Original ArticleClinical Profile and Long-Term Outcome in Neonatal Cerebral Sinus Venous Thrombosis
Introduction
Cerebral sinus venous thrombosis (CSVT) arises from a combination of venous stasis, blood vessel wall injury, and hypercoagulability, factors that are together known as the Virchow triad. A disruption in the Virchow triad can lead to occlusion of cerebral veins and sinuses.1 The incidence of pediatric CSVT is 0.67/100,000 children and ranges from 2.6 to 12 per 100,000 neonates per year.1,2 Neonates make up 50% of all pediatric cases and suffer substantial morbidity and mortality.2, 3, 4, 5, 6, 7, 8, 9, 10 CSVT risk factors include a combination of maternal, fetal, and neonatal factors.1 In 61% to 84% of neonates with CSVT, an acute systemic illness is a precedent.10 Clinical signs of CSVT are often nonspecific. However, seizures, encephalopathy,1,11 respiratory failure, abnormal tone and reflexes, poor feeding, and dehydration1,7,8,12,13 are all common presenting symptoms.
CSVT is associated with both short- and long-term morbidity and mortality. Survivors frequently develop epilepsy, sensorimotor impairments, and visual impairments.1,14,15 Neurodevelopmental and cognitive impairments are common, and neuropsychological testing is abnormal in 87% of CSVT survivors.3,16
Despite several studies examining the role of anticoagulation,8,14,15,17, 18, 19, 20 the treatment of neonatal CSVT remains controversial for a variety of reasons, including lack of randomized controlled trials. Consequently, it is essential to identify specific neurological prognostic factors to help guide management decisions to improve the long-term outcomes of patients with neonatal CSVT. To evaluate such factors, we performed a long-term retrospective cohort study of patients with neonatal CSVT at a single center.
Section snippets
Patient identification
This was a retrospective cohort study conducted at Texas Children's Hospital (TCH). Institutional Review Board approval by Baylor College of Medicine (H-39598) was obtained. Patients included term and preterm infants with diagnosis of CSVT between 2000 and 2018 and diagnosed with the following International Classification of Diseases (ICD)-9 and ICD-10 codes for any type of cerebral venous thrombosis: G08, G95.1, I63.6, I67.6, I82.8, I82.90, I82.91, I82.C, I82.C1, I82.C2, I82.C11, I82.C12,
Neonatal CSVT patient cohort selection and characteristics
A total of 67 neonatal patients with radiologically confirmed CSVT met the study's inclusion criteria (Fig 1). Of 67 patients, 46 were male and 21 were female. The majority of patients were white (Table 1), of which 24 were Hispanic. A total of 75% of patients were born at term, and 63% were born via spontaneous vaginal delivery. The age of presentation and reasons for presentation at the time of diagnosis were variable among the cohort (Table 2). Of 67 patients, seizures were the most common
Discussion
We present a cohort of 67 patients with neonatal CSVT and identified factors statistically associated with both favorable and unfavorable long-term neurological outcomes as determined by all-cause death, death due to CSVT, and PSOM score.
Neonates are at high risk for CSVT due to maternal and fetal/neonatal factors. Maternal factors implicated in CSVT include preeclampsia and diabetes.1 Transplacental transfer of circulating maternal antiphospholipid antibodies can persist into the newborn
Conclusion
Despite medical advances, neonatal CSVT remains a serious condition with a high morbidity and mortality. Important conclusions of this study were the that comorbid medical conditions, neurological examination at initial presentation, neurological examination at presentation, location of venous thrombosis, and type of cerebral injury all had an impact on long-term neurological outcome in neonatal CSVT whereas duration and type of treatment did not show an association. Therefore, further studies
Acknowledgments
We thank the families of the patients included in this study.
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2022, Seminars in Pediatric NeurologyCitation Excerpt :Although infrequently attributable to the CSVT, mortality is reported, particularly if associated with ICH.1,- –7,11,12,14-19,28,43,44,75 Most cohorts, pediatric and neonatal, show that approximately 1/3-2/3 of children have some neurological abnormality at discharge from hospital and long term (when data was available).1,3-6,9-12,14-19,43,44,75-78 It is however unclear if anticoagulants directly improve long-term clinical outcomes.
Neuroimaging of Neonatal Stroke: Venous Focus
2024, Radiographics
Statement of Ethics: The study received Institutional Review Board approval by Baylor College of Medicine (H-39598).
Conflict of Interest Statement: The authors have no conflicts of interest to declare.
Funding Sources: D.P. is supported by International Rett Syndrome Foundation (IRSF grant #37011) and Clinical Research Training Scholarship in Neuromuscular Disease partnered by the American Brain Foundation (ABF) and Muscle Study Group (MSG).
Author Contributions: I.H. wrote the manuscript and interpreted the data. C.K. interpreted and collected data. T.A.W. performed the statistical analyses. S.F.K. reviewed all imaging data. D.S.T., K.F., and E.A.E. performed data collection. J.J.R. and G.D.C. provided feedback on study design and interpretation of results. D.P. interpreted and collected data and conceived the study design.
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These authors contributed equally to this study.