OBJECTIVE: The aim of this study was to elucidate the long-term morbidity associated with untreated cerebral cavernous malformation (CCM). METHODS: From a prospectively maintained database of patients with CCM, we assess...OBJECTIVE: The aim of this study was to elucidate the long-term morbidity associated with untreated cerebral cavernous malformation (CCM). METHODS: From a prospectively maintained database of patients with CCM, we assessed functional outcome as measured using the modified Rankin Scale (mRS) score at baseline and the last untreated follow-up evaluation. Disability was defined as an mRS score ≥ 3. Kaplan-Meier curves were used to assess the time to disability, and Cox proportional hazards models were used to determine risk factors for disability. Furthermore, the predictive performance of a previously published nomogram for morbidity in CCM was assessed. RESULTS: The cohort consisted of 332 patients (58.4% female, mean age at diagnosis 44.5 years), with 19.8% having the familial form and 28.0% having a CCM located in the brainstem. Of 48 patients who presented with symptomatic hemorrhage (SH) and who did not undergo surgery or have a prospective SH, 27.1% had an mRS score ≥ 3 at diagnosis. By 1 year and 5 years, this improved to 6.2% and 4.7%, respectively. Over a mean follow-up duration of 6.6 years, 103 of 332 patients (31.0%) had one prospective SH and 48 (14.5%) had multiple prospective SHs. Patients with brainstem-located CCM had an 18.8% 5-year and 35.4% 10-year risk of disability compared with 4.1% and 7.5%, respectively, in patients with a nonbrainstem location (p < 0.0001). Multivariate analysis showed that a brainstem location, history of self-reported psychiatric disorder, and ≥ 2 SHs were predictive of disability at the last follow-up. External validation of a published nomogram demonstrated high specificity but limited sensitivity for predicting disability, with an area under the receiver operating curve of 0.687 for mRS scores ≥ 2 and 0.783 for mRS scores ≥ 3. CONCLUSIONS: Most patients improved after a first hemorrhage, with the most improvement occurring in year 1. Disability increased with each SH. Disability was associated with 2 or more SHs, a brainstem location, and a self-reported psychiatric diagnosis. Ten-year disability risk with a nonbrainstem CCM location was < 8%.
OBJECTIVE: Brain tumors are the most common solid neoplasm in children, accounting for 25% of pediatric cancer cases, with an incidence rate of 6.23 per 100,000. In addition, up to 2% of these patients will develop extra...OBJECTIVE: Brain tumors are the most common solid neoplasm in children, accounting for 25% of pediatric cancer cases, with an incidence rate of 6.23 per 100,000. In addition, up to 2% of these patients will develop extraneural metastases associated with a significantly poorer prognosis. The aim of this study was to investigate the incidence, presentation patterns, and neurosurgical considerations associated with extraneural metastases of primary pediatric brain tumors. METHODS: Using PRISMA guidelines, a search was conducted in the PubMed database to identify all full-text articles published in the English language up to May 2024 that described extraneural metastases in the pediatric population. The included studies comprised retrospective case reports and case series detailing the occurrence of extraneural metastasis following diagnosis of a primary brain tumor. Statistical differences between groups were assessed using the Mann-Whitney U-test for continuous variables and the chi-square test for categorical variables. RESULTS: Of 3811 articles reviewed, 124 articles that included 399 patients (male-to-female ratio of 1.46) with extraneural metastases were identified and analyzed. The mean age at diagnosis of the primary tumor was 8.78 (SD 4.92) years. Latency (the time from diagnosis of the primary tumor to appearance of extraneural metastases) increased with age, with a mean of 19.31 (SD 22.75) months. Medulloblastomas were the most frequent tumors leading to extraneural metastases. Surgical interventions, particularly tumor resection and shunt placement, played a crucial role in the management of the disease, with 79% of children undergoing surgery. Among these patients, placement of a ventriculoperitoneal shunt was associated with a significantly higher occurrence of abdominal metastases (p < 0.00001), whereas lymph node and lung metastases were more frequent in patients without shunts. CONCLUSIONS: As nearly all pediatric brain tumors carry a risk of extraneural metastases, these findings provide insights into the metastatic behavior of various tumors and highlight that surgery can influence the pattern of metastastic dissemination. This confirms the need for personalized follow-up and clinical management strategies based on tumor type, age, and surgical approach.
