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Cavernous angiomas of the central nervous system in children
R M Scott1, P Barnes, W Kupsky
1Department of Neurosurgery, Children's Hospital, Boston, Massachusetts.
Insights
This study details 19 pediatric patients with cavernous angiomas, highlighting surgical outcomes and pathological findings. Findings suggest unique growth mechanisms and emphasize the importance of complete resection for pediatric central nervous system vascular malformations.
Area of Science:
- Neurology
- Neurosurgery
- Pediatric Neurosurgery
Background:
- Cavernous angiomas (cerebral cavernous malformations) are vascular malformations of the central nervous system.
- Pediatric cases are less common, necessitating specific understanding of their presentation and management.
Observation:
- A surgical series of 19 pediatric patients (under 18) with pathologically verified cavernous angiomas.
- Lesions were located in various brain regions, including cerebral hemispheres, pons, midbrain, diencephalon, and spinal cord.
- Presenting symptoms included neurological deficits, seizures, irritability, and headache.
Findings:
- Intraoperative ultrasonography and stereotactic localization aided surgery for deep or small lesions.
- Pathology revealed complex histology with granulation tissue and hemorrhage, suggesting a growth mechanism.
- Good postoperative outcomes were observed, though incomplete resection led to rebleeding in two patients.
Implications:
- Complete resection is crucial for pediatric cavernous angiomas to prevent rebleeding.
- Understanding the unique pathology may inform future treatment strategies for these vascular malformations.
- Long-term follow-up is essential due to the unusual natural history of some cavernous angiomas.
Abstract:
A surgical series of 19 patients under the age of 18 years with pathologically verified cavernous angioma is presented. Most lesions were located in the cerebral hemispheres, but four were in the pons or midbrain, two in the diencephalon, and one in the spinal cord. Fourteen patients presented with an acute or progressing neurological deficit, three with seizures, one infant with irritability, and one with headache alone. Five patients had family histories of vascular malformations of the central nervous system, and five had multiple lesions. Surgery for small or deep lesions was aided considerably by intraoperative ultrasonographic or stereotactic localization techniques. Pathological examination of the resected malformations revealed a complex histology containing not only typical closely approximated cavernous vessels, but also areas of marked proliferation of granulation tissue and partially re-endothelialized hemorrhage, suggesting a mechanism for the apparent growth of certain cavernous angiomas. The postoperative results were good, with only one patient suffering a permanent worsening of neurological status after surgery. Incomplete resection was initially carried out in five patients, two of whom rebled within 1 year after operation. Long-term follow-up findings in these patients have emphasized the unusual history of certain of these malformations.