Endoscopic sphenoid sinus drainage in Lemierre syndrome

Lemierre syndrome is a rare condition arising from an invasive oropharyngeal infection, which leads to septic thrombophlebitis of the internal jugular vein and multi-organ septic embolization. Intracranial complications are rare but serious, including subdural empyema, cavernous sinus thrombosis, and internal carotid artery aneurysms. We report a patient with Lemierre syndrome with multiple intracranial complications despite aggressive antimicrobial therapy. The patient eventually required transsphenoidal endoscopic drainage of the sphenoid sinus to help eradicate the infectious source. We postulate that in patients with Lemierre syndrome with evidence of infection in the paranasal sinuses, endoscopic sinus drainage can be an adjunct to antimicrobial therapy in achieving infection control.     

Dural sinus thrombosis following head injury: report of two cases and review of the literature

Dural sinus thrombosis (DST) usually involves the sagittal, transverse and sigmoid sinuses and is more common in women due to pregnancy, puerperium and oral contraceptive use. Other etiologies include coagulopathies, infection and head injury. We have present two DST cases following head injury. The first case was a 35-year-old man hospitalized because of one-week history of headache and repeated vomiting after a mild head injury. Thrombosis of the superior sagittal sinus, right transverse and sigmoid sinuses and right jugular vein was determined on angiography. The second case was a 25- year-old man operated on for epidural hematoma at the posterior fossa. Meningitis developed and an abducens palsy was determined. Magnetic resonance imaging demonstrated thrombosis of the right transverse and sigmoid sinus. Low molecular weight heparin was administrated for three months. Both cases had good recovery, but one had recanalisation of the thrombosis. Intracranial hematomas, depressed skull fracture or skull fracture that cross the sinus can obstruct the blood flow in the sinus. Moreover, closed head injury may cause to DST. Because of undefinitive pathophysiology, a consensus was not obtained on overall strategy concerning conservative, radiosurgical, or surgical therapy yet.     

兔颈静脉血栓形成对颅内压的影响

目的 研究颈静脉血栓形成对颅内压的影响.方法 40只日本大耳兔随机分为左侧颈静脉血栓(LJVT)组、右侧颈静脉血栓(RJVT)组、双侧颈静脉血栓(BJVT)组、假手术(Sham)组,通过阻断相应血管血流并注入凝血酶建立血栓模型.通过颈部彩超测量造模前后各组动物颈静脉直径,并监测72 h内颅内压变化.造模后1周观察脑组织形态学改变.结果 与造模前比较,LJVT和RJVT造模后颅内压即升高(P<0.05),12h后降至造模前水平(P>0.05);BJVT造模后颅内压显著升高(P<0.01),72h仍处于较高水平(P<0.01); Sham组造模前后颅内压无明显差异(P> 0.05).结论 颈静脉血栓形成可引起颅内静脉回流受阻,进而导致颅内压升高.一侧颈静脉血栓可导致短暂颅内压升高,双侧颈静脉血栓颅内压升高严重而持久.     

Superior Sagittal Sinus Thrombosis in the Presence of Idiopathic Bilateral Internal Jugular Vein Stenosis

Cerebral venous outflow obstruction and anomalies in cerebral venous circulation predispose to dural sinus thrombosis. This case report illustrates the magnetic resonance and angiographic findings in a patient who had superior sagittal sinus thrombosis secondary to idiopathic bilateral internal jugular vein stenosis, a previously unrecognized entity. The findings suggest that bilateral stenosis of the internal jugular veins at their junction with the innominate veins causes obstruction to cerebral venous outflow leading to dural sinus thrombosis.     

