Zuccaro Graciela, Argañaraz Romina, Villasante Francisco, Ceciliano Alejandro
Department of Neurosurgery, Hospital Nacional de Pediatria Juan P. Garrahan, Buenos Aires, Argentina.
Childs Nerv Syst. 2010 Oct;26(10):1381-94. doi: 10.1007/s00381-010-1223-x. Epub 2010 Jul 25.
This study aims to analyze the clinical and radiological findings, timing and type of treatment, and outcome in children under 1 year of age that presented with neurosurgical vascular malformations.
A retrospective review of 23 children under 1 year of age with neurosurgical vascular malformations was performed at a single institution between 1999 and 2009.
The lesions found in this age group were: 10 vein of Galen aneurysmal malformations (VGAMs), 5 arteriovenous malformations (AVM), 2 pial arteriovenous fistulas (AVF; 1 in the brain and 1 in the spinal cord), 2 cavernous malformations, 2 dural sinus malformations (DSMs) in the posterior fossa with negative angiography which after surgery turned out to be embryonal malformations of dural sinuses, 1 sacular aneurysm, and 1 dural arteriovenous fistula (DAVF) that drained toward the vein of Galen. Of the 10 patients with VGAM, 8 presented choroidal type and 2 mural type. Two patients with choroidal VGAM were born in dramatically severe clinical condition; therefore, we decided to withhold aggressive treatment, and they died within 48 h after birth. The other eight patients with choroidal VGAM received endovascular treatment between 1 and 3 staged embolizations. In four of them, total occlusion was achieved and subtotal and partial in the others. One patient had complications and evolved with severe developmental delay. Another patient with partial occlusion died, and another patient with previous developmental delay stopped treatment because of parental decision making. Five patients evolved with normal development and one with mild delay. Only one patient required shunt. Hydrocephalus was solved after endovascular treatment in the rest of the patients. Regarding the five AVMs, four were treated with microsurgical approach achieving total resection of the lesion and normal developmental milestones except for one patient with brainstem AVM that was admitted with quadriparesis and coma. In the patient with basal ganglia AVM, the lesion spontaneously disappeared. From the two pial AVFs, the frontal one was microsurgically treated and the spinal one received endovascular session achieving 50% of lesion occlusion for which further treatment is needed. The two cavernous malformations were operated achieving total resection with normal development. The two children with DSM received surgical resection without complications and normal development. The rare case of sacular aneurysm at this age was occluded at the time of diagnostic procedure due to the bad clinical condition of the patient and the hematoma was removed immediately in the operating room. The only case of DAVF died of respiratory intercurrence after three sessions of endovascular treatment.
In this neonatal age group (first year of life), we can find the whole range of neurosurgical vascular pathology: VGAM, AVM, cavernous malformation, DSM, pial AVF, DAVF, and sacular aneurysm. VGAM is the most frequent vascular malformation during the first year of age. The early treatment of vascular malformations prevents its adverse effects on a developing brain. A multidisciplinary team composed by endovascular and surgical specialists is necessary to discuss and treat each case.
本研究旨在分析1岁以下患有神经外科血管畸形患儿的临床和影像学表现、治疗时机与类型以及治疗结果。
对1999年至2009年间在一家机构就诊的23例1岁以下患有神经外科血管畸形的患儿进行回顾性研究。
该年龄组发现的病变有:10例大脑大静脉动脉瘤样畸形(VGAM)、5例动静脉畸形(AVM)、2例软膜动静脉瘘(AVF;1例位于脑部,1例位于脊髓)、2例海绵状畸形、2例后颅窝硬脑膜窦畸形(DSM),血管造影阴性,术后证实为硬脑膜窦胚胎性畸形、1例囊状动脉瘤以及1例向大脑大静脉引流的硬脑膜动静脉瘘(DAVF)。10例VGAM患者中,8例为脉络丛型,2例为壁型。2例脉络丛型VGAM患儿出生时临床状况极为严重;因此,我们决定不进行积极治疗,他们在出生后48小时内死亡。其他8例脉络丛型VGAM患者接受了1至3期的血管内栓塞治疗。其中4例实现了完全闭塞,其他患者为次全闭塞和部分闭塞。1例患者出现并发症,伴有严重发育迟缓。另1例部分闭塞的患者死亡,另1例先前有发育迟缓的患者因家长决定而停止治疗。5例患者发育正常,1例轻度发育迟缓。仅1例患者需要分流。其余患者在血管内治疗后脑积水得到解决。对于5例AVM,4例采用显微手术方法治疗,实现了病变的完全切除,除1例脑干AVM患者以四肢瘫和昏迷入院外,其余患者发育里程碑正常。在基底节AVM患者中,病变自发消失。2例软膜AVF中,额叶的1例接受了显微手术治疗,脊髓的1例接受了血管内治疗,病变闭塞50%,需要进一步治疗。2例海绵状畸形均接受手术并实现完全切除,发育正常。2例DSM患儿接受手术切除,无并发症,发育正常。该年龄罕见的囊状动脉瘤病例因患者临床状况不佳,在诊断过程中即被闭塞,血肿在手术室立即被清除。唯一的DAVF病例在3次血管内治疗后死于呼吸并发症。
在这个新生儿年龄组(出生第一年),我们可以发现神经外科血管病变的全部类型:VGAM、AVM、海绵状畸形、DSM、软膜AVF、DAVF和囊状动脉瘤。VGAM是1岁以内最常见的血管畸形。血管畸形的早期治疗可防止其对发育中的大脑产生不良影响。由血管内和外科专家组成的多学科团队对于讨论和治疗每个病例是必要的。