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Editorial: Small incidentally found aneurysms

Robert A. Solomon

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Introduction: Management of ischemic cerebrovascular disease

Giuseppe Lanzino and Robert D. Brown Jr.

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Medulla oblongata edema associated with neurogenic pulmonary edema

Case report

Robert H. Brown Jr., Brian D. Beyerl, Richard Iseke, and Michael H. Lavyne

✓ Neurogenic pulmonary edema (NPE) occurs in association with central nervous system disease without underlying cardiopulmonary problems. It is characterized by profound pulmonary vascular congestion and a fulminant clinical course. Although several reports document a role for experimental brain-stem lesions in the production of NPE, there have been only two studies in man correlating specific brain-stem lesions with NPE. The authors report a case of NPE occurring in a patient with von Hippel-Lindau disease and a dorsal medullary syrinx with postoperative dorsal medullary edema. The anatomical location of this patient's lesion is reviewed in the context of alternative theories of the pathogenesis of NPE.

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Small (< 10-mm) incidentally found intracranial aneurysms, Part 1: reasons for detection, demographics, location, and risk factors in 212 consecutive patients

Ioannis Loumiotis, Anne Wagenbach, Robert D. Brown Jr., and Giuseppe Lanzino

Object

The widespread use of imaging techniques for evaluating nonspecific symptoms (vertigo, dizziness, memory concerns, unsteadiness, and the like) and focal neurological symptoms related to cerebrovascular disease has led to increased identification of asymptomatic incidentally discovered unruptured intracranial aneurysms (UIAs). The management of these incidental aneurysms is controversial and many factors need to be considered. The authors describe reasons leading to diagnosis, demographics, and risk factors in a large consecutive series of patients with small incidentally found UIAs.

Methods

The authors prospectively evaluated 335 patients harboring 478 small (< 10-mm) UIAs between January 2008 and May 2011. Patients with known aneurysms, possibly symptomatic aneurysms, arteriovenous malformation–related aneurysms, patients with a history of subarachnoid hemorrhage from another aneurysm, and patients harboring extradural aneurysms were excluded from the analysis. Only truly incidental small aneurysms (272 aneurysms in 212 patients) were considered for the present analysis. Data regarding the reason for detection, demographics, location, and presence of potential risk factors for aneurysm formation were prospectively collected.

Results

There were 158 female (74.5%) and 54 male (25.5%) patients whose mean age was 60.6 years (median 62 years). The most common reason for undergoing the imaging study that led to a diagnosis of the aneurysms was evaluation for nonspecific spells and symptoms related to focal cerebrovascular ischemia (43.4%), known/possible intracranial or neck pathology (24%), and headache (16%). The most common location (27%) of the aneurysm was the middle cerebral artery; the second most common (22%) was the paraclinoid internal carotid artery (excluding cavernous sinus aneurysms). Sixty-nine percent of patients were current or prior smokers, 60% had a diagnosis of hypertension, and 23% had one or more relatives with a history of intracranial aneurysms with or without subarachnoid hemorrhage.

Conclusions

Small incidental UIAs are more commonly diagnosed in elderly individuals during imaging performed to investigate ill-defined spells or focal cerebrovascular ischemic symptoms, or during the evaluation of known or probable unrelated intracranial/neck pathology. Hypertension, smoking, and family history of aneurysms are common in this patient population, and the presence of these risk factors has important implications for treatment recommendations. Although paraclinoid aneurysms (excluding intracavernous aneurysms) are uncommon in patients with ruptured intracranial aneurysms, this location is very common in patients with small incidental UIAs.

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Intracranial dural arteriovenous fistulae: angiographic predictors of intracranial hemorrhage and clinical outcome in nonsurgical patients

Robert D. Brown Jr., David O. Wiebers, and Douglas A. Nichols

✓ This long-term follow-up study of 54 patients clarifies the angiographic predictors of intracranial hemorrhage (ICH) and clinical outcome in individuals with unoperated intracranial dural arteriovenous fistulae (AVF's). All of these patients were examined at the Mayo Clinic between 1976 and 1989, and all available cerebral arteriograms were reviewed by a neuroradiologist. Follow-up information was obtained for 52 patients (96%) until death or treatment intervention, or for at least 1 year after diagnosis, with a mean follow-up period of 6.6 years.

Throughout this 6.6-year follow-up period, ICH related to dural AVF occurred in five of the 52 patients, for a crude risk of hemorrhage of 1.6% per year. The risk of hemorrhage at the time of mean follow-up examination was 1.8% per year. Angiographic examination revealed several characteristics that were considered potential predictors of ICH during the follow-up period. Lesions of the petrosal sinus and straight sinus had a higher propensity to bleed, although the small numbers in the series precluded a definite conclusion. A person suffering from a dural AVF with a venous varix on a draining vein had an increased risk of hemorrhage, whereas no hemorrhage was seen in the 20 patients without a varix (p < 0.05). Lesions draining into leptomeningeal veins had an increased occurrence of hemorrhage, although this increased risk was not statistically significant. Patients' initial symptoms were compared to those at follow-up evaluation. Pulsatile tinnitus improved in more than half of the 52 patients, and resolved in 75% of those showing some improvement. Individuals without a sinus or venous outflow occlusion at initial cerebral angiography were more likely to improve or remain stable (89%), whereas patients with an occlusion showed infrequent improvement (11%; p < 0.05).

