Patel Milin, Grewal Sahibjot Singh, Ahmed Rashid A, Rabinov James D, Regenhardt Robert W, Dmytriw Adam A
University of Ottawa, Ottawa, Canada.
Western University, London, Canada.
Neuroradiology. 2025 Jun 3. doi: 10.1007/s00234-025-03665-3.
Pure arterial malformations (PAMs) of the brain are rare vascular anomalies, typically identified incidentally and characterized by tortuous arterial loops. Their unclear etiology and management pose diagnostic and therapeutic challenges.
A systematic review of Medline, EMBASE, and Web of Science databases (1900-present) identified 15 studies involving 54 patients with PAM. Data on demographics, clinical presentations, diagnostic modalities, and treatments were extracted.
The mean age was 37.2 years (range: 10-77), with a female predominance (69%). Severe headaches were the most common symptom (43%), though some cases presented with neurological deficits (20%) or incidentally (32%). Imaging modalities such as digital subtraction angiography, MRI, and CT were pivotal in diagnosis. Most cases (81%) were managed conservatively, while 19% had interventions, including clipping and embolization. Surgical management was effective in alleviating symptoms in select cases of symptom progression.
Our findings highlight the variability in clinical presentations ranging from benign to acute emergencies, emphasizing the challenges associated with the diagnosis and management of PAMs. This review underscores the need for larger studies that explore the etiology, treatment efficacy, and long-term patient outcomes.
脑纯动脉畸形（PAMs）是罕见的血管异常，通常为偶然发现，其特征为迂曲的动脉环。其病因不明且治疗困难，给诊断和治疗带来挑战。
对Medline、EMBASE和Web of Science数据库（1900年至今）进行系统综述，确定了15项涉及54例PAM患者的研究。提取了有关人口统计学、临床表现、诊断方法和治疗的数据。
平均年龄为37.2岁（范围：10 - 77岁），女性占多数（69%）。严重头痛是最常见的症状（43%），不过有些病例表现为神经功能缺损（20%）或偶然发现（32%）。数字减影血管造影、MRI和CT等影像学检查方法对诊断至关重要。大多数病例（81%）采用保守治疗，而19%的病例进行了干预，包括夹闭和栓塞。手术治疗在某些症状进展的病例中有效缓解了症状。
我们的研究结果突出了临床表现从良性到急性紧急情况的变异性，强调了与PAMs诊断和治疗相关的挑战。本综述强调需要进行更大规模的研究，以探索病因、治疗效果和患者长期预后。
