Outcome Following Hemorrhage From Cranial Dural Arteriovenous Fistulae: Analysis of the Multicenter International CONDOR Registry.

Background and Purpose: Dural arteriovenous fistulae can present with hemorrhage, but there remains a paucity of data regarding subsequent outcomes. We sought to use the CONDOR (Consortium for Dural Arteriovenous Fistula Outcomes Research), a multi-institutional registry, to characterize the morbidity and mortality of dural arteriovenous fistula­related hemorrhage. Methods: A retrospective review of patients in CONDOR who presented with dural arteriovenous fistula­related hemorrhage was performed. Patient characteristics, clinical follow-up, and radiographic details were analyzed for associations with poor outcome (defined as modified Rankin Scale score ≥3). Results: The CONDOR dataset yielded 262 patients with incident hemorrhage, with median follow-up of 1.4 years. Poor outcome was observed in 17.0% (95% CI, 12.3%­21.7%) at follow-up, including a 3.6% (95% CI, 1.3%­6.0%) mortality. Age and anticoagulant use were associated with poor outcome on multivariable analysis (odds ratio, 1.04, odds ratio, 5.1 respectively). Subtype of hemorrhage and venous shunting pattern of the lesion did not affect outcome significantly. Conclusions: Within the CONDOR registry, dural arteriovenous fistula­related hemorrhage was associated with a relatively lower morbidity and mortality than published outcomes from other arterialized cerebrovascular lesions but still at clinically consequential rates.

Updates in the management of cranial dural arteriovenous fistula.

Dural arteriovenous fistula (dAVF) accounts for approximately 10% of all intracranial vascular malformations. While they can be benign lesions, the presence of retrograde venous drainage and cortical venous reflux makes the natural course of these lesions aggressive high risk of haemorrhage, neurological injury and mortality. Endovascular treatment is often the first line of treatment for dAVF. Both transarterial and transvenous approaches are used to cure dAVF. The selection of treatment approach depends on the angioarchitecture of the dAVF, the location, the direction of venous flow. Surgery and, to a lesser extent, stereotactic radiosurgery are used when endovascular approaches are impossible or unsuccessful.

Transarterial Onyx Embolization of Intracranial Dural Fistulas: A Prospective Cohort, Systematic Review, and Meta-Analysis.

BACKGROUND: Onyx is important embolic material in the endovascular treatment of intracranial dural arteriovenous fistula (DAVF). However, its impact on DAVF occlusion rates, morbidity, mortality, and complication rates is not fully examined. OBJECTIVE: To improve understanding of safety and effectiveness profiles associated with transarterial endovascular treatment using Onyx for intracranial DAVF. METHODS: We analyzed data from our prospective clinical registry and conducted a systematic review of all previous transarterial embolization studies using Onyx published between January 2005 and December 2015 in MEDLINE and EMBASE. RESULTS: In the prospective study, 41 transarterial procedures were performed in 33 consecutive patients harboring 36 DAVFs. Complete initial exclusion was obtained in 32 of 36 (88.9%) fistulas; 31 fistulas were followed up showing 4 (12.9%) recurrences. Procedure-related morbidity and mortality were 3% and 0%, respectively. The literature review identified 19 studies involving a total of 425 patients with 463 DAVFs. Meta-analysis, including our registry data, showed an initial complete occlusion rate of 82% (95% confidence interval [CI]: 74%, 88%; I2, 70.6%), and recurrence rate at midterm of 2% (95% CI: 0%, 5%; I2, 21.5%). Pooled postoperative neurological deficit, procedure-related morbidity, and mortality rates were 4% (95% CI: 2%, 6%; I2, 0%), 3% (95% CI: 1%, 5%; I2, 0%), and 0%, respectively. CONCLUSION: This meta-analysis suggests that transarterial embolization with Onyx is a safe treatment modality for DAVFs. Although Onyx showed a low recurrence rate at midterm, the long-term risk is poorly addressed in our study and should warrant a longer follow-up.