OBJECTIVE: This study aimed to evaluate the diagnostic yield from minimally invasive biopsies of pediatric brain tumors using endoscopic, robot-guided, and optical image-guided techniques. METHODS: A single-center retros...OBJECTIVE: This study aimed to evaluate the diagnostic yield from minimally invasive biopsies of pediatric brain tumors using endoscopic, robot-guided, and optical image-guided techniques. METHODS: A single-center retrospective review was conducted on the data of children (< 18 years of age) who underwent minimally invasive brain tumor biopsies using optical image-guided, endoscopic, or robot-assisted needle biopsies. Data collected consisted of demographic details, tumor location, simultaneous CSF diversion, and macroscopic details of collected specimens. Primary outcomes were histological and molecular diagnostic yield, histological accuracy, need for repeat biopsy, and reasons for biopsy failure. Adverse events were prospectively recorded. Statistical analysis was conducted in SPSS and Prism using Pearson's chi-square and Fisher's exact tests. RESULTS: Between January 2004 and February 2023, 188 biopsies were performed using endoscopic (41.5%), optical image-guided (37.2%), and robot-guided (21.3%) techniques. Tumor location was classified as suprasellar (28.2%), pineal region (20.2%), brainstem (19.1%), basal ganglia/thalamic (16.5%), supratentorial lobar (10.1%), intraventricular (5.3%), and cerebellar (0.5%). Histological diagnosis was achieved in 88.3% (166/188) and consisted of low-grade glioma (33.5%), high-grade glioma (31.9%), germ cell tumor (10.6%), embryonal tumor (5.3%), craniopharyngioma (2.1%), lymphoma (1.6%), pineoblastoma (1.6%), choroid plexus papilloma (0.5%), meningioma (0.5%), and pineal cyst (0.5%). Overall, 11.7% (22/188) of biopsies were nondiagnostic: 19.2% for endoscopic, 8.6% for optical image-guided, and 2.5% for robot-guided techniques. Thirteen (6.9%) patients required further biopsy. A molecular diagnosis was achieved in 82.0% (109/133) of eligible cases. All tumors with a molecular diagnosis had a histological diagnosis; of 24 patients for whom molecular diagnosis was not possible, 79.2% (19/24) had a histological diagnosis. Larger-sized biopsies were more likely to result in a positive diagnosis for histology and molecular analysis. Hemorrhage in the specimen did not affect results. Endoscopic biopsies were most likely to be small and yield a lower volume of core specimens. Histological diagnostic accuracy for glioma was more likely for high-grade tumors, larger-sized biopsies, and tumors in which a molecular diagnosis was achieved. CONCLUSIONS: Through the use of contemporary minimally invasive techniques, pediatric brain tumor biopsy has a histological diagnostic yield of 88.3% and molecular diagnostic yield of 82.0%. Robot-guided and optical image-guided techniques provided the highest diagnostic yield (97.5% and 91.4%, respectively, for histological analysis), while endoscopic biopsies had the highest rate of nondiagnostic results (19.2%), likely due to smaller specimen volumes.
OBJECTIVE: The pathophysiological distinctions and modifiable risk factors underlying ischemic versus hemorrhagic conversion in moyamoya disease (MMD) remain incompletely characterized, hindering subtype-specific managem...OBJECTIVE: The pathophysiological distinctions and modifiable risk factors underlying ischemic versus hemorrhagic conversion in moyamoya disease (MMD) remain incompletely characterized, hindering subtype-specific management. This study aimed to delineate subtype-specific risk patterns and validate biomarkers for asymptomatic progression. METHODS: The authors analyzed the medical records of 774 adult MMD patients from Nanjing Jinling Hospital (2010-2020) and Nanjing Drum Tower Hospital (2013-2020). MMD was stratified as asymptomatic (n = 139), ischemic (n = 450), or hemorrhagic (n = 185). Multivariable logistic regression and Cox proportional hazards models were used to evaluate metabolic profiles, angiographic features (posterior cerebral artery [PCA] involvement and choroidal and lenticulostriate anastomoses), and longitudinal outcomes. RESULTS: Symptomatic patients demonstrated higher PCA involvement prevalence versus asymptomatic patients (p < 0.001). Ischemic MMD was independently associated with male sex (OR 2.00, 95% CI 1.30-3.07; p = 0.002), hypertension (OR 2.30, 95% CI 1.49-3.54; p < 0.001), hypertriglyceridemia (OR 1.36, 95% CI 1.01-1.83; p = 0.04), hyperglycemia (OR 1.22, 95% CI 1.03-1.45; p = 0.02), and PCA involvement (OR 2.43, 95% CI 1.40-4.20; p = 0.001). Hemorrhagic MMD correlated with BMI (OR 0.88 per kg/m2, 95% CI 0.81-0.97; p = 0.007), hypercholesterolemia (OR 1.53, 95% CI 1.14-2.07; p = 0.005), choroidal anastomosis formation (OR 2.38, 95% CI 1.21-4.70; p = 0.01), and PCA involvement (OR 3.41, 95% CI 1.76-6.61; p < 0.001). During the median 44-month follow-up, asymptomatic patients with PCA involvement (adjusted hazard ratio [HR] 4.86, 95% CI 1.07-22.14; p = 0.04) or choroidal anastomosis (adjusted HR 5.92, 95% CI 1.27-27.62; p = 0.02) exhibited an elevated risk of symptomatic conversion. CONCLUSIONS: Ischemic MMD was independently associated with male predominance, hypertension, and dyslipidemia, while hemorrhagic transformation correlated with lower BMI, hypercholesterolemia, and choroidal anastomosis. PCA involvement and choroidal anastomosis emerged as critical biomarkers for asymptomatic risk stratification.
