Li Junrao, Wang Ting, Richard Seidu A, Zhang Changwei, Xie Xiaodong, Wang Chaohua
Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, China.
Department of Medicine, Princefield University, Ho-Volta Region, Ghana West Africa.
Medicine (Baltimore). 2020 Jun 26;99(26):e20991. doi: 10.1097/MD.0000000000020991.
Pial arteriovenous fistula (PAVF) occurs when intracranial arteries communicate directly with veins. PAVFs are very rare congenital vascular lesions that are commonly seen in infants and children. Arachnoid cysts are congenital cavitation often filled with cerebrospinal fluid. We present a very rare associated occurrence of bilateral pediatric PAVF and a giant arachnoid cyst presenting as torticollis in a child. So far, this is the first case.
A 6-year-old male child was referred to our facility from a local hospital due to severe torticollis.
An enhanced computed tomography scan revealed 2 slightly high-density masses at the anterior pontine cistern, right circumferential cistern, as well as left posterior occipital region. The same computed tomography scan revealed a giant arachnoid cyst in the left occipital as well as the temporal region with a thin cerebral cortex adjacent to the cyst.
Digital subtraction angiography confirmed that the 2 high-flow lesions were PAVFs. The patient was treated with a combination of detachable coils and Onyx Liquid Embolic System (Onyx HD-500) (Covidien/ev3 Neurovascular) via the transarterial endovascular route while the giant arachnoid cyst was managed conservatively.
The torticollis resolved 2 days after the procedure. He is currently well with no neurologic deficit.
We advocate that in cases of PAVF with accompanying cyst, the cyst should be managed conservatively if it is not associated with intracranial hemorrhage or focal neurologic deficit.
软脑膜动静脉瘘(PAVF)是指颅内动脉与静脉直接相通。PAVF是非常罕见的先天性血管病变,常见于婴幼儿。蛛网膜囊肿是通常充满脑脊液的先天性空洞。我们报告了一例非常罕见的双侧小儿PAVF与巨大蛛网膜囊肿并存且表现为斜颈的病例。到目前为止,这是首例。
一名6岁男童因严重斜颈从当地医院转诊至我院。
增强计算机断层扫描显示脑桥前池、右侧环池以及左枕后区域有2个略高密度肿块。同一次计算机断层扫描显示左枕叶及颞叶有一个巨大蛛网膜囊肿,囊肿旁脑皮质较薄。
数字减影血管造影证实这2个高流量病变为PAVF。患者通过经动脉血管内途径接受了可脱性弹簧圈和Onyx液体栓塞系统(Onyx HD - 500)(柯惠/ev3神经血管公司)联合治疗,而巨大蛛网膜囊肿则采取保守治疗。
术后2天斜颈缓解。他目前情况良好,无神经功能缺损。
我们主张,对于伴有囊肿的PAVF病例,如果囊肿与颅内出血或局灶性神经功能缺损无关,应采取保守治疗。