Department of Neurosurgery, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA.
Department of Radiology, Mayo Clinic, Rochester, MN, USA.
Acta Neurochir (Wien). 2021 Apr;163(4):1181-1189. doi: 10.1007/s00701-021-04736-z. Epub 2021 Feb 10.
Pure arterial malformations (PAMs) are rare vascular lesions characterized by dilated, tortuous arterial loops without venous shunting. The natural history of PAMs remains unclear. We report the mid-term radiological and longer-term clinical outcomes of the largest series of patients with PAM.
Retrospective review at a tertiary academic referral center for patients with a PAM. Follow-up clinical and radiological data were collected and analyzed for clinical symptoms and radiographic changes.
Twenty-five patients met the inclusion criteria. The mean age at presentation was 30.9 ± 14 years. Nineteen (76%) patients were female, and six (23.1%) were male. Eleven (44%) patients had ≥1 symptom at presentation, of which only 3 (12%) could be linked to PAM. The most common symptom was headache (n=8). PAMs involved a single vessel in 16 (64%) cases and ≥2 vessels in 9 cases (36%). Fifteen (60%) lesions were isolated to the anterior circulation compared to 6 (24%) in the posterior circulation. The most frequently involved vessel was the supraclinoid internal cerebral artery (36%). An associated saccular aneurysm was present in 32% of patients. Ten lesions were partially calcified. The mean radiographic and clinical follow-up was 21.9 ± 26.5 months and 44.6 ± 34.8 months, respectively. None of the patients developed new symptoms related to their lesion or radiographic progression over the duration of follow-up.
PAMs are found most frequently in young, asymptomatic females. PAMs have a benign natural history and are best managed conservatively with serial imaging.
纯动脉畸形（PAMs）是一种罕见的血管病变，其特征为扩张、迂曲的动脉环，无静脉分流。PAMs 的自然病程尚不清楚。我们报告了最大系列 PAMs 患者的中期影像学和长期临床结果。
在三级学术转诊中心对 PAM 患者进行回顾性研究。收集并分析了随访的临床和影像学数据，以了解临床症状和影像学变化。
25 名患者符合纳入标准。就诊时的平均年龄为 30.9 ± 14 岁。19 名（76%）患者为女性，6 名（23.1%）为男性。11 名（44%）患者在就诊时有≥1 个症状，但只有 3 个（12%）可以与 PAM 相关。最常见的症状是头痛（n=8）。PAMs 累及单支血管 16 例（64%），≥2 支血管 9 例（36%）。15 例（60%）病变局限于前循环，6 例（24%）病变局限于后循环。最常受累的血管是前循环的颈内动脉虹吸段（36%）。32%的患者存在伴发的囊状动脉瘤。10 个病变部分钙化。影像学和临床随访的平均时间分别为 21.9 ± 26.5 个月和 44.6 ± 34.8 个月。在随访期间，没有患者因病变或影像学进展而出现新的相关症状。
PAMs 最常发生在年轻、无症状的女性中。PAMs 具有良性自然病程，最好通过连续影像学检查进行保守治疗。
