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OBJECTIVE Ruptured blister, dissecting, and iatrogenic pseudoaneurysms are rare pathologies that pose significant challenges from a treatment standpoint. Endovascular treatment via flow diversion represents an increasingly popular option; however, drawbacks include the requirement for dual antiplatelet therapy and the potential for thromboembolic complications, particularly acute complications in the ruptured setting. The Pipeline Flex embolization device with Shield Technology (PED-Shield) offers reduced material thrombogenicity, which may aid in the treatment of ruptured internal carotid artery pseudoaneurysms. METHODS The authors conducted a multi-institution, retrospective case series to determine the safety and efficacy of PED-Shield for the treatment of ruptured blister, dissecting, and iatrogenic pseudoaneurysms of the internal carotid artery. Clinical, radiographic, treatment, and outcomes data were collected. RESULTS Thirty-three patients were included in the final analysis. Seventeen underwent placement of a single device, and 16 underwent placement of two devices. No thromboembolic complications occurred. Four patients were maintained on aspirin alone, and all others were treated with long-term dual antiplatelet therapy. Among patients with 3-month follow-up, 93.8% had a modified Rankin Scale score of 0–2. Complete occlusion at follow-up was observed in 82.6% of patients. CONCLUSIONS PED-Shield represents a new option for the treatment of ruptured blister, dissecting, and iatrogenic pseudoaneurysms of the internal carotid artery. The reduced material thrombogenicity appeared to improve the safety of the PED-Shield device, as this series demonstrated no thromboembolic complications even among patients treated with only single antiplatelet therapy. The efficacy of PED-Shield reported in this series, particularly with placement of two devices, demonstrates its potential as a first-line treatment option for these pathologies.

OBJECTIVE The aim of this study was to describe the efficacy, clinical outcomes, and complications of open cerebrovascular surgery, endovascular surgery, and conservative management of dolichoectatic vertebrobasilar aneurysms (DVBAs). METHODS Relevant articles were retrieved from PubMed, Scopus, Web of Science, and Cochrane databases according to PRISMA guidelines. A meta-analysis was conducted for clinical presentation, treatment protocols, and clinical outcomes—good (improved or stable clinical status) or poor (deteriorated clinical status or death)—and mortality rates. RESULTS The 9 identified articles described 41 cases (27.5%) of open cerebrovascular surgery, 61 endovascular procedures (40.9%), and 47 cases (31.5%) of conservative management for DVBAs. The total cohort had a good outcome rate of 51.9% (95% CI 28.3%–74.6%), a poor outcome rate of 45.5% (95% CI 23.0%–70.1%), and a mortality rate of 22.3% (95% CI 11.8%–38.0%). The treatment groups had comparable good clinical outcome rates (open cerebrovascular surgery group: 24.7% [95% CI 2.9%–78.2%]; endovascular surgery group: 69.0% [95% CI 28.7%–92.5%]; conservative management group: 57.7% [95% CI 13.0%–92.5%]; p = 0.19) and poor outcome rates (open vascular surgery group: 75.3% [95% CI 21.8%–97.1%]; endovascular surgery group: 27.2% [95% CI 5.6%–0.70.2%]; conservative management group: 39.9% [95% CI 9.1%–81.6%]; p = 0.15). The treatment groups also had comparable mortality rates (open vascular surgery group: 39.5% [95% CI 11.4%–76.8%]; endovascular surgery group: 15.8% [95% CI 4.4%–43.0%]; conservative management group: 19.2% [95% CI 6.8%–43.5%]; p = 0.23). CONCLUSIONS The current study of DVBAs illustrated poor outcomes and high mortality rates regardless of the treatment modality. The subgroup analysis showed heterogeneity among the subgroups and advice for personalized management.

OBJECTIVEThe authors of this study found that, given the latency period required for arteriovenous malformation (AVM) obliteration after stereotactic radiosurgery (SRS), a study with limited follow-up cannot assess the benefit of SRS for unruptured AVMs.METHODSThe authors reviewed their institutional experience with “ARUBA (A Randomized Trial of Unruptured Brain Arteriovenous Malformations)–eligible” AVMs treated with SRS between 1987 and 2016, with the primary outcome defined as stroke (ischemic or hemorrhagic) or death (AVM related or AVM unrelated). Patients with at least 3 years of follow-up in addition to those who experienced stroke or died during the latency period were included. Secondary outcome measures included obliteration rates, patients with new seizure disorders, and those with new focal deficits without stroke.RESULTSOf 233 patients included in this study, 32 had a stroke or died after SRS over the mean 8.4-year follow-up (14%). Utilizing the 10% stroke or death rate at a mean 2.8-year follow-up for untreated AVMs in ARUBA, the rate in the authors’ study is significantly lower than that anticipated at the 8.4-year follow-up for an untreated cohort (14% vs 30%, p = 0.0003). Notwithstanding obliteration, in this study, annualized rates of hemorrhage and stroke or death after 3 years following SRS were 0.4% and 0.8%, respectively. The overall obliteration rate was 72%; new seizure disorders, temporary new focal deficits without stroke, and permanent new focal deficits without stroke occurred in 2% of patients each.CONCLUSIONSAfter a sensible follow-up period exceeding the latency period, there is a lower rate of stroke/death for patients with treated, unruptured AVMs with SRS than for patients with untreated AVMs.

