Disappearance of globus pallidus hyperintensity in a patient with portal-systemic encephalopathy after occlusion of the shunt vessel]

A 67-year-old woman was admitted to our hospital with confusion and asterixis on January 23, 1994. She had had the same symptoms repeatedly. On admission she was disorientated, and had slurred speech and asterixis. Laboratory data showed hyperammonemia (84 micrograms/dl) with a poor ICG hepatic clearance (ICG15min = 32%), although hepatic failure did not exist. Abdominal ultrasonography, CT scan and liver biopsy showed no evidence of cirrhosis. Celiac arteriography revealed a large shunt vessel connecting the portal vein to the left renal vein. A 1.5 T magnetic resonance imaging (MRI) demonstrated a bilateral and symmetrical hyperintensity of the globus pallidus in the T1-weighted images. Portal-systemic encephalopathy recurred repeatedly in spite of the conservative therapy, and there was no evidence of the portal hypertension. Therefore, the operative procedure of ligation of the shunt vessel was done on February 21, 1995. After the operation, blood ammonia level and ICG hepatic clearance were normalized. She became free from encephalopathy. Twenty-seven months after the operation, the hyperintensity of the globus pallidus in the T1-weighted images completely disappeared. There have been several reports describing that the globus pallidus alterations on the T1-weighted images in patients with liver cirrhosis, manganese intoxication and portal-systemic encephalopathy. To our knowledge, this is the first case that the hyperintense signal of the basal ganglia in a patient with portal-systemic encephalopathy disappeared completely after occlusion of the shunt vessel.     

Histological characteristics of arterialized medullary vein in spinal dural arteriovenous fistulas related with clinical findings: report of five cases

The histological features of arterialized medullary vein (MV) in spinal dural arteriovenous fistulas (SDAVF) were studied in five consecutive patients who presented with progressive congestive myelopathy. Retrograde venous filling on preoperative angiography was recognized as being severe in 3 cases and moderate in 2 cases. Direct intradural interruption of the arterialized MV was performed in all patients. The arterialized MV was sampled and examined histologically to determine the percentage of the hyperplasia of venous wall (hypertrophic ratio). Histological examination of arterialized MV showed that hypertrophic alteration of venous wall structure was due to hyperplasia of elastic fibers, ranging from 41 to 82%. Patients with angiographically severe venous hypertension tended to have a higher hypertrophic ratio than patients with moderate venous hypertension. Our observations support the clinical concept that long-standing arterial stress in the spinal venous circulation causes histological alterations of spinal vascular structure associated with the progression of venous hypertension. We suggested that possibly the histological parameter can be used for predicting neurological recovery after occlusion of the fistulas.     

Hepatic myelopathy with spastic paraparesis

Progressive myelopathy is a rare neurological complication of chronic liver disease with portal hypertension and there is no special diagnostic tool for hepatic myelopathy. Neuropathological studies of the patients with hepatic myelopathy have demonstrated demyelination of the lateral corticospinal tracts with various degree of axonal loss. Transcranial magnetic stimulation (TMS) is widely utilized as an indicator of changes in exitability and conductivity of the motor pathways. TMS studies are also used for the diagnosis of hereditary spastic paraparesis in the literature. In this study, we described two patients who presented with spastic paraparesis; TMS studies suggested that they had myelopathy and diagnosed as hepatic myelopathy when all the other possible diagnoses were ruled out.     

A case of portal-systemic shunt encephalopathy due to congenital portal vein hypoplasia presenting with abnormal cerebral white matter lesions on the MRI]

A 49-year-old woman, without any past history of liver diseases and blood transfusion, was admitted to our service because of somnolence, and flapping tremor. Neurologically, she was drowsy and disoriented. She had bilateral pyramidal tract signs and flapping tremor. Although the laboratory examination showed marked hyperammonemia (217 micrograms/dl), neither abdominal CT nor liver biopsy showed any evidence of liver cirrhosis. An abdominal angiography showed portal vein hypoplasia associated with the portal-systemic shunt. A T2-weighted MRI showed the high intensity areas in the bilateral deep cerebral white matter, and the posterior limbs of the bilateral internal capsules. This is a rare case of portal-systemic shunt encephalopathy due to congenital portal vein hypoplasia presenting with abnormal cerebral white matter lesions on the MRI.     

