Laboratory and clinical retential features of patients followed with the diagnosis of sinus vein thrombosis between 01.01.2012-01.01.2022 to Ondokuz Mayis University Medical Faculty child hematology polyclinic

Objective: Cerebral sinus vein thrombosis (SVT) is a rare cerebrovascular disease characterized by partial or complete occlusion of the dural sinus and cerebral veins with progressive cerebral edema and increased intracranial pressure. It is being diagnosed more frequently with clinical awareness, sensitive neuroimaging techniques and increased susceptibility to synovenous thrombosis.The incidence of childhood SVT is estimated to be between 0.07 and 0.14 per 10,000 children per year. Risk factors are diverse and are associated with age, the presence of acute or chronic diseases and thrombophilia. The long-term outcome of SVT is variable and depends on the age at diagnosis, the presence of comorbid diseases and acute complications. The aim of this study was to investigate the clinical presentation, acquired and genetic risk factors, laboratory and imaging findings, treatments given, and outcome of pediatric patients diagnosed with SVT. Materials and Methods: In our study, 59 patients who were diagnosed with sinus vein thrombosis in Ondokuz Mayıs University, Faculty of Medicine, Pediatric Hematology Outpatient Clinic between 01.01.2012-01.01.2022 were retrospectively analyzed in terms of risk factors for acquired and genetic thrombophilia, clinical features, laboratory and radiological findings, treatment given, discharge and clinical status at follow-up. Results: Of 59 patients with SVT, 21 were female and 38 were male. The age range of the patients was 0-17 years and the mean age of the study group was 7 years and 2 months. 13 patients were in the neonatal period and 46 patients were 1 year and older. The most common symptom at presentation was headache (54.2%) (n:32). All patients aged 1 year and older had symptoms at presentation and 53.8% of the neonatal group had symptoms at presentation. While hemogram abnormality was detected in 37.2% (n:22) of the patients at the time of admission, infection was the second most common cause in 16.9% (n:10) of the patients. These infections were mastoiditis, otitis and CNS infection, respectively. No infection was observed in female patients. The presence of infection according to gender was statistically significant. The most common disease diagnosed during follow-up in the study group was Behçet's disease (11.8%) (n:7). In the thrombophilia gene panel, MTHFR gene mutation was the most common (81.3%) (n:48). The 2nd most common mutation was PAI mutation (62.7%) (n:37). 18.6% (n:11) of the patients had elevated homocysteine in the thrombophilia panel. Hereditary protein C and protein S were not found to be low in our study. In the neurologic examination of the patients, the 6th cranial nerve palsy was the most common (10.1%) (n:6). No neurologic examination findings were detected in 55.9% (n:33) of the study group. The most common SVT localization was the transverse sinus (69.4%) (n:41). All patients received anticoagulant therapy in the acute period and during follow-up. DMAH was the most commonly used anticoagulant. There were no deficit findings in 50 patients in 3-6 months and yearly follow-ups according to the clinical status of the patients. Discussion and Conclusion: Considering the studies conducted in Turkey, our study consisting of 59 patients was the study with the highest number of cases. The number of neonatal cases in our study group was lower than in the literature (n:13, 22%). It was thought that the lack of clinical findings specific to cerebral thrombosis in the neonatal period may lead to a deficiency in diagnosis. When the presenting symptom was analyzed according to age, similar results were obtained compared to the literature. Early diagnosis is important because the risk of acute complications and long-term sequelae can be significantly reduced with an appropriate treatment approach applied in the early period. Clinicians should be familiar with the clinical picture of SVT. Since patients with Behçet's disease are prone to thrombosis, SVT should be considered when there is clinical suspicion. Initiation of anticoagulant therapy as soon as possible under appropriate conditions reduces the risk of neurologic sequelae in the presence of thrombosis.