OBJECTIVEMany small series and technical reports chronicle the evolution of endovascular techniques for cranial dural arteriovenous fistulas (dAVFs) over the past 3 decades, but reports of large patient series are lacking. The authors provide a thorough analysis of clinical and angiographic outcomes across a large patient cohort.METHODSThe authors reviewed their endovascular database from January 1996 to September 2015 to identify patients harboring cranial dAVFs who were treated initially with endovascular approaches. They extracted demographic, presentation, angiographic, detailed treatment, and long-term follow-up data, and they evaluated natural history, initial angiographic occlusion, complications, recurrence, and symptomatic resolution rates.RESULTSAcross a cohort of 251 patients with 260 distinct dAVFs, the overall initial angiographic occlusion rate was 70%; recurrence or occult residual lesions were seen on subsequent angiography in 3% of cases. The overall complication rate was 8%, with permanent neurological complications occurring in 3% of cases. Among 102 patients with dAVFs without cortical venous reflux, rates of resolution/improvement of pulsatile tinnitus and ocular symptoms were 79% and 78%, respectively. Following the introduction of Onyx during the latter half of the study period, the number of treated dAVFs doubled; the initial angiographic occlusion rate increased significantly from 60% before the use of Onyx to 76% after (p = 0.01). In addition, during the latter period compared with the pre-Onyx period, the rate of dAVFs obliterated via a transarterial-only approach was significantly greater (43% vs 23%, p = 0.002), as was the number of dAVFs obliterated via a single arterial pedicle (29% vs 11%, p = 0.002).CONCLUSIONSOverall, in the Onyx era, the rate of initial angiographic occlusion was approximately 80%, as was the rate of meaningful clinical improvement in tinnitus and/or ocular symptoms after initial endovascular treatment of cranial dAVFs.

OBJECTIVEThe incidence of already common chronic subdural hematomas (CSDHs) and other nonacute subdural hematomas (NASHs) in the elderly is expected to rise as the population ages over the coming decades. Surgical management is associated with recurrence and exposes elderly patients to perioperative and operative risks. Middle meningeal artery (MMA) embolization offers the potential for a minimally invasive, less morbid treatment in this age group. The clinical and radiographic outcomes after MMA embolization treatment for NASHs have not been adequately described in elderly patients. In this paper, the authors describe the clinical and radiographic outcomes after 151 cases of MMA embolization for NASHs among 121 elderly patients.METHODSIn a retrospective review of a prospectively maintained database across 15 US academic centers, the authors identified patients aged ≥ 65 years who underwent MMA embolization for the treatment of NASHs between November 2017 and February 2020. Patient demographics, comorbidities, clinical and radiographic factors, treatment factors, and clinical outcomes were abstracted. Subgroup analysis was performed comparing elderly (age 65–79 years) and advanced elderly (age > 80 years) patients.RESULTSMMA embolization was successfully performed in 98% of NASHs (in 148 of 151 cases) in 121 patients. Seventy elderly patients underwent 87 embolization procedures, and 51 advanced elderly patients underwent 64 embolization procedures. Elderly and advanced elderly patients had similar rates of embolization for upfront (46% vs 61%), recurrent (39% vs 33%), and prophylactic (i.e., with concomitant surgical intervention; 15% vs 6%) NASH treatment. Transfemoral access was used in most patients, and the procedure time was approximately 1 hour in both groups. Particle embolization with supplemental coils was most common, used in 51% (44/87) and 44% (28/64) of attempts for the elderly and advanced elderly groups, respectively. NASH thickness decreased significantly from initial thickness to 6 weeks, with additional decrease in thickness observed in both groups at 90 days. At longest follow-up, the treated NASHs had stabilized or improved in 91% and 98% of the elderly and advanced elderly groups, respectively, with > 50% improvement seen in > 60% of patients for each group. Surgical rescue was necessary in 4.6% and 7.8% of cases, and the overall mortality was 8.6% and 3.9% for elderly and advanced elderly patients, respectively.CONCLUSIONSMMA embolization can be used safely and effectively as an alternative or adjunctive minimally invasive treatment for NASHs in elderly and advanced elderly patients.

