Torné Ramon, Rodríguez-Hernández Ana, Arikan Fuat, Romero-Chala Fabián, Cicuéndez Marta, Vilalta Jordi, Sahuquillo Juan
Department of Neurological Surgery, Vall d'Hebron University Hospital, Paseo Vall D'Hebron 119-129, 08035 Barcelona, Spain; Neurotraumatology and Neurosurgery Research Unit (UNINN), Vall d'Hebron Research Institute, Universitat Autonoma de Barcelona (UAB), Barcelona, Spain.
Department of Neurological Surgery, Vall d'Hebron University Hospital, Paseo Vall D'Hebron 119-129, 08035 Barcelona, Spain.
Clin Neurol Neurosurg. 2015 Jul;134:37-43. doi: 10.1016/j.clineuro.2015.04.003. Epub 2015 Apr 17.
Hydrocephalus associated with different types of intracranial arteriovenous malformations (AVMs) has been scarcely studied. In the present report we investigate this association with posterior fossa AVMs (pfAVMs). We hypothesized that there is an increased risk of hydrocephalus and required permanent cerebrospinal fluid (CSF) shunt in patients with pfAVMs that may be linked to the increased risk of bleeding of these lesions. We also review the factors associated with this increased risk of hemorrhagic presentation and we assess how it affects management strategies and functional outcomes in these patients.
Out of a prospective registry of 374 patients with brain AVMs diagnosed in our center from 1993 to 2013, 60 (16%) had a pfAVM. We described these patients' demographics, their AVM characteristics, clinical presentation, and hydrocephalus incidence and compared the results with those of the supratentorial AVM (spAVM) patients recorded during the same period.
Out of the 60 patients with pfAVMs, 10 (16.7%) presented AVMs located in the brainstem. Hemorrhagic presentation (49/60; 82%) was significantly higher in pfAVMs than in spAVMs (122/314; 38.8%; p<0.05). Hydrocephalus was a common complication in pfAVM patients who had a statistically significant higher need for both temporary external ventricular drain (EVD) (6.7 vs. 20%; p<0.05) and permanent CSF shunts (3.5 vs. 20%; p<0.05). The initial mortality was high (12/60; 20.3%) and half of these patients died before any treatment option could be offered. However, out of those who survived, 70% (42/60) had already shown good clinical outcome at the 6-month follow-up.
Hemorrhagic presentation and hydrocephalus have a higher incidence in pfAVM patients, which initially results in more neurological deficits and an elevated mortality even before receiving any treatment. However, a large number of survivors present good functional outcomes at early follow-up, justifying an aggressive management strategy with microsurgery as the first treatment option in most cases, and radiosurgery as an alternative, especially in brainstem AVMs.
与不同类型颅内动静脉畸形（AVM）相关的脑积水鲜有研究。在本报告中，我们研究后颅窝AVM（pfAVM）与脑积水的这种关联。我们假设，pfAVM患者发生脑积水及需要永久性脑脊液（CSF）分流的风险增加，这可能与这些病变出血风险增加有关。我们还回顾了与出血表现风险增加相关的因素，并评估其如何影响这些患者的治疗策略和功能结局。
在1993年至2013年于我们中心诊断的374例脑AVM患者的前瞻性登记中，60例（16%）有pfAVM。我们描述了这些患者的人口统计学特征、AVM特点、临床表现和脑积水发生率，并将结果与同期记录的幕上AVM（spAVM）患者的结果进行比较。
在60例pfAVM患者中，10例（16.7%）的AVM位于脑干。pfAVM患者的出血表现（49/60；82%）显著高于spAVM患者（122/314；38.8%；p<0.05）。脑积水是pfAVM患者的常见并发症，在临时外部脑室引流（EVD）（6.7%对20%；p<0.05）和永久性CSF分流（3.5%对20%；p<0.05）方面，其需求在统计学上显著更高。初始死亡率很高（12/60；20.3%），其中一半患者在能够提供任何治疗选择之前死亡。然而，在存活的患者中，70%（42/60）在6个月随访时已显示出良好的临床结局。
pfAVM患者的出血表现和脑积水发生率较高，这最初导致更多神经功能缺损，甚至在接受任何治疗之前死亡率就升高。然而，大量幸存者在早期随访时呈现良好的功能结局，这证明在大多数情况下采用积极的治疗策略是合理的，以显微手术作为首选治疗方案，放射外科作为替代方案，尤其是在脑干AVM中。