Cirugía precoz en la prevención de la sepsis del recién nacido con mielomeningocele / Precocious surgery in preventing the sepsis of newborn with meningomyelocele

El mielomeningocele es una malformación congénita por defecto del cierre del tubo neural, se produce en las primeras semanas de crecimiento intrauterino. Consiste en una masa quística que incluye tejido nervioso y meninges, acompañadas de una fusión incompleta de los arcos vertebrales. Produce severos daños neurales y puede asociarse a otras malformaciones. Su reparación, en las primeras horas de vida, es necesaria para evitar complicaciones que pueden comprometer la vida del niño o causar mayor discapacidad. Se reportó un caso de un recién nacido con diagnóstico de mielomeningocele fisurado, a nivel lumbar. Se intervino quirúrgicamente en las primeras 6 h de vida; se disecó el saco dural y se separaron las raíces, logrando el cierre completo de la duramadre. Se reparó la piel con afrontamiento de los bordes y adecuada cicatrización de la herida. Fue aplicada antibioticoterapia profiláctica con cefalosporina de tercera generación. El infante egresó a los 10 días de operado sin complicaciones neuroquirúgicas asociadas (AU).

Meningomyelocele is a congenital malformation by defect of neural tube closing, produced in the first weeks of intrauterine grow. It is a cystic mass that includes nervous tissues and meninges, together with an incomplete fusion of the vertebral arches. It produces severe neural damages and could be associated to other malformations. It is necessary to repair it during the first hours after birth to avoid complications that could compromise the childs life or cause more disability. The case of a new-born child diagnosed with fissured meningomyelocele at the lumbar level is presented. He was operated in the first six hours after birth; the dural sac was dissected and the roots separated, reaching the complete dura mater closure. The skin was repaired with edges affronting and adequate wound healing. Prophylactic antibiotic therapy with third generation cephalosporin was applied. The child was discharged 10 days alter the surgery without associated neurosurgical complications (AU).

Cirugía precoz en la prevención de la sepsis del recién nacido con mielomeningocele / Precocious surgery in preventing the sepsis of newborn with meningomyelocele

El mielomeningocele es una malformación congénita por defecto del cierre del tubo neural, se produce en las primeras semanas de crecimiento intrauterino. Consiste en una masa quística que incluye tejido nervioso y meninges, acompañadas de una fusión incompleta de los arcos vertebrales. Produce severos daños neurales y puede asociarse a otras malformaciones. Su reparación, en las primeras horas de vida, es necesaria para evitar complicaciones que pueden comprometer la vida del niño o causar mayor discapacidad. Se reportó un caso de un recién nacido con diagnóstico de mielomeningocele fisurado, a nivel lumbar. Se intervino quirúrgicamente en las primeras 6 h de vida; se disecó el saco dural y se separaron las raíces, logrando el cierre completo de la duramadre. Se reparó la piel con afrontamiento de los bordes y adecuada cicatrización de la herida. Fue aplicada antibioticoterapia profiláctica con cefalosporina de tercera generación. El infante egresó a los 10 días de operado sin complicaciones neuroquirúgicas asociadas (AU).

Meningomyelocele is a congenital malformation by defect of neural tube closing, produced in the first weeks of intrauterine grow. It is a cystic mass that includes nervous tissues and meninges, together with an incomplete fusion of the vertebral arches. It produces severe neural damages and could be associated to other malformations. It is necessary to repair it during the first hours after birth to avoid complications that could compromise the child’s life or cause more disability. The case of a new-born child diagnosed with fissured meningomyelocele at the lumbar level is presented. He was operated in the first six hours after birth; the dural sac was dissected and the roots separated, reaching the complete dura mater closure. The skin was repaired with edges affronting and adequate wound healing. Prophylactic antibiotic therapy with third generation cephalosporin was applied. The child was discharged 10 days alter the surgery without associated neurosurgical complications (AU).

Outcome after intracranial haemorrhage from dural arteriovenous fistulae; a systematic review and case-series.

