Trigeminocardiac reflex in a child during pre-Onyx DMSO injection for juvenile nasopharyngeal angiofibroma embolization. A case report.

We describe the occurrence of the trigeminocardiac reflex (TCR) during DMSO pre-flushing of the microcatheter in preparation for Onyx embolization via the internal maxillary artery. TCR has not been previously associated with embolization of extradural entities. Familiarity with this clinical reflex and its proper management may help in planning neurointerventional procedures involving DMSO injection in the trigeminal territory.

Safety and clinical efficacy of Onyx for embolization of extracranial head and neck vascular anomalies.

BACKGROUND AND PURPOSE: Onyx was developed for embolization of central nervous system AVMs but is increasingly used extracranially because of its unique physical properties. We review our experience and results with the use of Onyx for the treatment of fast-flow extracranial vascular lesions. MATERIALS AND METHODS: We retrospectively analyzed clinical and imaging records of 22 patients who underwent 71 extracranial embolizations from March 2007 through January 2010. The diagnoses were the following: cervicofacial AVM (n = 18), traumatic fistula (n = 3), and vessel laceration (n = 1). In 62 of 71 procedures (87%), Onyx was the sole embolic agent; it was delivered transarterially in 67/71 and percutaneously in 4/71 procedures. Clinical goals included amelioration of pain and control of bleeding. The clinical efficacy of embolization was judged by symptom control, and adverse events were assessed by clinical examination and history, both postembolization and 4 weeks postprocedure. RESULTS: Cessation of acute bleeding was achieved in 13/14 cases, with 1 case of immediate recurrent massive epistaxis prompting reintubation and further embolization. Control of subacute bleeding episodes and pain was achieved for all patients. Following staged embolization, 7 patients underwent surgical resection without significant blood loss. Surgeons reported high satisfaction with the intraoperative handling properties of Onyx. Transient swelling, local tenderness, or numbness was encountered after 7 procedures. There were no stuck catheters, vessel dissections, or vessel ruptures and no skin discoloration. CONCLUSIONS: Staged Onyx embolization was clinically efficacious in managing extracranial fast-flow vascular malformations and lesions, with low associated morbidity.

Hermann Friedberg's case report: an early description of CLOVES syndrome.

CLOVES syndrome is a recently described overgrowth disorder with complex vascular anomalies. Careful analysis of the case report by the German physician Hermann Friedberg "gigantism of the right lower limb" published in 1867 revealed that the report probably represents one of the first written accounts of CLOVES syndrome.

A novel association between RASA1 mutations and spinal arteriovenous anomalies.

BACKGROUND AND PURPOSE: CM-AVM is a recently recognized autosomal dominant disorder associated with mutations in RASA1. Arteriovenous lesions have been reported in the brain, limbs, and the face in 18.5% of patients. We report a novel association between RASA1 mutations and spinal arteriovenous anomalies. MATERIALS AND METHODS: In a collaborative study, 5 index patients (2 females, 3 males) with spinal AVMs or AVFs and cutaneous multifocal capillary lesions were investigated for the RASA1 gene mutation. RESULTS: All 5 patients were found to have RASA1 mutation (2 de novo, 3 familial), and all had multifocal capillary malformations at birth. Neurologic deficits developed at ages ranging from infancy to early adulthood. All spinal anomalies (2 AVMs at the conus, 1 AVM at the lumbosacral junction, and 1 cervical and 1 cervicothoracic AVF) were complex, extensive, and fast-flow lesions. All patients required treatment based on the clinical and/or radiologic appearance of the lesions. CONCLUSIONS: To our knowledge, an association of RASA1 mutation and spinal AVM/AVF has not been described. MR imaging screening of patients with characteristic CMs and neurologic symptoms presenting at a young age may be useful in detecting the presence of fast-flow intracranial or intraspinal arteriovenous anomalies before potentially significant neurologic insult has occurred.

The safety of dedicated-team catheter-based diagnostic cerebral angiography in the era of advanced noninvasive imaging.

