儿童期颅内动静脉畸形临床及形态学分析

Clinical and morphological analysis of intracranial arteriovenous malformation in childhood

目的：探讨儿童期颅内动静脉畸形（AVM）的临床特点及形态学表现。方法2012年1月-2013年6月收治14岁以下颅内 AVM 患儿14例，其中11例（78．6%）曾有自发性脑出血病史，头痛7例（50．0%），单侧肢体乏力5例（35．7%），2例（14．3%）伴有癫痫发作。回顾性分析患儿的临床特点、术前影像及全脑血管 DSA 资料。结果 DSA 显示终末支供血9例，另5例为穿支供血；单支供血7例，另7例多支供血的患儿中4例为前、后循环联合供血。病变在侧裂面及凸面5例，侧脑室4例，脑底面3例，半球内侧面2例。10例病灶大小为0～30 mm，4例为30～60 mm。根据 Spetzler-Martin 分级，Ⅰ级4例、Ⅱ级2例、Ⅲ级5例、Ⅳ级3例。结论儿童期颅内 AVM 病灶虽然体积小、评级低，但其发病早、易出血，需要临床积极的综合治疗。 DSA 造影发现多数儿童期 AVM 呈幼稚型，其自然病程需要进一步随访研究。

Objective To discuss the clinical manifestations and the morphological features of intracranial arteriovenous malformation （AVM） in childhood. Methods During the period from Jan. 2012 to June 2013, a total of 14 AVM patients under the age of 14 years were admitted to authors’ hospital. Of the 14 patients, 11 had a history of spontaneous intracerebral hemorrhage （78.6%）, 7 complained of headache （50.5% ）, 5 showed signs of unilateral limb weakness （35.7% ）, and 2 was accompanied with epileptic attacks （14.3% ）. The clinical characteristics, the preoperative imaging features and DSA findings were retrospectively analyzed. Results DSA showed that terminal feeding branches were visualized in 9 cases and blood supply from perforating branches was seen in the remaining 5 cases. In seven cases the feeding artery was single, while in the remaining 7 cases the feeding artery was multiple, and in four of them combination blood supply from both anterior and posterior circulation was found. The lesions were situated at the lateral or convex surface of cerebral hemisphere （n = 5）, lateral ventricle （n = 4）, cerebral bottom （n = 3） or medial surface of cerebral hemisphere （n = 2）. The lesion’s diameter was 〈 30 mm in 10 cases and （30 -60） mm in 4 cases. According to Spetzler- Martin grading, grade Ⅰ was seen in 4 cases, grade Ⅱ in 2 cases, grade Ⅲ in 5 cases and grade Ⅳ in 3 cases. Conclusion Although in childhood AVM lesion is small in size and lower in grading, its onset is early and the lesion is prone to bleeding. DSA findings indicate that most AVMs in childhood take the form of immature type. Nevertheless, its natural course needs to be further studied.