改良溶栓方案治疗脑静脉窦合并颈内静脉血栓形成

目的探讨改良溶栓方案【静脉窦内微量持续泵点尿激酶(100,000u/24h)】治疗脑静脉窦合并颈内静脉血栓形成的疗效。方法对11例患者进行机械性破栓、静脉窦内留置微导管行最低量尿激酶100,000u/24h静脉窦直接泵点滴48～96h治疗。术后积极治疗原发病,抗凝治疗6个月。术后随访6～12个月,平均10个月。结果 11例脑静脉窦合并颈内静脉血栓形成患者,脑静脉窦均获得再通(其中8例患者应用尿激酶100,000u/24h效果良好,3例患者在应用尿激酶100,000u/24h,48h复查后增量至250,000u/24h),1例颈内静脉未通,预后良好。结论改良溶栓方案可有效治疗脑静脉窦合并颈内静脉血栓形成,有待大宗病例进一步证实。     

Vernet's Syndrome Associated with Internal Jugular Vein Thrombosis

Our objective is to present a case of Vernet's syndrome (cranial nerve (CN) IX, X, and XI palsy) associated with cerebral venous thrombosis (CVT) in an internal jugular vein. The patient presented with acutely developed dysphagia. The weakness of the left sternocleidomastoid and trapezius muscles was observed. The initial magnetic resonance imaging and computed tomography (CT) with contrast enhancement showed contrast-filling defect in the left internal jugular vein inside the jugular foramen. The magnetic resonance venography with contrast enhancement revealed a partial filling defect in the left sigmoid sinus and total occlusion of the left internal jugular vein. Under the diagnosis of CVT associated with CN IX, X palsy, anticoagulation therapy with low-molecular-weighted heparin was initiated. Despite the continued anticoagulation therapy for 3 months, neither the burden of thrombosis in the left sigmoid sinus and internal jugular vein on neck CT nor dysphagia symptoms improved. Clinicians need to be aware of internal jugular venous thrombosis as one of the differential diagnoses in Vernet's syndrome in patients in a hypercoagulable state. Further reporting of similar cases is needed to confirm the association between CVT and Vernet's syndrome.     

白塞病相关颅内静脉窦血栓形成9例分析

目的 研究白塞病相关颅内静脉窦血栓形成（cerebral venous sinus thrombosis,CVST）的临床表现、血 栓好发部位、治疗及预后。 方法 回顾性分析2014年1月-2016年8月于首都医科大学附属北京同仁医院神经内科住院的白塞病 相关CVST患者的临床资料。 结果 共收集白塞病相关CVST患者9例,其中男性2例（22.2%）,女性7例（77.8%）,年龄21～47岁,中 位数是35.9岁;急性起病1例（11.1%）,慢性起病8例（88.9%）;头痛4例（44.4%）、头晕1例（11.1%）、 阵发性黑蒙3例（33.3%）、双眼视力下降5例（55.6%）、视盘水肿9例（100%）、复视3例（33.3%）、耳 鸣1例（11.1%）;乙状窦血栓形成7例（77.8%）、横窦血栓5例（55.6%）、直窦血栓1例（11.1%）、下矢状 窦血栓1例（11.1%）、颈内静脉血栓2例（22.2%）;仅1处静脉窦受累的4例（44.4%）,同时有2处及2处 以上静脉窦受累的5例（55.6%）;所有患者均予醋甲唑胺50 mg 2次/日口服,3例予糖皮质激素冲击 治疗,其中1例合并华法林抗凝治疗、2例进行了腰大池-腹腔分流手术。随访1～32个月,所有患者治疗 后均好转。 结论 白塞病可引起颅内静脉窦血栓形成,可累及单处或多处静脉窦,CVST患者需除外白塞病可能。     

Early Spontaneous Recanalization of Sigmoid Sinus Thrombosis Following a Closed Head Injury in a Pediatric Patient : A Case Report and Review of Literature

Cerebral venous sinus thrombosis (CVST) following a closed head injury in pediatric patients is a rare condition, and an early spontaneous recanalization of this condition is extremely rare. A 10-year-old boy was admitted with a mild, intermittent headache and nausea five days after a bicycle accident. The brain computed tomography showed an epidural hematoma at the right occipital area with pneumocephalus due to a fracture of the occipital skull bone. The brain magnetic resonance imaging and the magnetic resonance venography demonstrated a flow signal loss from the right sigmoid sinus to the right jugular vein. The diagnosis was sigmoid sinus thrombosis, so close observations were selected as a treatment for the patient because of his gradually improving symptoms; however, he complained of vomiting 14 days the after conservative treatment. The patient was readmitted for a further examination of his symptoms. The laboratory and the gastroenterological examinations were normal. Due to concern regarding the worsening of the sigmoid sinus thrombosis, the brain magnetic resonance venography was rechecked and it revealed the recanalization of the venous flow in the sigmoid sinus and in the jugular vein.     

