Multiple cerebral arteriovenous shunts in children: report of 13 cases

The authors present a series of 13 multiple arteriovenous malformations (MAVMs) in the pediatric population (16.9% of their overall series of brain AVMs in this group). Two types of MAVMs can be distinguished: congenital and acquired. Congenital MAVMs may be of the nidus or fistula type. They may be uni- or bilateral, placed in one or several cerebral lobes, separated or close one to another, or even systematized (Wiburn-Mason syndrome). The symptoms created by these MAVMs are the same as those encountered in the presence of other AVMs, with hemorrhage as revealing symptom in 31% of patients. The responsibility of one particular nidus in the onset of clinical signs is often difficult to determine. From an angioarchitectural point of view, it seems that venous drainage changes are mainly responsible for the symptomatology. The natural history of these MAVMs is difficult to assess; spontaneous regression has been noted in 15% of cases. Acquired cerebral MAVMs can be due to angiogenesis ("sprouting" or "non-sprouting") around a true AVM because of previous hemorrhage or ischemia, or to pial shunts associated with dural arteriovenous malformations. The treatment of MAVMs is difficult. Embolization seems to the authors the best therapeutic modality available, as surgery or radiosurgery are often unable to treat these multifocal lesions. Anatomical cure is rarely obtained; the therapeutic strategy has to be targeted on the symptomatic lesions.