OBJECTIVE Loeys-Dietz syndrome (LDS) is a heritable aortopathy associated with craniofacial abnormalities and dilatation and dissection of the aorta and its branches, as well as increased risk for intracranial aneurysms (ICAs). Given the rarity of the disease, the authors aimed to better define the natural history and role for the treatment for ICAs in these patients. METHODS The medical records of 83 patients with LDS were retrospectively reviewed to obtain clinical and genetic history and vascular imaging of the aorta, aortic branches, and intracranial vessels. Serial radiology reports and cervical and intracranial vascular images were reviewed to determine presence, location, and size of ICAs. RESULTS In total, 55 patients (66.3%) had at least two screening intracranial vascular examinations, and 19 (22.9%) had at least 1 ICA detected. Aneurysms were typically small (mean ± SD 3.2 ± 1.8 mm). ICAs were most often located in the cavernous carotid, followed by the ophthalmic and anterior cerebral artery vessels. The rate of ICA growth was 0.43 ± 0.53 mm/year, similar to that of the general population. Three patients underwent intervention for an ICA, with 1 procedure complicated by stroke and resulting in transient hemiparesis. Several illustrative cases detail the authors' experience with ICA growth, de novo aneurysm formation, and ICA intervention in this rare patient population. CONCLUSIONS ICAs in patients with LDS are common, are frequently small, and have a growth rate similar to that of unruptured ICAs in the general population. More aggressive or earlier intervention for asymptomatic ICAs identified in LDS patients compared with the general population is likely unwarranted based on the authors' experience at their institution.

中文翻译：

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Loeys-Dietz 综合征颅内动脉瘤的自然史和生长率：对治疗的影响。

目的 Loeys-Dietz 综合征 (LDS) 是一种遗传性主动脉病，与颅面异常、主动脉及其分支的扩张和夹层以及颅内动脉瘤 (ICA) 风险增加相关。鉴于该疾病的罕见性，作者旨在更好地定义 ICA 在这些患者中的自然史和治疗作用。方法回顾性分析83例LDS患者的病历，获取临床、遗传史以及主动脉、主动脉分支和颅内血管的血管影像。回顾了系列放射学报告以及颈部和颅内血管图像，以确定 ICA 的存在、位置和大小。结果 共有 55 例患者（66.3%）至少接受过两次颅内血管检查，19 例（22.9%）患者至少检测到 1 处 ICA。动脉瘤通常很小（平均值±SD 3.2±1.8毫米）。ICA 最常位于海绵状颈动脉，其次是眼动脉血管和大脑前动脉血管。ICA 生长速度为 0.43 ± 0.53 毫米/年，与一般人群相似。3 名患者接受了 ICA 干预，其中 1 名患者并发中风并导致短暂性偏瘫。几个说明性案例详细介绍了作者在这一罕见患者群体中 ICA 生长、新生动脉瘤形成和 ICA 干预方面的经验。结论 LDS 患者的 ICA 很常见，通常很小，并且其生长速度与普通人群中未破裂的 ICA 相似。根据作者在其机构的经验，与普通人群相比，对 LDS 患者中发现的无症状 ICA 进行更积极或更早的干预可能是没有根据的。