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Sporadic Meningioangiomatosis: A Series of Three Pediatric Cases
Raja Anand1, Richard J Garling1, Janet Poulik2
1Neurosurgery, Wayne State University School of Medicine.
Abstract:
Meningioangiomatosis (MA) is a rare benign, hamartomatous lesion within the leptomeninges and cerebral cortex. Three percent of intractable epileptic patients with tumor develop MA. It may be accompanied with neurofibromatosis type II, or it may occur sporadically. Three patients, age range of 2-16 years old, presented with episodes of seizure. The patients demonstrated no family history or stigmata of neurofibromatosis type II. Electroencephalogram (EEG) was unremarkable for epileptiform activity. Magnetic resonance imaging (MRI) revealed enhancing lesions within the frontal gyrus, the anterior cingulate gyrus, and the parietal lobe. Incomplete resection led to recurrence in one patient, and later, intraoperative ultrasound was used to achieve total resection in another patient. Each patient was seizure free on follow-up, and managed with anti-epileptic medication. Resection is the only curative treatment in 85% of MA cases. Complete resection is necessary for symptomatic treatment in cases of MA, as recurrence has been documented in this lesion. Intraoperative ultrasound is an effective imaging modality to ensure gross total resection of MA.
Insights
Meningioangiomatosis (MA) is a rare tumor causing seizures in children. Complete surgical resection is crucial for symptom resolution and preventing recurrence.
Area of Science:
- Neurology
- Neurosurgery
- Pediatric Epilepsy
Background:
- Meningioangiomatosis (MA) is a rare, benign, hamartomatous lesion affecting the leptomeninges and cerebral cortex.
- MA accounts for 3% of intractable epilepsy cases associated with tumors and can occur sporadically or with neurofibromatosis type II.
Observation:
- Three pediatric patients (ages 2-16) presented with seizures, lacking family history or stigmata of neurofibromatosis type II.
- Electroencephalogram (EEG) showed no epileptiform activity, while Magnetic Resonance Imaging (MRI) revealed enhancing lesions in the frontal, cingulate, and parietal lobes.
Findings:
- Incomplete resection resulted in recurrence in one patient.
- Intraoperative ultrasound facilitated complete resection in another patient, leading to seizure freedom post-operatively.
- Complete resection is curative in 85% of MA cases and essential for symptomatic management due to recurrence risk.
Implications:
- Complete surgical resection is the primary curative treatment for meningioangiomatosis.
- Intraoperative ultrasound is a valuable tool for ensuring gross total resection, minimizing recurrence.
- Effective management involves anti-epileptic medication and surgical intervention for symptomatic relief.
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