesions should be performed on the entire arterial vascular tree and regularly in the course of time, especially as the presence of cardiovascular risk factors increases with the age of these patients. The venous and lymphatic vascular damage in the literature and in our series of cases in University Hospital of Strasbourg description should also lead to the detection of these lesions. These vascular complications can be alone responsible for the reduction in life expectancy or responsible for serious morbidity. Improved screening of associated vascular lesions is necessary to enable the best prevention of cardiovascular complications. It is also to establish the prevalence of vascular anomalies, whether arterial, venous or lymphatic, to better understand the disease and its management. By collecting systematically karyotype leading to diagnosis, it may be possible to make a link between the genetic defect and heart or vascular disease.
Retreatment and Its Efficiency of Thalidomide for Vascular Malformation Patients With Failure of...
Gastrointestinal Bleeding of Unknown OriginGastrointestinal Vascular Malformation1 moreBackground: Repeated episodes of bleeding from gastrointestinal vascular malformations refractory to endoscopic or surgical therapy often pose a major therapeutic challenge. Methods: The investigators will perform a randomized, double blind, placebo controlled study of thalidomide as a retreatment therapy for recurrent gastrointestinal bleeding due to vascular malformation. Patients with failure of first course treatment of thalidomide will be randomly grouped, prescribed a second four-month course regimen of 25 mg of thalidomide or placebo orally four times daily. All patients will be monitored for at least one year. The primary end point is defined as the patients whose rebleeds decrease from baseline by ≥ 50% at 12 months and the cessation of bleeding. Rebleeding is defined based on a positive fecal occult blood test (FOBT) (monoclonal colloidal gold color technology) at any visit after treatment. Secondary outcomes include the participants dependent on blood transfusions and changes from baseline in transfused packed red cell units, bleeding episodes, and hemoglobin levels at 12 months. Statistical significance is defined at P < 0.05.
Efficacy Study of Thalidomide in Gastrointestinal Vascular Malformation Related Bleeding
Gastrointestinal HemorrhageVascular MalformationBackground: Repeated bleeding from gastrointestinal vascular malformations remains to be a major therapeutic challenge. Methods: The investigators performed a randomised, double-blind, placebo-controlled, single centre study to assess the long-term efficacy and safety of thalidomide 100mg qn p.o. or placebo 100 mg qn p.o. administration for 4 months in subjects with recurrent gastrointestinal bleeding due to vascular malformations. Patients with at least six episodes of bleeding in the prior year due to vascular malformation were randomly grouped, prescribed a four-month regimen of either 100mg of thalidomide or 100 mg of placebo orally one time daily, and monitored for at least one year. The primary end point was defined as the patients whose rebleeds decreased from baseline by ≥ 50% at 12 months. Rebleeding was defined based on a positive fecal occult blood test (FOBT) (monoclonal colloidal gold color technology) at any visit after treatment. Secondary outcomes included the changes from baseline in participants dependent on blood transfusions and transfused packed red cell units, bleeding episodes, bleeding durations, and hemoglobin levels at 12 months. Statistical significance was defined at P < 0.05.
A New Approach in Laser Surgery Using the Regenerative Solution in Children Diagnosed With Vascular...
Vascular DiseasesVascular Malformation4 moreLaser treatment (LT) is the first-line treatment for Vascular Pathology. However even when LT is based on the selective photothermolysis it causes the first-degree burns. While being typically benign by affecting only the epidermis, or outer layer of skin, the burn site is remaining red, dry, and very painful. As Haemoblock contains nanoparticles of silver and is known for both bactericidal and bacteriostatic effects, it likely decreases the potential for infection postoperatively. Furthermore, after fibrin replaces the superficial structure "Hemoblock-albumin", the polyacrylate matrix is plasmolyzed which initiates the cascade of signals required for the tissue regeneration processes. Objective of the study was to examine the effect of the Regenerative Solution "Hemoblock" in lowering postoperative complications in children diagnosed with Vascular Pathology undergoing a laser surgery if delivered with transdermal patches.
Using Ferumoxytol-Enhanced MRI to Measure Inflammation in Patients With Brain Tumors or Other Conditions...
Brain InjuryCentral Nervous System Degenerative Disorder7 moreThis pilot clinical trial study will assess the inflammatory response of brain tumors or other central nervous system conditions in pediatric and adult patients using ferumoxytol-enhanced MRI. Imaging features will be correlated with the number of inflammatory cells (macrophages) at histopathology. Determining the extent of inflammation associated with pathologies in the central nervous system may be helpful for diagnostic and prognostic purposes as well as monitoring treatment response of current and future immunotherapies.
Pregnancy in Women With Rare Multisystemic Vascular Diseases: COGRare5 Study
Vascular AnomalyOsler Rendu Disease4 moreThere are no prospective studies of pregnancies for the diseases studied here in (Heredity Hemorrhagic Telangiectasia, Marfan syndrome, primary lower limb lymphedema, superficial arteriovenous malformations, and cerebro-spinal arteriovenous malformations) although complications of these can present life-threatening health problems for the mother and her baby. The purpose of this National prospective study is to obtain greater insight into obstetrical complications associated with rare maternal vascular genetic disorders in order to improve prevention and to reduce risk of death. In this context, experts and patient associations consider that there is a need to make real progress in the formulation of recommendations based on scientific data.
Safety of Apollo Micro Catheter in Pediatric Patients
Brain Arteriovenous MalformationBrain Vascular Malformations1 moreThis is a clinical trial/study for patients diagnosed with brain arteriovenous malformation (AVM). An AVM is an abnormal connection between the arteries and veins of the brain. Patients considered for this trial are 21 years and younger with AVM suitable for embolization treatment (a procedure used to block the abnormal connection between the arteries and veins of the brain) with Onyx Liquid Embolic System (Onyx LES) or TruFill n-Butyl Cyanoacrylate (n-BCA) Liquid Embolic System (both are liquid substances used in the embolization procedure to block the abnormal connection).
Vascular Malformations and Abnormalities of Growth
AbnormalityTo take part in this study you/your child have a vascular malformation, a type of blood vessel disorder whose cause is unknown. The investigators will do this by looking at changes in the genes and proteins in the cells of the malformation as well as normal cells. The investigators are doing this research because currently there is no known cause of vascular malformations and no way to know whether or not other health problems will occur in addition to the malformation. Through this research we hope to create standard methods for doctors to examine and treat people with vascular anomalies.
Vascular Anomaly Pathology and Genomics Biopsy Study
Vascular MalformationsVascular Anomaly5 moreThe purpose of this research is to gather information on the safety and effectiveness of core biopsy of vascular anomalies for clinical pathology and clinical genomics studies.