OBJECTIVE: During endoscopic endonasal surgery (EES), inferolateral trunk (ILT) sacrifice may be required to efficiently and safely achieve tumor resection within the lateral compartment (LC) of the cavernous sinus (CS)....OBJECTIVE: During endoscopic endonasal surgery (EES), inferolateral trunk (ILT) sacrifice may be required to efficiently and safely achieve tumor resection within the lateral compartment (LC) of the cavernous sinus (CS). The authors investigated the surgical anatomy and variations of the ILT, aiming to provide practical information to safely expose, coagulate, and transect this artery during EES. METHODS: In this anatomical study, 24 postmortem, lightly embalmed, colored silicone-injected human head specimens were dissected and 41 sides were examined. The origin, course, branching pattern, and relation of the ILT with surrounding structures were investigated. Clinical charts of patients surgically treated for pituitary adenomas (PAs) with LC invasion from July 2018 to April 2023 at the authors' institution were also retrospectively analyzed. Illustrative cases are provided. RESULTS: The ILT was found in 93% (38/41) of sides, mainly arising from the inferolateral aspect (91%, 30/33 sides) of either the middle or posterior third (82%, 27/33 sides) of the horizontal segment of the internal carotid artery. After a short common trunk (mean length 3 mm), the artery divided into 2 (21%, 8/38) or, more frequently, 3 (74%, 28/38) branches, supplying blood to cranial nerves (CNs) III, IV, V1, V2, V3, and VI and the Gasserian ganglion. While the sympathetic plexus was always located anterior to the ILT, CN VI was found anterior to the ILT in 82% (31/38) of sides. The lateral parasellar ligament (LPL) enwrapped the ILT and its branches in 43% (15/35) of sides. In the coronal plane, the ILT origin was found at the level of the sellar floor (0 ± 1 mm) and the LPL (0 ± 2 mm), both of which can serve as surgical landmarks during lateral transcavernous EES. In the case series of 25 EESs for PAs with LC invasion, the ILT was sacrificed in 5 cases (20%) without any permanent postoperative CN deficits. CONCLUSIONS: This study served as a detailed anatomical investigation of the ILT, which is crucial when accessing the LC of the CS. The authors proposed two reliable landmarks to identify the ILT intraoperatively: the sellar floor and the LPL. Furthermore, investigations confirmed that the ILT can be sacrificed without causing permanent CN deficits given the existence of a collateral supply.
OBJECTIVE: In patients with low-grade gliomas (LGGs), reallocations of cortical functions (i.e., plasticity) evolve over the course of the disease, allowing serial resections while preserving patients' neurological statu...OBJECTIVE: In patients with low-grade gliomas (LGGs), reallocations of cortical functions (i.e., plasticity) evolve over the course of the disease, allowing serial resections while preserving patients' neurological status. This study aimed to capture evolving patterns of LGG-induced plasticity by means of longitudinal measures of cortical functions based on serial intrasurgical direct electrical stimulation (DES) mappings and navigated transcranial magnetic stimulation (nTMS) mapping. It further assessed nTMS prediction accuracy using DES measures as a reference. METHODS: In patients with confirmed LGG, cortical functional remodeling was assessed through initial intraoperative awake DES mapping (DESI) and DES remapping (DESII), typically spaced several years apart. An additional session of nTMS mapping mirroring intrasurgical functional testing was performed before DESII. In particular, nTMS mapping was guided by preexistent DESI functional maps to maximize stimulation coverage in areas susceptible to presenting with functional reorganizations. Probabilistic functional maps accounting for functional and nonfunctional responses were computed through a multistep voxelwise approach to measure cortical reallocations between DESI and DESII and nTMS prediction accuracy. RESULTS: Eight patients were prospectively enrolled (median DESI-DESII time interval 7.0 years). Overall, 2268 nTMS and 244 DES cortical sites were recorded. DESII systematically highlighted evolving plasticity. The nTMS functional convergence index, based on comparisons with DESII results, was heterogeneous across cortical structures. Converging nTMS/DESII measures were associated with nonfunctional nTMS responses (91.13%). Evolving patterns of plasticity were effectively revealed by nonfunctional nTMS mapping, except within the ventral premotor cortex (vPMC). Further, nTMS prediction accuracy was high within the pars triangularis, supramarginal gyrus, and supplementary motor area (R2 = 0.50) and low within the vPMC (R2 = 0.0005). CONCLUSIONS: These results provide a unique overview of evolving patterns of cortical plasticity in LGG patients. Although nTMS may help provide longitudinal metrics of plasticity in brain tumor patients, several challenges must be addressed before routine clinical applications.