Microsurgical interruption of dural arteriovenous fistula at the foramen magnum.

We report two rare cases of progressive congestive myelopathy caused by dural arteriovenous fistulae (DAVF) at the foramen magnum. The first, a 69-year-old male with a 2-year history of progressive myelopathy, had symptoms unrecognized due to a past history that included spinal caries and congenital dislocation of the hip. The second, a 60-year-old male, had a recurrence of the myelopathy three months after endovascular occlusion of the DAVF feeding artery. Both patients were successfully treated by direct microsurgical interruption of the arterialized medullary vein with functional and radiological improvement. The clinical manifestations of DAVF at the foramen magnum are nonspecific, mimicking those of cervical spondylotic myelopathy or cervical neoplasm. Accurate and early diagnosis followed by complete obliteration of the fistula is mandatory to avoid permanent neurological deficit.     

Percutaneous ventriculoatrial shunt: experience with 179 cases

BACKGROUND AND PURPOSE: The aim of the study was to assess the impact of percutaneous placement of a distal catheter on treatment results in patients with hydrocephalus undergoing ventriculoatrial shunt (VA) implantation. MATERIAL AND METHODS: This retrospective study included 184 patients aged 14-80 years (mean: 48 yrs) with hydrocephalus who were treated with VA shunt between 1990-2003. In 179 patients the cardiac catheter was introduced by catheterization of the internal jugular vein, rarely the subclavian or external jugular vein, using the Seldinger technique and standard equipment for central vein catheterization. The mean follow-up time was 64 months. RESULTS: The most frequent complication related to the method used was an incidental puncture of the carotid artery (5% of cases). Pneumothorax was observed in two cases. None of these complications caused permanent sequelae. Early (<6 months) postoperative complications included shunt infection in the neck region (2 cases), impatience of the cardiac catheter (3), disconnection between the outlet and the cardiac catheter with its subsequent migration (1) and kinking of the distal catheter (1). Late (>6 months) complications included infection along the distal catheter (2 cases), occlusion of the cardiac catheter (4) and its disconnection (1). The mean duration of the procedure was 40 minutes. CONCLUSIONS: Percutaneous placement of the cardiac catheter is a safe procedure which shortens the duration of the operation and minimizes tissue traumatization. This method may contribute to a decreased risk of infection. VA shunt with presented modification may be used in those patients for whom implantation of ventriculoperitoneal shunt is contraindicated.     

门静脉及肠系膜上静脉内有实性闭塞性血栓时：腔门静脉半转位术条件下同种异体肝移植1例

1例53岁男性患者,有乙型肝炎病史20余年,因查体发现肝右后叶占位以原发性肝癌在解放军总医院行肝右后叶切除术,术后行肝介入治疗2次。3年半后半肿瘤复发,于2004-04-28在青岛大学医学院附属烟台毓璜顶医院再次手术行肝右前叶肿瘤切除,同时因肝硬化门静脉高压症行脾切除术加贲门周围血管离断术,14个月后肿瘤再次复发,再次入院行肝移植。术中见门静脉、肠系膜上静脉内血栓闭塞呈实性,术中取出门静脉内栓子做病理检查为机化血栓。手术不能行常规原位肝移植术,采用肝移植的特殊术式-腔门静脉半转位术。30 d后患者顺利康复出院。170 d后患者发生反复发作呕血、黑便和失血性休克,以黑便为主,共发生5次出血并住院治疗,14个月后死于肿瘤脑、肺转移。提示施行改良腔门静脉半转位术或扩张的内脏静脉血管搭桥术对预防消化道出血成为一种必要的补救措施。另外,在门静脉高压症切脾和断流术后,门静脉系统血栓形成发病率高,早期及时的抗凝治疗预防门静脉系统血栓形成,为日后肝移植术创造条件、改善患者生活质量是必要的。     

