Randall T. Higashida

Intracranial Aneurysms in Childhood: 27-Year Single-Institution Experience

BACKGROUND AND PURPOSE: Pediatric aneurysms are rare and, thus, relatively poorly understood as compared to those in adults. Our aim was to characterize the clinical, imaging, treatment, and outcome data of patients younger than 19 years diagnosed with intracranial aneurysms at a tertiary care institution. MATERIALS AND METHODS: We performed a retrospective medical record review of pediatric patients examined at our university hospital between 1981 and 2008. RESULTS: We evaluated 77 patients (mean age, 12 years; 40 female, 37 male) with 103 intracranial aneurysms. Patients presented with headache (45%), cranial neuropathies (16%), nausea/vomiting (15%), vision changes (13%), trauma (13%), seizure (4%), or sensory changes (3%). Subarachnoid hemorrhage occurred in 25 patients. Thirty-one fusiform aneurysms occurred in 25 patients. Forty-seven saccular aneurysms occurred in 35 patients. Twelve infectious aneurysms occurred in 6 patients. Fifteen traumatic aneurysms occurred in 12 patients. Fifty-nine patients underwent treatment of their aneurysms; 18 patients’ conditions were managed conservatively. Nineteen patients underwent primary endovascular coiling, 1 patient had endovascular stent-assisted coiling, 11 patients underwent endovascular parent artery occlusion, 19 patients underwent surgical clipping, and 10 patients had aneurysms trapped and bypassed. Mortality was 1.3%. Morbidity included 8% infarction and 4% new-onset seizures. Six patients developed new aneurysms or had enlargement of untreated aneurysms. CONCLUSIONS: In our experience, intracranial aneurysms of childhood show a female predilection and predominantly saccular morphology. In neurovascular centers where microneurosurgical and endovascular options are available, most children with intracranial aneurysms can be successfully treated with low morbidity and mortality. Fusiform aneurysms require a combined microneurosurgical and endovascular approach more often than saccular aneurysms. The development of new aneurysms in pediatric patients during limited follow-up warrants further investigation.

Endovascular Treatment of Medically Refractory Cerebral Vasospasm Following Aneurysmal Subarachnoid Hemorrhage

BACKGROUND AND PURPOSE: CV following aneurysmal SAH is a significant cause of morbidity and mortality. We review our experiences using PTA and IA verapamil infusion for treating medically refractory cases. MATERIALS AND METHODS: We performed a retrospective review of patients with SAH admitted from July 2003 to January 2008. RESULTS: Of 546 patients admitted within 72 hours of symptom onset, 231 patients (42%) developed symptomatic CV and 189 patients (35%) required endovascular therapy. A total of 346 endovascular sessions were performed consisting of 1 single angioplasty, 286 IA verapamil infusions, and 59 combined treatments. PTA was performed on 151 vessel segments, and IA verapamil was infused in 720 vessel segments. IA verapamil doses ranged from 2.0 to 30.0 mg per vessel segment and from 3.0 to 55.0 mg per treatment session. Repeat treatments were necessary in 102 patients (54%) for persistent, recurrent, or worsening CV. There were 6 treatment-related complications, of which 2 resulted in clinical worsening. No deaths were attributable to endovascular therapy. At follow-up, 115 patients (61%) had a good outcome and 55 patients (29%) had a poor outcome. Sixteen patients died from causes related to SAH, while 3 died from other medical complications. CONCLUSIONS: Endovascular treatments are an integral part of managing patients with medically refractory CV. In our experience, PTA and IA verapamil are safe, with a low complication rate, but further studies are required to determine appropriate patient selection and treatment efficacy.

