Berman M F, Sciacca R R, Pile-Spellman J, Stapf C, Connolly E S, Mohr J P, Young W L
Department of Anesthesiology, Columbia University College of Physicians & Surgeons, New York, New York 10032, USA.
Neurosurgery. 2000 Aug;47(2):389-96; discussion 397. doi: 10.1097/00006123-200008000-00023.
Common estimates of the prevalence rate for pial arteriovenous malformations (AVMs) of the brain vary widely, and their accuracy is questionable. Our objective was to critically review the original sources from which these rates were derived and to establish best estimates for both the incidence and prevalence of the disease.
We reviewed all of the relevant original literature: autopsy series, the Cooperative Study of Intracranial Aneurysms and Subarachnoid Hemorrhage and related analyses, and other population-based studies. We also modeled the confidence intervals of estimates for a process of low prevalence such as AVMs.
Many of the prevalence estimates (500-600/100,000 population) were based on autopsy data, a source that is inherently biased. Other estimates (140/100,000 population) originated from an inappropriate analysis of data from the Cooperative Study. The most reliable information comes from a population-based study of Olmsted County, MN, but prevalence data specific to AVMs was not found in that study.
The estimates for AVM prevalence that are published in the medical literature are unfounded. Because of the rarity of the disease and the existence of asymptomatic patients, establishing a true prevalence rate is not feasible. Owing to variation in the detection rate of asymptomatic AVMs, the most reliable estimate for the occurrence of the disease is the detection rate for symptomatic lesions: 0.94 per 100,000 person-years (95% confidence interval, 0.57-1.30/100,000 person-years). This figure is derived from a single population-based study, but it is supported by a reanalysis of other data sources. The prevalence of detected, active (at risk) AVM disease is unknown, but it can be inferred from incidence data to be lower than 10.3 per 100,000 population.
脑软膜动静脉畸形（AVM）患病率的常见估计值差异很大，其准确性值得怀疑。我们的目的是严格审查得出这些患病率的原始资料来源，并确定该疾病发病率和患病率的最佳估计值。
我们查阅了所有相关的原始文献：尸检系列、颅内动脉瘤和蛛网膜下腔出血合作研究及相关分析，以及其他基于人群的研究。我们还对诸如AVM这种低患病率疾病的估计值置信区间进行了建模。
许多患病率估计值（每10万人中500 - 600例）基于尸检数据，而尸检数据本身存在偏差。其他估计值（每10万人中140例）源于对合作研究数据的不当分析。最可靠的信息来自明尼苏达州奥尔姆斯特德县的一项基于人群的研究，但该研究未找到特定于AVM的患病率数据。
医学文献中公布的AVM患病率估计值毫无根据。由于该疾病罕见且存在无症状患者，确定真实的患病率不可行。由于无症状AVM检测率的差异，该疾病发生的最可靠估计值是有症状病变的检测率：每10万人年0.94例（95%置信区间，每10万人年0.57 - 1.30例）。这个数字来自一项单一的基于人群的研究，但得到了对其他数据源重新分析的支持。已检测到的、活跃（有风险）的AVM疾病的患病率未知，但可从发病率数据推断低于每10万人中10.3例。