Dural arteriovenous fistulae (DAVFs) are a rare cause of intracranial haemorrhage. We aimed to investigate outcome of patients with intracranial haemorrhage from a DAVF. We performed a systematic literature search for studies reporting outcome after intracranial haemorrhage caused by a DAVF. We used predefined selection criteria and assessed the quality of the studies. In addition, we studied outcome in all patients with DAVF who had presented with intracranial haemorrhage at two university centers in the Netherlands, between January 2007 and April 2012. We calculated case fatality and proportions of patients with poor outcome (defined as modified Rankin Scale ≥ 3 or Glasgow Outcome Scale ≤ 3) during follow-up. We investigated mean age, sex, mid-year of study and percentage of patients with parenchymal haemorrhage as determinants of case fatality and poor outcome. The literature search yielded 16 studies, all but two retrospective and all hospital-based. Combined with our cohort of 29 patients the total number of patients with DAVF-related intracranial haemorrhage was 326 (58% intracerebral haemorrhage). At a median follow-up of 12 months case fatality was 4.7% (95% CI 2.5-7.5; 17 cohorts) and the proportion of patients with poor outcome 8.3% (95% CI 3.1-15.7; nine cohorts). We found no effect of mean age, sex, mid-year of the cohorts and percentage of patients with parenchymal haemorrhage on either outcome. Hospital based case-series suggest a relatively low risk of death and poor outcome in patients with intracranial haemorrhage due to rupture of a DAVF. These risks may be underestimated because of bias.

Spinal pial (type IV) arteriovenous fistulae: a systematic pooled analysis of demographics, hemorrhage risk, and treatment results.

BACKGROUND: Demographics, hemorrhage risk, and results of surgical and endovascular treatment of spinal pial (type IV) arteriovenous fistulae (AVFs) across a large patient group have not been previously reported. OBJECTIVE: To report demographics, hemorrhage rates, and treatment results for these AVFs. METHODS: We performed a pooled analysis via the PubMed and Embase databases through November 2012. Individualized patient data were extracted and analyzed using Cox proportional hazards regression to obtain hazard ratios for hemorrhage risk factors and pooled for baseline demographics and treatment results. RESULTS: We extracted information on 213 patients from 28 studies. Only 1% of lesions were incidental; 93% of patients presented with neurologic deficits and 36% with hemorrhage. Patients with type IVa lesions were significantly older (mean age, 46.9 years) and demonstrated a male sex predilection (68% male). Patients with type IVc lesions were significantly younger (mean age, 18.7 years), had no sex predilection, and had the highest prevalence of syndromic conditions (29% of cases). The annual hemorrhage rate was 2.5% (95% confidence interval [CI]: 1.4%-4.7%), increasing to 5.6% for hemorrhagic fistulae (95% CI: 3.0%-10.7%; hazard ratio: 6.31; 95% CI: 0.69-57.4; P = .10). Patient sex, fistula location, and fistula subclass were not significant risk factors for hemorrhage. The surgical obliteration rate was 88%; 68% of patients improved, 26% were the same, and 6% were worse. The endovascular obliteration rate was 74%; 75% of patients improved, 14% were the same, and 11% were worse. CONCLUSION: We demonstrate the utility of the Anson-Spetzler a-c subclassification and underscore the efficacy of surgical and endovascular spinal AVF treatment.

Early and long-term excess mortality in 227 patients with intracranial dural arteriovenous fistulas.

OBJECT: The aim of this study was to assess the early and long-term excess mortality in patients with intracranial dural arteriovenous fistula (DAVF) compared with a matched general Finnish population in an unselected, population-based series. METHODS: The authors identified 227 patients with DAVFs admitted to 2 of the 5 Departments of Neurosurgery in Finland--Helsinki and Kuopio University Hospitals--between 1944 and 2006. All patients were followed until death or the end of 2009. Long-term excess mortality was estimated using the relative survival ratio compared with the general Finnish population matched by age, sex, and calendar year. RESULTS: The median follow-up period was 10 years (range 0-44 years). Two-thirds (67%) of the DAVFs were located in the region of transverse and sigmoid sinuses. Cortical venous drainage (CVD) was present in 28% of the DAVFs (18% transverse and sigmoid sinus, 42% others). Of the 61 deaths counted, 11 (18%) were during the first 12 months and were mainly caused by treatment complications (5 of 11, 45%). The 1-year survivors presenting with hemorrhage experienced excess mortality until 7 years from admission. However, DAVFs with CVD were associated with significant, continuous excess mortality. There were more cerebrovascular and cardiovascular deaths in this group of patients than expected in the general Finnish population. Location other than transverse and sigmoid sinuses was also associated with excess mortality. CONCLUSIONS: In the patients with DAVF there was excess mortality during the first 12 months, mainly due to treatment complications. Thereafter, their overall long-term survival became similar to that of the matched general population. However, DAVFs with CVD and those located in regions other than transverse and sigmoid sinuses were associated with marked long-term excess mortality after the first 12 months.

