- •In patients with spontaneous intracranial hypotension (SIH), cerebral venous thrombosis (CVT) has rarely been observed with an incidence of 1–2%.
- •The first-line therapy for CVT is anticoagulation. Epidural blood patch (EBP) is the main treatment option for SIH.
- •Anticoagulation potential benefit in CVT associated to SIH must be carefully evaluated taking account of the intracranial haemorrhage risk.
- •There is currently insufficient evidence to support definitive treatment guidelines of CVT in SIH.
- •Effective and prompt EBP, even without anticoagulation therapy, might lead to a good prognosis in selected SIH/CVT cases.
The occurrence of cerebral venous thrombosis (CVT) in patients with spontaneous intracranial hypotension (SIH) raises difficult practical questions regarding the management of the two conditions. The first-line therapy for CVT is anticoagulation (AC); however, its potential benefit in SIH/CVT patients, especially if complicated by subdural haematoma, must be carefully evaluated taking account of the intracranial haemorrhage risk. Venous system recanalization and good prognosis in SIH/CVT patients treated with epidural blood patch (EBP), the main treatment option for SIH, have been already described.
We reviewed our cases of SIH complicated by CVT among a cohort of 445 SIH patients observed and treated during the last years. All published case reports and case series reporting patients with SIH and CVT were also ascertained and reviewed.
Eight (2%) out of 445 patients suffering with SIH, were also diagnosed with CVT. All patients observed had orthostatic headache, three of them experienced a change in their headache pattern over the SIH course. Six out of eight patients received both AC and EBP treatments. Two patients were treated using only AC or EBP. A bilateral subdural haematoma enlargement after 1 month of AC was observed in one case. Complete CVT recanalization after treatment was obtained in three patients, including two with multiple CVT at baseline; partial CVT recanalization was achieved in two patients. Three patients experienced no CVT recanalization. After 6–48 months' follow-up all patients were still asymptomatic.
The use of AC therapy should be weighed against the intracranial haemorrage risk and should be monitored carefully if initiated. Effective and prompt EBP, even without AC therapy, might lead to a good prognosis in selected cases.
Abbreviations:cerebral venous thrombosis ((CVT)), spontaneous intracranial hypotension ((SIH)), anticoagulation ((AC)), epidural blood patch ((EBP)), low cerebrospinal fluid ((CSF)), International Classification of Headache Disorders 3rd edition ((ICHD-3)), numeric rating scale ((NRS)), computed tomography ((CT)), intravenous ((iv)), magnetic resonance imaging ((MRI)), magnetic resonance venography ((MRV)), dural artero-venous fistula ((DAVF)), Digital Subtraction Angiography ((DSA)), subdural hematomas ((SDH))
To read this article in full you will need to make a payment
Purchase one-time access:Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
One-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:Subscribe to Journal of the Neurological Sciences
Already a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
- Spontaneous intracranial hypotension.Continuum (Minneap Minn). 2015; 21: 1086-1108
- Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension.JAMA. 2006; 295: 2286-2296
- The status of diagnosis and treatment to intracranial hypotension, including SIH.J. Headache Pain. 2017; 18: 4
- Hyperprolactinemia due to spontaneous intracranial hypotension.J. Neurosurg. 2015; 122: 1020-1025
- Management of spontaneous intracranial hypotension during pregnancy: a case series.Headache. 2020; 60: 1777-1787
- The international classification of headache disorders, 3rd edition.Cephalalgia. 2018; 38: 1-211
- Spontaneous intracranial hypotension with brain sagging causing “prayer headache”.Rev. Neurol. (Paris). 2020; 177 (S0035-3787(20)30624-X): 321-323
