Tag: natural history

Posted on

Spinal juvenile (Type III) extradural-intradural arteriovenous malformations

Spinal extradural-intradural AVMs

 J Neurosurg Spine 20:452–458, 2014

Owing to their rarity, demographics, natural history, and treatment, results for spinal juvenile (Type III) extradural-intradural arteriovenous malformations (AVMs) are frequently only provided in case report format.

Methods. A pooled analysis was performed utilizing the PubMed database through April 2013. Individualized patient data were extracted to elucidate demographics, hemorrhage risk, and treatment result information.

Results. Twenty-nine studies describing 51 patients were included. The mean age at presentation was 15.0 ± 10.5 years with a slight male predilection (63%, 1.7:1 sex ratio). Presentation modality included progressive deficits in 35%, hemorrhage in 31%, acute deficits not attributed to hemorrhage in 22%, and asymptomatic/incidental in 12% of patients. The annual hemorrhage rate was 2.1%; statistically significant risk factors for hemorrhage included presentation age (HR 0.39 [95% CI 0.18–0.87]) and associated aneurysms (HR 8.74 [95% CI 1.76–43.31]). Seventy-seven percent of patients underwent treatment; after a mean follow-up of 2.6 ± 3.2 years, 73% were improved, 10% were the same, and 17% were worse neurologically. Of 25 cases with described angiographic results, 8 lesions were obliterated (32%). Of these 25 patients, 8 had AVMs with associated aneurysms, and the aneurysm was obliterated in all 8 patients. Over the course of 57.9 patient-years of follow-up, including 55.3 patient-years for partially treated AVMs, no hemorrhages were described, reflecting a trend toward protection from hemorrhage after treatment (p = 0.12, likelihood ratio test).

Conclusions. Spinal juvenile (Type III) extradural-intradural AVMs commonly present symptomatically. Associated arterial aneurysms increase their hemorrhage risk, and protection from hemorrhage may be achieved from partial obliteration of these lesions, particularly if targeted toward associated aneurysms.

Posted on

Management of subdural hygromas associated with arachnoid cysts

Subdural hygromas associated with cysts

J Neurosurg Pediatrics 12:434–443, 2013

Arachnoid cysts may occasionally be associated with subdural hygromas. The management of these concurrent findings is controversial.

Methods. The authors reviewed their experience with arachnoid cysts and identified 8 patients with intracranial arachnoid cysts and an associated subdural hygroma. The medical records and images for these patients were also examined.

Results. In total, 8 patients presented with concurrent subdural hygroma and arachnoid cyst. Of these 8 patients, 6 presented with headaches and 4 had nausea and vomiting. Six patients had a history of trauma. One patient was treated surgically at the time of initial presentation, and 7 patients were managed without surgery. All patients experienced complete resolution of their presenting signs and symptoms.

Conclusions. Subdural hygroma may lead to symptomatic presentation for otherwise asymptomatic arachnoid cysts. The natural course of cyst-associated subdural hygromas, even when symptomatic, is generally benign, and symptom resolution can be expected in most cases. The authors suggest that symptomatic hygroma is not an absolute indication for surgical treatment and that expectant management can result in good outcomes in many cases.

Posted on

Spinal Pial (Type IV) Arteriovenous Fistulae

Spinal Pial (Type IV) Arteriovenous Fistulae

Neurosurgery 73:141–151, 2013

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.

Posted on

Natural history of DAVFs without cortical venous drainage

J Neurosurg 117:539–545, 2012

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.

Posted on

Hemorrhage From Arteriovenous Malformations During Pregnancy

Neurosurgery 71:349–356, 2012 DOI: 10.1227/NEU.0b013e318256c34b

Previous hemorrhage, deep venous drainage, and deep location are established risk factors for arteriovenous malformation (AVM) hemorrhage. Although pregnancy is an assumed risk factor, there is a relative paucity of data to support this neurosurgical tenet.

OBJECTIVE: To elucidate the hemorrhage rate of AVMs during pregnancy.