OBJECT Outcomes of microsurgical treatment of arteriovenous malformations (AVMs) in children are infrequently reported across large cohorts. METHODS The authors undertook a retrospective review of departmental and hospital databases to obtain the medical data of all patients up to 18 years of age who were diagnosed with cerebral AVMs. Demographic and AVM angioarchitectural characteristics were analyzed, and for the patients who underwent surgery, the authors also analyzed the estimated intraoperative blood loss, postoperative angiographically confirmed obliteration rates, and neurological complications and outcomes classified according to the modified Rankin Scale (mRS). RESULTS Of 117 children with cerebral AVMs, 94 underwent microsurgical resection (80%). Twenty (21%) of these 94 patients underwent adjunctive preoperative embolization. The overall postoperative angiographically confirmed obliteration rate was 94%. As part of a new protocol, the last 50 patients in this series underwent immediate perioperative angiography, improving the subsequent obliteration rate from 86% to 100% (p = 0.01). No other factors, such as a hemorrhagic AVM, size of the AVM, location, drainage, or Spetzler-Martin grade, had a statistically significant impact on the obliteration rate. Perioperative neurological deficits occurred in 17% of the patients, but the vast majority of these (77%) were predictable visual field cuts. Arteriovenous malformations that were hemorrhagic or located in noneloquent regions were each associated with lower rates of postoperative neurological complications (p = 0.05 and 0.002, respectively). In total, 94% of the children had good functional outcomes (mRS Scores 0–2), and these outcomes were significantly influenced by the mRS score on presentation before surgery (p = 0.01). A review of 1- and 5-year follow-up data indicated an overall annual hemorrhage rate of 0.3% and a recurrence rate of 0.9%. CONCLUSIONS Microsurgical resection of AVMs in children is associated with high rates of angiographically confirmed obliteration and low rates of significant neurological complications. Implementation of a protocol using perioperative angiography in this series led to complete radiographically confirmed obliteration of all AVMs, with low annual repeat hemorrhage and recurrence rates.

Object 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.

OBJECTIVEPenetrating cerebrovascular injury (PCVI) is a subset of traumatic brain injury (TBI) comprising a broad spectrum of cerebrovascular pathology, including traumatic pseudoaneurysms, direct arterial injury, venous sinus stenosis or occlusion, and traumatic dural arteriovenous fistulas. These can result in immediate or delayed vascular injury and consequent neurological morbidity. Current TBI guidelines recommend cerebrovascular imaging for detection, but there is no consensus on the optimum modality. The aim of this retrospective cohort study was to compare CT angiography (CTA) and digital subtraction angiography (DSA) for the diagnosis of PCVI.METHODSThe records of all patients presenting to two level I trauma centers in the United States between January 2010 and July 2016 with penetrating head or neck trauma were reviewed. Only those who had undergone both CTA and DSA were included. Clinical and neuroimaging data were collected, and PCVIs were stratified using a modified Biffl grading scheme. DSA and CTA results were then compared.RESULTSOf 312 patients with penetrating trauma over the study period, 56 patients (91% male, mean age 32 years) with PCVI met inclusion criteria and constituted the study cohort. The mechanism of injury was a gunshot wound in 86% (48/56) of patients. Twenty-four (43%) patients had sustained an angiographically confirmed arterial or venous injury. Compared with DSA as the gold standard, CTA had a sensitivity and specificity of 72% and 63%, respectively, for identifying PCVI. CTA had a positive predictive value of 61% and negative predictive value of 70%. Seven patients (13%) required immediate endovascular treatment of PCVI; in 3 (43%) of these patients, the injury was not identified on CTA. Twenty-two patients (39%) underwent delayed DSA an average of 25 days after injury; 2 (9%) of these patients were found to harbor new pathological conditions requiring treatment.CONCLUSIONSIn this retrospective analysis of PCVI at two large trauma centers, CTA demonstrated low sensitivity, specificity, and positive and negative predictive values for the diagnosis of PCVI. These findings suggest that DSA provides better accuracy than CTA in the diagnosis of both immediate and delayed PCVI and should be considered for patients experiencing penetrating head or neck trauma.

