Abstract
Cerebral vein thrombosis (CVT) and splanchnic vein thrombosis (SVT) are two manifestations of venous thromboembolism (VTE) at unusual sites. They have an incidence at least 25–50 times lower than usual site VTE, but represent true clinical challenges. Recent evidence on the epidemiology, risk factors, prognosis, and treatment of CVT and SVT has been published in the last two decades, thus contributing to a better understanding of these diseases. The improvement in imaging techniques and a higher degree of clinical suspicion may have led to the observed increased frequency, whereas a better knowledge of provoking mechanisms could have contributed to reducing the proportion of events classified as unprovoked or idiopathic (13–21% of CVT, 15–27% of SVT). Few small randomized clinical trials and a number of observational studies, although hampered by heterogeneous therapeutic approaches, shed light on the safety and effectiveness of anticoagulant therapy in these populations. However, there are still some grey areas that warrant future research. In this narrative review, we discuss recent advances and therapeutic challenges in CVT and SVT.
Highlights
Venous thromboembolism (VTE) can potentially occur in any venous segment
The Cerebral Vein Thrombosis International Study (CEVETIS) included 706 cerebral vein thrombosis (CVT) patients, of whom 85% were treated with heparin in the acute phase and 84% were prescribed with vitamin K antagonist (VKA) for a median duration of 12 months [3]
splanchnic vein thrombosis (SVT) refers to thrombosis occurring in the veins draining the abdominal viscera, and includes portal vein thrombosis (PVT), mesenteric veins thrombosis (MVT), splenic vein thrombosis, and Budd–Chiari syndrome (BCS) [47]
Summary
The most common disorders are represented by deep vein thrombosis (DVT) of the lower limbs and pulmonary embolism (PE). Unusual manifestations of VTE include DVT of the upper extremities, and splanchnic, renal, ovarian, cerebral, and retinal veins thrombosis. Unusual site VTE has an incidence at least 25–50 times lower than usual site VTE, but often represent a true clinical challenge. There is paucity of large clinical trials in the literature and, solid evidence to drive patient management. In this narrative review, we will discuss recent advances and therapeutic challenges in cerebral vein thrombosis (CVT) and splanchnic vein thrombosis (SVT), starting from the presentation of two clinical cases
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