Sildenafil for the Treatment of Lymphatic Malformations
Lymphatic MalformationsLymphatic DiseasesA Phase 2 study to evaluate safety and efficacy of sildenafil taken orally to improve or resolve lymphatic malformations in children. Subjects may receive either placebo or treatment in an oral dosage with an open label extension for subjects who received placebo. The study treatment assignment will be randomized in a double blind fashion. MRI examination will evaluate change in lesion volume due to treatment. Other safety and efficacy measures will be taken through the 32-week study duration. Funding Source - FDA OOPD
Safety and Efficacy Study of Sirolimus in Complicated Vascular Anomalies
Kaposiform HemangioendotheliomasTufted Angioma7 moreThe purpose of this study is to determine if the use of sirolimus in the treatment of children and young adults with complicated vascular anomalies will prove to be safe and provide objective response resulting in improved clinical status and quality of life. Funding Source - FDA OOPD (Food and Drug Administration - Office of Orphan Products Development)
Airway Vascular Lesions
HemangiomasVenous Malformations1 moreControversy exists in the treatment of airway hemangiomas ranging from tracheotomy, various lasers, to open removal, none of which have proven to be a gold standard. Venous malformations of the airway are difficult to treat and also require laser therapy or open removal and often repeated treatments are required. Similarly airway lymphatic malformations can require multiple treatment modalities as well as multiple procedures and are infrequently eradicated when extensive. New uses for various lasers and differing treatment protocols are frequently developed. Review of the treatment modalities and efficacy of these methods is required. The goal of this research is to determine protocols for treatment of airway vascular malformation and to evaluate our results from treatment of these lesions.
Diagnosis of Hemangiomas and Vascular Malformations
HemangiomasVascular Malformations4 moreTo develop a non-invasive, simple and affordable, measurement technique to differentiate hemangiomas from subtypes of VMS. The purpose of this study is to determine the best office based diagnositc instrument for differentiating the subtype of vascular lesions. The instrument must be simple and affordable so it can be used by primary car physicians in the doctor office. The procedure will be based on non-invasive and unharmful measurements that will be compared to the diagnosis from specialized physicians in this field and the final pathology.
Lymphatic Function in Patients With a Fontan Circulation From Brazil
Univentricular HeartLymphatic Abnormalities1 moreIt is suggested that the elevated central venous pressure in patients with a Fontan circulation can provoke manifestation of lymphatic dysfunction, met as plastic bronchitis, peripheral oedema and most feared protein-losing enteropathy (PLE). An explorative study from our department at Aarhus University Hospital revealed that the function of the lymphatic vasculature in 10 young Fontan patients with no complications was abnormal compared to healthy controls. However, to further describe and confirm these findings we had to investigate the lymphatic circulation in a larger, older and more complicated group of Fontan patients. The hypothesis is that, patients with a univentricular circulation have a reduced functionality of the lymphatic vasculature and which predisposes them to developing complications such as edema and PLE.
Lymphatic Morphology of Fontan Patients
Lymphatic AbnormalityPlastic Bronchitis3 moreThe Fontan procedure has revolutionized the treatment of patients born with a congenital univentricular heart defect. However, over time, it is associated with severe lymphatic complications such as plastic bronchitis, protein-losing enteropathy (PLE) and peripheral edema. The hypothesis is that patients with a univentricular circulation have a changed morphology which may be associated with both the degree of lymphatic complications and their physical capacity. The morphology will be described using T2-weighted non-contrast MRI.
Lymphatic Function in Patients With a Fontan-Kreutzer Circulation
Lymphatic AbnormalitiesLymphatic Edema1 moreThe lymphatics regulate the interstitial fluid by removing excessive fluid. It represents an extremely important step in the prevention of edema. The Fontan-Kreutzer procedure has revolutionized the treatment of univentricular hearts. However, it is associated with severe complications such as protein-losing enteropathy (PLE) and peripheral edema that may involve the lymphatic circulation. Our hypothesis is that patients with a univentricular circulation have a reduced functionality of the lymphatic vasculature, which predisposes them to developing complications such as edema and PLE. The functional state of lymphatics is investigated using near infrared fluorescence imaging, NIRF. The anatomy is described using non-contrast MRI and the capillary filtration rate is measured using plethysmography.