Galcn静脉动脉瘤样扩张——附8例报告

目的:报告8例Galen静脉动脉瘤样扩张(VGAD),并对其分型、血流动力学变化所致的临床症状及治疗进行分析和讨论.方法:自1986年5月～1996年5月,在4000例脑血管造影中检出VGAD8例,进一步检查:CT3例,MRI2例,全部行全脑血管造影.行栓塞治疗6例,栓塞加X-刀治疗1例,未治1例. 栓塞材料为IBCA或NBCA.结果:症状消失2例,好转4例,无效1例.结论:已证明此种栓塞技术对于VGAD的治疗是有效的,重要的是要了解VGAD的分型和有关血流动力学变化,常需多次栓塞.     

面静脉-眼上静脉入路填塞海绵窦

目的评价经面静脉-眼上静脉入路填塞海绵窦治疗颈动脉海绵窦瘘（CCF）的有效性。方法经股静脉-面静脉-眼上静脉入路到达患侧海绵窦，用GDC或EDC，游离弹簧圈，真丝线段等多种栓塞材料填塞海绵窦，同时闭塞瘘口。面静脉插管困难者，在下颌角附近切开皮肤显露面静脉，直视下穿刺面静脉放置相应导管，再经眼上静脉到达患侧海绵窦并将其填塞。结果经面静脉-跟上静脉入路对14例，16侧海绵窦进行了栓塞治疗，其中5例为外伤性、直接CCF（A型），经动脉途径球囊栓塞后复发，或微弹簧圈栓塞未能成功，或经岩下窦入路未能成功，9例为自发性、间接CCF（D型8例，C型1例）。13例经股静脉-面静脉-眼上静脉途径，1例通过直视下面静脉穿刺。11例栓塞治疗后即刻造影显示瘘消失，2例残留低流量的岩下窦引流，另有1例在微导管进入面静脉后，而静脉痉挛闭塞，未能继续进行栓塞治疗，造影仍见瘘存在，但眼静脉出现明显的造影剂滞留。1例A型CCF在球囊栓塞后出现外展神经麻痹，经面静脉-眼上静脉栓塞后亦无改善。因面静脉痉挛闭塞未能栓塞成功者，于术后即感眼部症状加重，但第2天感症状缓解，术后第21天症状明显改善，造影检查发现瘘门已经消失，术后1个月病人眼部症状完全消失。其他病例在栓塞术后眼部症状明显改善，最后消失。随访3个月至21个月未见复发。2例残留瘘口者，1例于3个月和12个月进行2次造影复查，另1例于3个月造影复查，瘘的流量均无明显变化，因无临床症状未再进行治疗。其他病例未进行造影复查。结论经面静脉-眼上静脉栓塞治疗CCF安全有效，对于A型CCF，可作为经动脉途径治疗失败后的补救措施，而对于B、C、D型CCF，应作为首选治疗。     

Spinal dural arteriovenous fistula

PURPOSE OF REVIEW: To summarize clinical key points, diagnostic features, and results of imaging and therapy of spinal dural arteriovenous fistula (SDAVF). RECENT FINDINGS: SDAVF accounts for 70% of spinal arteriovenous malformation with an annual incidence of 5-10 cases per million. At least 80% of patients are male, and more than 66% of patients are in the sixth and seventh decade of life indicating preponderance of gender and age. Thrombophilia is not a predisposing factor of disease. Clinical course is predominated by symptoms of congestive myelopathy, but subarachnoid hemorrhage may occur. Double SDAVF is a rare problem in the management of disease. Magnetic resonance imaging has replaced myelography as screening procedure. Contrast-enhanced magnetic resonance angiography and multislice computerized tomographic angiography may facilitate diagnostic procedure, however, spinal angiography is still required to confirm diagnosis. Treatment by permanent occlusion of fistula results in clinical improvement in 70% of cases. Microsurgical shunt interruption has proven secure and reliable. Endovascular shunt embolization has been established as a standardized procedure, but occlusion rates are still lower than in surgical treatment. SUMMARY: Advances have been made in diagnosis and treatment of SDAVF, but the disease is still not completely understood.     