METHODS: We reviewed the records of 54 women with an angiographic diagnosis of an AVM at our institution. Annual hemorrhage rates were calculated as the ratio of the number of bleeds to total number of patient-years of follow-up. Patient-years of followup were tallied assuming lesion presence from birth until AVM obliteration. The Cox proportional hazards model for hemorrhage with pregnancy as the time-dependent variable was used to calculate the hazard ratio.

RESULTS: Five hemorrhages in 4 patients occurred over 62 pregnancies, yielding a hemorrhage rate of 8.1% per pregnancy or 10.8% per year. Over the remaining 2461.3 patient-years of follow-up, only 28 hemorrhages occurred, yielding an annual hemorrhage rate of 1.1%. The hazard ratio for hemorrhage during pregnancy was 7.91 (P = 2.23 x 10-4), increasing to 18.12 (P = 7.31 x 10-5) when limiting the analysis to patient follow-up up to age 40.

CONCLUSION: Because of the increased risk of hemorrhage from AVMs during pregnancy, we recommend intervention in women who desire to bear children, particularly if the AVM has bled. If the AVM is discovered during pregnancy, we recommend early intervention if it has ruptured; if it is unruptured, we recommend comprehensive counseling, weighing risks of intervention against continuation of pregnancy without intervention.

 

Posted on

The Natural History of Cranial Dural Arteriovenous Fistulae With Cortical Venous Reflux—The Significance of Venous Ectasia

Neurosurgery 70:312–319, 2012 DOI: 10.1227/NEU.0b013e318230966f

The quoted risk of hemorrhage from dural arteriovenous fistulae with cortical venous reflux varies widely, and the influence of angiographic grade on clinical course has not previously been reported.

OBJECTIVE: To assess the risk of hemorrhage and the influence of angiographic grade on this risk, compared with known predictors of hemorrhage such as presentation.

METHODS: Seventy-five fistulae with cortical venous reflux identified in our arteriovenous malformations clinic between 1992 and 2007 were followed up clinically, and their angiograms were reviewed.

RESULTS: There were 8 hemorrhages in 90 years of follow-up. The annual incidence of hemorrhage before any treatment was 13%, and 4.7% after partial treatment, giving an overall incidence of 8.9% before definitive treatment. Borden and Cognard grades were poor discriminators of risk for lesions with the exception of Cognard type IV lesions. These lesions, characterized by venous ectasia, had a 7-fold increase in the incidence of hemorrhage (3.5% no ectasia vs 27% with ectasia). Patients presenting with hemorrhage (20%) or nonhemorrhagic neurological deficit (22%) had a higher incidence of hemorrhage than those with a benign presentation (4.3%), but this may be directly linked to the presence of venous ectasia.

CONCLUSION: In this series untreated dural arteriovenous fistulae with cortical venous reflux had a 13% annual incidence of hemorrhage after diagnosis. There was a significant difference between those with and without venous ectasia. This should be confirmed by further studies, but probably defines a high-risk subgroup of patients that requires rapid intervention.

Posted on

Natural history of Chiari malformation Type I

J Neurosurg Pediatrics 8:214–221, 2011.  DOI: 10.3171/2011.5.PEDS1122

The natural history of the Chiari malformation Type I (CM-I) is incompletely understood. The authors report on the outcome of a large group of patients with CM-I that were initially selected for nonsurgical management.

Methods. The authors identified 147 patients in whom CM-I was diagnosed on MR imaging, who were not offered surgery at the time of diagnosis, and in whom at least 1 year of clinical and MR imaging follow-up was available after the initial CM-I diagnosis. These patients were included in an outcome analysis.

Results. Patients were followed clinically and by MR imaging for a mean duration of 4.6 and 3.8 years, respectively. Of the 147 patients, 9 had new symptoms attributed to the CM-I during the follow-up interval. During this time, development of a spinal cord syrinx occurred in 8 patients; 5 of these patients had a prior diagnosis of a presyrinx state or a dilated central canal. Spontaneous resolution of a syrinx occurred in 3 patients. Multiple CSF flow studies were obtained in 74 patients. Of these patients, 23 had improvement in CSF flow, 39 had no change, and 12 showed worsening CSF flow at the foramen magnum. There was no significant change in the mean amount of cerebellar tonsillar herniation over the follow-up period. Fourteen patients underwent surgical treatment for CM-I. There were no differences in initial cerebellar tonsillar herniation or CSF flow at the foramen magnum in those who ultimately underwent surgery compared with those who did not.

