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Central nervous system cavernomas in the pediatric age group
C Mottolese1, M Hermier, H Stan
1Department of Pediatric Neurosurgery, Université Claude-Bernard Lyon-I, Hĵpital Neurologique et Neurochirurgique P. Wertheimer, France.
Insights
Pediatric central nervous system (CNS) cavernomas present a high hemorrhagic risk and unique imaging features in children. Surgical excision is the primary treatment for symptomatic lesions, though more research on natural history is needed.
Area of Science:
- Pediatric Neurology
- Neurosurgery
- Neuroradiology
Background:
- Central nervous system (CNS) cavernomas are challenging pediatric lesions.
- Adult cavernoma natural history is known, but pediatric data is limited.
- Previous studies often combined adult and pediatric cases.
Purpose of the Study:
- Focus on pediatric CNS cavernoma clinical, imaging, and therapeutic features.
- Highlight specificities of cavernomas in the pediatric age group.
- Propose an imaging classification for pediatric cerebral cavernomas.
Main Methods:
- Literature review and single-center experience (36 operated, 7 unoperated pediatric cases).
- Analysis of clinical presentation, imaging findings, and treatment outcomes.
- Development of a pediatric-specific imaging classification.
Main Results:
- Pediatric cavernomas have a high hemorrhagic risk, similar to adults.
- Lesions often present with sudden intracerebral hematoma and neurological deficits.
- Proposed imaging classification aids diagnosis, identifying sharply demarcated lesions.
- Surgical excision is effective for symptomatic and growing lesions.
Conclusions:
- Pediatric CNS cavernomas require specific diagnostic and therapeutic approaches.
- Microsurgical excision offers good outcomes for operated children.
- Further research on the natural history of pediatric cavernomas is essential for optimal management.
Abstract:
Pediatric CNS cavernomas still are diagnostically and therapeutically challenging lesions. With the help of magnetic resonance imaging, the natural history of cavernomas now guiding therapeutic strategies is well documented in adults but remains poorly known in the pediatric age group, since most previous studies dealt with adult and pediatric patients together. This paper focuses on clinical, imaging, and therapeutic features and differential diagnosis of CNS cavernomas with an emphasis on their specificities in the pediatric age group. It is based upon a critical review of the literature and our single-center experience with 36 children (35 with cerebral cavernomas and one with spinal cord cavernoma) operated on during the period of 1985-1999 as well as with seven additional unoperated pediatric cases. Our experience resembles that of other authors regarding the high hemorrhagic risk in children compared to adults. These angiographically occult vascular malformations are often revealed by the sudden onset of intracerebral hematoma with acute focal neurologic deficits, concomitant manifestations, and/or signs of raised intracranial pressure. True epilepsy is less common and may be related to chronic or recurrent microbleeding. Evocative imaging findings are also somewhat different in the two age groups, and we propose here an imaging classification of cerebral cavernomas based on both morphological and signal characteristics that is applicable to the pediatric age group. A sharply demarcated spherical intracerebral hematoma or heterogeneous lesion should always make one consider the hypothesis of a cavernoma. For symptomatic lesions and most rapidly growing asymptomatic lesions, the treatment of choice is complete microsurgical excision preceded by careful anatomical and functional evaluation. Improvements in surgical techniques and anesthesiology over recent years have brought good results in most operated children. The limited role of radiosurgery in the management of pediatric cerebral cavernomas is discussed. There is still a need for well-conducted specific evaluation of the natural history of these lesions in the pediatric age group to aid in systematic research, follow-up, and therapeutic strategies for asymptomatic cavernomas.