Pediatric Intracranial Nongalenic Pial Arteriovenous Fistulas: Clinical Features, Angioarchitecture, and Outcomes

BACKGROUND AND PURPOSE: NGAVFs are rare vascular malformations usually presenting in infancy or childhood. We sought to identify clinical and angiographic predictors of clinical outcome for these lesions. MATERIALS AND METHODS: Retrospective review of a neurointerventional data base identified 386 pediatric patients with intracranial AVFs and AVMs, from which a cohort of 25 patients with NGAVF were selected for medical record and imaging analysis. RESULTS: NGAVFs constituted 7.3% of pediatric intracranial vascular lesions with a nondural arteriovenous shunt. Seven of 8 patients who presented in the first month of life had CHF and harbored large, complex fistulas with multiple sites of arteriovenous shunting. Single-hole fistulas predominated later in childhood and more frequently presented with seizures, hemorrhage, or focal neurologic deficits. More treatment procedures were performed in subjects presenting at ≤2 years of age compared with older children (median = 3 versus 2, P = .041), and in those harboring a multi-hole fistula versus those with a single-hole fistula (median = 3 versus 2, P = .003). Eighteen patients (72%) had complete posttreatment elimination of NGAVF shunting. Compared with patients presenting at >2 years of age, patients presenting in the first 2 years of life were more likely to have a multi-hole fistula (100% versus 25%, P = .0001) and to have a poor clinical outcome (54% versus 0%, P = .0052), defined as a pediatric mRS of ≥3. CONCLUSIONS: The morbidity of NGAVF appears higher than previously reported despite a somewhat higher rate of angiographic cure. Poor clinical outcome occurred primarily in patients with multi-hole NGAVFs presenting at ≤2 years of age.

Assessment of vasculature of meningiomas and the effects of embolization with intra-arterial MR perfusion imaging : A feasibility study

BACKGROUND AND PURPOSE: Embolization of meningiomas has emerged as a preoperative adjuvant therapy that has proved effective in mitigating blood loss during surgical resection. Arterial supply to these tumors is typically identified by diffuse areas of parenchymal staining after selective x-ray angiograms. We investigate the benefits that selective injection of MR contrast may have in identifying vascular territories and determining the effects of embolization therapy. MATERIALS AND METHODS: Selective intra-arterial (IA) injection of dilute MR contrast media was used to assess the vascular distribution territories of meningeal tumors before and after embolization therapy. Regions of the tumor that experienced loss of signal intensity after localized contrast injections into the external and common carotid as well as vertebral arteries were used to quantify the specific vessels volume of distribution. Assessments were made before and after embolization to reveal changes in the vascular supply of the tumor. MR findings were compared with radiographic evaluation of tumor vascular supply on the basis of conventional x-ray angiography. RESULTS: MR proved to be an excellent means to assess tissue fed by selected arteries and clearly demonstrated the treated and untreated portions of the neoplasm after therapy. In some instances, MR revealed postembolization residual enhancement of the tumor that was difficult to appreciate on x-ray angiograms. Very low contrast dose was necessary, which made repeated assessment during therapy practical. CONCLUSION: MR perfusion imaging with selective IA injection of dilute contrast can reveal the distribution territory of vessels. Changes in tumor vasculature could be detected after embolization, which reveal the volumetric fraction of the tumor affected by the therapy.

MR Imaging of Partially Thrombosed Cerebral Aneurysms: Characteristics and Evolution

Noninvasive imaging of partially thrombosed aneurysm is important because it delineates the true lumen and provides information about the walls. The authors examined 9 patients twice each, separated by 4–22 months, and found thrombus in all MR imaging sequences. Thrombus was better seen on T1-weighted images whereas differentiation between lumen and thrombus was better depicted on the steady-state sequence. Peripheral high T1 signal in the clot was typical and all clots remained stable during the time period between imaging studies. Thus, MR imaging may be used to evaluate aneurysm size, thrombosed portions, and growth and may affect treatment decisions in these patients. BACKGROUND AND PURPOSE: A comprehensive evaluation of aneurysmal morphometry requires appreciation of both the vascular lumen and the intraluminal thrombus. MR imaging methods can both evaluate the lumen and directly image the vessel wall. We investigated the ability of T1-weighted, T2-weighted, and steady-state MR imaging techniques to delineate thrombus morphology and reveal changes with time. MATERIALS AND METHODS: Nine patients with fusiform basilar or intracranial vertebral artery aneurysms that contained intraluminal thrombus were studied with MR imaging. All patients underwent at least 2 imaging sessions, which were separated by 4–22 months. Analysis of signal intensity to determine the mean signal intensity from thrombus, blood, CSF, and brain in matched regions was performed. Aneurysm maximal diameter and cross-sectional area were determined with and without thrombus. RESULTS: Thrombus was identified on all image sequences, and its general appearance was consistent between imaging sessions. Thrombus produced the highest and most consistent signal intensities with T1-weighted and steady-state techniques, though the latter showed superior contrast between luminal blood and thrombus. Heterogeneity within clot was evident in 4/9 of patients, with peripheral hyperintensity being a common feature. CONCLUSIONS: Steady-state imaging was found to be superior to T1- and T2-weighted imaging for delineating and characterizing intraluminal thrombus within aneurysms. The imaging characteristics of intraluminal thrombus proved to be very consistent for long periods. Assessment of overall aneurysm size, including thrombosed portions, permits more accurate evaluation of aneurysm growth and concomitantly may permit more informed clinical decision-making with regard to the timing and need for aneurysm treatment.

