Carotid Artery Tortuosity Is Associated with Connective Tissue Diseases.

BACKGROUND AND PURPOSE: There is a general assumption in the cerebrovascular literature that there is an association between carotid artery tortuosity and connective tissues disease; however, this has not been firmly established. The purpose of this study was to determine the prevalence of carotid artery tortuosity in patients with connective tissue diseases relative to matched controls. MATERIALS AND METHODS: Patients with previous CTA or MRA and a diagnosis of connective tissue diseases were identified and compared with a cohort of age-matched controls. Radiologists blinded to the diagnosis reviewed the images and evaluated the presence of carotid artery tortuosity (including loops, kinks, or coils). Continuous variables were compared using the Student t test, and categoric variables with χ2 tests. RESULTS: One hundred forty-three patients with connective tissue disease and 143 controls were included in this study. Specific diagnoses included Marfan (n = 33), nonvascular Ehlers-Danlos (n = 36), Ehlers-Danlos vascular-type (n = 32), neurofibromatosis type 1 (n = 26), and Loeys-Dietz (n = 16) syndromes. The presence of carotid tortuosity was 44% in connective tissue disease and 16% in controls (P < .001). Of tortuosity manifestations, coils were most prevalent (23% versus 3%; P < .001). Among the various connective tissue diseases, the rates of any carotid tortuosity were 88% for Marfan syndrome, 63% for Loeys-Dietz syndrome, 42% for neurofibromatosis type 1, and 19% for both vascular- and nonvascular-type Ehlers-Danlos syndrome. The positive predictive value of the combination of aortic aneurysm and carotid tortuosity being associated with connective tissue disease was 95.4%. The specificity was 98.6%. CONCLUSIONS: Carotid artery tortuosity is highly associated with connective tissue diseases, particularly Marfan syndrome, Loeys-Dietz syndrome, and neurofibromatosis type 1. Such findings are relevant in risk assessment for vascular complications in connective tissue disease, endovascular treatment planning, and in understanding the pathomechanisms of vascular tortuosity in general.

Clinical and Imaging Characteristics of Diffuse Intracranial Dolichoectasia.

BACKGROUND AND PURPOSE: Among patients with vertebrobasilar dolichoectasia is a subset of patients with disease affecting the anterior circulation as well. We hypothesized that multivessel intracranial dolichoectasia may represent a distinct phenotype from single-territory vertebrobasilar dolichoectasia. The purpose of this study was to characterize clinical characteristics and angiographic features of this proposed distinct phenotype termed "diffuse intracranial dolichoectasia" and compare them with those in patients with isolated vertebrobasilar dolichoectasia. MATERIALS AND METHODS: We retrospectively reviewed a consecutive series of patients with diffuse intracranial dolichoectasia and compared their demographics, vascular risk factors, additional aneurysm prevalence, and clinical outcomes with a group of patients with vertebrobasilar dolichoectasia. "Diffuse intracranial dolichoectasia" was defined as aneurysmal dilation of entire vascular segments involving ≥2 intracranial vascular beds. Categoric and continuous variables were compared by using χ2 and Student t tests, respectively. RESULTS: Twenty-five patients had diffuse intracranial dolichoectasia, and 139 had vertebrobasilar dolichoectasia. Patients with diffuse intracranial dolichoectasia were older than those with vertebrobasilar dolichoectasia (70.9 ± 14.2 years versus 60.4 ± 12.5 years, P = .0002) and had a higher prevalence of abdominal aortic aneurysms (62.5% versus 14.3%, P = .01), other visceral aneurysms (25.0% versus 0%, P < .0001), and smoking (68.0% versus 15.9%, P < .0001). Patients with diffuse intracranial dolichoectasia were more likely to have aneurysm growth (46.2% versus 21.5%, P = .09) and rupture (20% versus 3.5%, P = .007) at follow-up. Patients with diffuse intracranial dolichoectasia were less likely to have good neurologic function at follow-up (24.0% versus 57.6%, P = .004) and were more likely to have aneurysm-related death (24.0% versus 7.2%, P = .02). CONCLUSIONS: The natural history of patients with diffuse intracranial dolichoectasia is significantly worse than that in those with isolated vertebrobasilar dolichoectasia. Many patients with diffuse intracranial dolichoectasia had additional saccular and abdominal aortic aneurysms. These findings suggest that diffuse intracranial dolichoectasia may be a distinct vascular phenotype secondary to a systemic arteriopathy affecting multiple vascular beds.

Clinical Outcomes of Patients with Delayed Diagnosis of Spinal Dural Arteriovenous Fistulas.