- spontaneous intracranial hypotension syndrome with hearing loss and pachymeningeal enhancement in the internal acoustic canal: neuroimaging correlations.J. Craniofac. Surg. 2010; 21: 1660-1661
- Early pain relief from orthostatic headache and hearing changes in spontaneous intracranial hypotension after epidural blood patch.Acta Neurol. Belg. 2016; 116: 503-508
- Intracranial hypotension mimicking cervical radiculopathy.Eur. Neurol. J. 2011; 3: 46-47
- Spontaneous intracranial hypotension: review and expert opinion.Acta Neurol. Belg. 2020; 120: 9-18
- Early coma awakening after epidural blood patch.J. Clin. Neurosci. 2020; 71: 295-296
- Spontaneous intracranial hypotension.Curr. Pain Headache Rep. 2007; 11: 56-61
- Headaches due to low and high intracranial pressure.Continuum 24 (Minneap Minn). 2018; : 1066-1091
- Factors affecting cerebrospinal fluid opening pressure in patients with spontaneous intracranial hypotension.J Zhejiang Univ Sci B. 2018; 18: 577-585
- How common is normal cerebrospinal fluid pressure in spontaneous intracranial hypotension?.Cephalalgia. 2016; 36: 1209-1217
- Cerebral venous thrombosis in spontaneous intracranial hypotension.Headache. 2008; 48: 1511-1519
- Cerebral venous thrombosis in spontaneous intracranial hypotension: a report on 4 cases and a review of the literature.Headache. 2018; 58: 1244-1255
- Dural sinus thrombosis in spontaneous intracranial hypotension.J. Neurol. 2006; 253: 1197-1202
- Thrombosis of the cerebral veins and sinuses.N. Engl. J. Med. 2005; 353: 314-315
- Subdural hematoma in a patient with spontaneous intracranial hypotension and cerebral venous thrombosis.Neurol. Sci. 2010; 31: 669-672
- Mechanical thrombectomy in cerebral venous thrombosis: systematic review of 185 cases.Stroke. 2015; 46: 1263-1268
- Epidural blood patch in Trendelenburg position pre-medicated with acetazolamide to treat spontaneous intracranial hypotension.Eur. J. Neurol. 2010; 17: 715-719
- Lumbar epidural blood patch: effectiveness on orthostatic headache and MRI predictive factors in 101 consecutive patients affected by spontaneous intracranial hypotension.J. Neurosurg. 2019; 8: 1-9
- Cerebral venous thrombosis: an update.Lancet Neurol. 2007; 6: 162-170
- The Monro-Kellie hypothesis: applications in CSF volume depletion.Neurology. 2001; 56: 1746-1748
- May intracranial hypotension be a cause of venous sinus thrombosis?.Eur. Neurol. 2004; 51: 113-115
- In vivo detection of venous sinus distension due to intracranial hypotension in small animal using pulsed-laser-diode photoacoustic tomography.J. Biophotonics. 2020; 13e201960162
- New concept of cerebrospinal fluid dynamics in cerebral venous sinus thrombosis.Med. Hypotheses. 2008; 70: 143-147
- Diagnosis and management of cerebral venous thrombosis: a statement for healthcare professionals from the American Heart Association/American Stroke Association.Stroke. 2011; 42: 1158-1192
- Cerebral sinus venous thrombosis in two patients with spontaneous intracranial hypotension.Cerebrovasc. Dis. 2004; 17: 9-12
- Dural puncture and corticotherapy as 708 risk factors for cerebral venous sinus thrombosis.Eur. J. Neurol. 2000; 7: 123-124
- Delayed cerebral venous thrombosis in a patient with spontaneous intracranial hypotension.Cephalalgia. 2007; 27: 1176-1178
- Spontaneous intracranial hypotension. Clinical and neuroimaging findings in six cases with literature review.Eur. J. Radiol. 2009; 69: 253-259
- Cerebral venous thrombosis in spontaneous intracranial hypotension.Headache. 2008; 48: 1511-1519
- Postoperative sagittal sinus thrombosis after spinal anesthesia.Anesth. Analg. 1986; 65: 541-542
- Dural puncture headache suspected, cortical vein thrombosis diagnosed.Anesth. Analg. 1987; 66: 285
- Images from headache. Cerebral venous thrombosis complicating lumbar puncture.Headache. 2009; 29: 276-277
- Risk factors of cerebral vein and sinus thrombosis.Front. Neurol. Neurosci. 2008; 23: 23-54