Pediatric Intracranial Aneurysms: New and Enlarging Aneurysms after Index Aneurysm Treatment or Observation

Although de novo intracranial aneurysms are very rare, their incidence is increased in children with other aneurysms. These authors sought to determine the factors that result in new or rapidly enlarging aneurysms in children. They reviewed 114 aneurysms not associated with other vascular malformations and found that 8.4% of children developed new or enlarging aneurysms. Nearly all of these patients had originally presented with fusiform aneurysms. Other features that lead to new or enlarging aneurysms included multiple aneurysms at presentation and immunosuppression. New aneurysms generally occurred 4 years after the initial one was diagnosed and at locations distal to the initial site. BACKGROUND AND PURPOSE: Children with brain aneurysms may be at higher risk than adults to develop new or enlarging aneurysms in a relatively short time. We sought to identify comorbidities and angiographic features in children that predict new aneurysm formation or enlargement of untreated aneurysms. MATERIALS AND METHODS: Retrospective analysis of the University of California–San Francisco Pediatric Aneurysm Cohort data base including medical records and imaging studies was performed. RESULTS: Of 83 patients harboring 114 intracranial aneurysms not associated with brain arteriovenous malformations or intracranial arteriovenous fistulas, 9 (8.4%) developed new or enlarging brain aneurysms an average of 4.2 years after initial presentation. Comorbidities that may be related to aneurysm formation were significantly higher in patients who developed new aneurysms (89%) as opposed to patients who did not develop new or enlarging aneurysms (41%; RR, 9.5; 95% CI, 1.9%–48%; P = .0099). Patients with multiple aneurysms at initial presentation were more likely than patients with a single aneurysm at presentation to develop a new or enlarging aneurysm (RR, 6.2; 95% CI, 2.1%–185; P = .0058). Patients who initially presented with at least 1 fusiform aneurysm were more likely to develop a new or enlarging aneurysm than patients who did not present with a fusiform aneurysm (RR, 22; 95% CI, 3.6%–68%; P = .00050). Index aneurysm treatment with parent artery occlusion also was associated with higher risk of new aneurysm formation (RR, 4.2; 95% CI, 1.3%–13%; P = .024). New aneurysms did not necessarily arise near index aneurysms. The only fatality in the series was due to subarachnoid hemorrhage from a new posterior circulation aneurysm arising 20 months after index anterior circulation aneurysm treatment in an immunosuppressed patient. CONCLUSIONS: Patients who presented with a fusiform aneurysm had a significantly greater incidence of developing a new aneurysm or enlargement of an index aneurysm than did those who presented with a saccular aneurysm. In our patient cohort, 8 of the 9 children who eventually developed new or enlarging brain aneurysms initially presented with fusiform aneurysm morphology. Other comorbidities or multiple aneurysms were also common in these patients at initial presentation.

