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)

ISTH Guideline 2020

 

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