Central nervous system cavernomas in the pediatric age group

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.

Intracranial cavernous malformation in children: a single-centered experience with 30 consecutive cases

“…In the current study, 56.6% of patients suffered from seizures, which is significantly higher than in earlier studies 2,[6][7][8]10,17,19 , although Acciarri et al 9 reported 70% of seizures in a series of 42 children with CMs.…”

“…Table 2 summarizes previous studies on pediatric CM. Note that only ten series were according to the selection criteria and therefore included in this review 2,3,[6][7][8][9][10]12,17,19 .…”

“…Patients with lesions located superficially in the cerebral hemispheres more frequently are present with seizures. On the other hand, patients with lesions 8,19,22 . Supratentorial location accounts for about 80.0%, while the other 20.0% are located in the posterior cerebral fossa 3,8,19,22 .…”

“…A deep location in the basal ganglia, hypothalamus, or ventricular system is rare 8,19,22 . In this series, regarding only the symptomatic lesions, 23 patients (76.6%) presented supratentorial CM and seven (23.3%) infratentorial.…”

“…Pediatric CMs may have characteristics that differ from those of adults in clinical presentation and treatment, such as higher rates of hemorrhage [6][7][8][9][10] and larger dimensions 9 . Even though there are not many reports about exclusive pediatric CMs, only a few large series have yet been reported, and ideal group classification has not yet been defined.…”