Niimi Yasunari
Department of Neuroendovascular Therapy, St Luke's International Hospital, Tokyo, Japan.
Pediatr Int. 2017 Mar;59(3):247-257. doi: 10.1111/ped.13159.
Intracranial arteriovenous shunts (ICAVS) in young children are characterized by frequent high-flow fistulas. In association with high-flow fistulas and the physiological condition of the developing brain and heart, each ICAVS type tends to present at a certain age with unique symptoms. Vein of Galen aneurysmal malformation (VGAM) and dural sinus malformation with arteriovenous (AV) shunt tend to present in the neonate with high output cardiac failure. In infancy, VGAM, pial arteriovenous fistula (AVF) and infantile dural AVF (DAVF) tend to present with hydrodynamic disorder such as macrocephaly, ventriculomegaly, prominent facial veins, and developmental delay. Pial AVF, AV malformation, and infantile DAVF can present with focal neurological signs such as seizure or hemorrhage at older ages. Endovascular treatment is currently the first choice of treatment for most pediatric ICAVS. The treatment goal should be defined on a patient-by-patient basis, according to the unique physiological condition of the child.
幼儿颅内动静脉分流（ICAVS）的特点是频繁出现高流量瘘。与高流量瘘以及发育中的脑和心脏的生理状况相关，每种ICAVS类型往往在特定年龄出现独特症状。大脑大静脉瘤样畸形（VGAM）和伴有动静脉（AV）分流的硬脑膜窦畸形往往在新生儿期出现高输出量心力衰竭。在婴儿期，VGAM、软膜动静脉瘘（AVF）和婴儿硬脑膜动静脉瘘（DAVF）往往表现为脑积水相关病症，如巨头畸形、脑室扩大、面部静脉突出和发育迟缓。软膜AVF、AV畸形和婴儿DAVF在较大年龄时可出现局灶性神经体征，如癫痫发作或出血。血管内治疗目前是大多数小儿ICAVS的首选治疗方法。治疗目标应根据患儿独特的生理状况，逐例确定。