BACKGROUND AND PURPOSE: Given the current high quality and usefulness of noninvasive cerebrovascular imaging, invasive angiographic evaluation of the cerebrovascular system is justified if the procedural risk for a neurologic complication is far below the anticipated benefit. The purpose of this study was to evaluate the safety of diagnostic cerebral angiography provided by a dedicated neurointerventional team in a high-volume university hospital. MATERIALS AND METHODS: A consecutive cohort of 1715 patients undergoing diagnostic cerebral angiography at our institution from 2000 to 2008 was retrospectively assessed for incidence of stroke or TIA related to cerebral angiography. In the subgroup of patients (n = 40) who serendipitously underwent DWI within the first 30 days after cerebral angiography, the presence of new DWI hyperintensities found in territories explored during angiography was tabulated. Complications related to the catheter technique and sheath placement were also studied. RESULTS: No stroke or permanent neurologic deficit was seen in any of the 1715 patients undergoing diagnostic neuroangiography. One patient experienced a TIA. Nonneurologic complications without long-term sequelae occurred in 9 patients. Two patients had punctate areas of restricted diffusion in territories that had been angiographically explored. CONCLUSIONS: Within a high-volume neurointerventional practice, the risk for neurologic complications related to catheter-based diagnostic cerebral angiography can approach zero. As the absolute number of invasive diagnostic procedures diminishes with time, diagnostic cerebral angiography remains a useful tool while providing a foundation for neuroendovascular interventions, and should preferably be performed in institutions with high-volume operators also capable of managing unanticipated complicating adverse events.

The use of Onyx for embolization of central nervous system arteriovenous lesions in pediatric patients.

BACKGROUND AND PURPOSE: The safety and efficacy of Onyx for the embolization of central nervous system (CNS) arteriovenous (AV) lesions have been widely reported in adults. However, data describing the use of this agent in children are limited. This study presents our experience with Onyx in the treatment of CNS AV lesions in pediatric patients. MATERIALS AND METHODS: We retrospectively analyzed clinical and imaging records of 15 pediatric patients who underwent 36 transarterial embolizations by using Onyx for CNS AV lesions, from March 2007 through April 2009 at our institution. Underlying pathologies included brain AV malformations (AVMs) (n = 7), vein of Galen malformations (n = 4), dural AV fistulas (n = 2), and spinal AVMs (n = 2). For 7 procedures in very high-flow lesions, detachable coils were deployed before Onyx embolization, whereas in 29 procedures, Onyx was the sole embolic agent. The efficacy of embolization was judged by the residuum of AV shunting within the target region. RESULTS: Embolization was complete in 2 patients, nearly complete in 9 patients, and partial (and ongoing) in 4 patients. Following staged embolization, 7 patients underwent surgical resection without significant blood loss and with good functional outcome in all cases. Clinically silent non-target embolization was encountered in 2 of 36 procedures. After 3 of the 36 embolizations, patients developed transient neurologic symptoms, all of which resolved to baseline within 24 hours. There were no non-neurologic adverse events. There was no imaging evidence of infarct or hemorrhage. CONCLUSIONS: Onyx embolization of pediatric CNS AV lesions can be an efficacious treatment technique, with extremely low associated morbidity.

Microglial activation and intracerebral hemorrhage.

INTRODUCTION: Microglia activate upon injury, migrate to the injury site, proliferate locally, undergo morphological and gene expression changes, and phagocytose injured and dying cells. Cytokines and proteases secreted by these cells contribute to the injury and edema formed. We studied the injury outcome after local elimination/paralysis of microglia. METHODS: Adult male mice were subjected to intracerebral hemorrhage (ICH) by intra-caudate injection of either collagenase or autologous blood. Mice survived for different periods of time, and were subsequently evaluated for neurological deficits, size of the hematoma, and microglia activation. Mice expressing an fms-GFP transgene or the CD11b-HSVTK transgene were also used. For elimination of monocytes/macrophages, CD11b-HSVTK mice were treated with ganciclovir prior to hemorrhage. Modifiers of microglial activation were also used. RESULTS: Induction of ICH resulted in robust microglia activation and recruitment of macrophages. Inactivation of these cells, genetically or pharmacologically, pointed to a critical role of the time of such inactivation, indicating that their role is distinct at different time points following injury. Edema formation is decreased when microglia activation is inhibited, and neurological outcomes are improved. CONCLUSIONS: Microglia, as immunomodulatory cells, have the ability to modify the final presentation of ICH.