An unusual case of neuro-Behçet’s disease presenting with co-occurence of cerebral venous sinus thrombosis with basilar artery occlusion

Non-parenchymal neuro-Behçet disease generally affects cerebral venous sinuses, whereas intracranial intracerebral arterial involvement has been rarely reported. But co-involvement of both intracranial intracerebral artery and venous vascular systems in a patient at the same time has not been mentioned before. To the best of our knowledge, this case involving a 25-year-old male with a 7-year history of Behçet disease is the first reported of this type of involvement. He developed occlusion of the basilar artery together with thrombosis of the left sigmoid sinus, distal internal jugular vein, and straight sinus. He was successfully treated with a combination of high-dose steroid and cyclophosphamide. Cranial magnetic resonance angiography demonstrated the resolution of these abnormalities.     

外伤性颅内静脉窦血栓形成的MRI诊断

目的探讨磁共振成像(MRI)结合磁共振静脉成像(MRV)在外伤性颅脑静脉窦血栓形成中的诊断价值。方法回顾性分析28例经临床及影像学诊断为外伤性颅脑静脉窦血栓形成的 MRI与MRV资料。磁共振均行常规SE序列T1WI、T2WI以及液性衰减反转恢复序列(FLAIR)扫描,并全部行二维时间飞跃法静脉成像(2d-TOF MRV)检查。结果外伤性静脉窦血栓形成最常累及上矢状窦、下矢状窦、横窦,表现为受累静脉窦流空效应消失,为不同信号的血栓所阻断。MRV则表现为受累静脉窦管腔的狭窄、中断以及属支的迂曲扩张。结论 MRI结合MRV能无创性的同时显示外伤性颅脑静脉窦血栓形成与脑挫裂伤,为外伤性颅脑静脉窦血栓形成的首选检查方法。     

Abducens nerve palsy due to inferior petrosal sinus thrombosis

Isolated unilateral abducens nerve palsy is usually due to ischemia, trauma or neoplasm. Dorello’s canal is the space between the petrous apex and superolateral portion of the clivus, bound superiorly by Gruber’s ligament. The abducens nerve travels with inferior petrosal sinus (IPS) though the Dorello’s canal before entering the cavernous sinus. A 31-year-old man presented with neck pain, and binocular horizontal diplopia, worse looking towards left and at distance. He had a history of intravenous drug abuse but no history of hypertension or diabetes. On examination, he had complete left 6th nerve palsy with normal fundi, pupils, and other cranial nerves. Methicillin-resistant Staphylococcus aureus bacteremia was detected with naïve tricuspid valve endocarditis and multiple septic emboli to lungs with infarcts. His cerebrospinal fluid was normal. MRI of the brain was normal. MRV of head and neck showed thrombosis of the left internal jugular vein, left sigmoid sinus and left inferior petrosal sinus with normal cavernous sinus and no evidence of mastoiditis. He was treated with broad spectrum antibiotics. He was not anticoagulated for fear of pulmonary hemorrhage from pulmonary infarcts. Although cerebral venous sinus thrombosis commonly presents with elevated intracranial pressure, isolated ipsilateral 6th nerve palsy from its compression in Dorello’s canal due to thrombosis of the ipsilateral inferior petrosal sinus is extremely rare. To our knowledge, only two patients have been reported with isolated abducens palsy due to IPS thrombosis; one caused by septic emboli and the other developed it during IPS cortisol level sampling.     

TCD动态监测静脉窦血栓形成五例

目的评价静脉窦血栓形成患者经颅多普勒超声(TCD)检测静脉侧枝的开放和再通情况以及颅内压变化。方法用TCD 2MHz探头检测5例静脉窦血栓形成患者的颅内静脉的血流速度以及动脉频谱形态的变化。结果 5例静脉窦血栓形成患者的颅内静脉(大脑中深静脉、基底静脉)的血流速度均明显增高(139cm/s,118 cm/s,99 cm/s,103 cm/s,58cm/s),动脉频谱呈高阻力,经过治疗,随着病情的好转,颅内静脉血流速度下降,颅内动脉频谱由高阻力型恢复正常。结论 TCD能可靠无创、准确地检测颅内静脉,在病程中通过多次的TCD检查,可以评价静脉窦血栓形成静脉侧枝的开放和再通情况以及颅内压变化。     

