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Induction and Micro-CT Imaging of Cerebral Cavernous Malformations in Mouse Model
Published on: September 4, 2017
[Central nervous system cavernomas in children]
G Lena1, J Ternier, A Paz-Paredes
1Unité fonctionnelle de neurochirurgie pédiatrique, hôpital des enfants de La Timone, 286 rue Saint-Pierre, 13385 Marseille cedex 05, France. gabriel.lena@ap-hm.fr
Insights
Cavernomas are vascular malformations in children, often presenting with hemorrhage or neurological deficits. Surgical intervention for symptomatic or hemorrhagic lesions significantly improves outcomes, with most children remaining neurologically intact post-surgery.
Area of Science:
- Pediatric Neurology
- Vascular Malformations
- Neurosurgery
Context:
- Cavernomas are a type of cerebral vascular malformation found in children.
- Hemorrhage is a frequent complication in pediatric cavernomas, occurring in up to 78% of cases.
- Lesions are predominantly supratentorial (79.4%), with a significant proportion in the brainstem.
Purpose:
- To review the clinical presentation, location, management, and outcomes of pediatric cavernomas.
- To highlight the efficacy of surgical management for symptomatic or hemorrhagic lesions.
- To provide insights into the current consensus on cavernoma treatment.
Summary:
- Pediatric cavernomas manifest with hemorrhage (27.3-78%), epilepsy (16-60%), and neurological deficits (22.7%).
- Management favors medical treatment for asymptomatic cases and surgical intervention for symptomatic/hemorrhagic lesions.
- Surgical outcomes are encouraging, with approximately 70% of children remaining neurologically intact post-operatively.
Impact:
- Advances in neuroimaging and surgical techniques have improved the management of pediatric cavernomas.
- Surgical intervention for epilepsy associated with cavernomas shows promising results, especially for non-temporal lobe lesions.
- Effective management strategies lead to favorable neurological outcomes in the majority of pediatric patients.
Unlabelled:
Cavernomas represent 1.7 to 18% of all vascular malformations in children and 25% are observed in children under 18 years of age. Cases observed in neonates and infants have been published, but the mean age varies from 9.1 to 10.2 years. There is no predominance between boys and girls.
Clinical Presentation:
In children, hemorrhage is a common manifestation with an incidence varying from 27.3 to 78% versus 8 to 37% in adult patients. Isolated headaches occur in 2.8% of patients and elevated ICP is observed in 20.1%. Epilepsy is reported in 16 to 60% of children, depending on the series. Neurological deficits are observed in 22.7% of patients and are more severe for deep-seated and brainstem cavernoma. About 14.2% of the cases are discovered fortuitously in asymptomatic patients. Spinal cord deficits are observed in 5% of the cases.
Location:
Using data in the literature plus our personal series of 57 cases, 79.4% of lesions are in the supratentorial compartment and 20.6% in the posterior fossa, the majority located in the brainstem, most of them in the pons. Spinal cord cavernomas represent 5% and multiples cavernomas (12.6%) of the reviewed cases.
Management:
Appropriate management of cavernomas has long been a subject of much debate. Today, a consensus has been reached to favor medical management of asymptomatic and non hemorrhagic lesions and surgical management of symptomatic and/or hemorrhagic cavernomas whatever the localization. Progress in neuroimaging, surgical mapping, intraoperative monitoring and microsurgical techniques has greatly contributed to improved approach to those lesions.
Results:
Results obtained in 217 cases were reviewed. Near 70% of the children are neurologically intact, 19.3% are improved or stable, 2.7% worsened and 1.13% died. Results for epilepsy are very encouraging, surgery is efficient in almost all the children except for temporal lobe cavernomas where invasive presurgical evaluation is recommended. Deep-seated and brainstem cavernomas can safely be removed in most of the cases. Only two children died from recurrent hemorrhage due to residual lesions.
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