A rare brainstem hemorrhage during transvenous embolization of a cavernous dural arteriovenous fistula

Intravascular treatment of cavernous dural arteriovenous fistula (dAVF) is usually safe and effective. However, we describe a patient with a rare brainstem hemorrhage during transvenous embolization (TVE). A 79-year-old woman suffered from left chemosis and diplopia. Cerebral angiography revealed a left cavernous dAVF with cortical venous drainage. The patient underwent TVE of the cavernous sinus (CS) via the left inferior petrosal sinus. Superior petrosal sinus (SPS) outflow occlusion was performed to avoid venous congestion, followed by superficial middle cerebral vein outflow occlusion, selective shunt occlusion of the middle meningeal artery, and superior orbital vein outflow occlusion. The patient's condition suddenly deteriorated during CS packing. A CT scan revealed a massive brainstem hemorrhage. Cerebral angiography did not show SPS reopening or redistributed drainage to the posterior fossa. Thus, TVE for cavernous dAVF can result in life-threatening vascular complications. Well-planned treatment strategies could avert this rare complication.     

Thrombosis and thrombophilebitis of the internal jugular vein as a very rare complication of the ventriculoatrial shunt

A 21-year-old man presented with a sausage shaped mass lesion, located in the right anterolateral region of the neck. He had been operated on for hydrocephalus and thoraco-lumbar myelomeningocele at the age of 10 days and 2 months, respectively. Although he was asymptomatic at the age of 10 years, ventriculoatrial (VA) shunt was considered non-functional. Doppler ultrasound showed thrombosis of the right internal jugular vein. Cervical magnetic resonance imaging displayed thrombosis and thrombophilebitis of the right internal jugular vein with loss of flow pattern. VA shunt was removed under general anesthesia and shunt material sent for culture. Staphylococcus aureus grew from the culture of shunt material. A cephalosporin (cefuroxime axetil) was administered and the neck mass disappeared in 4 weeks. He has been symptom free for the last 5 years. VA shunts for hydrocephalus have some well-defined specific complications. In this paper, a case with VA shunt related thrombosis and thrombophilebitis of internal jugular vein was presented and management of VA shunt related cardiovascular complications were discussed.     

脑内静脉窦血栓形成三例报告及文献复习

目的 探讨脑内静脉窦血栓形成（CVST）的临床表现、诊断和治疗。方法 回顾性分析3例经影像学检查诊断为CVST的患者，均行抗凝治疗和脑室-腹腔分流，随访1～2年。结果 CVST主要症状为外伤、感染后头痛，颅内压增高，视力减退，精神症状；神经影像学表现为CT增强扫描后可见静脉窦内“空三角征”，MRI示静脉窦正常流空影的形态消失，出现长T2、短T1信号；MRV示静脉窦血流不连续或缺失，梗阻远端侧支循环血管建立或其他引流静脉异常扩张；3例患者经抗凝及脑室-腹腔分流后症状均有不同程度的缓解。结论 对临床急性起病的颅高压征，伴或不伴局灶性神经功能缺损病人，应高度怀疑CVST，及时行MRI及MRV或DSA检查，明确诊断，尽早治疗。抗凝和溶栓治疗为治疗CVST的首选治疗方法，对于经济有困难的CVST患者，行抗凝及脑室-腹腔分流短期内疗效满意，不失为一种治疗方法的选择。     

A case of spinal dural AVF treated by endovascular embolization]