闭合性颅脑损伤后乙状窦栓塞的诊断与治疗

目的讨论闭合性颅脑损伤后乙状窦栓塞的诊断方法及治疗原则。方法采用扩大版的科技引文索引检索系统获得1995年1月至2010年1月关于闭合性颅脑损伤后乙状窦栓塞的7项研究中的15名患者,结合本组1例,共16例。通过分析,明确其临床概况、诊断方法、治疗原则及预后。结果 16例闭合性颅脑损伤后乙状窦栓塞患者平均年龄20.9岁,伤后GCS评分13~15分,剧烈头痛、呕吐等颅内压增高症状明显。12例栓塞在右侧,4例在左侧。12例合并有横窦或颈静脉栓塞。13例有颅骨骨折表现,且骨折与乙状窦栓塞均为同侧。10例仅对症治疗,5例接受抗凝治疗(其中2例还接受了手术治疗),另有1例仅接受了手术治疗。所有患者预后良好。结论闭合性颅脑损伤后出现无法解释的颅内压增高表现时应高度怀疑乙状窦栓塞,CTA、磁共振静脉造影以及DSA可以明确乙状窦栓塞的诊断。大部分患者经观察对症治疗可取得满意效果,部分患者可在监测凝血功能条件下行抗凝治疗。     

Lemierre’s Syndrome presenting with neurological and pulmonary symptoms: Case report and review of the literature

Lemierre’s Syndrome (LS) is a potentially life-threatening condition, characterized by clinical or radiologic evidence of internal jugular vein thrombosis following an oropharyngeal infection, most commonly by Fusobacterium necrophorum. A high index of suspicion and early recognition is important for successful management and to prevent systemic complications like multiorgan failure with extremely high morbidity, prolonged hospitalization and, not uncommonly, death. We are reporting a rare case of LS that was complicated with internal jugular vein and cavernous sinus thrombosis along with lung metastatic lesions, which was diagnosed and treated at our institute.     

Neonatal thrombosis of the jugular veins: long-term repercussions on cerebral development (two cases)

Bilateral obstruction of the internal jugular veins is rare in the neonatal period and no long-term follow-up has been reported yet. We report two cases. The first developped an extensive thrombosis of superior veina cava related to a central veinous line, the second an unilateral thrombosis of the transverse-sigmoid sinus associated to a constitutional hypoplasia of the contralateral jugular vein. In the latter case, no cause or risk factor was noticeable, except for a forceps delivery. In both cases the clinical course was dominated by the development of a prominent collateral network of cervico-facial veins and by a progressive macrocrania. According to MRImaging, the latter was not related to a dilation of CSF spaces, but to a macro-encephaly, either by inflation of the vascular veinous compartment inside the parenchyma or by genuine brain's overgrowth. Long-term follow-up showed a grossly normal course, both from the neurological and the scholar point of view. However, slight neuropsychological anomalies were noticed, bringing some shade on the prognosis.     

Endovascular treatment for dural arteriovenous fistulas at the jugular foramen

We report the technique and results of the endovascular treatment of jugular foramen dural arteriovenous fistulas (DAVFs) in 4 (3 men and 1 women, mean age 50.75 years) symptomatic patients. The jugular foramen DAVFs accounted for 5.9% of intracranial DAVFs. Three patients presented with pulsatile tinnitus and 1 patient presented with intracranial hemorrhage. Angiography demonstrated an AV fistula at the jugular foramen, mostly arising from the middle meningeal, ascending pharyngeal and vertebral arteries with direct drainage to the internal jugular vein. All patients underwent transarterial embolization using Onyx-18. Complete shunt obliteration was achieved in 3 patients; and shunt reduction, in 1 patient, who was cured with additional surgery. Our study suggests that in jugular foramen DAVF transarterial embolization with Onyx should be considered when access is available.     