Borden-Shucart Type I dural arteriovenous fistulas: clinical course including risk of conversion to higher-grade fistulas.

OBJECT: The goal of this study was to determine the clinical course of Borden-Shucart Type I cranial dural arteriovenous fistulas (DAVFs) and to calculate the annual rate of conversion of these lesions to more aggressive fistulas that have cortical venous drainage (CVD). METHODS: A retrospective chart review was conducted of all patients harboring DAVFs who were seen at the authors' institution between 1997 and 2009. Twenty-three patients with Type I DAVFs who had available clinical follow-up were identified. Angiographic and clinical data from these patients were reviewed. Neurological outcome and status of presenting symptoms were assessed during long-term follow-up. RESULTS: Of the 23 patients, 13 underwent endovascular treatment for intolerable tinnitus or ophthalmological symptoms, and 10 did not undergo treatment. Three untreated patients died of unrelated causes. In those who were treated, complete DAVF obliteration was achieved in 4 patients, and palliative reduction in DAVF flow was achieved in 9 patients. Of the 19 patients without radiographic cure, no patient developed intracranial hemorrhage or nonhemorrhagic neurological deficits (NHNDs), and no patient died of DAVF-related causes over a mean follow-up of 5.6 years. One patient experienced a spontaneous, asymptomatic obliteration of a partially treated DAVF in late follow-up, and 2 patients experienced a symptomatic conversion of their DAVF to a higher-grade fistula with CVD in late follow-up. The annual rate of conversion to a higher-grade DAVF based on Kaplan-Meier cumulative event-free survival analysis was 1.0%. The annual rate of intracranial hemorrhage, NHND, and DAVF-related death was 0.0%. CONCLUSIONS: A small number of Type I DAVFs will convert to more aggressive DAVFs with CVD over time. This conversion to a higher-grade DAVF is typically heralded by a change in patient symptoms. Follow-up vascular imaging is important, particularly in the setting of recurrent or new symptoms.

Transvenous embolization of intracranial dural arteriovenous shunts through occluded venous segments: experience in 51 Patients.

BACKGROUND AND PURPOSE: DAVFs with cortical venous reflux carry a high risk of morbidity and mortality. Endovascular treatment options include transarterial embolization with a liquid embolic agent or transvenous access with occlusion of the involved venous segment, which may prove difficult if the venous access route is thrombosed. The aim of this article is to describe the technique and results of the transvenous approach via thrombosed venous segments for occlusion of DAVFs. MATERIALS AND METHODS: Our study was a retrospective analysis of 51 patients treated with a transvenous approach through an occluded sinus that was reopened by gentle rotational advancement of a 0.035-inch guidewire, which opened a path for a subsequently inserted microcatheter. RESULTS: Of 607 patients with DAVFs, the transvenous reopening technique was attempted in 62 patients in 65 sessions and was successful in 51 patients and 53 sessions. Immediate occlusion was seen in 42 patients; on follow-up, occlusion was seen in 49 patients, whereas 2 patients had reduced flow without cortical venous reflux. No permanent procedure-related morbidity was noted. CONCLUSIONS: The reopening technique to gain access to isolated venous pouches or the cavernous sinus for the treatment of DAVFs is a safe and effective treatment, which should be considered if transarterial approaches fail or are anticipated to result only in an incomplete anatomic cure.

Predictors of outcome in childhood intracerebral hemorrhage: a prospective consecutive cohort study.

