可调压分流管治疗颅内静脉异常回流所致高颅压性脑积水

目的 探讨可调压分流管治疗先天性颅内静脉回流异常所致脑积水的应用价值.方法 2例男性患儿行CT、MRI提示脑室系统扩张,MRV及DSA检查发现颅内静脉窦广泛闭塞.腰穿压力显著升高.手术均采用强生可调压分流管行脑室-腹腔体外可调压分流术治疗.结果 2例患者术中均将分流阀压力设定为200 mmH2O,术后逐步调低,调低至180 mmH2O均出现不同程度低颅压症状,调高后症状缓解,2例患者术后智力及运动功能显著提高,复查CT提示脑室减小.结论 先天性颅内静脉异常回流导致的高颅压性脑积水非常少见,治疗经验甚少.可调压分流显然是治疗颅内静脉回流导致高颅压性脑积水一种最佳治疗方法,对压力调节,需通过较长时间多次调节来逐步达到一个合适的个体压力.     

可调压分流管治疗颅内静脉异常回流所致高颅压性脑积水

目的 探讨可调压分流管治疗先天性颅内静脉回流异常所致脑积水的应用价值.方法 2例男性患儿行CT、MRI提示脑室系统扩张,MRV及DSA检查发现颅内静脉窦广泛闭塞.腰穿压力显著升高.手术均采用强生可调压分流管行脑室-腹腔体外可调压分流术治疗.结果 2例患者术中均将分流阀压力设定为200 mmH2O,术后逐步调低,调低至180 mmH2O均出现不同程度低颅压症状,调高后症状缓解,2例患者术后智力及运动功能显著提高,复查CT提示脑室减小.结论 先天性颅内静脉异常回流导致的高颅压性脑积水非常少见,治疗经验甚少.可调压分流显然是治疗颅内静脉回流导致高颅压性脑积水一种最佳治疗方法,对压力调节,需通过较长时间多次调节来逐步达到一个合适的个体压力.     

颅内静脉异常回流致脑积水一例及文献复习

目的 探讨先天性颅内静脉回流异常所致脑积水的临床特点和治疗方法.方法 对1例先天性颅内静脉回流异常所致脑积水患者,经CT、MRI、MRV及DSA检查明确诊断后,行脑室-腹腔体外可调压分流术治疗,对其病史结合文献进行回顾分析.结果 先天性颅内静脉回流异常导致的脑积水临床上非常罕见,患儿常有头颈部皮肤毛细血管扩张,对高颅压耐受较好,回流静脉受损可致颅内压调节失代偿,DSA检查能够明确颅内静脉回流的异常路径,体外可调压脑室-腹腔分流可随时根据患儿反应调节分流压力,分流压力一般需维持在较高水平,以保证静脉回流.结论 出生后有头颈部毛细血管扩张者需考虑先天性颅内静脉回流异常,DSA检查有助于明确诊断,体外可调压脑室-腹腔分流可安全有效地控制先天性颅内静脉回流异常所致脑积水引起的头颅进行性增大.     

脑静脉窦内支架植入术中球囊扩张状态下行脑血管造影术的临床意义

目的探讨脑静脉窦内支架植入术中于球囊扩张状态下行脑血管造影术以预测术后穿支静脉回流情况的临床价值。方法共93例脑静脉窦内支架植入术患者(包括51例脑静脉窦狭窄合并颅内高压和42例源于脑静脉窦狭窄的搏动性耳鸣),63例直接行脑血管造影术测量横窦和乙状窦直径并根据测量结果选择支架,30例于球囊扩张状态下行狭窄侧颈动脉或椎动脉造影术以明确穿支静脉回流情况,回流缓慢者选择较脑静脉窦直径小1~2 mm的支架。结果脑静脉窦内支架植入术成功率达100%(93/93)。63例根据脑血管造影术测量的横窦和乙状窦直径而选择支架患者中45例选择9 mm×40 mm支架、15例选择8 mm×40 mm支架、3例选择7 mm×40 mm支架,支架平均直径(8.67±0.68)mm;术后11例(17.46%)出现穿支静脉回流缓慢。余30例于球囊扩张状态下行颈动脉或椎动脉造影术而选择支架的患者中3例选择8 mm×40 mm支架、11例选择7 mm×40 mm支架、16例选择6 mm×40 mm支架,支架平均直径(7.57±0.67)mm;术后仅1例(3.33%)出现穿支静脉回流缓慢。两组患者选择的支架直径(t=15.632,P=0.001)和术后穿支静脉闭塞发生率(校正χ2=60.065,P=0.001)差异均有统计学意义。结论脑静脉窦内支架植入术后穿支静脉闭塞是较为常见的并发症,术中于球囊扩张状态下行脑血管造影术可以有效预测穿支静脉闭塞的可能性,并为选择适宜直径的支架提供依据。     

