儿童出血型烟雾病的临床特征和手术疗效观察

目的探讨儿童出血型烟雾病的临床特征及脑硬膜颞浅动脉血管融通术（EDAS）的治疗效果。方法回顾性分析2002年12月—2011年12月25例出血型烟雾病患儿的临床及影像学资料。对25例行双侧EDAS,应用正电子发射断层显像术（PET）和DSA评估手术前后脑代谢和血管重建情况。结果①出血型烟雾病患儿占本院同期收治的儿童烟雾病患儿的5.56%（25/450）。男∶女=1∶1.27（11/14）。17例（68%）以脑出血为首发症状,8例在出血前有脑缺血症状。6例在出血急性期（1周）出现脑缺血症状。脑实质出血5例（20%）,单纯脑室出血20例（80%）。合并动脉瘤4例。②50侧半球有30侧存在脉络膜前动脉和后交通动脉的扩张。其中脑出血侧占63%（19/30）,非出血侧占37%（11/30）,P=0.021。术前PET检查显示,出血侧脑代谢降低22例,正常3例;非出血侧脑代谢降低21例,正常4例。③术后随访1～8年,平均为（3.8±2.1）年。25例随访期均未发生再出血。7例原有神经功能缺损症状均未加重,随访期间没有新发缺血症状;6例患儿有间断头痛、肢体无力等缺血症状,其余患儿未诉不适。11例术后6～18个月行DSA及PET检查。DSA示颞浅动脉与颅内血管形成交通,出血侧有10例（10/11）,非出血侧有9例（9/11）。PET示出血侧10例脑代谢明显改善,1例无明显变化;非出血侧8例明显改变,3例无明显变化。结论脉络膜前动脉和后交通动脉的扩张是儿童烟雾病出血的重要原因之一。EDAS能有效地改善患儿的脑代谢,重建脑血流;同时有可能预防再出血。

Observation of clinical characteristics and surgical efficacy of hemorrhagic moyamoya disease in children

Objective To observe the clinical characteristics of hemorrhagic moyamoya disease in children and the therapeutic effect of encephalo-duro-arterio-synangiosis(EDAS) on it.Methods The clinical and imaging data of 25 children with hemorrhagic moyamoya disease from December 2002 to December 2011 were analyzed retrospectively,and their clinical characteristics were analyzed.The bilateral EDAS were performed in all the children.Cerebral metabolism and vessel revascularization were evaluated with positron emission tomography(PET) and digital silhouette angiography(DSA) before and after procedures.Results ①Hemorrhagic moyamoya disease in children accounted for 5.56%(25/450) of all the children admitted in hospital over the same period.The male to female ratio was 1 ∶ 1.27(11/14).Cerebral hemorrhage was the first symptom in 17 children(68%) and 8 had the symptom of cerebral ischemia before cerebral hemorrhage.Six children had symptoms of cerebral ischemia in the acute stage(1 week).Five(20%) had parenchymal hemorrhage,and 20(80%) had intraventricular hemorrhage alone.Four had aneurysmal intracranial hemorrhage.②30 of the 50 hemispheres had dilation of anterior choroidal artery and posterior communicating artery.Among them,the hemorrhagic hemispheres accounted for 63%(19/30),and the non-hemorrhagic hemispheres accounted for 37%(11/30)(P=0.021).Preoperative PET examination showed that the cerebral metabolism in 22 children were reduced on the hemorrhagic hemisphere,and 3 were normal;the cerebral metabolism were reduced in 21 on the hemorrhagic hemisphere,and 4 were normal.③25 children were followed up for 1 to 8 years(mean 3.8±2.1 years) and none of them occurred rebleeding during the follow-up period.The original neurological deficit symptom in 7 children did not aggravate.No new ischemic symptoms occurred during the follow-up period;6 children complained of ischemic symptoms,such as intermittent headache and limb weakness,and the others did not complain of any discomfort.DSA and PET scans were performed in 11 children from 6 to 18 months after procedure.DSA showed that the superficial temporal arteries communicated with the intracranial blood vessels in 10 children(10/11) on the hemorrhagic hemisphere and in 9(9/11) on the non-hemorrhagic hemisphere.PET showed that the cerebral metabolism was improved significantly in 10 children on the hemorrhagic hemisphere;one had no significant change;8 had significant change and 3 had no significant change on the non-hemorrhagic hemisphere.Conclusion The dilation of anterior choroidal artery and posterior communicating artery is one of the important reasons of hemorrhage in children with moyamoya disease.EDAS may effectively improve the cerebral metabolism and reconstruction of cerebral blood flow in children,at the same time,it may prevent rebleeding.