Interobserver agreement in the magnetic resonance location of cerebral vein and dural sinus thrombosis

The interobserver variation in the magnetic resonance (MR) location of cerebral vein and dural sinus thrombosis (CVT) has not been previously reported. Four independent observers rated a convenience sample of 40 MR/MR angiographies to assess whether or not each dural sinus and major cerebral veins were occluded. Interobserver reliability was measured using κ statistics. Interobserver agreement was comparable between the six pairs of raters. Agreement was excellent for thrombosis of the deep cerebral venous system ( κ  = 1.00), cerebellar veins ( κ  = 1.00), superior saggital sinus ( κ range: 0.82–1) and right jugular vein ( κ range: 0.84–0.95); good to excellent for the right transverse/sigmoid sinus ( κ range: 0.75–0.90) and the left jugular vein ( κ range: 0.65–0.85); moderate to excellent for the left lateral sinus ( κ range: 0.59–0.78) and the straight sinus ( κ range: 0.59–0.92); poor to good for the cortical veins ( κ range: 0.02–0.65). Agreement between observers varies with the location of CVT. It is good or excellent for most of the occluded sinus and veins, except for the cortical veins. This study suggests that information on the location of CVT can be reliably collected and used in multicentre studies.     

Thrombosis of the superior cerebral vein with hemorrhagic cerebral infarction--serial MRI and pathological study of a case

We reported an autopsy case of thrombotic occlusion of the superior cerebral vein with hemorrhagic laminar necrosis of the right parietal cortex. A 68-year-old woman was admitted to our hospital because of a severe headache and left hemiplegia of acute onset. There was a past history of hypertension, fever of unknown origin, leukocytopenia and nasal dermatitis. Magnetic resonance images (MRI) disclosed thrombosis of the superior sagittal sinus and of the right parietal cortical vein as well as right parieto-occipital cerebral infarction. Although she improved with mild sequelae, the subsequent MRI showed a recurrent thrombosis of the superior sagittal sinus. Ten months after the onset she died suddenly, presumably due to acute myocardial infarction. Pathologically, thrombotic occlusion of the right parietal cortical vein, recurrent thrombosis of the superior sagittal sinus and old hemorrhagic cortical laminar necrosis of the right parietal region were revealed. Moreover, intracranial arteritis and phlebitis were observed, as well as arteriolitis in the peripheral nerves. In our case, MRI was useful for the diagnosis and following the course of cerebral venous thrombosis. Cerebral noninfective vasculitis may well have caused the venous thrombosis.     

Dural scalp and intracranial hemangiomas causing hydrocephalus and venous sinus thrombosis in an infant

Cutaneous scalp hemangiomas may herald the presence of occult intracranial hemangiomas. A previously healthy 4-month-old girl presented with a bleeding scalp hemangioma, a bulging fontanel, and anemia. Magnetic resonance imaging (MRI) of the brain revealed hydrocephalus along with multiple intracranial hemangiomas. These lesions compressed the jugular foramina, resulting in venous sinus thrombosis involving the right transverse sinus, the left sigmoid sinus, and the torcular herophili. The patient had no family history of phakomatoses or other genetic abnormalities. A thrombophilia work-up result was unremarkable. The patient was treated with prednisolone (10 mg twice daily) and low molecular weight heparin (1 mg/kg/dose) twice daily. This treatment decreased the size of her cutaneous and intracranial hemangiomas and led to the resolution of her venous sinus thromboses and hydrocephalus. Innocuous scalp hemangioma in an infant may herald more concerning intracranial pathology, which can be treated effectively if diagnosed with appropriate imaging studies.     

脑静脉血栓的治疗方法探讨

目的 探讨脑静脉血栓的治疗方法。方法对133例脑静脉血栓单独或联合采用经颈动脉溶栓、静脉窦内留置微导管连续溶栓、静脉窦成形（支架置入或球囊扩张）等方法进行治疗，同时辅以全身抗凝治疗。结果治疗后颅内压均明显下降，除１例ICP降至350mmH2O者失明，1例行静脉窦球囊扩张成形者（拒绝接受静脉窦支架置入）由术前760mmH2O降至450mmH2O外．其余病人ICP基本稳定在200-260mmH2O。颅内出血严重致脑疝死亡2例。溶栓后发生脑出血3例．均经手术治愈。结论同时应用血管内溶栓和全身抗凝是治疗脑静脉血栓较为可靠和安全的方法；血栓部位不同者治疗方案应个性化。