颅内静脉窦血栓形成的临床与磁共振诊断5例报告

目的分析颅内静脉窦血栓形成的临床特点,磁共振成像及磁共振血管造影对其诊断的价值。方法分析5例磁共振检查确诊的颅内静脉窦血栓形成病人的临床资料及影像学特点,其中男1例,女4例,年龄22～40岁。病后3～19天行磁共振检查,主要观察水肿、静脉性便死或出血、静脉窦内异常信号,部分病人复查时可见再通。结果5例颅内静脉窦血栓形成皆有明确原因,临床表现多样,主要为亚急性颅内压增高、抽搐、肢体瘫痪或昏迷。MRI常规SE序列T1、T2加权示窦内高信号,脑实质水肿或合并出血、硬死。磁共振脑血管造影（MRA）的PC法示静脉窦高血流信号丢失,2例分别于病后24、31天复查MRI,结果示再通。经降颅内压、抗凝、合并感染者予抗生素、激素治疗,5例均治愈。结论颅内静脉窦血栓形成是较为少见的脑血管病,且病情重,MRI／MRA可帮助确诊。早期治疗能明显改善预后。     

硬脑膜动-静脉瘘合并髓静脉扩张的临床及影像学表现(附1例报告及文献复习)

目的报道1例硬脑膜动-静脉瘘(DAVF)合并髓静脉扩张病例的临床资料,探讨DAVF合并髓静脉扩张的临床及影像学表现。方法回顾性收集1例DAVF合并髓静脉扩张患者的临床及影像学资料,并结合文献复习进行分析。结果患者以症状性癫痫起病。头颅MRI表现为脑白质内迂曲、扩张血管影,并继发性脑梗死和脑水肿;全脑数字减影血管造影(DSA)提示右侧颈内动脉造影静脉期上矢状窦前1/3不显影,静脉晚期颅内多发髓静脉迂曲增粗,经室管膜下静脉-大脑内静脉回流,右侧颈外动脉造影示DAVF形成,大脑上静脉逆向回流,左侧颈内动脉造影示左侧发育性静脉畸形。经血管内介入栓塞DAVF治疗后患者病情改善。结论DAVF合并髓静脉扩张少见,可继发脑实质病变进而出现神经功能缺损症状与体征;影像学表现为头颅MRI提示髓静脉扩张涉及的疾病谱较多,需仔细鉴别;MRI和DSA联合检查能更好评价脑实质和血管情况。     

孕产妇合并颅内静脉窦血栓形成的诊治体会

目的探讨孕产妇合并颅内静脉窦血栓形成的诊断和治疗。方法选择2005-05-2015-09在我院治疗的孕产妇合并颅内静脉窦血栓患者15例,回顾分析其临床资料。结果妊娠早期发病2例患者中1例经抗凝治疗后无效,转行静脉窦内接触性尿激酶溶栓治疗;妊娠晚期3例患者均行剖宫产终止妊娠,术后24h给予抗凝治疗,1例发生发生意识障碍转行血管内溶栓治疗。产褥期发病10例中经抗凝治疗无效2例,2例伴癫痫发作转行血管内溶栓治疗,1例出现颅内静脉窦广泛血栓合并脑内出血,经抢救无效死亡。结论孕产妇出现抽搐、急性和亚急性头痛等症状需警惕出现颅内静脉窦血栓,符合静脉窦引流区域的脑实质异常信号为颅内静脉窦血栓形成的诊断依据,给予抗凝和溶栓等治疗以改善孕产妇结局。     

