Macroglossia due to venous malformation

Massive vascular malformation involving tongue can cause significant functional impairment. In this report we describe a rare case of extensive venous malformation involving tongue leading to obstructive sleep apnoea, inability to speak, eat, severe discomfort due to exposure induced dryness and ulceration. Multimodality treatment approach comprising of sclerotherapy, electrocautery and radiation therapy was used in this case.     

症状性大脑中动脉缺血患者脑血管储备能力的CT灌注成像研究

目的采用5％CO2吸入CT灌注成像研究症状性大脑中动脉（MCA）缺血患者MCA分布区脑血管储备能力。资料与方法临床诊断为症状性MCA缺血且无心、肺疾病患者20例,基础态CT灌注成像TTP参数图显示有MCA供血区TTP延长。首先进行常规脑灌注CT检查（基础态）;20min后,面罩吸入5％CO2,持续吸入2min后再次行脑灌注CT检查（激发态）。感兴趣层面选择基底节层面和放射冠层面,层厚10mm。计算机后处理获得脑血流量（CBF）参数图后,于MCA分布区皮层多点手绘感兴趣区,分别计算双侧皮层CBF,计算双侧CBF变化率r=（CBF激发态-CBF基础态）／CBF基础态×100％;比较双侧激发后CBF改变,分析患侧MCA分布区脑血管储备能力。结果患侧MCA分布区皮层CBF明显低于健侧。健侧MCA分布区基础态和激发态CBF可见不同程度的改变：升高17例,降低3例。对17例激发后升高的患者进行分析,根据计算将患侧CO：激发反应分为：Ⅰ型,激发后CBF升高;Ⅱ型,激发后CBF变化不明显;Ⅲ型,激发后CBF降低。结论5％CO2吸入CT灌注成像激发试验可以评估脑血管储备能力,具有重要的临床价值。     

PC-MRI在Ⅰ型Chiari畸形枕大池成形术后疗效评估中的应用

目的探讨磁共振相位对比电影成像(PC-MRI)技术在Ⅰ型Chiari畸形枕大池成形术后疗效评估中的应用价值。方法2018年6月至2020年12月前瞻性收集Ⅰ型Chiari畸形21例,均行枕大池成形术治疗,术前及术后6个月行PC-MRI检查,分析中脑导水管及脑桥腹侧脑脊液平均流量(MF)、头向峰值流速(Vumax)及足向峰值流速(Vdmax)。术后6个月,根据症状缓解与否,分为症状缓解组和无缓解组。结果术后6个月,15例术后症状消失或缓解(缓解组),6例症状无明显改善(无缓解组)。缓解组中脑导水管、脑桥腹侧Vdmax和Vumax较术前均明显降低(P<0.05),而MF则明显增加(P<0.05)。无缓解组手术前后中脑水管及脑桥腹侧脑脊液MF、Vdmax和Vumax均明显变化(P>0.05)。结论 PC-MRI可定量分析脑脊液流动情况,对Ⅰ型Chiari畸形术后疗效评估具有一定价值。     

硬化剂治疗口腔颌面部静脉畸形疗效的对比研究

目的:评价平阳霉素(PYM)单独使用或联合鱼肝油酸钠(SM)和地塞米松(DXM)瘤内注射治疗口腔颌面部静脉畸形的疗效和安全性。方法:选择100例口腔颌面部静脉畸形患者,随机分为两组。PYM组50例瘤腔内单独注射PYM;联合用药组50例,用DXM 5 mg/ml加2%利多卡因4ml瘤内注射,尔后再据年龄、瘤体大小,局部情况注射0.5～2.0mlSM。均5～7d注射1次,3～5次为一个疗程。分析其疗效和安全性。结果:治疗后经6～48个月随访。PYM组有效率84.0%,联合用药组有效率96.0%。明显高于PYM组(P<0.05)。PYM组有5例(10.0%)出现发热,联合用药组无发热病例(P<0.05)。结论:PYM、DXM、SM联合注射治疗口腔颌面部静脉畸形,具有疗程短、不良反应小等优点,是一种安全、可靠、简便的治疗方法,其疗效优于PYM单独瘤内注射治疗。     

