后颅窝骨性扩大术治疗Chiari畸形合并脊髓空洞症

目的　介绍后颅窝骨性扩大术治疗Chiari畸形合并脊髓空洞症。方法　该手术分为两种亚术式 ,一种是枕颈固定扩大术 :取自体髂骨 5cm× 10cm ,打磨成“凹”字形。上端为齐头端 ,固定在后颅窝减压窗缘。缺口端为下端 ,固定在枢椎棘突上 ,将扩大的硬脑 (脊 )膜修补筋膜片固定在植骨片内面 ,使筋膜片贴附在植骨片内面 ,形成帐篷状 ;另一种是单纯骨性扩大术 :取自体髂骨 6cm× 8cm的梯形骨片 ,并利用较厚的髂骨缘打磨成弧形。植骨片固定在后颅窝减压窗上 ,弧形端构成枕骨大孔后缘。将扩大的硬脑 (脊 )膜修补筋膜片也固定在植骨片内面 ,使筋膜片贴附在植骨片内面 ,也形成帐篷状。结果　本组病例随访 2～ 7年 ,术后症状体征缓解总有效率为91.6% ,肌力比以前提高 2～ 3级。结论　该术式比其他治疗Chiari畸形合并脊髓空洞症的术式 ,治疗效果显著 ,疗效切实可靠     

增殖细胞核抗原在血管瘤和血管畸形中的表达及其临床意义

目的探讨不同类型血管瘤、血管畸形和同一类型不同时期的内皮细胞增殖情况及肥大细胞计数的变化。方法采用免疫组织化学方法及细胞特染技术,对120例婴幼儿和28例成人血管瘤标本进行了增殖细胞核抗原(PCNA)检测和肥大细胞计数比较。结果增生期毛细血管瘤、混合型血管瘤PCNA表达为阳性,其他均为阴性。经统计学处理,各类增生期血管瘤PCNA表达阳性率及肥大细胞计数均明显高于消退期婴幼儿血管瘤及各类血管畸形,有显著差异(P<0.01)。结论PCNA的表达对血管瘤和血管畸形的鉴别诊断及治疗选择具有指导意义;血管瘤的内皮细胞增生与肥大细胞增多有密切关系。     

Bilateral periventricular and subcortical heterotopia in a man with refractory epilepsy

PURPOSE: To report a novel malformation in a male subject with refractory partial seizures. METHODS: Magnetic resonance imaging (MRI) and data reformatting in a subject referred for management of partial seizures. RESULTS: The patient had four distinct partial seizure types, without learning disability. MRI demonstrated the novel association of bilateral laminar subcortical heterotopia, bilateral temporal periventricular heterotopia, and hippocampal malformation. CONCLUSIONS: This previously unreported complex bilateral neocortical and archicortical malformation in a male patient cannot be explained by known genetic causes of heterotopia, raising the possibility of a novel gene involved in brain formation.     

DNA Fragmentation in Central Nervous System Vascular Malformations

Recent studies have shown that apoptosis plays an important role in vascular remodeling. We examined central nervous system vascular malformations for the presence of DNA fragmentation which is the evidence of apoptosis. We hypothesize that vascular remodeling through apoptosis may be responsible for recurrence or hemorrhage in these lesions. We examined the specimens of central nervous system vascular malformations by in situ end labeling (ISEL) of fragmented DNA. Moreover, we examined the expression of Caspase-3 which is apoptosis-related proteins in these lesions by immunohistochemistry. DNA fragmentation was observed in all 15 arteriovenous malformation (AVM) specimens. ISEL-positive cells were mainly distributed in the endothelium, media and perivascular tissue. In cavernous hemangioma (CH), DNA fragmentation was also observed in all 5 specimens. ISEL-positive cells were distributed in the endothelium, subendothelium and intercavernous matrix. Thirteen out of 15 AVM lesions stained positive for Caspase-3. Caspase-3 immunoreactivity was mainly distributed in the endothelium, media and perivascular tissue. This distribution was similar to that of ISEL positive cells. As for CHs, all 5 lesions stained positive for Caspase-3. Caspase-3 immunoreactivity was distributed in the endothelium, subendothelium and intercavernous matrix. Our findings indicate that apoptotic cell death and vascular remodeling play a role in the development and maintenance of vascular malformations.     

