Search

Object The four types of cerebrovascular malformations may sometimes be combined and more often occur in pairs; triads are exceptional. The authors present six patients with the clinicoradiographic profile of mixed vascular malformations of the brainstem, including cavernous malformation (CM), capillary telangiectasia, and developmental venous anomaly (DVA). Methods Five patients (one of whom was a child) suffered from hemorrhage, suggesting that this complex association has a high bleeding potential. Progressive growth, rebleeding, and de novo occurrence of the associated CM were documented in three cases. Magnetic resonance imaging of the brain was obtained in all patients by using one or more of the following modalities: T1-weighted sequences before and after gadolinium administration; T2-weighted sequences; T2-weighted fluid attenuated inversion recovery; T1-weighted fast spin echo; and diffusion weighted, diffusion tensor, and perfusion imaging in three cases. Results Three patients were surgically treated with the intention of excising the hemorrhagic lesion, but only two patients had their malformations successfully removed. In the third case, diffuse pontine telangiectasia precluded the safe excision of the CM. Histological examination demonstrated a blended pathological milieu characterized by coalescent telangiectasia and venules associated with loculated endothelial chambers resembling an immature or de novo CM. Three patients were treated conservatively; recurrent minor hemorrhage occurred in one case. The authors found these malformations to be arranged in two basic relationships: CM inside the telangiectasia and CM in the radicles of the DVA. Stenosis of the main venous collector and dilation of the medullary veins were important findings. Conclusions The pathogenesis of this malformation may be referred to a developmental deviance of the brainstem capillary–venous network associated with transitional vessels and loculated endothelial vascular spaces related to genetic and acquired origins, probably in a restrictive venous outflow milieu.

Object. Glioneuronal tumors (ganglioglioma, dysembryoplastic neuroepithelial tumors [DNTs]) are commonly associated with partial seizures. The optimal surgical treatment of such tumors, however, has not been fully established; it is still unclear whether lesionectomy itself can be used to control seizures or if epileptogenic areas adjacent to the tumor should also be removed. To address this uncertainty, the authors analyzed seizure outcome in a series of children with epileptogenic glioneuronal tumors that had been treated only by lesionectomy. Methods. The authors retrospectively reviewed 15 children surgically treated for glioneuronal tumors associated with epilepsy. Patients ranged in age from 3 to 18 years (mean 12.6 years); there were 12 boys and three girls. The interval between onset of seizures and surgery ranged from 0.5 to 16 years (mean 6.1 years). Ten patients (66.6%) suffered complex partial seizures and five (33.3%) simple partial seizures. Seizure frequency varied from several per day to one per month. Nine tumors (60%) were temporal and six extratemporal; in all patients resection was limited to the tumor. The follow-up duration ranged from 1 to 11 years (mean 5.6 years). Gross-total removal was achieved in 13 patients and subtotal in two. The histological diagnosis was ganglioglioma in 11 cases and DNT in four. At last follow up 13 patients (86.6%) were Engel Class I, one was Engel Class II, and one was Engel Class III. Conclusions. The results of this study indicate that lesionectomy may provide good long-term seizure control in the majority of children with epileptogenic glioneuronal tumors.