面颈部静脉畸形的综合治疗

目的:探讨多种方法综合治疗口腔及面颈部大面积静脉畸形的临床效果。方法:根据患者的年龄、病变部位、病变大小制订个体化治疗方案,采用多种药物联合注射,结合病变周围环形缝扎、激光、手术等方法综合治疗口腔颌面部大面积静脉畸形134例。结果:134例中治疗效果优79例(58.96%),良45例(33.58%),显效10例(7.46%),差0例。结论:采用多种方法综合治疗口腔颌面部大面积静脉畸形,对提高疗效、改善面部形态、维持局部功能、减少不良反应具有重要意义。     

刺络拔罐法对减低中风后上肢肌张力增高的临床研究

目的　观察刺络拔罐法对脑血管意外患者肌张力增高的改善情况。方法　将已出现肌张力增高的脑血管意外患者6 2例 ,随机分为刺络拔罐组 31例、对照组 31例。结果　刺络拔罐组在治疗后的肌张力较治疗前有显著降低 (P<0 .0 0 1) ,并且疗效优于对照组 (P<0 .0 5 )。结论　刺络拔罐法可显著改善脑血管意外患者肌张力增高的情况。     

Thirty-One Autopsy Cases of Trisomy 18: Clinical Features and Pathological Findings

The clinical features and morphological findings in 31 Japanese infants with trisomy 18 are presented. The majority were small-for-date infants. There was no sex predominance in our series, as opposed to male female ratios of 1:3 reported in the literature. The average age at death was greater in females than in males. Cardiovascular anomalies were consistently present; ventricular septar defect and patent ductus arteriosus being the most common malformations. Various other internal malformations including the Arnold-Chiari malformation were observed.     

Birth outcomes of patients with isolated anorectal malformations: A population‐based case‐control study

In most patients affected by isolated anorectal malformation (IARM) the etiology is largely unknown. Thus, the aim of our project was to analyze possible risk factors for IARM. In the first step, birth outcomes of cases with IARM were analyzed on the basis of maternal socio‐demographic variables, and these data are presented in this paper. Gestational age at delivery, birthweight, preterm birth, low birthweight and small for gestational age of cases with IARM were evaluated in the function of maternal age, birth/pregnancy order, marital and employment status of mothers in the population‐based large dataset of the Hungarian Case‐Control Surveillance of Congenital Abnormalities, 1980–1996. The study samples included 231 live‐born cases with IARM, 361 matched and 38 151 population controls without any defect. IARMs are more frequent in males, twins and newborn infants with low birthweight and small‐for‐gestational‐age, the latter being the consequence of intrauterine growth restriction. In addition, mothers of cases were younger but with higher birth order, and had lower socio‐economic status. These maternal variables are characteristic for the gypsy population in Hungary. The higher proportion of gypsy women among the mothers of cases with IARM was confirmed during the home visits of the study. Male sex and intrauterine growth restriction of cases, in addition to low socioeconomic status and gypsy origin of mothers may have a role in the risk of IARMs.     

伽玛刀治疗血管性病变后脑水肿发生率的分析

目的探讨不同的血管性疾病γ刀治疗后脑水肿的发生率。方法回顾十余年内经γ刀治疗的血管性病变358例,采用χ^2检验探讨γ刀术后不同血管性病变及不同随访时间脑水肿发生率的差异性。结果γ刀术后,动静脉畸形（AVM）组水肿阳性率为47.5%,海绵状血管瘤组为25.9%,术后水肿发生率有显著性差异（P〈0.01）。不同随访阶段水肿阳性率分别为：≤1年为47.3%,1～3年为38.0%,3～5年为37.1%,〉5年为25.0%,不同随访阶段脑水肿发生率有显著性差异（P〈0.01）。结论脑水肿是γ刀治疗后最重要的并发症之一,术后1年内水肿的发生率最高。     

产前超声在先天性心脏大血管畸形中的诊断价值

目的 探讨先天性心脏大血管畸形的超声特点及其产前诊断方法.方法 随机选取本院2012年3月至2015年3月受检胎儿2260例,对其行胎儿心脏四腔心切面联合胎儿头侧偏转法以对心脏畸形进行快速筛查.对疑似心脏畸形者行大动脉短轴切面、主动脉弓切面、肺动脉长轴切面、左右心室流出道、三血管气管平面超声检查,并对内部血流情况、血管内径大小、排列关系等进行观察记录.结果 本次研究中共计检出82例先天性心脏大血管畸形,其中以血管排列异常、血流性质异常、血管内径大小异常及血管数目异常四种超声线索最为常见.结 论产前超声检查是一种有效的先天性心脏大血管畸形诊断方法,其中大动脉数目异常、内径大小异常、血流异常及血管排列异常均可作为临床诊断的可靠线索.