重症颅内静脉窦血栓形成的血管内小剂量持续溶栓治疗

目的探讨重症颅内静脉窦血栓形成血管内接触性溶栓的合理给药方案、安全性、疗效。方法对6例重症颅内静脉窦血栓形成患者,行股静脉插管至静脉窦内接触性溶栓治疗,小剂量、持续给药2~3d,平均每天60万U尿激酶。辅以低分子肝素全身抗凝治疗2~3w。结果经治疗,静脉窦血栓均在2~3d内完全溶解,静脉窦恢复通畅,所有患者神志转清晰,颅内高压症状消失,无颅内出血及全身性不良反应。出院时未留下任何神经系统后遗症,随访3个月,未出现新症状及血栓再形成。结论对重症颅内静脉窦血栓形成的病例,行血管内小剂量持续溶栓治疗,可快速使闭塞的静脉窦恢复畅通,副作用小、安全可靠。     

小儿颅内静脉窦血栓形成12例临床分析

目的 总结小儿颅内静脉窦血栓形成(CVST)的病因、临床表现和影像学特征,以期早期诊断和及时治疗.方法 对复旦大学附属儿科医院于2008年8月至2012年5月收治住院的12例CVST患儿的临床表现、实验室资料、影像学特征、治疗经过及短期预后进行回顾性分析.结果 (1)病因:12例患儿中,7例病因不明,临床表现为亚急性或者慢性头痛进行性或急性加重;2例与感染相关;1例为颅内肿瘤术后;另外2例发生在肾病综合征激素治疗过程中.确诊前有7例发生误诊.(2)诊断:12例患儿均行头颅磁共振及磁共振静脉成像检查,明确诊断为颅内静脉窦血栓.(3)短期预后:所有患儿确诊后均予以抗凝治疗,11例临床症状好转;7例病因不明的患儿中确诊时4例存在不同程度的视力障碍,治疗后短期视力无改善;1例患儿确诊后至外院行数字减影血管造影及球囊扩张,后治疗无效死亡.结论 小儿CVST临床表现缺乏特异性,误诊率高,认识其临床特征并及时行磁共振静脉成像检查是诊断的关键.抗凝治疗是一种安全且有效的方法.     

血管内局部溶栓治疗颅内静脉窦血栓

目的评价局部溶栓与机械碎栓治疗静脉窦血栓的影像学资料与治疗效果。方法对7例静脉窦血栓病人经股静脉穿刺,将微导管选择性插入已闭塞的静脉窦内。6例行静脉窦局部灌注尿激酶或基因重组组织型纤溶酶原激活剂,1例采用球囊机械碎栓术;3例伴脑皮质或深部静脉血栓者同时经颈动脉给予溶栓药。结果造影示6例已闭塞的静脉窦部分再通,1例无明显再通;3例侧支静脉回流增多。1例溶栓后原有脑内血肿增大,导致偏瘫加重;1例术后发生无症状出血性静脉梗死。随访10个月~3年,6例mRankin评分0~1分,1例mRankin评分2分。结论血管内局部溶栓治疗静脉窦血栓,静脉窦完全再通率低,但血管部分再通及侧支静脉回流增多,可改善病人的临床预后。     

可调压分流管治疗颅内静脉异常回流所致高颅压性脑积水

目的 探讨磁共振相位电影对比成像法在内镜下导水管成形术治疗导水管梗阻性脑积水的应用价值.方法 对23例诊断为导水管梗阻性脑积水的患者,术前常规采用磁共振相位电影对比法进一步确诊;手术采用电子软性神经内镜下导水管成形术,术中对导水管阻塞程度进行评估.术后1周及随访均采用磁共振相位电影对比法复查来测量导水管脑脊液流速流量以确定导水管是否开通.结果 23例术前磁共振相位电影对比法未见导水管脑脊液流动患者,术中见导水管完全闭塞或直径小于1 mm2;23例患者成形术均获成功,导水管扩张平均在4 mm左右,术后1周电影成像检查导水管平均流速为(4.74±1.77)cm/s,在随访期间,2例再次出现颅高压症状的患者,电影成像显示导水管未见脑脊液流动,二次内镜下探查见导水管重新闭塞.结论 磁共振相位电影对比法通过测量导水管内脑脊液流速流量来精确判断导水管开通情况,可以作为导水管梗阻性脑积水术前诊断及导水管成形术后疗效判断及随访的重要工具.

