From the Guidelines
Portal venous sinus disorder treatment should prioritize anticoagulation therapy with low molecular weight heparin, such as enoxaparin 4000 IU daily, to prevent the occurrence of portal vein thrombosis (PVT) and its complications, as recommended by the EASL clinical practice guidelines 1.
Treatment Overview
The treatment of portal venous sinus disorder, also known as portal vein thrombosis (PVT), aims to prevent the progression of thrombosis, promote recanalization of the portal vein, and manage complications such as portal hypertension and variceal bleeding.
- Anticoagulation therapy is the cornerstone of treatment, with low molecular weight heparin (LMWH) being the preferred initial treatment, followed by oral anticoagulants such as warfarin or direct oral anticoagulants like rivaroxaban 1.
- The duration of anticoagulation therapy varies from 3-6 months for provoked cases to indefinite therapy for unprovoked or recurrent thrombosis 1.
- Patients should be monitored regularly for bleeding complications and efficacy through blood tests, and lifestyle modifications such as avoiding alcohol, maintaining a healthy weight, and managing underlying conditions are important adjunctive measures 1.
Anticoagulation Therapy
- Low molecular weight heparin (LMWH) is recommended as the initial anticoagulant therapy, with a dose of 4000 IU daily, as shown to be effective in preventing PVT without increasing bleeding complications 1.
- Oral anticoagulants such as warfarin, with a target INR of 2-3, or direct oral anticoagulants like rivaroxaban, with a dose of 15-20mg daily, can be used for long-term anticoagulation therapy 1.
- The choice of anticoagulant and duration of therapy should be individualized based on the patient's risk factors, underlying conditions, and response to treatment 1.
Management of Complications
- Portal hypertension and variceal bleeding can be managed with beta blockers, endoscopic variceal band ligation, or sclerotherapy, according to the guidelines for cirrhosis 1.
- TIPS (transjugular intrahepatic portosystemic shunt) can be considered in patients with refractory variceal bleeding or portal hypertensive gastropathy 1.
- Surgical portosystemic shunting or mesenterico-Rex shunt can be considered in selected patients with extrahepatic portal vein obstruction (EHPVO) 1.
From the Research
Portal Sinus Venous Disorder
- The term "portal sinus venous disorder" is not a standard medical term, but it appears to be related to veno-occlusive disease (VOD) or sinusoidal obstruction syndrome (SOS) 2, 3.
- VOD/SOS is a potentially life-threatening complication of hematopoietic stem cell transplantation (HSCT) and can also occur in other settings, such as after oxaliplatin-containing chemotherapy for colorectal cancer 3.
- The diagnosis of VOD/SOS is based on clinical criteria, including jaundice, right upper-quadrant pain, and ascites, and can be confirmed by liver biopsy or imaging studies 2, 3.
Risk Factors
- Several risk factors have been identified for the development of VOD/SOS, including:
- Pre-existing hepatic disease 2, 4
- Second myeloablative transplant 2
- Allogeneic transplant for leukemia beyond second relapse 2
- Conditioning with busulfan-containing regimens 2
- Prior treatment with gemtuzumab ozogamicin 2, 4
- Diagnosis of primary hemophagocytic lymphohistiocytosis, adrenoleucodystrophy, or osteopetrosis 2
- Acute kidney injury, increased international normalized ratio, female sex (in children), and platelet refractoriness 4
Treatment and Prevention
- Defibrotide is recommended for the prevention and treatment of VOD/SOS in adults and children 2, 3.
- Ursodeoxycholic acid may be used for prophylaxis 2, 3.
- Anticoagulant therapy, such as direct oral anticoagulants or vitamin K antagonists, may be used to prevent and treat venous thromboembolism, including portal vein thrombosis 5, 6.
- Liver transplantation may be considered in severe cases of VOD/SOS 3.