Overview
Meningiomatosis refers to the presence of multiple meningiomas, often involving both dural layers and potentially extracranial locations, leading to complex clinical presentations and management challenges 1.Diagnosis
Imaging studies (CT, MRI) essential for identifying multiple meningiomas and associated abnormalities like fibrous dysplasia 1.
Angiography may be required to detect vascular complications such as intraosseous pseudoaneurysms 1.
Specific findings include en plaque meningiomas and vascular malformations fed by branches of the middle meningeal artery 1.Management
First-line treatments: Surgical resection for symptomatic or large meningiomas 1.
Adjunctive treatments: Embolization techniques (e.g., Onyx) for managing vascular complications like intraosseous pseudoaneurysms 1.
Targeted interventions: Address specific comorbidities; for instance, managing fibrous dysplasia and associated syndromes 1.Special Populations
Pediatrics: Unique considerations due to ongoing bone development; tailored surgical approaches and embolization techniques may be necessary 1.
Comorbidities: Patients with syndromes like McCune-Albright syndrome require multidisciplinary care addressing both meningiomatosis and associated conditions 1.Key Recommendations
Utilize advanced imaging (CT, MRI) for comprehensive diagnosis of meningiomatosis and associated vascular anomalies 1. (Evidence: Moderate)
Consider endovascular embolization (e.g., Onyx) for managing vascular complications such as intraosseous pseudoaneurysms 1. (Evidence: Weak)
Tailor management strategies to address coexisting syndromes and developmental stages, particularly in pediatric patients 1. (Evidence: Expert opinion)References
1 Settecase F, Nicholson AD, Amans MR, Higashida RT, Halbach VV, Cooke DL et al.. Onyx embolization of an intraosseous pseudoaneurysm of the middle meningeal artery in a patient with meningiomatosis, McCune-Albright syndrome, and gray platelet syndrome. Journal of neurosurgery. Pediatrics 2016. link