BACKGROUND AND PURPOSE: Spinal dural arteriovenous fistulas are commonly missed on imaging or misdiagnosed as inflammatory or neoplastic processes. We reviewed a consecutive series of spinal dural arteriovenous fistulas referred to our institution that were missed or misdiagnosed on initial imaging and studied the clinical consequences of missing or misdiagnosing the lesion. MATERIALS AND METHODS: We reviewed spinal dural arteriovenous fistulas diagnosed at our institution between January 1, 2000, and November 1, 2014. A lesion was defined as "misdiagnosed" if initial MR imaging or CT myelography demonstrated characteristic imaging features of spinal dural arteriovenous fistula but the patient was clinically or radiologically misdiagnosed. Outcomes included length of delay of diagnosis, increased disability (increase in mRS or Aminoff motor disability of ≥1 point) between initial imaging evaluation and diagnosis date, and posttreatment disability. RESULTS: Fifty-three consecutive spinal dural arteriovenous fistulas that were initially misdiagnosed despite having characteristic imaging findings on MR imaging or CT myelography were included in our study. Eight patients (18.9%) underwent spinal angiography before referral, which was interpreted as having negative findings but was either incomplete (6 cases) or retrospectively demonstrated the spinal dural arteriovenous fistulas (2 cases). The median time of delayed diagnosis was 6 months (interquartile range, 2-14 months). Fifty-one patients (96.2%) had increased disability between the initial study, which demonstrated features of a spinal dural arteriovenous fistula, and diagnosis. Thirty-two patients (60.4%) developed a new requirement for a walker or wheelchair. Following treatment, 21 patients (41.2%) had an improvement of 1 point on the mRS or Aminoff motor disability scale. CONCLUSIONS: Delayed diagnosis of spinal dural arteriovenous fistula with characteristic imaging features results in high rates of additional disability that are often irreversible despite surgical or endovascular treatment of the fistula.

Mortality in cerebral venous thrombosis: results from the national inpatient sample database.

BACKGROUND: Outcomes of cerebral venous thrombosis (CVT) vary from full recovery to death. Few studies have been performed examining epidemiologic and medical risk factors associated with high mortality in CVT. In this study, we examined the National Inpatient Sample (NIS) to determine the epidemiologic and medical risk factors associated with increased mortality from CVT. MATERIALS AND METHODS: Using the NIS from 2001 to 2008, patients who suffered from CVT were identified using the ICD-9 codes 437.6 (nonpyogenic thrombosis of intracranial venous sinus), 325 (phlebitis and thrombophlebitis of intracranial venous sinuses) and 671.5 (peripartum phlebitis and thrombosis, cerebral venous thrombosis, thrombosis of intracranial venous sinus). We analyzed the associations of demographic factors, risk factors, comorbidities, complications of CVT, and therapeutic interventions with in-hospital mortality. We performed a multivariate logistic regression analysis to determine which variables were independently associated with in-hospital mortality. RESULTS: 11,400 patients were hospitalized with CVT between 2001 and 2008. Two-hundred and thirty-two (2.0%) suffered in-hospital mortality. Patients 15-49 years old had the lowest mortality rate (1.5%) compared with 2.8% for patients aged 50-64 (p < 0.001) and 6.1% for patients ≥65 years old (p < 0.001). The most common condition associated with CVT was pregnancy/puerperium (24.6%), and these women had a low mortality rate (0.4%). On multivariate analysis, the comorbidity most strongly associated with increased risk of mortality was sepsis (mortality rate 15.6%, OR = 7.5, 95% CI = 4.79-11.53, p < 0.001). Malignancy, underlying autoimmune disease and substance abuse were also independently associated with mortality, but with lower mortality rates (<5%). Complications associated with increased risk of mortality included paralysis (8.0%, OR = 3.4, 95% CI = 3.17-6.96, p < 0.001), intracranial hemorrhage (8.7%, OR = 5.4, 95% CI = 4.38-7.96, p < 0.001), and hydrocephalus (15.0%, OR = 3.2, 95% CI = 5.54-15.11, p = 0.004). Demographic variables associated with decreased mortality on multivariate analysis were male gender (2.1%, OR = 0.62, 95% CI = 0.43-0.87, p = 0.006) and Asian/Pacific Islander race (OR = 0.00, 95% CI = 0-0.27, p < 001). CONCLUSIONS: CVT is associated with a low in-hospital mortality rate. Amongst patients suffering CVT, male gender and Asian/Pacific Islander race were independently associated with lower odds of in-hospital mortality when compared to their female and white counterparts, respectively. Septic patients with CVT have the greatest risk of in-hospital mortality. Hydrocephalus, intracranial hemorrhage, and motor deficits are also associated with higher risk of death. Our results build on previous evidence that serves to define a group of patients with CVT at high risk of early death.

Better outcomes with treatment by coiling relative to clipping of unruptured intracranial aneurysms in the United States, 2001-2008.

BACKGROUND AND PURPOSE: Endovascular therapy has increasingly become an acceptable option for treatment of unruptured aneurysms. To better understand the recent trends in the use of and outcomes related to coiling compared with clipping for unruptured aneurysms in the United States, we evaluated the NIS. MATERIALS AND METHODS: Hospitalizations for clipping or coiling of unruptured cerebral aneurysms from 2001 to 2008 were identified by cross-matching ICD codes for the diagnosis of unruptured aneurysm (437.3) with procedural codes for clipping (39.51) or coiling (39.52, 39.79, or 39.72) of cerebral aneurysms and excluding all patients with a diagnosis of subarachnoid hemorrhage (430) and intracerebral hemorrhage (431). Mortality and discharge to a long-term facility were evaluated for both clipping and coiling patient populations. RESULTS: The fraction of unruptured aneurysms treated with coiling increased from 20% in 2001 to 63% in 2008. For surgical clipping, the percentage of patients discharged to long-term facilities was 14.0% (4184/29,918) compared with 4.9% (1655/34,125) of coiled patients (P < .0001). Clipped patients also had a higher mortality rate because 344 (1.2%) clipped patients died compared with 215 (0.6%) coiled patients (P < .0001). Between 2001 and 2008, the overall number of adverse outcomes from treatment had decreased from 14.8% to 7.6%. CONCLUSIONS: The use of endovascular coiling relative to surgical clipping of unruptured intracranial aneurysms is associated with decreasing periprocedural morbidity and mortality among patients treated in the United States from 2001 to 2008.