Rodesch G, Malherbe V, Alvarez H, Zerah M, Devictor D, Lasjaunias P
Hôpital Bicêtre, Le Kremlin Bicêtre, France.
Childs Nerv Syst. 1995 Apr;11(4):231-41. doi: 10.1007/BF00277659.
We present 26 consecutive cases of nongalenic pial arteriovenous malformations (PAVMs) diagnosed in the neonatal period or in infancy. No diagnosis was made antenatally. Presenting symptoms in neonates were systemic cardiac manifestations (54%), seizures (31%), and hemorrhages (15%). In infants, hemorrhagic strokes and hydrodynamic disorders (external or internal hydrocephaly, macrocephaly, atrophy) both occurred in 38% of cases. Systemic cardiac manifestations and seizures were rare at that age (respectively 16% and 8%). Sixty-two percent of neonates and 31% of infants already had neurocognitive disorders (assessed by pediatric neurocognitive testing: Brunet-Leizine and Denver tests) when referred. The venous drainage and its anomalies (ectasias, stenoses, thromboses) were the main causes of symptoms. Atrophy and leukomalacic lesions occurred rapidly; they express local hydrovenous disorders and are specific to this population group. Untreated neonates and infants have a poor prognosis. Endovascular treatment, although partial and challenging in all instances, represents the treatment of choice in our series. Of the eight neonates treated, one improved to normal (12.5%), while four remained stable (50%): two neurologically normal, two with mild neurological deficit. Three (37%) died despite embolization (heart failure, multiorgan failure, postoperative death). Transient neurological complications occurred in two cases (25%): hemiparesis in one patient with a rolandic and in one with a thalamic AVM. Of the eight infants successfully embolized, one was significantly improved (12.5%) and is now neurologically normal, while five remained stable (62.5%): four neurologically normal, one with mild neurological deficit. One died between two sessions of embolization from intracerebral hemorrhage (12.5%). Hemianopsy occurred in one case (12.5%) after embolization of an occipital AVM. In one additional case in a normal child we failed to embolize the last small pial AVM of four after the three others had spontaneously thrombosed. With a minimal follow-up of 18 months and a maximum of 7 years, the review of our series shows 53% of the initial group of neonates and infants growing neurologically normal after therapeutic management in our institution; 23.5% died despite treatment, and the remaining 23.5% present minor neurological deficit. When targeted at the points of angioarchitectural weakness, embolization contributes to stabilizing a lesion. It should be undertaken rapidly to avoid loss of brain substance secondary to hemorrhage, atrophy, or leukomalacia, and to allow neurocognitive recovery and normal brain maturation. In our experience, these lesions are the most aggressive ones for the maturing brain, and the most difficult to approach technically. They represent a new therapeutic field and have their own specific anatomy and physiology.
我们报告了26例在新生儿期或婴儿期诊断出的非盖伦型软膜动静脉畸形（PAVM）。产前均未作出诊断。新生儿的首发症状为全身性心脏表现（54%）、癫痫发作（31%）和出血（15%）。在婴儿中，出血性中风和流体动力学障碍（外部或内部脑积水、巨头畸形、萎缩）在38%的病例中均有发生。全身性心脏表现和癫痫发作在该年龄段较为罕见（分别为16%和8%）。62%的新生儿和31%的婴儿在转诊时已存在神经认知障碍（通过儿科神经认知测试：布鲁内 - 勒津和丹佛测试评估）。静脉引流及其异常（扩张、狭窄、血栓形成）是症状的主要原因。萎缩和脑白质软化病变迅速出现；它们表现为局部静脉性疾病，且是该人群特有的。未经治疗的新生儿和婴儿预后较差。血管内治疗虽然在所有情况下都是部分性且具有挑战性，但在我们的系列研究中是首选治疗方法。在接受治疗的8例新生儿中，1例改善至正常（12.5%），4例病情稳定（50%）：2例神经功能正常，2例有轻度神经功能缺损。3例（37%）尽管进行了栓塞仍死亡（心力衰竭、多器官衰竭、术后死亡）。2例（25%）出现短暂性神经并发症：1例额叶动静脉畸形患者和1例丘脑动静脉畸形患者发生偏瘫。在成功栓塞的8例婴儿中，1例显著改善（12.5%）且目前神经功能正常，5例病情稳定（62.5%）：4例神经功能正常，1例有轻度神经功能缺损。1例在两次栓塞治疗期间因脑出血死亡（12.5%）。1例枕叶动静脉畸形栓塞后出现偏盲（12.5%）。在另外1例正常儿童中，在其他3个小的软膜动静脉畸形自发血栓形成后，我们未能栓塞最后1个小的软膜动静脉畸形。经过至少18个月、最长7年的随访，对我们系列研究的回顾显示，在我们机构接受治疗的最初一组新生儿和婴儿中，53%神经功能正常生长；23.5%尽管接受了治疗仍死亡；其余23.5%有轻度神经功能缺损。当针对血管构筑薄弱点进行栓塞时，有助于稳定病变。应迅速进行栓塞以避免因出血、萎缩或脑白质软化导致脑实质丧失，并促进神经认知恢复和大脑正常发育。根据我们的经验，这些病变对发育中的大脑最具侵袭性，在技术上也最难处理。它们代表了一个新的治疗领域，有其自身特定的解剖学和生理学特点。