Surgery for Syringomyelia: An Analysis Based on 163 Surgical Cases

Summary ? Object. The authors analyzed the cases of 163 patients with syringomyelia to assess the appropriate surgical procedure. Methods. Depending on the aetiological factors and treatment considerations the series was classified into three groups. Group I were cases where there was no definite demonstrable aetiological factor; Group II cases had basilar invagination and/or Chiari malformation; and Group III consisted of cases where the syrinx was secondary to an obvious aetiology, such as a mass lesion either in the posterior cranial fossa or in the spine or a severe kyphotic spinal deformity. Post-traumatic syringomyelia and syrinx in association with spina bifida were not studied. Conclusions. We concluded that for Group I cases syringosubarachnoid shunting is the ideal form of treatment. In Group II cases foramen magnum bony decompression is satisfactory and physiological. Good results were obtained even in cases where either a foramen magnum decompression alone or in combination with a syringo-subarachnoid shunt was done. Only syringosubarachnoid shunt (without a foramen magnum decompression) in Group II cases was found to produce poor outcome. Group III cases should be treated for the primary aetiological problem. Only syrinx drainage procedure without treatment of aetiology in these cases produced poor results. It was observed that clinical outcome rather than radiological improvement is the reliable indicator of the surgical result.     

Animal Model for Cerebral Arteriovenous Malformation

Summary  Background. The present study was conducted to establish an animal model for the investigation of the pathophysiology and haemodynamics of cerebral arteriovenous malformation (AVM) but also to assess therapeutic aspects.  Method. For anatomic and haemodynamic reasons, dogs were chosen as the animal model. An arteriovenous fistula was created by interposing a segment of the superficial temporal artery between one of the main branches of the middle cerebral artery and the dorsal sagittal sinus. A temporal muscle graft supplied by this artery was implanted intracerebrally in the ischaemic area.  Findings. The angiographic and histopathologic findings obtained in the animal model are comparable with the situation found in intracerebral AVM in humans.  Interpretation. The animal model of intracerebral AVM established in this study allows for further investigation of the pathophysiology and dynamics of this disorder. It may help to develop better therapeutic options and thus improve the prognosis of affected patients.     

氯乙烯对大鼠精子生成量和畸形率的影响

[目的 ]探讨氯乙烯 (vinylchloride ,VC)对雄性大鼠精子生成和畸形发生的影响。 [方法 ] 68只SD雄性大鼠随机分成 4组 ,分别为 5 ,10 ,2 0mg/kg 3个染毒组和对照组 ,进行 12周亚慢性实验 ,分别在染毒 3 ,6,9周每组随机处理 3只大鼠观察睾丸的精子头计数、每日精子生成量 (dailyspermproduction ,DSP)、精子畸形发生率和睾丸组织碱性磷酸酶(ALP)、乳酸脱氢酶同工酶 (LDHx)的动态变化。 [结果 ]染毒结束时 ,各染毒组睾丸精子头计数和DSP均低于对照组 (P<0 .0 5 ) ;高剂量组ALP和LDHx活性分别为 71.60 μmol/(min·gpro)和 173 6.0 2 μmol/(min·gpro) ,都低于对照组 (P <0 0 5 )。 [结论 ] 2 0mg/kg氯乙烯经 12周亚慢性染毒对雄性大鼠具有一定的生殖毒性。     

先天性肛门直肠畸形Hoxc-8基因表达的研究及其意义

目的　对先天性肛门直肠畸形 (congenitalanorectalmalformations,CAM )末端直肠组织Hoxc 8基因表达水平的测定以探讨其分子水平的发病机制。方法　应用RT PCR(逆转录 聚合酶链反应 )技术检测 32例CAM的末端直肠组织及 8例对照组直肠标本的Hoxc 8基因表达 ,以 β actin基因 (是一种在组织中表达稳定的基因 )作为阳性内对照 ,观察Hoxc 8基因的表达水平。结果　CAM组Hoxc 8表达的相对比值为 0 .892± 0 .0 6 8,对照组则为 1.0 0 6± 0 .0 82。Hoxc 8在CAM组的表达与对照组比较差异有非常显著性意义 ,P <0 .0 1。结论　CAM的发生与Hoxc 8基因密切相关 ,该基因在肛门直肠组织中表达减弱可能参与导致其相应的靶器官发育异常而产生畸形。     

B超监测在子宫畸形合并早孕的人工流产术中的应用

目的:评价B超在子宫畸形合并早孕的人工流产术中的应用价值。方法:收集2003年8月~2004年8月妇产科门诊1 741例人工流产妇女中48例子宫畸形合并早孕患者,在B超监测下行人工流产术。结果:48例手术均为1次成功,无漏吸、吸宫不全、子宫穿孔等并发症发生,手术时间短、出血少。结论:对于子宫畸形合并早孕患者应用B超监测完成人工流产手术安全,病人痛苦小,值得推广。     

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.