Long-Term Outcome in the Repair of Spinal Cord Perimedullary Arteriovenous Fistulas

BACKGROUND AND PURPOSE: The natural history of PMAVFs, also known as type IV spinal cord AVFs, is incompletely understood. Both open surgical and endovascular approaches have been described as treatment modalities for this disease. The goal of this study was to evaluate the long-term outcome of patients with PMAVFs treated at a single tertiary care institution. MATERIALS AND METHODS: We conducted a retrospective study of 32 patients with PMAVFs, evaluated between 1983 and 2009. Data were gathered by reviewing outpatient clinic notes, operative and radiologic reports, and spinal angiograms. The PMAVFs were categorized into 1 of 3 types based on the angiographic imaging criteria. Pretreatment and posttreatment ambulation and micturition symptoms were quantified by using the ALS. RESULTS: Thirty patients underwent corrective procedures, 4 by embolization alone, 11 by surgery alone, and 15 with a combination of the 2. Twenty-eight patients underwent follow-up spinal angiography, with residual shunt noted in 6 patients. The mean follow-up period was 54 months (range, 1–228 months). Analysis of the ALS scores revealed that treatment of PMAVFs, independent of technique, resulted in significant improvement in ambulation but inconsistent changes in micturition. In addition, residual fistula at the time of the follow-up angiogram was associated with worsened neurologic status or lack of improvement. Outcome analysis based on fistula type showed dramatic improvement in ALS ambulation scores (62%) for type 3 fistulas, compared with types 1 and 2 (26% and 27%, respectively). CONCLUSIONS: Significant improvement in ambulation but in not micturition was observed following treatment. Residual fistula on follow-up angiography was associated with progressive worsening or lack of improvement in neurologic function. Patients with type 3 fistulas were shown to benefit most from treatment, with marked improvement in posttreatment ambulation scores. As endovascular and surgical techniques continue to evolve, further studies are warranted.

Comprehensive Management of Arteriovenous Malformations of the Brain and Spine

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Pial Artery Supply as an Anatomic Risk Factor for Ischemic Stroke in the Treatment of Intracranial Dural Arteriovenous Fistulas

BACKGROUND AND PURPOSE: Although intracranial dural arteriovenous fistulas are principally supplied by dural branches of the external carotid, internal carotid, and vertebral arteries, they can also be fed by pial arteries that supply the brain. We sought to determine the frequency of neurologic deficits following treatment of intracranial dural arteriovenous fistulas with and without pial artery supply. MATERIALS AND METHODS: One hundred twenty-two consecutive patients who underwent treatment for intracranial dural arteriovenous fistulas at our hospital from 2008 to 2015 were retrospectively reviewed. Patient data were examined for posttreatment neurologic deficits; patients with such deficits were evaluated for imaging evidence of cerebral infarction. Data were analyzed with multivariable logistic regression. RESULTS: Of 122 treated patients, 29 (23.8%) had dural arteriovenous fistulas with pial artery supply and 93 (76.2%) had dural arteriovenous fistulas without pial arterial supply. Of patients with pial artery supply, 4 (13.8%) had posttreatment neurologic deficits, compared with 2 patients (2.2%) without pial artery supply (P = .04). Imaging confirmed that 3 patients with pial artery supply (10.3%) had cerebral infarcts, compared with only 1 patient without pial artery supply (1.1%, P = .03). Increasing patient age was also positively associated with pial supply and treatment-related complications. CONCLUSIONS: Patients with dural arteriovenous fistulas supplied by the pial arteries were more likely to experience posttreatment complications, including ischemic strokes, than patients with no pial artery supply. The approach to dural arteriovenous fistula treatment should be made on a case-by-case basis so that the risk of complications can be minimized.

Intravascular Treatment of Aneurysms and Angioplasty of Arterial Vasospasm

Intracranial arterial vasospasm due to aneurysmal subarachnoid hemorrhage (SAH) remains a leading cause of major morbidity and mortality among cerebrovascular disorders.1–3 Despite recent advances in medical and surgical therapy, including calcium antagonists, early removal of thrombus, and cisternal irrigation with thrombolytic agents, it is estimated that 20% to 30% of patients with an acute SAH will develop vasospasm leading to stroke or death.3–8 Recent advances in interventional neurovascular radiology have now allowed patients with symptomatic vasospasm to be treated by intravascular balloon angioplasty techniques in selected cases. This chapter describes our current clinical protocol for patient selection, angiographic technique, and clinical results from treatment of patients with symptomatic arterial vasospasm by balloon dilatation therapy.