Object Cavernous malformations (CMs) of the basal ganglia are relatively rare lesions that can lead to considerable neurological impairment because of their eloquent location. The authors reviewed the clinical course and surgical outcome of a series of children with basal ganglia CMs. Methods The authors retrospectively reviewed the operative experience of the senior author (R.M.S.) and the 1997–2011 database of Boston Children's Hospital for children with CM of the basal ganglia (which includes CM of the caudate and/or lentiform nucleus and excludes CM of the thalamus). They evaluated baseline demographics, presenting signs, operative outcomes, and condition at long-term follow-up visits and compared these characteristics among patients who underwent surgery and those who were observed. Results Of 180 children with a diagnosis of CM, 11 (6%) had CM of the basal ganglia. The mean age at diagnosis was 9.3 years, and the male/female ratio was 1.8:1. Presenting signs were as follows: hemorrhage (8 children), incidental lesions (2), and seizures (1); 2 children had choreiform movement disorders. Treatment was observation or surgery. Observation was chosen for 5 children either because the lesions were asymptomatic (2 children) or because the risk for neurological dysfunction after attempted excision was believed to be high (3 children). These 5 children were observed over a combined total of 30.4 patient-years; none experienced neurological deterioration or symptomatic hemorrhage from their lesions. The other 6 children underwent microsurgical resection of the lesion because they were symptomatic from hemorrhage or increasing mass effect. All 6 of these children had hemorrhagic lesions, of which the smallest dimension was at least 1.5 cm. Of these 6 lesions, 5 were excised completely, and over a combined total of 46 patient-years of follow-up, no rebleeding or late neurological deterioration after surgery was reported. Conclusions In this patient population, the natural history of small and asymptomatic CMs of the basal ganglia was benign. The children with large (> 1.5 cm) symptomatic lesions underwent excision; neurological impairment was apparently minimal, and no hemorrhage or neurological deterioration occurred later.

Object The purpose of this report was to provide overall arteriovenous malformation (AVM) hemorrhage rates and, with enhanced statistical power, to elucidate significant risk factors for hemorrhage. Methods The authors performed a meta-analysis via the PubMed database through January 2012 using the terms “AVM,” “arteriovenous malformation,” “natural history,” “bleed,” and “hemorrhage.” Additional studies were identified through reference searches in each reviewed article. English language studies providing annual hemorrhage rates for AVMs were included. Data extraction, performed independently by the authors, included demographic data, hemorrhage rates, and hazard ratios for hemorrhage risk factors. The analysis was performed using a random effects model. Results Nine natural history studies with 3923 patients and 18,423 patient-years of follow-up were identified for analysis. The overall annual hemorrhage rate was 3.0% (95% CI 2.7%–3.4%). The rate of hemorrhage was 2.2% (95% CI 1.7%–2.7%) for unruptured AVMs and 4.5% (95% CI 3.7%–5.5%) for ruptured AVMs. Prior hemorrhage (HR 3.2, 95% CI 2.1–4.3), deep AVM location (HR 2.4, 95% CI 1.4–3.4), exclusively deep venous drainage (HR 2.4, 95% CI 1.1–3.8), and associated aneurysms (HR 1.8, 95% CI 1.6–2.0) were statistically significant risk factors for hemorrhage. Any deep venous drainage (HR 1.3, 95% CI 0.9–1.75) and female sex (HR 1.4, 95% CI 0.6–2.1) demonstrated a trend toward an increased risk of hemorrhage that was not statistically significant. Small AVM size and older patient age were not significant risk factors for hemorrhage. Conclusions Arteriovenous malformations with prior hemorrhage, deep location, exclusively deep venous drainage, and associated aneurysms have greater annual hemorrhage rates than their counterparts, influencing surgical decision making and the selection of radiosurgery for these lesions.

✓ In Cushing disease, a pituitary corticotroph neoplasm causes secondary adrenal hypercortisolism. This condition has known morbidity and mortality, underscoring the need for an efficient and accurate diagnostic approach. An 11 p.m. salivary cortisol level is a modern, simple initial screening tool for the diagnosis of Cushing syndrome. Confirmation with a 24-hour urinary free cortisol test and/or a low-dose dexamethasone suppression test may subsequently be performed. Patients with repeatedly equivocal results should be reevaluated after several months or undergo a corticotropin-releasing hormone (CRH) stimulation test following low-dose dexamethasone suppression to help rule out pseudo-Cushing states. The presence of low morning serum adrenocorticotropic hormone (ACTH) levels then distinguishes primary adrenal hypercortisolism from Cushing disease and the ectopic ACTH syndrome. Patients with moderate ACTH levels can undergo CRH stimulation testing to clarify the underlying disease because those with an ACTH-independent disorder have blunted subsequent ACTH levels. Once ACTH-dependent hypercortisolemia is detected, magnetic resonance (MR) imaging of the pituitary gland can be performed to detect a pituitary neoplasm. Normal or equivocal MR imaging results revealing small pituitary lesions should be followed up with inferior petrosal sinus sampling, a highly specific measure for the diagnosis of Cushing disease in experienced hands. If necessary, body imaging may be used in turn to detect sources of ectopic ACTH.