Management of delayed edema formation after fibrinolytic therapy for intracerebral hematomas: preliminary experimental data.

OBJECTIVE: Fibrinolytic therapy for spontaneous intracerebral hemorrhage using recombinant tissue plasminogen activator (rtPA) is considered a viable alternative to microsurgical hematoma removal. However, experimental data suggest that rtPA is neurotoxic and evokes a late perihematomal edema. We present preliminary data focusing on the avoidance of late edema formation after lysis of an intracerebral hematoma in a porcine model. METHODS: Twenty pigs underwent placement of a frontal intracerebral hematoma with a minimum volume of 1 mL. Half of the pigs were subjected to rtPA clot lysis and MK-801 injection for blockage of the NMDA receptor-mediated rtPA-enhanced excitotoxic pathway. The remaining 10 pigs received desmoteplase (DSPA) for clot lysis, which is known to be a less neurotoxic fibrinolytic agent than rtPA. MRI on the day of surgery and on postoperative days 4 and 10 was used to assess hematoma and edema volumes. RESULTS: Late edema formation could be prevented in both the MK-801/rtPA and DSPA pigs. CONCLUSION: The benefits of fibrinolytic therapy for intracerebral hematomas appear to be counterbalanced by late edema formation. MK-801 infusion as an adjunct to rtPA lysis, or the use of DSPA instead of rtPA, prevents late edema and therefore has the potential to further improve results after clot lysis.

Delayed diagnosis of spinal dural arteriovenous fistula in the absence of pathological vessels on MRI.

The authors report on a 69-year-old man presenting with progressive leg weakness and gait ataxia over two years. A central intramedullary cord lesion ranging from T8-12 on MR imaging was misdiagnosed as a low-grade glioma and a biopsy was attempted followed by temporary clinical deterioration. Selective spinal angiography revealed a spinal dural arteriovenous (AV) fistula on the left L3 nerve root sheath despite the absence of pathological vessels on MR imaging. The fistula was successfully treated by microsurgical interruption of the arterialized intradural vein. The present case should remind us to include selective spinal angiography in our diagnostic work-up in patients predisposed for spinal dural AV fistula by male sex, advanced age and clinical presentation of slowly progressive sensorimotor symptoms with myelopathy on MR imaging, even in the absence of any pathological vascular structures.

Technical pitfalls in a porcine brain retraction model. The impact of brain spatula on the retracted brain tissue in a porcine model: a feasibility study and its technical pitfalls.

We describe technical pitfalls of a porcine brain injury model for identifying primary and secondary pathological sequelae following brain retraction by brain spatula. In 16 anaesthetised male pigs, the right frontal brain was retracted in the interhemispheric fissure by a brain spatulum with varying pressures applied by the gravitational force of weights from 10 to 70 g for a duration of 30 min. The retracted brain tissue was monitored for changes in intracranial pressure and perfusion of the cortex using a Laser Doppler Perfusion Imager (MoorLDI). To evaluate the extent of oedema and cortical contusions, MRI was performed 30 min and 72 h after brain retraction. Following the MR scan, the retracted brain areas were histopathologically assessed using H&E and Fluoro-Jade B staining for neuronal damage. Sinus occlusion occurred in four animals, resulting in bilateral cortical contusions and extensive brain oedema. Retracting the brain with weights of 70 g (n = 4) caused extensive oedema on FLAIR images that correlated clinically with a hemiparesis in three animals. Morphologically, an increased number of Fluoro-Jade B-positive neurons were found. A sequential decrease in weights prevented functional deficits in animals. A retraction pressure applied by 10-g weights (n = 7) caused a mean rise in intracranial pressure to 4.0 +/- 3.1 mm Hg, and a decrement in mean cortical perfusion from 740.8 +/- 41.5 to 693.8 +/- 72.4 PU/cm2 (P < 0.24). A meticulous dissection of the interhemispheric fissure and a reduction of weights to 10 g were found to be mandatory to study the cortical impact caused by brain spatula reproducibly.