- Sinus thrombosis in a patient with intracranial hypotension: a suggested hypothesis of venous stasis. A case report.Interv. Neuroradiol. 2011; 17: 248-251
- Delayed subdural hematoma and cerebral venous thrombosis in a patient with spontaneous intracranial hypotension.Neurol. Sci. 2011; 32: 981-983
- Cerebral venous thrombosis in two patients with spontaneous intracranial hypotension.Case Rep. Neurol. Med. 2014; 2014: 528268
- Spontaneous CSF leak complicated by venous thrombosis and dural arteriovenous fistula.Cephalalgia. 2005; 25: 751-753
- An interesting case of headache.Ann. Indian Acad. Neurol. 2011; 14: 130-132
- Spontaneous intracranial hypotension and venous sinus thrombosis.Pract. Neurol. 2013; 13: 120-124
- Cerebral venous thrombosis caused by spontaneous intracranial hypotension: two cases.Eur. J. Neurol. 2007; 14: 1296-1298
- Headache due to spontaneous intracranial hypotension and subsequent cerebral vein thrombosis.Headache. 2012; 52: 1592-1596
- Subdural haematoma in patients with spontaneous intracranial hypotension.Cephalalgia. 2007; 27: 133-138
- A case of convexity subarachnoid haemorrhage treated with epidural blood patch.Neurol. Sci. 2012; 33: 715-716
- Can spontaneous intracranial hypotension cause venous sinus thrombosis?.J. Headache Pain. 2007; 8: 200-201
- Association of cerebral venous thrombosis and intracranial hypotension: review of 3 cases.J. Stroke Cerebrovasc. Dis. 2017; 26: e165-e169
- Spontaneous occult intracranial hypotension precipitating life-threatening cerebral venous thrombosis: case report.J. Neurosurg Spine. 2018; 28: 669-678
- Spontaneous intracranial hypotension as a cause of subdural hematoma in a patient with cerebral venous thrombosis on anticoagulation treatment.J. Clin. Neurol. 2020; 16: 327-329
- My headache does not get better when I lie down: spontaneous intracranial hypotension complicated by venous thrombosis.Headache. 2008; 48: 149-152
- Spontaneous intracranial hypotension followed by dural sinus thrombosis: a case report.Clin. Neurol. Neurosurg. 2010; 112: 498-500
- Dural enhancement detected by magnetic resonance imaging reflecting the underlying causes of cerebral venous sinus thrombosis.Chin. Med. J. 2012; 125: 1513-1516
- Pachymeningeal enhancement on MRI: a venous phenomena not always related to intracranial hypotension (resolving pachymeningeal enhancement and cerebral vein thrombosis).Headache. 2013; 53: 673-675
- A case of cerebral venous thrombosis accompanying with intracranial hypotension: headache that changing character.Agri. 2013; 25: 141-144
- Spontaneous intracranial hypotension complicated by cerebral venous thrombosis.Radiol. Case Rep. 2018; 13: 834-838
- Spontaneous intracranial hypotension with isolated cortical vein thrombosis and subarachnoid haemorrhage.Cephalalgia. 2007; 27: 1413-1417
- Spontaneous intracranial hypotension associated with dural sinus thrombosis.Neurol. Med. Chir. (Tokyo). 2007; 47: 555-558
- Superior sagittal sinus thrombosis as a rare complication of spontaneous intracranial hypotension syndrome: a case report and review of the literature.Int. J. Neurosci. 2019; 129: 401-405
- Spontaneous intracranial hypotension is associated with a risk of venous sinus thrombosis and subdural hematoma.Cerebrovasc. Dis. 2007; 23: 315-317
- Cerebral venous thrombosis and subdural collection in a comatose patient: do not forget intracranial hypotension, A Case Report.Headache. 2020; 60: 2583-2588
- Spontaneous intracranial hypotension associated with cerebral venous thrombosis detected by a sudden seizure: a case report.JA Clin. Rep. 2020; 6: 59
- Cerebral venous thrombosis: an update.Curr. Neurol. Neurosci. Rep. 2019; 19: 74
- Deep cerebral venous thrombosis treatment.Clin. Neuroradiol. 2020; 30: 661-670
Published online: April 18, 2021
Accepted: April 16, 2021
Received in revised form: March 30, 2021
Received: January 14, 2021
© 2021 Elsevier B.V. All rights reserved.