Related Experiment Video
Updated: Aug 3, 2026
Endoscopic Third Ventriculostomy and Pineal Biopsy from a Single Entry Point
Published on: June 28, 2024
Paediatric Behçet's disease with sinus venous thrombosis: experience from three centres in Turkey
Selcan Demir1, Ceyhun Acari2, Ozge Basaran3
1Division of Rheumatology, Department of Paediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Insights
Juvenile Behçet's disease patients with cerebral venous sinus thrombosis (CVST) often present with headache. Combined immunosuppressant and steroid treatment is crucial for managing pediatric CVST in Behçet's disease, reducing relapses and adverse effects.
Area of Science:
- Pediatric Rheumatology
- Pediatric Neurology
- Vascular Neurology
Background:
- Juvenile Behçet's disease (BD) is a rare multisystemic inflammatory disorder.
- Cerebral venous sinus thrombosis (CVST) is a serious neurological complication that can occur in juvenile BD.
- Understanding the clinical characteristics and outcomes of juvenile BD with CVST is crucial for effective management.
Purpose of the Study:
- To describe the clinical features and outcomes of pediatric patients with CVST and juvenile BD.
- To review existing literature on juvenile BD associated with CVST.
- To evaluate treatment strategies and their effectiveness in this patient population.
Main Methods:
- Retrospective review of 12 pediatric patients with CVST meeting Paediatric Behçet's Disease (PEDBD) criteria from three Turkish referral centers.
- Systematic literature review of published cases of pediatric CVST associated with BD.
- Analysis of clinical characteristics, treatment regimens, and patient outcomes.
Main Results:
- Headache was the most common symptom (100%) at CVST diagnosis in the study group.
- The transverse sinus was the most frequently affected sinus (75%).
- Combined immunosuppressant (azathioprine) and steroid treatment led to remission in most patients, with only one relapse in a patient not receiving azathioprine.
Conclusions:
- Neuroimaging plays a vital role in diagnosing neuro-Behçet's disease (NBD).
- Combination therapy with immunosuppressants and steroids is essential for managing pediatric CVST in BD, mitigating corticosteroid side effects and preventing relapses.
- Further multicenter prospective studies are needed to optimize treatment protocols for pediatric CVST in BD.
Objectives:
To report our experiences of the juvenile Behçet's disease (BD) patients with cerebral venous sinus thrombosis (CVST) and to review previous studies reporting the clinical characteristics and outcomes of juvenile BD with CVST.
Methods:
Clinical characteristics and outcomes of paediatric patients with CVST who met the Paediatric Behçet's Disease (PEDBD) classification criteria for juvenile BD from 3 referral centres in Turkey were reviewed retrospectively. A systematic review of literature of all published data was conducted.
Results:
The study group consisted of 12 juvenile BD patients with CVST. At the time of CVST diagnosis, the most common symptom was headache (100%), followed by vomiting (25%), blurred vision (16.7%), and disturbances in eye movements (16.7%). Six (50%) patients presented with CVST. Transverse sinus was the most frequently affected sinus (9/12, 75%) followed by superior sagittal sinus. The mean (±2SD) BDCAF at the CVST diagnosis was 6 (±3.8). Four children (33.3%) had another venous thrombosis apart from CVST. All patients received pulse methylprednisolone for three consecutive days continued with oral prednisolone. Steroid treatment was tapered and discontinued minimum in six months. Eleven patients received azathioprine concomitant to steroid treatment at the time of CVST. All the patients received anticoagulant therapy concomitantly. Only one patient who did not receive azathioprine relapsed. Median follow-up period was 4 years (IQR: 2-5.4). In the literature review, we identified nine articles, describing 35 pediatric CVST patients associated with BD. Thirty patients achieved remission, while five patients had residual neurologic deficit.
Conclusions:
Neuroimaging is very important in the diagnosis of NBD. We suggest that treatment with immunosuppressants and steroid treatment is essential to decrease the adverse events of corticosteroids in the pediatric population and decrease relapses. Further multicenter studies with prospective follow-up may guide us in better management of these patients.
