Meningioangiomatosis without neurofibromatosis: a clinical analysis
George I Jallo1, Karl Kothbauer, Vikas Mehta
1Division of Pediatric Neurosurgery, Johns Hopkins University, Baltimore, Maryland 21287, USA. gjallo1@jhmi.edu
Object:
Meningioangiomatosis is an uncommon clinical entity. This lesion has been reported at time of autopsy in patients with neurofibromatosis (NF) and in case reports of patients without NF Type 2 (NF2). The authors report a series of six patients with meningioangiomatosis who do not have NF2 and describe the clinical presentation, diagnosis of disease, and treatment. They also review the literature concerning this entity.
Methods:
Six patients with meningioangiomatosis were treated at the authors' institutions from 1994 to 2001. The mean age of the patients was 10.7 years (range 5-14 years). All of the children presented with a seizure disorder. Surgery was performed in all children, and a gross-total resection was accomplished. All patients exhibited clinical improvement. At last follow up (mean 6.3 years) all patients are free of seizures and are not taking anticonvulsant medications. No signs of recurrence have been noted on imaging studies.
Conclusions:
The authors advocate a gross-total resection of meningioangiomatosis for the treatment of seizure disorder in this population.
Insights
Meningioangiomatosis, a rare condition, often presents with seizures in children. Complete surgical removal of the lesion offers a promising treatment, leading to seizure freedom without medication.
Area of Science:
- Neurology
- Neurosurgery
- Pediatric Neurology
Background:
- Meningioangiomatosis is an uncommon vascular tumor.
- It is occasionally associated with neurofibromatosis Type 2 (NF2).
- This study focuses on patients without NF2.
Observation:
- Six pediatric patients without NF2 were treated for meningioangiomatosis.
- All patients presented with intractable seizures.
- The mean age of presentation was 10.7 years.
Findings:
- Surgical gross-total resection was performed in all six patients.
- All patients experienced significant clinical improvement post-surgery.
- At a mean follow-up of 6.3 years, all patients were seizure-free and off anticonvulsant medication.
Implications:
- Gross-total resection is an effective treatment for pediatric meningioangiomatosis.
- Complete surgical removal can resolve seizure disorders associated with this condition.
- This approach offers a favorable long-term prognosis for affected children.