Here we report a case of spinal dural arteriovenous fistula(AVF) treated by endovascular embolization. A 58-year-old female presented with progressive intermittent claudication and numbness of the lower extremities. MRI showed swelling of the spinal cord with intramedullary high signal intensity on T2-weighted image and intramedullary enhancement, suggested spinal cord myelopathy. Myelography demonstrated the dilated serpentine vessels in the subarachnoid space and focal filling defect. Angiography showed spinal dural AVF fed by bilateral lateral sacral artery. The draining vein was posterior spinal vein. Endovascular embolization using liquid material was performed under general anesthesia. The injection of glue included the distal feeding artery, the shunt itself and the initial part of draining vein. A complete cure was achieved, with a normal postoperative angiogram. MRI returned to normal with complete disappearance of T2 high signal, cord enlargement and enhancement by contrast medium. It was suggested that venous congestion induced the transient spinal ischemia, manifested as intermittent claudication. Endovascular embolization using liquid material was safe and quite effective for spinal dural AVF.     

肝硬化的脊髓病变：肝硬化与非肝硬化尸体胸腰髓形态及神经元功能蛋白表达比较

依据国内外现有文献报道的肝硬化存在脊髓外侧索损伤的现象,实验对肝硬化与非肝硬化尸体的胸、腰髓形态、功能蛋白表达及其相关血管变化等进行了解剖观察与比较分析。结果显示,与非肝硬化尸体相比,肝硬化患者尸体的肝门静脉管径增粗,全胃肠管腔阶段性积血;椎管内静脉丛和腰髓明显淤血;胸、腰髓前角神经元胞体变小;少数神经元固缩;Nissel体密集细小,呈脱颗粒样改变,胞核不清晰;前角神经元胞体和白质侧索纤维束神经丝蛋白和突触素含量减少。提示肝硬化门静脉高压血流动力学改变导致的椎管和脊髓血循环异常可能是肝性脊髓病的最重要因素。     

Multiple cerebral embolism caused by a patent foramen ovale and a uterine myoma]

Recently, patent foramen ovale(PFO) has been highlighted as an important risk factor of cerebral infarctions in young adults. We report a patient of multiple cerebral embolism associated with PFO and deep venous thrombosis caused by a uterine myoma. A 40-year-old woman suddenly suffered from right hemiparesis with motor aphasia. Brain angiography showed an occlusion of M2 portion of the left middle cerebral artery, but atherosclerotic changes were not seen. She developed left facial paresis 23 days later and admitted to our hospital. Brain MRI revealed multiple cerebral infarcts in the left insular cortex, the deep white matter of the right frontal lobe, and bilateral thalamus. Hypoxia with the perfusion defects of S1 and S2 sections of the right lung demonstrated by scintigraphy suggested pulmonary embolism. Transesophageal echocardiography showed a PFO with spontaneous left-to-right shunt and right-to-left shunt evoked by the Valsalva maneuver. Although venography could not detect thrombi, it revealed severe compression of the right external iliac vein by a uterine myoma. These findings suggested thrombi in the right external iliac vein were the embolic source when combined with elevated coagulation markers. An uterine myoma should be considered as an important risk factor for an embolic source in case of cerebral embolism with PFO.     

In retinal vein occlusion platelet response to thrombin is increased

Introduction

Retinal vein occlusion is a major cause of ocular morbidity. The precise mechanism leading to thrombosis in retinal vein occlusion has not yet been clearly elucidated. Several risk factors have been identified, including hypertension diabetes, history of cardiovascular disease, hypercholesterolemia, hyperhomocysteinaemia, increased ocular pressure and glaucoma. Although thrombus formation in the vein plays a significant role in the onset of retinal vein occlusion, the relationship between platelet aggregation and retinal vein occlusion remains to be clarified.

Materials and Methods

In the present study the platelet response to thrombin in a selected group of retinal vein occlusion patients was investigated. Retinal vein occlusion patients were compared to a group of healthy subjects matched for age, sex, clinical and metabolic characteristics. In resting and activated platelets of both groups of subjects total protein tyrosine phosphorylation, p38MAPK and cytosolic phospholipase A₂ phosphorylation, arachidonic acid release, intracellular calcium levels, thromboxane B₂ and superoxide anion formation were measured.

