A literature review demonstrated a wide variation of timing for anticoagulation initiation in patients with CVT and intracranial hemorrhage. Most started anticoagulation within 24 hours of admission with similar functional neurological recovery. Current guidelines on the treatment of CVT, even with intracranial hemorrhage, recommend anticoagulation. Most reports in the literature state initiation of anticoagulation within 24 hours. However, the literature does not definitively state when to initiate anticoagulation in a patient with CVT, intracranial hemorrhage, thrombectomy, and decompressive hemicraniectomy 2).
Given that there is usually an underlying cause for the disease, tests may be performed to look for these. The disease may be complicated by raised intracranial pressure, which may warrant surgical intervention such as the placement of a shunt.
There are several other terms for the condition, such as cerebral venous and sinus thrombosis, (superior) sagittal sinus thrombosis, dural sinus thrombosis and intracranial venous thrombosis as well as the older term cerebral thrombophlebitis.
● Contraindication to anticoagulation and/or anti-platelet therapy including hemorrhagic infarct 3).
● Impending risk of stroke.
Chemical Thrombolysis: A catheter may be advanced to the involved sinus or close to it, through the femoral vein. The advantage of local administration is that, a larger amount of tPA actually reaches the clot vs systemic administration through a peripheral vein. Usually, 2–5mg are administered through the thrombus and then an infusion started at a rate of 1 mg/hr, usually for 12 hours. If clot burden is still there on angiography, the infusion may be continued for longer, until the clot resolves.
For CST, the infusion may be prepared in a concentration of 1 mg/10 ml (0.1 mg/ml), for a rate of 10 ml/hr.
Mechanical Thrombolysis: Similar to arterial embolic stroke, devices such as Stentriever or Penumbra may be used for clot extraction. Additionally, devices intended for other sites e.g., clot extraction from dialysis fistula, have also been used in cranial sinuses 4).
The challenge during endovascular intervention is negotiating the sigmoid-transverse sinus junction especially when using bulkier catheters e.g., AngioJet.
Ding JY, Pan LQ, Hu YY, Rajah GB, Zhou D, Bai CB, Ya JY, Wang ZA, Jin KX, Guan JW, Ding YC, Ji XM, Meng R. Batroxobin in combination with anticoagulation may promote venous sinus recanalization in cerebral venous thrombosis: A real-world experience. CNS Neurosci Ther. 2019 May;25(5):638-646. doi: 10.1111/cns.13093. Epub 2019 Jan 23. PubMed PMID: 30675757; PubMed Central PMCID: PMC6488911.
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The natural history of untreated cerebral AVMs in children is worse than in adults, in relation to a longer life expectation, a higher annual risk of AVM bleeding (3.2% vs. 2.2%) and a higher incidence of posterior fossa and basal ganglia AVMs, most of which present with massive haemorrhages 8).
The management of pediatric bAVMs is particularly challenging. In general, the treatment options are conservative treatment, microsurgery, endovascular therapy (EVT), gamma kniferadiosurgery (GKRS), proton-beam stereotactic radiosurgery (PSRS), or a combination of the above.
In 2019 Meling et al., performed a systematic review, according to the PRISMA guidelines, with the result that none of the options seem to offer a clear advantage over the others when used alone. Microsurgery provides the highest obliteration rate, but has higher incidence of neurological complications. EVT may play a role when used as adjuvant therapy, but as a stand-alone therapy, the efficacy is low and the long-term side effects of radiation from the multiple sessions required in deep-seated pediatric bAVMs are still unknown. GKRS has a low risk of complication, but the obliteration rates still leave much to be desired. Finally, PSRS offers promising results with a more accurate radiation that avoids the surrounding tissue, but data is limited due to its recent introduction. Overall, a multi-modal approach, or even an active surveillance, might be the most suitable when facing deep-seated bAVM, considering the difficulty of their management and the high risk of complications in the pediatric population 9).
In 2016 El-Ghanem et al., published a Review of the Existing Literature:
Microsurgical resection remains the gold standard for the treatment of all accessible pediatric AVMs. Embolization and radiosurgery should be considered as an adjunctive therapy. Embolization provides a useful adjunct therapy to microsurgery by preventing significant blood loss and to radiosurgery by decreasing the volume of the AVM. Radiosurgery has been described to provide an alternative treatment approach in certain circumstances either as a primary or adjuvant therapy 10).