Periodic surveillance of nosocomial infections in a neurosurgery intensive care unit.

BACKGROUND: We assessed data on the epidemiology of nosocomial infections (NIs) in a 14-bed neurosurgical intensive care unit (NSICU) and used surveillance data for the promotion of quality improvement activities. PATIENTS AND METHODS: Prospective periodic surveillance was performed over five 3-month periods between July 1998 and October 2002 on all patients admitted with a length of stay > 24 hours. RESULTS: 763 patients with a total of 4,512 patient days and a mean length of stay of 5.9 days were enrolled within the 15-month study period. A total of 93 NIs were identified in 82 patients. Urinary tract infections (24.7%), pneumonia (23.6%), and bloodstream infections (17.2%) were the most frequent NIs recorded. Device-associated incidence rates were 6.0 (3.8-9.0, CI(95%)) for urinary tract infection, 4.4 (2.4-7.4, CI(95%)) for bloodstream infection, and 10.3 (6.3-15.9, CI(95%)) for pneumonia per 1,000 days at risk. For improvement of infection control-related processes, evidence-based infection control guidelines were established and an NSICU nurse was designated to be responsible for infection control issues on the ward. In addition, several infection control problems arose during the observation periods and were rapidly responded to by introducing specific intervention strategies. CONCLUSION: Periodic surveillance is a valuable tool for assessing the epidemiology of NIs in the NSICU setting as well as for promoting the initiation of quality improvement activities.

Are the configuration and neck morphology of experimental aneurysms predictable? A technical approach.

Aneurysm configuration and neck morphology are important factors in the decision for cerebral aneurysm therapy, i.e., clipping versus coiling. The aim of our study was to create various aneurysm configurations in a predictable and reproducible way in an animal model. In our recently proposed endovascular approach to produce bifurcation aneurysms in the rabbit, the right common carotid artery (CCA) is surgically exposed and distally ligated, and a sheath is advanced retrogradely into the CCA, the base of which is proximally occluded using a Fogarty balloon. Subsequently, elastase is injected via a microcatheter that is placed directly distal to the balloon and allowed to incubate for 20 min. After removal of the sheath, saccular aneurysms begin to form within 2 weeks. For greater variability in aneurysm size and neck morphology, we modified two parameters of this formerly established elastase-induced aneurysm model--the distance between the balloon and sheath and the level of balloon position--before the elastase was endoluminally incubated in 15 rabbits. Three weeks after aneurysm induction, the size and configuration of the aneurysms were controlled using DSA. Our results confirm that balloon occlusion in the brachiocephalic trunk results in broad-based aneurysms, whereas balloon occlusion in the CCA gives rise to circumscribed aneurysm necks. By increasing the distance between the balloon and sheath, the rabbits developed significantly larger aneurysms. The balloon-sheath distance and the level of balloon occlusion proved to be parameters whose modifications result in predictable and reproducible aneurysm variants that can be used for the testing of endovascular devices.

Haemorrhagic tracheal necrosis as a lethal complication of an aneurysm model in rabbits via endoluminal incubation with elastase.

BACKGROUND: We describe a lethal complication of an aneurysm model in rabbits for saccular aneurysmal creation via endoluminal incubation with elastase. METHOD: In 24 anaesthetized female New Zealand White rabbits, the right common carotid artery (CCA) was ligated distally to the arteriotomy. A 4F sheath was then placed retrograde into the CCA, and its origin was occluded endoluminally using a 2F Fogarty balloon. Elastase was incubated above the balloon in the separated vessel lumen for the duration of 20 minutes. Two weeks later, digital subtraction angiography was performed for aneurysm control. Two animals were then sacrificed and the aneurysm studied on histology. All animals that died within the experiment were examined post-mortem. FINDINGS: Following this protocol, an aneurysm with a mean size of 7.6 x 3.2 mm could be created in 11 out of 24 animals. 9 out of 13 animals with lethal outcome died from haemorrhagic necrosis of the trachea with subsequent pulmonary complications. DSA releaved an arterial branch originating from the proximal CCA in a near 90 degree-angle aiming at the trachea. INTERPRETATION: The endoluminal incubation with elastase is suitable for aneurysm creation of reproducible size that are suited to test new endovascular devices such as stents and new coils. One should always be aware of an arterial branch of the CCA supplying the trachea. In case of elastase instillation into this branch, lethal haemorrhagic necrosis of the trachea occurs. Bearing this complication in mind, we have experienced a minimal loss of animals in subsequent studies.

