From the Guidelines
Portal sinus venous disorder, also known as portal vein thrombosis (PVT), should be treated with immediate anticoagulation using low molecular weight heparin (LMWH) in the absence of major contraindications to anticoagulation. This approach is based on the recommendations from the European Association for the Study of the Liver (EASL) clinical practice guidelines for vascular diseases of the liver 1. The guidelines suggest that anticoagulation therapy should be initiated as soon as possible, with LMWH being the preferred initial treatment, and oral anticoagulants such as warfarin or direct oral anticoagulants (DOACs) being used for long-term anticoagulant treatment.
Key Recommendations
- Initiate immediate anticoagulation with LMWH in the absence of major contraindications to anticoagulation 1
- Use Doppler ultrasound as the first line investigation for acute PVT, and CT for diagnostic confirmation and assessment of extension 1
- Establish or rule out underlying cirrhosis or obliterative portal venopathy 1
- Consider intestinal infarction in patients with persisting severe abdominal pain, rectal bleeding, moderate or massive ascites, or multiorgan dysfunction 1
- Screen for gastroesophageal varices in unrecanalised patients 1
Treatment Duration and Monitoring
- Anticoagulation therapy should be given for at least 6 months 1
- Perform a CT scan to assess recanalisation of the portal venous system at 6-12 months follow-up 1
- Regular ultrasound monitoring is essential to assess recanalization
- Lifestyle modifications include avoiding alcohol, maintaining hydration, and prompt treatment of infections
Complications and Management
- Complications include portal hypertension, esophageal varices, and ascites, which require specific management
- Patients should be evaluated for underlying liver disease, prothrombotic disorders, or abdominal inflammation
- Early diagnosis and treatment are crucial to prevent progression to chronic portal hypertension and its complications 1
From the Research
Portal Sinus Venous Disorder
- Portal sinus venous disorder, also known as cerebral sinus venous thrombosis (CSVT), is a relatively rare neurological condition that can be potentially fatal 2.
- The condition is characterized by the formation of a blood clot in the cerebral sinuses, which can lead to increased intracranial pressure, cerebral edema, and even death 3.
- The diagnosis of CSVT is often made using magnetic resonance imaging (MRI) with magnetic resonance venogram (MRV), which is considered the gold standard for diagnosis 2.
Causes and Risk Factors
- The causes of portal sinus venous disorder are multifactorial, and can include hypercoagulable states, trauma, infection, and certain medical conditions such as liver cirrhosis and hepatic or pancreatic malignancy 4, 5.
- Porto-sinusoidal vascular disease (PSVD) is a novel clinical entity characterized by typical histological changes with or without portal hypertension (PH) in the absence of cirrhosis, and has been proposed as a distinct clinical entity 5, 6.
Treatment and Management
- Anticoagulation with heparin or low-molecular-weight heparin is the mainstay of treatment for CSVT, and endovascular management is indicated for those cases with severe symptoms or worsening of symptoms despite anticoagulation therapy 3, 2.
- Thrombectomy and thrombolytic therapy may also be used in certain cases, and the choice of treatment depends on the individual patient's condition and the severity of the disorder 3, 4.
- Early diagnosis and treatment are crucial in reducing morbidity and mortality, and improving outcomes in patients with portal sinus venous disorder 2.