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Unruptured intracranial aneurysms and arteriovenous malformations: frequency of intracranial hemorrhage and relationship of lesions

Robert D. Brown Jr., David O. Wiebers, and Glenn S. Forbes

✓ Among 91 patients with unruptured intracranial arteriovenous malformations (AVM's), 16 patients had 26 unruptured intracranial saccular aneurysms. An actuarial analysis showed the risk of intracranial hemorrhage among patients with coexisting aneurysm and AVM to be 7% per year at 5 years following diagnosis compared to 1.7% for patients with AVM alone. The difference in length of survival free of hemorrhage was significant (log-rank, p < 0.0007). Several angiographic and clinical parameters were investigated to better understand the relationship of these lesions. The aneurysms occurred in similar percentages in patients with small, medium, and large AVM's. Twenty-five aneurysms were on arteries feeding the malformation system, almost equally distributed proximally and distally. Eleven aneurysms were atypical in location, and all arose from primary or secondary branch feeders to the malformation; 24 were on enlarged feeding arteries. Eleven (16%) of the 67 patients with high-flow AVM's had associated aneurysms, compared with five (21%) of the 24 patients with low-flow AVM's. Four (16%) of 25 low-shunt malformations and 12 (18%) of 65 high-shunt malformations had associated aneurysms. All five aneurysms associated with low-shunt malformations were on a direct arterial feeder of the malformation. These data suggest that the intracranial AVM's predispose to aneurysm formation within AVM feeding systems and that the mechanism is not simply based upon the high blood flow or high arteriovenous shunt in these systems.

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Contemporary cohort of cerebral cavernous malformations: natural history and utility of follow-up MRI

Kelly D. Flemming, Robert D. Brown Jr., and Giuseppe Lanzino

OBJECTIVE

This study reports the natural history of cerebral cavernous malformations (CCMs) in a contemporary cohort with prospectively collected data from multiple sources, access to follow-up imaging, integrated electronic medical records, and detailed imaging review by study investigators. The authors aimed to define the first prospective symptomatic hemorrhage (SH) and severe SH rates, determine the risk of a second prospective SH, and identify risk factors for SH.

METHODS

From a prospectively maintained database of adult patients with radiologically defined CCM, those with radiation-induced CCM, those who underwent surgery within 3 months postdiagnosis, and those with < 1 year of follow-up were excluded. The patients’ medical history and radiological features of the CCM were recorded at the time of diagnosis. Follow-up annual written surveys were completed for 5 years after the initial diagnosis and then semiannually thereafter in addition to medical record and follow-up imaging review. Outcomes of interest included SH and severe SH.

RESULTS

Of 315 patients, 58.7% were female and 19.7% had familial CCMs. At diagnosis, 37.1% of patients had ruptured CCMs and 28.9% of the CCMs were located in the brainstem. The 5-year cumulative rates of prospective SH and severe SH in those with ruptured CCMs at diagnosis were 41.2% and 12.8%, respectively, compared with 6.1% and 2.5% in patients with unruptured CCMs at diagnosis (p < 0.0001). Risk factors for prospective SH included a ruptured CCM at diagnosis and persistent or new hyperintensity on T1-weighted MRI performed > 3 months after baseline MRI. For those with a ruptured CCM at diagnosis, the risk of developing a second prospective SH was similar to that of developing a first SH.

CONCLUSIONS

In a contemporary cohort of adult patients with CCM, the authors report 5-year SH and severe SH rates, rates of second prospective hemorrhage, and predictors of SH. Persistent or new hyperintensity on T1-weighted MRI may be a useful marker of disease activity.

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Editorial: Cavernous malformations

E. Sander Connolly Jr.

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Editorial: International Study of Unruptured Intracranial Aneurysms

E. Sander Connolly Jr.

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Endovascular treatment of ruptured posterior circulation aneurysms using electrolytically detachable coils

Douglas A. Nichols, Robert D. Brown Jr., Kent R. Thielen, Fredric B. Meyer, John L. D. Atkinson, and David G. Piepgras

✓ The authors report their experience using electrolytically detachable coils for the treatment of ruptured posterior circulation aneurysms. Twenty-six patients with 28 posterior circulation aneurysms were treated. All patients were referred for endovascular treatment by experienced vascular neurosurgeons. Patients underwent follow-up angiography immediately after treatment, 1 to 6 weeks posttreatment, and 6 months posttreatment. Six-month follow-up angiograms obtained in 19 patients with 20 aneurysms demonstrated that 18 (90%) of the 20 aneurysms were 99 to 100% occluded, one aneurysm (5%) was approximately 90% occluded, and one aneurysm (5%) was approximately 75% occluded. The patient with the aneurysm that was approximately 75% occluded needed additional treatment, consisting of parent artery balloon occlusion, and was considered a treatment failure (3.8% of patients). There was one treatment-associated mortality (3.8%) but no treatment-associated serious neurological or nonneurological morbidity in the patient group. There was no recurrent aneurysm rupture during treatment or during the mean 27-month follow-up period.

Endovascular treatment of ruptured posterior circulation aneurysms with electrolytically detachable coils can be accomplished with low morbidity and mortality rates. The primary goal of treatment—preventing recurrent aneurysm—can be achieved over the short term.

Endovascular coil occlusion will play an important role in the treatment of ruptured posterior circulation aneurysms, particularly if long-term efficacy in preventing recurrent aneurysm hemorrhage can be documented.