What is the appropriate acute and subsequent management for a transgender woman on feminizing hormone therapy who presents with cerebral venous sinus thrombosis (CVST) complicated by intracranial hemorrhage (ICH)?

Management of Cerebral Venous Sinus Thrombosis with Intracranial Hemorrhage in a Transgender Patient

Initiate immediate anticoagulation with either intravenous unfractionated heparin or subcutaneous low-molecular-weight heparin, even in the presence of intracranial hemorrhage, as the hemorrhage is a consequence of the thrombosis itself and anticoagulation prevents further thrombus propagation and reduces mortality. 1, 2, 3

Acute Management Algorithm

Immediate Diagnostic Confirmation

• Obtain urgent MRI with MR venography (MRV) or CT with CT venography (CTV) to confirm CVST and assess extent of thrombosis and hemorrhage 1
• If MRI unavailable, multidetector CTV is acceptable as initial test in emergency setting 1
• Perform baseline severity assessment using National Institutes of Health Stroke Scale or Glasgow Coma Scale 1

Critical Decision Point: Anticoagulation Despite Hemorrhage

The presence of intracranial hemorrhage occurring as a consequence of CVST is NOT a contraindication to anticoagulation. 1, 2, 3 This is the most common pitfall—withholding anticoagulation due to hemorrhage on imaging is a critical error, as hemorrhagic venous infarction is an indication for, not against, anticoagulation. 2

Initial Anticoagulation Protocol

Preferred regimen: Low-molecular-weight heparin (LMWH) 2, 3

• Enoxaparin 1.0 mg/kg subcutaneously twice daily OR 1.5 mg/kg once daily 2
• Dalteparin 200 U/kg once daily 2

Alternative regimen: Unfractionated heparin (UFH) 2, 3

• Initial bolus: 5000 IU intravenous 2
• Continuous infusion: approximately 30,000 IU over 24 hours 2
• Adjust to maintain aPTT at 1.5-2.5 times baseline 2
• Use UFH when LMWH contraindicated, unavailable, severe renal failure (creatinine clearance <30 mL/min), or when thrombolytic therapy may be needed 2

Monitoring for Deterioration

Perform serial neurological assessments every 2-4 hours initially to detect: 2, 3

• Worsening level of consciousness
• New focal neurological deficits
• New or worsening seizures
• Signs of increased intracranial pressure

Obtain repeat CT head at 24-48 hours to assess for hematoma expansion or new hemorrhage 1

Management of Complications

If Patient Deteriorates Despite Adequate Anticoagulation

Consider escalation in this order: 1, 2

1. Endovascular therapy (mechanical thrombectomy with or without local thrombolysis) for patients with:

   • Absolute contraindications to anticoagulation 1
   • Failure of initial therapeutic anticoagulation 1
   • Progressive neurological deterioration despite adequate anticoagulation 2

2. Decompressive hemicraniectomy for patients with: 1, 2

   • Severe mass effect causing progressive neurological deterioration
   • Large intracerebral hemorrhage with midline shift
   • This is a lifesaving procedure in selected cases 1

Seizure Management

• Administer anticonvulsants if seizures occur 2, 3
• Consider prophylactic antiepileptics in patients with hemispheric lesions 4

Elevated Intracranial Pressure

• Manage elevated ICP aggressively with osmotic diuretics, hyperventilation if needed 5
• Avoid routine corticosteroids unless significant white matter edema causing mass effect (dexamethasone 4-8 mg/day oral or IV only in specific scenarios) 3

Transition to Oral Anticoagulation

Begin oral anticoagulation early while continuing parenteral therapy: 2, 3

• Continue parenteral anticoagulation minimum 5 days AND until INR ≥2.0 for at least 24 hours 2
• Target INR 2.0-3.0 (target 2.5) if using warfarin 2
• Duration of anticoagulation: minimum 3 months 2, 3

Duration Based on Etiology

• Provoked CVST (e.g., hormone therapy): 3-6 months 2, 3
• Unprovoked/idiopathic CVST: 6-12 months 1
• High-risk thrombophilia or recurrent events: Consider indefinite anticoagulation 1, 3

Specific Considerations for Transgender Patients

Discontinue feminizing hormone therapy immediately 3

• Estrogen-containing hormone therapy is a significant prothrombotic risk factor 6
• This is analogous to discontinuing oral contraceptives in cisgender women with CVST 3

Investigate underlying prothrombotic conditions: 3

• Obtain thrombophilia workup (Factor V Leiden, prothrombin gene mutation, antiphospholipid antibodies, protein C/S deficiency, antithrombin deficiency)
• This affects long-term anticoagulation duration 3

Future hormone therapy considerations:

• Transdermal estrogen may have lower thrombotic risk than oral formulations, but consultation with endocrinology is essential before any resumption
• Some patients may require lifelong anticoagulation if hormone therapy is deemed essential for quality of life

Follow-Up Protocol

Imaging surveillance: 1, 3

• Follow-up CT venography or MR venography at 3-6 months to assess recanalization 1, 3
• Note: Duration of anticoagulation is NOT based on presence or absence of recanalization 1

Clinical monitoring: 3

• Neurological assessment during first year
• Ophthalmological follow-up to monitor for visual loss from increased intracranial pressure 3
• Assess for cognitive and neurological sequelae requiring rehabilitation 3

Common Pitfalls to Avoid

1. Do NOT withhold anticoagulation because of hemorrhage on imaging—this is the most critical error 2, 3
2. Do NOT wait for hemorrhage to resolve before starting anticoagulation—immediate treatment is essential 1
3. Do NOT assume clinical improvement means anticoagulation can be shortened—minimum 3 months regardless of clinical course 2, 3
4. Do NOT restart hormone therapy without comprehensive thrombophilia evaluation and risk-benefit discussion 3