Conclusions. In patients with CM-Is that are selected for nonsurgical management, the natural history is usually benign, although spontaneous improvement and worsening are occasionally seen.

Posted on

The natural history of intracranial cavernous malformations

Neurosurg Focus 30 (6):E24, 2011, DOI: 10.3171/2011.3.FOCUS1165

Literature reports on the natural history of cerebral cavernous malformations (CMs) are numerous, with considerable variability in lesion epidemiology, hemorrhage rates, and risk factors for hemorrhage.

In this review, the authors performed a meta-analysis of 11 natural history studies. The overall male-to-female ratio was 1:1, and the mean age at presentation was 30.6 years. Overall, 37% of patients presented with seizures, 36% with hemorrhage, 23% with headaches, 22% with focal neurological deficits, and 10% were asymptomatic. Some patients had more than one symptom. Seizure presentation was most prevalent among supratentorial CMs, while focal neurological deficits were common in patients with infratentorial CMs. By location, CMs were in the cerebral hemispheres (66%), brainstem (18%), basal ganglia or thalamus (8%), cerebellum (6%), and other (2.5% [combined supra- and infratentorial, callosal or insular]). Overall, 19% of patients harbored multiple intracranial CMs, and 9% had radiographically apparent associated developmental venous anomalies. An overall annual hemorrhage rate of 2.4% per patient-year (range 1.6%–3.1%) was identified across 3 studies.

Prior hemorrhage and female sex were risk factors for bleeding, while CM size and multiplicity did not affect hemorrhage rates. Although not impacting the hemorrhage rate itself, deep location was a risk factor for increased clinical aggressiveness.

Posted on

The natural history of intracranial meningiomas

J Neurosurg 114:1250–1256, 2011. DOI: 10.3171/2010.12.JNS101623

Despite the increased detection of incidental or small meningiomas, the lesion’s natural history is largely unknown.

Methods. One year or longer of follow-up was conducted in 244 patients with 273 meningiomas managed conservatively by a single surgeon between 2003 and 2008. Data were stratified according to age, sex, tumor location, symptoms, initial tumor diameter, calcification, MR imaging intensity, and edema. Linear tumor growth was defined as a 2-mm or larger increase in the maximum diameter in any direction of the tumor. Volumetric analysis (ImageJ version 1.43) was also conducted in 154 of 273 meningiomas for which complete radiological data were available in the form of DICOM files throughout the follow-up period. A volume increase greater than 8.2% was regarded as significant because the preliminary volumetry based on 20 randomly selected meningiomas showed that the average SD was 4.1%.

Results. Linear growth was observed in 120 tumors (44.0%) with a mean follow-up of 3.8 years. Factors related to tumor growth were age of 60 or younger (p = 0.0004), absence of calcification (p = 0.027), MR imaging T2 signal hyperintensity (p = 0.021), and edema (p = 0.018). Kaplan-Meier analysis and Cox proportional hazards regression analysis revealed that age 60 or younger (hazard ratio [HR] 1.54, 95% CI 1.05–2.30, p = 0.026), initial tumor diameter greater than 25 mm (HR 2.23, 95% CI 1.44–3.38, p = 0.0004), and the absence of calcification (HR 4.57, 95% CI 2.69–8.20, p < 0.0001) were factors associated with a short time to progression. Volumetric growth was seen in 74.0% of the cases. Factors associated with a higher annual growth rate were male sex (p = 0.0002), initial tumor diameter greater than 25 mm (p < 0.0001), MR imaging T2 signal hyperintensity (p = 0.0001), presence of symptoms (p = 0.037), and edema (p < 0.0001).

Conclusions. Although the authors could obtain variable results depending on the measurement method, the data demonstrate patients younger than 60 years of age and those with meningiomas characterized by hyperintensity on T2-weighted MR imaging, no calcification, diameter greater than 25 mm, and edema need to be observed more closely. Volumetry was more sensitive to detecting tumor growth than measuring the linear diameter.

Proudly powered by WordPress