Intracranial haemorrhage is the presenting clinical manifestation in 75-80% of paediatric patients and is associated with a high morbidity and mortality 11).
A prospectively maintained database of children between January 1997 and October 2012 for bAVMs was retrospectively queried to identify all consecutive ruptured bAVMs treated by surgery, embolization, and radiosurgery. The impact of baseline clinical and bAVM characteristics on clinical outcome, rebleeding rate, annual bleeding rate, and bAVM obliteration was studied using univariate and multivariate Cox regression analysis.
One hundred six children with ruptured bAVMs were followed up for a total of 480.5 patient-years (mean, 4.5 years). Thirteen rebleeding events occurred, corresponding to an annual bleeding rate of 2.71±1.32%, significantly higher in the first year (3.88±1.39%) than thereafter (2.22±1.38%; P<0.001) and in the case of associated aneurysms (relative risk, 2.68; P=0.004) or any deep venous drainage (relative risk, 2.97; P=0.002), in univariate and multivariate analysis. Partial embolization was associated with a higher annual bleeding rate, whereas initial surgery for intracerebral hemorrhage evacuation was associated with a lower risk of rebleeding.
Associated aneurysms and any deep venous drainage are independent risk factors for rebleeding in pediatric ruptured bAVMs. Immediate surgery or total embolization might be advantageous for children harboring such characteristics, whereas radiosurgery might be targeted at patients without such characteristics 12).
Meling TR, Patet G. What is the best therapeutic approach to a pediatric patient with a deep-seated brain AVM? Neurosurg Rev. 2019 Apr 13. doi: 10.1007/s10143-019-01101-8. [Epub ahead of print] Review. PubMed PMID: 30980204.
Because of the rarity of the disease and the existence of asymptomatic
patients, establishing a true prevalence rate is not feasible. Owing to
variation in the detection rate of asymptomatic AVMs, the most reliable
estimate for the occurrence of the disease is the detection rate for
symptomatic lesions: 0.94 per 100,000 person-years (95% confidence interval,
0.57-1.30/100,000 person-years). This figure is derived from a single
population-based study, but it is supported by a reanalysis of other
data sources. The prevalence of detected, active (at risk) AVM disease
is unknown, but it can be inferred from incidence data to be lower than
10.3 per 100,000 population.
AVMs account for between 1 and 2% of all strokes, 3% of strokes in young
adults, 9% of subarachnoid haemorrhages and, of all primary
intracerebral haemorrhages, they are responsible for 4% overall, but for
as much as one-third in young adults. AVMs are far less common causes
of first presentations with unprovoked seizures (1%), and of people
presenting with headaches in the absence of neurological signs (0.3%).
At the time of detection, at least 15% of people affected by AVMs are
asymptomatic, about one-fifth present with seizures and for
approximately two-thirds of them the dominant mode of presentation is
with intracranial haemorrhage. The limited high quality data available
on prognosis suggest that long-term crude annual case fatality is
1-1.5%, the crude annual risk of first occurrence of haemorrhage from an
unruptured AVM is approximately 2%, but the risk of recurrent
haemorrhage may be as high as 18% in the first year, with uncertainty
about the risk thereafter. For untreated AVMs, the annual risk of
developing de novo seizures is 1%. There is a pressing need for large,
prospective studies of the frequency and clinical course of AVMs in
well-defined, stable populations, taking account of their prognostic
According to reports, 0.1% of the population harbors an AVM
Both sexes are affected equally. AVMs are the leading cause of
nontraumatic intracerebral hemorrhage in people less than 35 years old
Most lesions reach attention in patients in their 40’s and 75% of the hemorrhagic presentations occur before the age of 50 years
According to autopsy studies, only 12% of AVMs become symptomatic during life
They are the most frequently encountered structural cause of spontaneous
intracerebral hemorrhage in childhood, excluding hemorrhages of
AVMs are seen more frequently on MRI with advancing age in children and young adults
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