VTE at Unusual Sites (bsh 2012)
Cerebral Venous Sinus Thrombosis
1% of all strokes. 75% of cases are female.
Sx: headache, stroke-like symptoms, intracranial hypertension, haemorrhagic infarcts
Associations: Head/neck infection, asparaginase, cancer, áHb, áplt, COCP, obesity
Ix: MR Venography
Management:
7 days LMWH (ICH is not a contraindication)
Followed by
Warfarin for 3 months if provoked – low risk of recurrence
Warfarin for >3 months if unprovoked, persisting risks, persisting abnormal MRI or AT/Prot C/Prot S deficiencies.
Retinal Vein Occlusion (RVO)
Sx: Acute painless visual loss
Associations: Hypertension, Diabetes, hypercholesterolaemia
Management:
Routine anticoagulation not beneficial
Possible role for LMWH in acute central RVO
Laser therapy, intraocular steroids and antiangiogenics
Upper Extremity DVT (UEDVT)
10% of all DVT
Primary – Idiopathic, Thoracic Outlet Syndrome (e.g. 1st rib)
Secondary – CVC line, Plaster cast, Lemierre Syndrome (Jugular vein thrombosis in infection)
Ix: USS or venography
Outcome: Unprovoked cases - 2% recurrence in 5 years, compared to 19% for lower limb DVT
Management:
Optimal duration of treatment not known
3-6 months associated with reduced risk of recurrence
Consider 6 weeks for line-associated if line removed
SVC Thrombosis
Management:
Angioplasty and stenting if severe symptoms in a non-malignant case
Long term anticoagulation if risk factors persist
Consider radiotherapy in malignant cases
IVC Thrombosis
Ix: Look for IVCT in cases of bilateral DVT
Outcome: Natural history is of permanent blockage with growth of collaterals
Management:
Anticoagulate as for DVT
Consider catheter-delivered thrombolysis or endovascular surgery
Intra-Abdominal / splanchnic Vein Thombosis (SVT) (Portal, hepatic, mesenteric, splenic)
Site of Thrombosis
Portal Vein Thrombosis (PVT)
Male 2x as common as female.
Incidence increasing, ?more incidental diagnosis on imaging.
Worsens portal hypertension and GI bleeding common at diagnosis
High long-term risk of recurrence/bleeding
Most commonly associated with cirrhosis, solid organ malignancy and intrabdo inflammation - but MPN in up to 30% of cases (half of which may not have any other features of MPN at diagnosis (Blood 2012))
Other causes: AI disease, APS, PNH, pregnancy, hormonal therapies, heritable thrombophilia
Budd Chiari Syndrome (hepatic vein)
Pain + ascites + hepatomegaly
50% are MPN, 30% JAK2+
Mesenteric Vein
Most commonly 2o to bowel ischaemia / inflammation
Splenic Vein
Rare in isolation
Haem investigations for unexplained splanchnic thrombosis:
JAK2 + PNH
Not thrombophilia screen (does not change management)
Management Principle:
Anticoagulation improves recanalisation, reduces recurrence and bleeding in both cirrhotic and non-cirrhotic patients
Early OGD to check for varices if portal hypertension suspected
If long-term AC, need regular re-assessment of bleeding risk
AC considerations
LMWH?
The traditional, preferred choice
DOAC?
Off label
Beware contraindications of liver failure & GI bleeding risk.
But high rate of thrombus resolution in acute, non-cirrhotic SVT with a lower rate of bleeding
RIVASVT abstract 2021 - non-cirrhotic SVT treated with rivaroxaban, appears safe
Retrospective 2019 - DOAC = LMWH in non-cirrhotic PVT
Warfarin?
Potential difficulty monitoring INR in patient with cirrhosis
Choice / Length of AC
Cirrhosis-related SVT
LMWH preferred
3-6 months AC, consider indefinite treatment (DOAC/VKA) if bleeding risk acceptable
(Some will re-scan and base decision on degree of recanalisation - unlike usual DVT/PE practice)
Solid cancer-related SVT
LMWH or DOAC. LMWH preferred if intraluminal gastric or GU cancer.
Other cause SVT, including incidental diagnoses
DOAC preferred
3-6 months treatment and then re-assess
Long term AC if underlying MPN, PNH, Budd Chiari or other persistent provoking factors
Renal Vein Thrombosis
No routine investigation for thrombophilia
Anticoagulation depends on the underlying cause and risk of haemorrhage
Ovarian Vein Thrombosis
Postpartum OVT – treat with 3-6 months AC
Incidental OVT found after TAH-BSO does not require treatment
Penile Vein Thrombosis
No treatment required
Superficial Lower Limb Vein Thrombosis (SVT)
Ix: Rule out DVT
Outcome:
Management:
AC if within 3cm of the superficial femoral junction (SFJ)
LMWH for 30 days if SVT + risk factors for extension
10 days NSAIDs for other SVTs