Lu Alex Y, Winkler Ethan A, Garcia Joseph H, Raygor Kunal P, Fullerton Heather J, Fox Christine K, Kim Helen, Auguste Kurtis I, Sun Peter P, Hetts Steven W, Lawton Michael T, Abla Adib A, Gupta Nalin
Departments of1Neurological Surgery.
2Department of Neurosurgery, Barrow Neurological Institute, Phoenix, Arizona.
J Neurosurg Pediatr. 2023 Feb 17;31(5):463-468. doi: 10.3171/2023.1.PEDS22541. Print 2023 May 1.
Patients with unruptured brain arteriovenous malformations (AVMs) may present with headaches, seizures, and/or neurological deficits. A smaller number of cases may be discovered incidentally. These lesions remain incompletely understood due to their sparse reporting. Herein, the authors describe the largest series to date comparing the presentation, angioarchitecture, and management of incidental versus symptomatic unruptured AVMs in children.
The authors performed a retrospective analysis of patients who presented with brain AVMs from 1998 to 2022 at the University of California, San Francisco. Inclusion criteria were age ≤ 18 years at the time of presentation and an angiographically proven unruptured AVM that had been diagnosed postnatally.
Of 76 children with unruptured AVMs, 66 (86.8%) presented with headaches, seizures, and/or neurological deficit. Ten AVMs (13.1%) were incidentally discovered through unrelated disease workup (50%), cranial trauma (40%), or research study participation (10%). Compared with patients with symptomatic unruptured AVMs, patients with incidental unruptured AVMs had a smaller mean ± SD maximum nidus diameter (2.82 ± 1.1 vs 3.98 ± 1.52 cm, p = 0.025) and fewer had deep venous drainage (20% of patients vs 61%, p = 0.036). They also presented at an earlier age (10 ± 5.2 vs 13.5 ± 4 years, p = 0.043) and with longer duration to first treatment (541 ± 922 vs 196 ± 448 days, p = 0.005). During the observation period, 1 patient developed recurring headaches and demonstrated AVM nidus growth. Four AVMs greater than 3 cm in size or in a deep location were treated with radiosurgery. Six other AVMs were treated with resection, with 2 receiving preoperative embolization. Eight AVMs (80%) were obliterated on last follow-up. Postprocedural complications included 2 transient neurological deficits after resection and 1 case of delayed seizure development after radiosurgery. The mean follow-up period was 5.7 ± 5.7 years without any hemorrhage episodes.
A substantial proportion of pediatric patients with unruptured AVMs are discovered incidentally. With earlier presentation and more elementary angioarchitecture than symptomatic unruptured AVMs, these incidental lesions provide a snapshot into the natural history of AVM before symptom development or rupture.
未破裂脑动静脉畸形（AVM）患者可能出现头痛、癫痫发作和/或神经功能缺损。少数病例可能是偶然发现的。由于相关报道较少，这些病变仍未被完全了解。在此，作者描述了迄今为止最大规模的系列研究，比较儿童偶然发现的与有症状的未破裂AVM的临床表现、血管结构和治疗情况。
作者对1998年至2022年在加利福尼亚大学旧金山分校就诊的脑AVM患者进行了回顾性分析。纳入标准为就诊时年龄≤18岁，且经血管造影证实为出生后诊断的未破裂AVM。
76例未破裂AVM患儿中，66例（86.8%）出现头痛、癫痫发作和/或神经功能缺损。10例AVM（13.1%）是在无关疾病检查（50%）、颅脑外伤（40%）或参与研究（10%）过程中偶然发现的。与有症状的未破裂AVM患者相比，偶然发现的未破裂AVM患者的平均最大瘤巢直径±标准差较小（2.82±1.1 vs 3.98±1.52 cm，p = 0.025），深静脉引流的患者较少（20% vs 61%，p = 0.036）。他们就诊时年龄更小（10±5.2 vs 13.5±4岁，p = 0.043），首次治疗的时间间隔更长（541±922 vs 196±448天，p = 0.005）。在观察期内，1例患者出现复发性头痛，且AVM瘤巢增大。4例直径大于3 cm或位于深部的AVM接受了放射外科治疗。另外6例AVM接受了切除术，其中2例接受了术前栓塞。8例AVM（80%）在最后一次随访时闭塞。术后并发症包括2例切除术后短暂性神经功能缺损和1例放射外科治疗后迟发性癫痫发作。平均随访期为5.7±5.7年，无任何出血事件。
相当一部分未破裂AVM的儿科患者是偶然发现的。与有症状的未破裂AVM相比，这些偶然发现的病变出现更早，血管结构更简单，为AVM在出现症状或破裂之前的自然病程提供了一个快照。
