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Comprehensive Endovascular and Open Surgical Management of Cerebral Arteriovenous Malformations
Published on: October 20, 2017
Clusters of Pediatric Brain Arteriovenous Malformations Link Spatial Distribution, Clinical Presentation, and
Sandro Benichi1,2,3,4,5,6, Joseph Benzakoun7,8, Grégoire Boulouis9
1Department of Pediatric Neurosurgery, Necker University Hospital, Paris, France. sandro.benichi@aphp.fr.
Insights
Pediatric brain arteriovenous malformations (PbAVMs) can be classified into three distinct clusters based on angioarchitecture and location. These clusters show unique presentations, clinical courses, and outcomes, aiding in understanding and managing this condition.
Area of Science:
- Neurology
- Radiology
- Pediatrics
Background:
- Pediatric brain arteriovenous malformations (PbAVMs) are a significant cause of intracerebral hemorrhage and neurological issues in children.
- Understanding the relationship between PbAVM characteristics and clinical outcomes is crucial for effective management.
Purpose of the Study:
- To investigate the links between the angioarchitecture, spatial distribution, and clinical presentation of pediatric brain arteriovenous malformations.
- To develop a 3D MRI-based atlas and identify distinct clusters of PbAVMs.
Main Methods:
- Retrospective analysis of 245 consecutive PbAVMs in pediatric patients (2007-2023).
- Hierarchical clustering based on quantitative imaging data to delineate PbAVM clusters.
- Analysis of clinical parameters including presentation, cure rates, recurrent bleeding, proliferation, and location.
Main Results:
- Three distinct PbAVM clusters were identified: small slow flow, fast flow, and giant.
- Cluster 1 (small slow flow) had a high rupture rate. Cluster 2 (fast flow) was associated with epilepsy and thalamic location. Cluster 3 (giant) showed higher rates of recurrent bleeding and proliferation.
- Specific locations were linked to clinical presentations: motor cortex (epilepsy), right thalamus (neurological deficit), and left parietal lobe (headaches).
Conclusions:
- PbAVM angioarchitecture and spatial distribution define distinct clinical phenotypes.
- Classification into small slow flow, fast flow, and giant clusters provides insights into prognosis and management strategies.
- This clustering approach aids in predicting outcomes and tailoring treatment for pediatric brain arteriovenous malformations.
Abstract:
Pediatric brain arteriovenous malformation (PbAVM) is a potentially life-threatening condition accounting for intracerebral hemorrhage and other neurological complications. Our study aimed to elucidate the relationships between PbAVM angioarchitecture, spatial distribution, and clinical presentation. This retrospective study included all consecutive PbAVMs managed in a tertiary pediatric center (2007-2023). We performed a hierarchical clustering based on quantitative imaging data and constructed a 3D MRI-based atlas. We derived and analyzed clusters for initial presentation, clinical parameters (cure, recurrent bleeding, proliferation/recurrence, seizures) and location. We included 245 pediatric brain arteriovenous malformations in 234 patients (74.8% ruptured, mean age 9.9±4 years). Hierarchical clustering delineated three clusters of PbAVMs. Cluster 1 included ruptured small slow flow PbAVMs (n = 162, 82% ruptured). Cluster 2 included ruptured and epileptic fast flow PbAVMs (n = 69, 53% ruptured, 23% epileptic), more frequently located within the right thalamus. Cluster 3 included giant PbAVMs (n = 14, 21.5% ruptured, 57% epileptic), more frequently located within the left posterior insula and right premotor area. Cluster 2 PbAVMs achieved lower rate of exclusion than Cluster 1 (HRcure=0.34±0.23, p = 3.7.10- 6). Cluster 3 exhibited null complete exclusion, more frequent recurrent bleeding (HRrupture=6.83±0.61, p = 0.001) and proliferation over time (HRproliferation=11.1±0.49, p = 1.10- 6). The probabilistic analyses showed association between PbAVMs within primary motor cortex and epileptic presentation, PbAVMs within right thalamus and neurological deficit at presentation, and PbAVMs within left parietal lobe and headaches. We identified three clusters (small slow flow, fast flow, giant) of PbAVMs that demonstrated unique initial presentation, clinical course, outcomes and spatial distribution.
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