BACKGROUND AND PURPOSE: The purposes of this study were to describe features of children with intracerebral hemorrhage (ICH) and to determine predictors of short-term outcome in a single-center prospective cohort study. METHODS: A single-center prospective consecutive cohort study was conducted of spontaneous ICH in children aged 1 to 18 years from January 2006 to June 2008. Exclusion criteria were inciting trauma; intracranial tumor; isolated epidural, subdural, intraventricular, or subarachnoid hemorrhage; hemorrhagic transformation of ischemic stroke; and cerebral sinovenous thrombosis. Hospitalization records were abstracted. Follow-up assessments included outcome scores using the Pediatric Stroke Outcome Measure and King's Outcome Scale for Childhood Head Injury. ICH volumes and total brain volumes were measured by manual tracing. RESULTS: Twenty-two patients, median age 10.3 years (range, 4.2 to 16.6 years), had presenting symptoms of headache in 77%, focal deficits 50%, altered mental status 50%, and seizures 41%. Vascular malformations caused hemorrhage in 91%. Surgical treatment (hematoma evacuation, lesion embolization or excision) was performed during acute hospitalization in 50%. One patient died acutely. At a median follow-up of 3.5 months (range, 0.3 to 7.5 months), 71% of survivors had neurological deficits; 55% had clinically significant disability. Outcome based on Pediatric Stroke Outcome Measure and King's Outcome Scale for Childhood Head Injury scores was worse in patients with ICH volume >2% of total brain volume (P=0.023) and altered mental status at presentation (P=0.005). CONCLUSIONS: Spontaneous childhood ICH was due mostly to vascular malformations. Acute surgical intervention was commonly performed. Although death was rare, 71% of survivors had persisting neurological deficits. Larger ICH volume and altered mental status predicted clinically significant disability.

Hypoplasia or occlusion of the ipsilateral cranial venous drainage is associated with early fatal edema of middle cerebral artery infarction.

BACKGROUND AND PURPOSE: Thrombosis of the cerebral venous sinus may cause venous congestion, cerebral edema, and infarction. The role of cerebrovenous disorders in arterial ischemic stroke is unknown. The objective of this study was to examine the contribution of ipsilateral cranial venous abnormalities to the development of cerebral edema in middle cerebral artery infarction. METHODS: This is a retrospective study of consecutive patients with large middle cerebral artery infarction admitted to our neurocritical care unit from January 2007 to October 2008. Medical records, laboratory data, and imaging of cerebral edema and cranial venous sinuses were analyzed. RESULTS: Of the 14 patients identified to have large middle cerebral artery infarction and images of cranial venous drainages, 5 (35.7%) had fatal edema with clinical signs of transtentorial herniation. Four of the 5 patients developed fatal edema within 48 hours of ictus and were found to have abnormal ipsilateral cranial venous drainage, including atresia of the transverse sinus (one), occlusion of the internal jugular vein (one), and hypoplasia of the transverse sinus and internal jugular vein (2). The fifth patient had symmetrical bilateral cranial venous drainages and fatal edema at Day 5. Of the 9 patients with nonmalignant middle cerebral artery infarction, all had ipsilateral dominant or symmetrical bilateral venous drainages. CONCLUSIONS: In this small case series, we demonstrated that only the patients with hypoplasia or occlusion of the ipsilateral cranial venous drainage developed early fatal edema after large middle cerebral artery infarction. Our results suggest a role of cranial venous outflow abnormalities in the development of brain edema after arterial ischemic stroke.

Bias from requiring explicit consent from all participants in observational research: prospective, population based study.

OBJECTIVE: To evaluate the differences between adults who consent to participate in observational research, and those who do not. DESIGN: Prospective, population based cohort study. SETTING: Primary and secondary care throughout Scotland. PARTICIPANTS: 187 adults (aged > or = 16 years) resident in Scotland at the time of their first diagnosis of a brain arteriovenous malformation in 1999-2002. INTERVENTION: Postal consent form sent via participants' general practitioner. MAIN OUTCOME MEASURES: Differences between consenters and non-consenters in demographic and clinical features at first presentation, and outcome during follow-up. RESULTS: 111 adults (59%) consented to participate in the study. These consenters were not significantly different from non-consenters in age, sex, or socioeconomic status at first presentation. However, consenters were significantly more likely than non-consenters to present alive and independent, and with a seizure. During follow-up, consenters were significantly more likely to receive interventional treatment. Although consenters' survival was significantly better, they were more likely to have a seizure during follow-up. Presentation with intracranial haemorrhage conferred a higher risk of subsequent haemorrhage when the whole cohort was analysed, but not when it was restricted to consenters. CONCLUSIONS: We have found differences between adults who consent to participate in observational records-based research and those who do not, or cannot, consent. Blanket requirements for explicit consent for the use of individuals' identifiable data can bias disease registers, epidemiological studies, and health services research.

Endovascular treatment for dural arteriovenous fistulae of the superior petrosal sinus.