Results

Results show that platelets of patients were more responsive to thrombin than healthy subjects. In resting or in thrombin stimulated platelets of patients total protein tyrosine phosphorylation, p38MAPK and cytosolic phospholipase A₂ phosphorylation were increased. Also arachidonic acid release, thromboxane B₂ and superoxide anion formation were higher in patients than in healthy subjects. In addition intracellular calcium rise induced by thrombin was increased in patients.

Conclusions

Altogether data suggest that platelet hyperaggregability inducing thrombus formation might be an important factor in the onset and/or development of retinal vein occlusion.     

Chronic cerebral venous hypertension model in rats

Although cerebral venous hypertension is known as an important determinant factor for clinical manifestation and outcome in patients with dural arteriovenous malformation (AVM), the pathophysiology of that condition is not well understood. We have created a chronic rat model by cervical arteriovenous fistularization with jugular vein occlusion and examined effect of cerebral venous hypertension on cerebral blood flow regulation. This model may be suitable for investigating mechanisms of cerebrovascular alteration after venous hypertension.     

Bilateral carotid occlusion and the sympathetic regulation of insulin secretion

After a 2-min bilateral carotid arterial occlusion (BCO) in puppies, a centrally originating, sympathetic discharge takes place which increases heart rate and blood pressure. We examined the specificity of this sympathetic neural outflow by determining whether it also caused a direct neurally mediated inhibition of insulin release from the pancreas. The effects of this BCO on portal venous insulin concentrations, as well as on heart rate and blood pressure, were examined during i.v. glucose infusions of 0 (saline), 5 and 15 mg/kg X min-1. To determine changes in splanchnic blood flow and to more closely estimate pancreatic insulin secretion rates, a major vein draining the pancreas, the gastroduodenal, was catheterized. Blood flows and the amount of insulin traversing this vein per min (insulin flow rate) were followed before, during and after BCO. BCO decreased portal vein insulin concentrations during i.v. glucose infusions of 5 and 15 mg/kg X min-1, but not when saline was infused. Since bilateral splanchnicotomy altered this result little and since BCO increased blood flow and the insulin flow rate in the gastroduodenal vein, it appears that the lower portal venous insulin concentrations during BCO are secondary, not to sympathetically induced decrease in insulin secretion rates but, to dilution of pancreatic effluent blood. We conclude that while BCO causes appropriate changes in heart rate and blood pressure, this central stimulus to the sympathetic system does not provide a direct neuroendocrine reflex change in insulin secretion. BCO alters portal venous insulin concentration indirectly, and the alteration depends on the plasma glucose concentration and an enhancement in the splanchnic blood flow.     

A critical analysis of 'normal' radionucleotide shuntograms in patients subsequently requiring surgery

BACKGROUND: Shuntograms are performed when patients present with symptoms suggestive of, but inconclusive for, shunt malfunction, without confirmatory radiological evidence. METHODS: Shuntograms over the past 3.5 years were reviewed. Patient records were reviewed for revision in proximity to a negative (normal) study. RESULTS: One hundred and fifteen out of 149 tests were negative. Thirty-four surgeries (in 31 patients) occurred subsequent to a negative shuntogram. In 18 out of 34 revisions the shunt was functional: 13 surgeries were for overdrainage, 4 were for unrelated reasons with shunt function confirmed incidentally and 1 was an exploration for cognitive deterioration. In 16 cases (13 patients) the shunt was not functional: 12 had proximal catheter occlusion in which, on subsequent review, there was no ventricular reflux present and the remaining had distal malfunctions. CONCLUSIONS: The false negative rate for shuntograms was 16 out of 115 (14%) with proximal occlusion most common. This estimate of the predictive value of a normal flow study may influence the decision to revise a shunt.