Abstract：

Objective To evaluate the application of phase-contrast cine magnetic resonance imaging (MRI) in endoscopic aqueductoplasty for patients with obstructive hydrocephalus. Methods The clinical diagnosis of hydrocephalus due to aqueduct obstruction in 23 patients was confirmed by phasecontrast cine MRI examination. The patients were treated with endoscopic aqueductoplasty. MRI was repeated during follow- up period. The cerebrospinal fluid (CSF) flow velocity in aqueduct was measured to determine whether the aqueduct was obstructed. Results The Results of phase -contrast cine MRI examinations indicated that there was no CSF flow in aqueduct in any patient. Aqueductoplasty was successfully performed in all patients. After one week, the Results of phase - contrast cine MRI examinations showed an average CSF flow velocity of (4.74 ± 1.77) cm/s. During follow - up period, intracranial hypertension recurred in two patients in whom CSF flow was not seen inside the aqueduct by phase - contrast cine MRI scan and the aqueduct re - occlusion was revealed by endoscopic exploration. Conclusions By measuring CSF flow velocity, phase - contrast cine MRI could accurately identify whether the aqueduct is obstructed. It should play an important role in the diagnosis of obstructive hydrocephalus and evaluation of theeffectiveness of aqueductoplasty, and it could be used for follow - up evaluation as well.     

Shapiro Shulman syndrome: a forgotten condition

We report on a girl with cutaneous angioma and hydrocephalus who presented the characteristics of the condition described by Shapiro and Shulman. At birth, she manifested extensive pink discoloration on her face, scalp, and back. During infancy, she developed hydrocephalus, which later stabilized. Magnetic resonance angiography revealed anomalous intracranial venous drainage, which led to development of her hydrocephalus. Various authors described such a process in the 1970s and 1980s, interpreting it as a variant of Sturge-Weber syndrome, and listing it with other neurocutaneous syndromes associated with vascular nevi, but few references to this condition appear in the recent literature. We think it exists as a syndrome, and that it is not a variant of Sturge-Weber syndrome. The cutaneous anomaly has characteristics closer to those of vascular birthmarks that fade than those of the port wine stain itself, and seems a direct consequence of abnormal venous drainage rather than a primarily capillary malformation.     

Facial palsy in cerebral venous thrombosis : transcranial stimulation and pathophysiological considerations

BACKGROUND: Cranial nerve palsy in cerebral sinovenous thrombosis (CVT) is rare, its pathophysiology remains unclear, and data from electrophysiological examinations in such patients are missing. CASE DESCRIPTION: We report the case of a 17-year-old woman with familial protein S deficiency who was admitted with extensive multiple CVT. Two weeks after onset of symptoms, she developed isolated right peripheral facial palsy, and MR venography showed segmental occlusion of the ipsilateral transverse sinus. Complete recovery of facial palsy occurred concomitant with recanalization of the transverse sinus. Facial neurography, including transcranial magnetic stimulation of the facial nerve and related motor cortex, ruled out a coincidental idiopathic palsy and revealed conduction block proximal to the facial canal. CONCLUSIONS: Facial palsy in our patient was caused by transient neurapraxia in the intracranial segment of the nerve. We suggest that elevated venous transmural pressure in the nerve's satellite vein, which belongs to the affected drainage territory of the transverse sinus, might have caused venous blood-brain barrier dysfunction in the intrinsic vascular system of the nerve, with leakage of fluids and ions into the endoneurial space and thus an increase in interstitial resistance.     