Object Type I spinal dural arteriovenous fistulas (SDAVFs) are low-flow vascular shunts fed by radicular arteries in patients who most often present with myelopathy. Although some fistulas are amenable to endovascular embolization, nearly all can be treated with direct microsurgical obliteration. Methods The authors reviewed their experience in treating 214 craniospinal arteriovenous malformations and/or fistulas over the last 8 years. Of these, 19 were spinal (9%), of which 15 (79%) were Type I SDAVFs. The authors reviewed the patients' epidemiological characteristics, presenting symptoms, and SDAVF angioarchitecture in all cases. They subsequently analyzed surgical obliteration rates and outcomes of all 11 patients who underwent fistula microsurgical obliteration. Results In all patients who underwent microsurgical treatment, complete angiographic obliteration of the fistula was achieved. At follow-up, 10 (91%) of 11 patients exhibited improvement, 1 patient (9%) was the same, and no patients were worse. Specifically, 8 (73%) of 11 patients had improvement in strength and sensation, 5 (71%) of 7 had improvement of bowel/bladder function, and 3 (60%) of 5 had improvement of preoperative paresthesias. There were no wound infections, CSF leaks, or permanent neurological deficits. Conclusions Microsurgical treatment of SDAVF provides direct access to the fistula point, allowing for high obliteration rates with excellent long-term improvement of preoperative deficits and limited periprocedural complications.

Object The management of spinal vascular malformations has undergone significant evolution with the advent of advanced endovascular and angiographic technology. Three-dimensional rotational spinal angiography is an advanced tool that allows the surgeon to gain a better appreciation of the anatomy of these spinal vascular lesions and their relation to surrounding structures. This article describes the use of rotational angiography and 3D reconstructions in the diagnosis and management of spinal vascular malformations. Methods The authors present representative cases involving surgical treatment planning for spinal vascular malformations with focus on the utility and technique of rotational spinal angiography. They report the use of rotational spinal angiography for a heterogeneous collection of vascular pathological conditions. Results Eight patients underwent rotational spinal angiography in addition to digital subtraction angiography (DSA) for the diagnosis and characterization of various spinal vascular lesions. Postprocessed images were used to characterize the lesion in relation to surrounding bone and to enhance the surgeon's ability to precisely localize and obliterate the abnormality. The reconstructions provided superior anatomical detail compared with traditional DSA. No associated complications from the rotational angiography were noted, and there was no statistically significant difference in the amount of radiation exposure to patients undergoing rotational angiography relative to traditional angiography. Conclusions The use of rotational spinal angiography provides a rapid and powerful diagnostic tool, superior to conventional DSA in the diagnosis and preoperative planning of a variety of spinal vascular pathology. A more detailed understanding of the anatomy of such lesions provided by this technique may improve the safety of the surgical approach.

Although originally the subject of rare case reports, intramedullary spinal cord cavernous malformations (CMs) have recently surfaced in an increasing number of case series and natural history reports in the literature. The authors reviewed 27 publications with 352 patients to consolidate modern epidemiological, natural history, and clinical and surgical data to facilitate decision making when managing these challenging vascular malformations. The mean age at presentation was 42 years without a sex predilection. Thirty-eight percent of the cases were cervical, 57% thoracic, 4% lumbar, and 1% unspecified location. Nine percent of the patients had a family history of CNS CMs. Twenty-seven percent of the patients had an associated cranial CM. On presentation 63% of the patients had motor deficits, 65% had sensory deficits, 27% had pain, and 11% had bowel or bladder dysfunction. Presentation was acute in 30%, recurrent in 16%, and progressive in 54% of cases. An overall annual hemorrhage rate was calculated as 2.5% for 92 patients followed up for a total of 2571 patient-years. Across 24 reviewed surgical series, a 91% complete resection rate was found. Transient morbidity was seen in 36% of cases. Sixty-one percent of patients improved, 27% were unchanged, and 12% were worse at the long-term follow-up. Using this information, the authors review surgical nuances in treating these lesions and propose a management algorithm.