OBJECTIVE: To determine the usefulness of endovascular embolization for treatment of dural arteriovenous fistulae of the superior petrosal sinus. METHODS: We performed a retrospective review of 18 patients treated during a 16-year period. Transarterial and/or transvenous embolizations were performed as a preoperative adjunct or definitive therapy. Clinical follow-up status was supplemented by telephone interviews to determine Glasgow Outcome Scale scores. RESULTS: Fourteen patients (78%) were treated with a combination of endovascular therapy and open surgery, and 4 were treated by embolization alone (22%). Angiographic cure was achieved in all patients (100%). Thirty-day morbidity and mortality were 11 and 0%, respectively. The mean follow-up period was 5.4 years. At the latest follow-up examination, all patients had returned to independent clinical status (Glasgow Outcome Scale scores of 1 or 2). CONCLUSION: Endovascular treatment of dural arteriovenous fistulae of the superior petrosal sinus can result in cure when access to the site of the fistula can be achieved. Preoperative embolization is a safe and effective adjunct to minimize bleeding during open neurosurgery.

Vein of Galen aneurysmal malformation: diagnosis and treatment of 13 children with extended clinical follow-up.

BACKGROUND AND PURPOSE: We herein present our experience in diagnosing and treating 13 children with vein of Galen aneurysmal malformations (VGAM), with an emphasis on possible prognostic indicators, endovascular strategies, factors affecting treatment during the neonatal period, and long-term follow-up. With this review, we hope to identify those factors that have the most significant prognostic value in determining long-term outcomes in children with VGAM. METHODS: We retrospectively reviewed the radiology studies, hospital charts, and outpatient clinic chart notes (when applicable) of 13 children evaluated and treated for VGAM at a single tertiary care pediatric hospital. Clinical presentation, diagnostic methods, treatment strategies, and outcome were documented for each child. The present neurologic status and level of function of each patient was determined by review of the outpatient charts and direct contact with the clinicians who were conducting the follow-up. Outcome was graded on a 5-point scale, ranging from 0 (death) to 4 (normal), taking into account only neurologic and developmental characteristics. RESULTS: Eight of 13 patients presented as neonates with congestive heart failure. The other five patients ranged in age from 4 months to 13 years at the time of presentation. The five patients presenting outside of the neonatal period achieved normal or near-normal outcomes. Two of the eight patients presenting during the neonatal period achieved normal or near-normal outcomes, one experienced significant impairment, and the other five died. We were unable to identify significant differences in outcome on the basis of differences in treatment strategies. CONCLUSION: Our experience confirms that children with VGAM presenting during the neonatal period have a generally much worse prognosis than do those presenting later in childhood. Complicating factors in the management and treatment of these children are discussed in light of their impact on outcome.

Clinical course of cranial dural arteriovenous fistulas with long-term persistent cortical venous reflux.

BACKGROUND AND PURPOSE: The natural history of aggressive (Borden 2 and 3) cranial dural arteriovenous fistulas (DAVFs) is not well described. Reported annual mortality and hemorrhage rates vary widely and range up to 20% per year. A consecutive single-center cohort of 236 cases that presented with a cranial DAVF between June 1984 and May 2001 was reviewed for the consequences of long-term persistent cortical venous reflux (CVR). METHODS: A group of 118 cranial DAVFs was selected for the presence of CVR. All patients were offered treatment aimed at the disconnection of the CVR. Patients who declined or had partial treatment with persistence of the CVR had long-term clinical and angiographic follow-up to study the disease course of this select group. RESULTS: Treatment was instituted in 101 of the 118 patients (85.6%). Three patients were lost to follow-up. The remaining 14 nontreated patients (11.9%) and the partially treated patients (n=6) were assessed clinically and angiographically over time. The mean follow-up in this select group was 4.3 years (86.9 patient-years). During follow-up, 7 patients suffered an intracranial hemorrhage (35%). The incidence of nonhemorrhagic neurological deficit was 30%. Nine patients (45%) died: 6 patients expired after a hemorrhage, and 3 patients died of progressive neurological deterioration. Two patients demonstrated a spontaneous closure of the DAVF (10%). CONCLUSIONS: Persistence of the CVR in cranial DAVFs yields an annual mortality rate of 10.4%. Excluding events at presentation, in this series the annual risk for hemorrhage or nonhemorrhagic neurological deficit during follow-up was 8.1% and 6.9%, respectively, resulting in an annual event rate of 15.0%.