Successful endovascular repair of an unusual right-to-left shunt presenting with cerebral ischemia

Ischemic stroke due to congenital cardiopulmonary vasculature anomalies is rare in adults. We report a 54-year-old man with a stroke due to a unique right-to-left shunt who underwent successful endovascular treatment. This patient developed acute onset of right arm weakness with facial droop and aphasia which improved after intravenous thrombolysis. An MRI showed acute cerebral ischemia in the left middle cerebral artery and left posterior cerebral artery distribution. The patient developed recurrent stroke symptoms during agitated saline injection while undergoing a transthoracic echocardiogram which showed right-to-left shunting. Chest CT scan and conventional angiography revealed near occlusion of the superior vena cava. Head and upper limb venous return drained via a large left vertical vein into an anomalous left pulmonary vein into the left atrium. He underwent endovascular surgery to relieve the superior vena cava obstruction and to occlude the source of right-to-left shunt. While rare, congenital cardiopulmonary vascular anomalies may result in ischemic stroke in adults. CT angiography may be necessary to evaluate cardiopulmonary vasculature when right-to-left shunting is discovered on echocardiography in the setting of ischemic stroke. With large right-to-left shunts, agitated saline should be avoided.     

Infantile spasms as an adverse outcome of neonatal cortical sinovenous thrombosis

Cerebral sinovenous thrombosis is a rare but potentially serious condition often occurring in children with nonspecific presenting features. Much remains to be learned about the long-term outcome of infants with cerebral sinovenous thrombosis. We report a series of four patients taken from a prospective database of neonates with sinovenous thrombosis who subsequently developed infantile spasms, three with hypsarrythmia on electroencephalography and one with multiple independent spike foci. The first patient presented at 2 weeks of age with hypernatremia, dehydration, and seizures. He was found to have extensive thrombosis and hemorrhagic infarction of the right basal ganglia. The second patient presented at 5 weeks of life and was found to have sagittal sinus thrombosis with bilateral intracranial hemorrhage. The third patient presented with seizures on day 1 of life and was found to have venous thrombosis involving the torcular, extending into the sagittal sinus. The fourth patient presented at 3 weeks with lethargy and seizures. He was diagnosed with bacterial meningitis and also had extensive sinus thrombosis. All patients developed infantile spasms at ages 9, 7, 11, and 10 months, respectively. This is the first report in the English literature describing infantile spasms as a possible outcome of sinovenous thrombosis in early infancy.     

The complementary role of angiography and CT in the diagnosis of cerebral sinovenous occlusion

Three patients with angiographic and CT findings of cerebral sinovenous occlusion are described. Filling defects within the venous pathways are well demonstrated by angiography. Some of the most characteristic CR signs are here described. Full evaluation, including the clinical history, angiography and computed tomography is essential.

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Sommario Vengono descritti tre pazienti con segni angiografici e tomodensitometrici di trombosi venosa del circolo intracranico. I difetti di ri empimento del circolo venoso son ben dimostrati dall'angiografia. Si decrivono alcuni degli aspetti tomodensitometrici più caratteristici. Viene sottolineata inoltre l'utilità di una completa valutazione clinica e neurocardiologica di questa patologia.

     

Neonatal arterial ischemic stroke and sinovenous thrombosis associated with meningitis

This series describes 5 neonates with meningitis associated with arterial ischemic stroke or cerebral sinovenous thrombosis identified from a tertiary children's hospital perinatal stroke database. A retrospective chart review was performed to collect data on clinical presentations, type of meningitis, radiographic and electroencephalographic findings, neonatal intensive care unit course and complications, additional risk factors associated with stroke, and outcomes. The proportion of arterial ischemic stroke and sinovenous thrombosis in the database associated with meningitis was calculated. Neonates came to medical attention because of seizures (2), hydrocephalus (1), and behavior changes (2). The median age of presentation was birth (range, 0-18 days). Meningitis was bacterial in 4 and viral in 1. The median time from symptom onset to identification of the causative organisms of meningitis was 16.8 days (range, 13-23 days) and from symptom onset to diagnosis of stroke was 11 days (range, 4-18 days). One child had arterial ischemic stroke. Four had cerebral sinovenous thrombosis. One child died in the neonatal intensive care unit. Outcome data were available for 3 of the 4 surviving children; all had some degree of neurological deficit. All 5 of the neonates described had risk factors for perinatal thrombosis or embolization in addition to meningitis. Meningitis is associated with 1.6% (1/63) of cases of arterial ischemic stroke and 7.7% (4/52) of cases of cerebral sinovenous thrombosis in our perinatal stroke database. Further work is needed to clarify when infarction occurs during meningitis and which children are at highest risk.