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Objectives Cerebral arterio-venous malformations (AVM) are considered congenital lesions, emerging as an important cause of haemorrhagic stroke in children. The potential influence of age on clinical presentation and angio-architecture have been analysed extensively in the last years. Yet, comparative studies comprising ascending age groups may be limited in their conclusions, especially when comparing young children and adults. It is the aim of this study to evaluate characteristic clinical and angiographic features of AVM within a paediatric subgroup and their correlation to age at presentation. Patients and methods Between 1990–2015, 46 children harbouring AVMs were treated at our institution. Clinical presentation, radiological data, treatment strategies and outcome were evaluated retrospectively. Results Of 46 consecutive patients, 18 were male and 28 female patients. Mean age was 11.6 ± 4.3 years, ranging from 2 to 17 years. 35 patients (76%) presented with haemorrhage. Seizures were found in 6 patients (13%) and progressive or transient focal neurological deficits in 4 individuals (9%). There was one incidental patient, only. Mean age of children presenting with haemorrhage was significantly lower as compared to those without a history of intracranial bleeding (p = 0.1). The size of the AVM was small (n = 27, 59%), corresponding a grade I AVM in the majority of patients (N = 28, 61%). 41 patients (89%) underwent treatment of their AVM by an interdisciplinary approach achieving complete elimination of the lesion in 34 patients (83%). 34 patients (83%) showed at least a favourable outcome (mRS ≤ 2) at last follow-up. An excellent recovery (mRS 0–1) was noted in 28 patients (68%). Conclusion From our data we suggest that patients’ age impacts the clinical presentation. Particularly young children seem to bear a higher risk for haemorrhage from their AVM. Treatment of paediatric AVMs can be achieved safely in experienced hands with a high rate of complete elimination and good clinical outcome.

Background: The natural history and treatment of brain arteriovenous malformations (AVMs) is the object of ongoing debates and discussions. To capture the entirety of these complex lesions, associated vascular pathologies, such as associated aneurysms (AAs), have to be implemented in future risk stratification models, as they are believed to represent additional risk factors for intracranial hemorrhage. The present study aims to determine AA characteristics in posterior fossa AVMs and to compare with AAs accompanying supratentorial AVMs, with special focus on aneurysm size. Methods: Patients with cerebral AVMs, treated in our department between 1990 and 2013, were analyzed retrospectively. Only patients with flow-related AAs of the feeding arteries were evaluated. Thus, patients harboring intranidal, venous or remote aneurysms were excluded. Results: Of 485 patients with cerebral AVM, 76 patients harbored an AVM of the posterior fossa. Among those, 22 individuals exhibited a total of 35 AAs (n = 8 patients with multiple AAs). Most common location of AAs was the posterior inferior cerebellar artery (n = 20, 57%) and mean AA diameter was 7.9 mm (SD 5.5). In the subgroup of patients with a single AA, mean aneurysm size in posterior fossa AVMs was with 7.8 mm (SD 6.0; range 2-25 mm) significantly larger than the mean size of AAs with supratentorial AVMs (4.8 mm, SD 3.0; range 2-20 mm; p = 0.048). Intracranial hemorrhage was found in 18 of 22 patients (82%) with infratentorial AVMs, and of these, 11 patients suffered from aneurysm rupture. In 14 patients bearing a single AA, 8 (57%) had sustained hemorrhage from aneurysm rupture. The mean diameter of AAs was as supposed in the ruptured group with 9.8 mm (SD 6.9; range 4-25 mm) significantly larger than in the unruptured AA group exhibiting a mean of 5.0 mm (SD 3.3; range 2-10 mm; p = 0.038). Patients with posterior fossa AVMs and AAs were significantly older as compared to those patients with supratentorial lesions (57.1, SD 12.6 vs. 45.8 years, SD 15.9 years; p = 0.004), which was also evident in the subgroup of patients with single AAs (55.2, SD 11.7 vs. 45.8 years, SD 14.9 years; p = 0.038). Conclusions: AAs of posterior fossa AVMs are larger in diameter than aneurysms accompanying supratentorial AVMs. AA size influences risk for hemorrhage, which, together with the high number of hemorrhagic events in posterior fossa AVMs, justifies treating these pathologies. The higher age of patients with AVMs of the posterior fossa might be one reason for larger AAs in this cohort, when compared to patients with supratentorial AVMs and AAs.