Delayed oedema in the pyramidal tracts remote from intracerebral missile path following gunshot injury.

A 60-year-old man developed a severe left hemiparesis and central facial palsy, accompanied by somnolence and dysarthria 9 days after a gunshot wound to the right temporal region, from which he slowly recovered over 3 months. MRI disclosed bilateral oedema of the pyramidal tracts. This was interpreted as a consequence of the impact of the pressure wave caused by the bullet, after excluding an infectious or vascular cause.

Endovascular treatment of experimentally induced aneurysms in rabbits using stents: a feasibility study.

Although Guglielmi detachable coil (GDC) systems have been generally accepted for treatment of intracranial aneurysms, primary stenting of aneurysms using porous stents or implantation of coils after stent placement remains experimental. Testing of these new methods requires an animal model which imitates human aneurysms in size, configuration and neck morphology. We assessed in detail the technical requirements of and steps for transfemoral stent treatment of experimentally induced aneurysms at the top of the brachiocephalic trunk in rabbits. We created aneurysms in ten rabbits by distal ligation and intraluminal digestion of the right common carotid artery with elastase. We treated five animals with porous stents alone, and five with stents plus coiling via the meshes of the stent, which permitted dense packing of coils. No complications related to the procedures occurred. In all animals, even in those treated solely with porous stents, total occlusion of the aneurysm was achieved. Our animal model can be suitable for testing the biocompatibility and occlusion rate of new methods and devices for the treatment of experimental aneurysms.

Giant cavernoma of the brain stem: value of delayed MR imaging after contrast injection.

Cavernous angiomas are vascular malformations composed of slowly perfused, sinusoidal vessels which can be located in any part of the central nervous system. Whereas diagnosis is mostly straightforward in typical cases, some lesions may present in unusual locations or with unusual imaging characteristics. Because of the slow perfusion, contrast enhancement is not regarded as a characteristic imaging feature of cavernomas. We report a large brain stem cavernoma with signs of recent bleeding, in which the differential diagnosis against other mass lesions was facilitated by the demonstration of slow, but intense, contrast enhancement on MRI 1 h after contrast injection. We conclude that contrast enhancement in delayed images may contribute to a safe diagnosis of cavernous haemangiomas and should be performed in atypical cases.

Giant cavernoma of the brain stem: value of delayed MR imaging after contrast injection.

Cavernous angiomas are vascular malformations composed of slowly perfused, sinusoidal vessels which can be located in any part of the central nervous system. Whereas diagnosis is mostly straightforward in typical cases, some lesions may present in unusual locations or with unusual imaging characteristics. Because of the slow perfusion, contrast enhancement is not regarded as a characteristic imaging feature of cavernomas. We report a large brain stem cavernoma with signs of recent bleeding, in which the differential diagnosis against other mass lesions was facilitated by the demonstration of slow, but intense, contrast enhancement on MRI 1 h after contrast injection. We conclude that contrast enhancement in delayed images may contribute to a safe diagnosis of cavernous haemangiomas and should be performed in atypical cases.

Endovascular treatment of experimental aneurysms in rabbits using Guglielmi detachable coils -- a feasibility study.

The Guglielmi detachable coil (GDC) has been generally accepted for treatment of intracranial aneurysms. Preclinical testing of new coil developments requires animal models of aneurysms which imitate human aneurysms in size, configuration and neck morphology. We assessed in detail the technical requirements and steps for transfemoral treatment of experimentally induced aneurysms at the top of the brachiocephalic trunk (TBC) in rabbits. We created aneurysms in five rabbits by distal ligation and intraluminal incubation of the right common carotid artery with elastase. All animals were treated successfully 2-3 weeks after induction of the aneurysm, with dense packing of the coils. No complications related to the procedures occurred. The study demonstrates that our animal model can be a suitable method for testing the biocompatibility and occlusion rate of new embolic materials.