rivaroxaban and Vascular-Malformations

Low-flow vascular malformations are rare congenital anomalies due to errors in vascular development and may be associated with known pathogenic genetic variants. Slow flow through the blood vessels can lead to localized intralesional thromboses, which can cause debilitating pain and impair quality of life. We present a case of venous malformation due to a variant in the TEK gene in a 38-year-old woman in whom treatment with low dose rivaroxaban was successful in controlling symptoms of chronic localized intravascular coagulation.

Topics: Adult; Female; Humans; Pain; Quality of Life; Rivaroxaban; Vascular Malformations

Orbital venolymphatic malformations are rare vascular malformations that typically appear early in life and harbor acute and chronic threats to vision. Historically, there are four categories of management: observation, medication, sclerotherapy, and surgery. Currently, there is neither a gold standard for treatment nor randomized control trials comparing treatments.The authors present a 20-year-old male who presented with spontaneous hemorrhage of an orbital venolymphatic malformation occurring with increased frequency and involving more of the posterior orbit. Surgery and sclerotherapy were not feasible options due to the extensive intraorbital and intracranial involvement of the venolymphatic malformation. Systemic steroids treated symptoms but was not curative. To this end, a combination of sirolimus, an mTOR inhibitor, and rivaroxaban, a factor Xa inhibitor, were used to reduce the size of the lesion and minimize the risk of thromboembolic events. This treatment has successfully kept the patient's symptoms in remission for greater than 2 years.

Topics: Adult; Humans; Male; Orbital Diseases; Rivaroxaban; Sclerotherapy; Sirolimus; Vascular Malformations; Young Adult

Slow-flow vascular malformations (VM) can be associated with localized intravascular coagulopathy (LIC) that is characterized by elevated D-Dimer levels and low fibrinogen and platelets. This can lead to bleeding and clotting tendencies, which can give rise to functional limitations such as pain and swelling and even progress to disseminated intravascular coagulopathy.. We conducted a chart review of four patients with evidence of LIC who were started on rivaroxaban. We found an improvement of D-Dimer and/or fibrinogen levels in all four patients. They also had an improvement of pain and functionality.. We report on four patients in whom anticoagulation with a direct oral anticoagulant, rivaroxaban, was effective in controlling signs and symptoms of consumptive coagulopathy with no evidence of bleeding from the use of rivaroxaban.

Topics: Administration, Oral; Adolescent; Adult; Anticoagulants; Blood Coagulation Disorders; Blood Flow Velocity; Factor Xa Inhibitors; Female; Fibrin Fibrinogen Degradation Products; Fibrinogen; Humans; Male; Pain; Rivaroxaban; Treatment Outcome; Vascular Malformations; Young Adult

The source of GI bleeding may elude us despite exhaustive testing in some cases. Bleeding in these cases is often related to a vascular lesion that is discernible only when actively bleeding. The objective of this study was to determine the efficacy and safety of endoscopy combined with the administration of antiplatelet and/or anticoagulant agents to stimulate bleeding in order to define a source.. A retrospective review of a database of device-assisted enteroscopy (DAE) procedures was completed to identify cases in which provocation with antiplatelet or anticoagulant agents was used as part of a GI bleeding evaluation. Procedures were divided into 3 groups based on the method of provocation: patients with a history of bleeding associated with an antiplatelet/anticoagulant (provocation-experienced); patients naïve to these medications (provocation-naïve); and cases of recurrent, overt GI bleeding in which a combination of clopidogrel and intravenous heparin was administered for provocation (Lousiana State University [LSU] protocol).. A review of 824 DAE procedures was completed to identify a total of 38 instances in which provocation was attempted in 27 patients. These cases were subdivided into 13 provocation-experienced procedures, 18 provocation-naïve procedures, and 7 LSU protocol procedures. The diagnostic yield of provocative testing per procedure was 53% in the provocation-experienced group, 27% in the provocation-naïve group, and 71% in the full protocol group. Provocative testing was revealing in 15 of 27 patients; angioectasias and Dieulafoy lesions were the most common pathologies. Provocative testing was not beneficial in 4 patients who were eventually diagnosed with bleeding caused by intestinal angioectasias (3) and an aorto-enteric fistula (1). There were no adverse events.. Provocative testing combined with endoscopy can be justified as an option in the diagnostic algorithm of complex cases of GI bleeding when intermittent bleeding related to a vascular lesion, such as an angioectasia or Dieulafoy, is suspected. However, this novel technique should be considered only after standard management has failed to define a bleeding source, and bleeding continues to recur. This is the first reported case series of provocative testing combined with endoscopy.

Topics: Aged; Aged, 80 and over; Anticoagulants; Balloon Enteroscopy; Clopidogrel; Female; Gastrointestinal Hemorrhage; Heparin; Humans; Male; Middle Aged; Platelet Aggregation Inhibitors; Retrospective Studies; Rivaroxaban; Ticlopidine; Vascular Malformations

Topics: Computed Tomography Angiography; Embolization, Therapeutic; Factor Xa Inhibitors; Female; Gastrointestinal Hemorrhage; Humans; Melena; Middle Aged; Pulmonary Disease, Chronic Obstructive; Risk Factors; Rivaroxaban; Treatment Outcome; Vascular Malformations; Venous Thrombosis

The localized activation of coagulation in vascular malformations can lead to a consumptive coagulopathy characterized by elevated D-dimers and a consumption of fibrinogen and platelets, eventually giving rise to a bleeding tendency. By reducing coagulation activation, anticoagulant treatment with heparin is able to limit this haemostatic dysregulation and the associated bleeding diathesis. Here, we present a case of a consumptive coagulopathy due to a large venous malformation with a sustained correction of the fibrinogen depletion and associated bleeding tendency both with subcutaneous enoxaparin and with the oral factor Xa inhibitor rivaroxaban.

Topics: Administration, Oral; Adult; Disseminated Intravascular Coagulation; Factor Xa Inhibitors; Fibrin Fibrinogen Degradation Products; Humans; Male; Morpholines; Rivaroxaban; Thiophenes; Vascular Malformations