Neonatal cerebral sinovenous thrombosis

doi:10.1016/B978-0-444-64029-1.00012-6

Handbook of Clinical Neurology

Volume 162, 2019, Pages 267-280

https://doi.org/10.1016/B978-0-444-64029-1.00012-6 Get rights and content

Abstract

Cerebral sinovenous thrombosis (CSVT) is a focal or diffuse disruption of cerebral blood flow secondary to occlusion of cerebral veins and/or sinuses. The challenge of CSVT during the neonatal age has led to a great interest in this condition among neonatologists, child neurologists, and pediatric neuroradiologists. The highly variable clinical spectra, etiologies, and prognosis require fine medical skills and a high level of suspicion. Nevertheless, the diagnosis is often delayed or missed altogether. Differences in brain vulnerability at different stages of maturation may explain the spectrum of associated brain lesions, which varies with gestational age. Treatment is controversial and reported clinical outcomes vary widely. The controversial treatment of CSVT with anticoagulant therapy is based only on case series and expert consensus, there is lack of safety data.

Section snippets

Anatomy of Veins

The veins of the brain have no muscular tissue in their thin walls and possess no valves. They emerge from the brain and lie in the subarachnoid space. They pierce the arachnoid and the meningeal layer of the dura and drain into the cranial venous sinuses. The cerebral venous system can be divided into a superficial and a deep system. The superficial system comprises sagittal sinuses and cortical veins, which drain superficial surfaces of both cerebral hemispheres. The deep system consists of

Incidence

The estimated annual incidence of CSVT is 3–4 cases per 2 million adults and 7 cases per 1 million neonates, even though its incidence is not known accurately because epidemiologic studies are scant (Martinelli et al., 2010).

The yearly incidence of pediatric CSVT is 0.67 cases per 100,000 children, while it ranges from 2.6 to 12/100,000 term neonates/year. Neonates are at a high risk for this condition and account for 18%–51% of all reported pediatric cases (deVeber et al., 2001). The incidence

Risk factors

Risk factors for the development of CSVT may be maternal and/or fetal and neonatal, but both conditions are likely to result from a combination of predisposing factors.

As in any thrombotic process, risk factors are associated with the classical Virchow triad of thrombogenesis: hypercoagulability, vessel wall damage, and blood stasis (Saposnik et al., 2011; Jia et al., 2012). It may be associated with inherited and acquired risk factors; however, this categorization is fairly artificial, because

Clinical Signs

Clinical data of CSVT is extraordinarily variable depending on different factors, such as the location and extension of the thrombosis, the extent of the venous occlusion, patient age, and the nature of the underlying disease or predisposing cause. It also seems reasonable to consider that the velocity of clot formation within venous vessels and the related complications may influence the different symptoms.

One of the most frequent clinical presentations is neonatal encephalopathy, not to be

Neonatal CSVT and Hypothermia for Asphyxiated Infants

Early presentation of symptoms of CSVT (even at birth) may suggest the possibility for a fetal origin and a relationship with birth asphyxia (Barron et al., 1992; Ramenghi et al., 2009). The extent of the problem could be underestimated because the clinical signs and symptoms are similar to those of hypoxic–ischemic encephalopathy. Neonatal CSVT more often occurs in the superficial venous system of the brain. Theoretically, slowing of blood flow in the cranial venous sinuses may be caused by

Difference Between Preterm and Term Babies With CSVT

A different spectrum of associated brain lesions could be recognized in a large group of neonates with CSVT, depending on their gestational age at the time of developing CSVT.

Extensive white matter lesions throughout the periventricular white matter were the predominant pattern of injury associated with CSVT in preterm infants, while an IVH associated with a predominantly unilateral thalamic hemorrhage and periventricular white matter lesions (PWMLs) were most often seen in the full-term

Imaging

Imaging in CSVT plays two main roles: detection of sinus thrombosis and identification of associated lesions. The diagnosis must be established rapidly, as early treatment can dramatically reduce further development or propagation of the thrombus (Moharir et al., 2011). However, direct signs of CSVT may be difficult to confirm with US with a high false-negative rate (Nwosu et al., 2008) and experience is required in the use of Doppler to assess venous sinus flow (Govaert et al., 2009). US may,

Treatment

The controversy surrounding treatment of neonatal CSVT with anticoagulant therapy arises from the following concerns:

(1)
lack of safety data,
(2)
occurrence of spontaneous intracranial hemorrhage,
(3)
difficulty in deciding the duration of anticoagulant therapy, and
(4)
absence of long-term randomized controlled trial (RCT) studies.

The majority of recommendations are based on case series and expert consensus (Cnossen et al., 2009).

Only recent reports support the safety of anticoagulant therapy in neonatal CSVT (

Propagation

Thrombus propagation is a frequent event (1 in 3) in the acute phase in anticoagulant therapy-untreated neonates and appears to increase the risk of brain injury; 40% of neonates who propagated their thrombus developed new venous infarction. Predictors of propagation included lack of anticoagulant therapy and absence of perinatal risk factors, while a trend was noted for multiple sinus involvement at diagnosis and propagation. Gender and hypercoagulable state did not predict propagation.

Outcomes

There is limited and discordant literature regarding outcomes and predictors of outcomes in neonates with CSVT. Furthermore, lack of standardized neurodevelopmental follow-up influence outcome data, but the most likely explanation is the spectrum of lesions sustained. Rates of impairment are reported as approximately 50% (Golomb, 2009; Yang et al., 2010).

Mortality in the Berfelo cohort (19%) is higher than previously reported and may reflect better identification of the most severely affected

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