OBJECTIVE: The aim of this study was to evaluate the role of hemodynamic parameters in predicting the efficacy of microvascular decompression (MVD) in patients with classic trigeminal neuralgia (CTN) using computational...OBJECTIVE: The aim of this study was to evaluate the role of hemodynamic parameters in predicting the efficacy of microvascular decompression (MVD) in patients with classic trigeminal neuralgia (CTN) using computational fluid dynamics (CFD). METHODS: Patients with unilateral CTN were recruited from May 2022 to December 2023. Preoperative time-of-flight MR angiography was used to identify neurovascular compression sites. CFD simulations were performed to analyze hemodynamic parameters such as peak systolic flow (PSF), peak systolic pressure drop (PSPD), maximum wall shear stress (WSS), and oscillatory shear index (OSI). Logistic regression analysis was used to develop predictive models for MVD efficacy. RESULTS: Fifty-six patients were included (28 in the effective MVD group and 28 in the ineffective MVD group). The effective group exhibited significantly lower PSF (mean 0.202 [SD 0.136] vs 0.306 [SD 0.142] ml/sec, p = 0.007) and higher PSPD (mean 33.239 [SD 20.122] vs 22.864 [SD 15.624] Pa, p = 0.036), maximum WSS (median 3.231 [interquartile range (IQR) 2.084-4.359] vs 2.197 [IQR 1.592-3.445] Pa, p = 0.024), and OSI (median 0.001 [IQR 0.001-0.002] vs 0.001 [IQR 0.001-0.001], p = 0.029). Logistic regression analysis identified PSF and maximum WSS as significant predictors of MVD efficacy. The developed prediction models showed high accuracy, with model 2 (using the backward logistic regression method) achieving an area under the receiver operating characteristic curve of 0.920 and both sensitivity and specificity of 90%. CONCLUSIONS: Hemodynamic parameters, particularly PSF and maximum WSS, significantly predict MVD efficacy in CTN. Integrating these parameters into clinical practice could improve surgical outcomes and guide personalized treatment strategies.
OBJECTIVE: The primary aim of this Italian multicenter study was to determine the incidence of complications associated with pituitary neuroendocrine tumor (PitNET) removal in the modern era of endoscopic pituitary surge...OBJECTIVE: The primary aim of this Italian multicenter study was to determine the incidence of complications associated with pituitary neuroendocrine tumor (PitNET) removal in the modern era of endoscopic pituitary surgery. The secondary objective was to evaluate the influence of surgical experience, patients, and tumor features on the occurrence of each specific complication, as well as options for management and avoidance. METHODS: This retrospective study included the contributions of 10 Italian neurosurgical departments. Respondents were asked to provide the number of endoscopic endonasal approaches (EEAs) performed for PitNET removal from 2013 to 2023 and to identify any surgical complications observed. Main tumor and surgical features associated with the occurrence of surgical complications were evaluated. Respondents were placed into 3 surgical experience groups, based on the number of endoscopic PitNET removals performed during the study period. RESULTS: From a total of 3356 operations for PitNET removal via an EEA, the incidence of surgical complications was 11.92% (400/3356). Worsening of anterior pituitary function (4.80%) and permanent diabetes insipidus (2.32%) were the complications that occurred the most often. Postoperative CSF leak occurred in 50 cases (1.49%). Other significant complications, such as carotid artery injury, epistaxis, sphenoid sinusitis, septal perforation, residual tumor hemorrhage, intracranial hematoma, vasospasm, subarachnoid hemorrhage, cranial nerve injuries, visual worsening, meningitis, and tension pneumocephalus occurred with incidence rates between 0.06% and 0.89%. Among 350 patients experiencing at least one surgical complication, 58.86% harbored a nonfunctioning adenoma and 17.71% a recurrent tumor. The overall rate of postoperative complications in relation to surgical experience exhibited a U-shaped progression, with a significantly higher rate (p < 0.001) in centers performing fewer than 200 procedures (117/654, 17.89%) compared to centers performing 200-500 operations (88/900, 9.78%) and those with caseloads exceeding 500 (195/1802, 10.82%). CONCLUSIONS: This study demonstrated a reduced overall rate of complications in EEAs for PitNET removal related to advancements in surgical technique, technological progress, and surgeon experience, which have allowed an expansion of indications to more complex cases. Within the U-shaped relationship between surgical experience and complication rate, likely due to the prevalence of PitNETs at higher risk of surgical morbidity in centers with more experience, CSF leakage was found to be the only complication continuously improving with surgical experience.
OBJECTIVE: Maximizing extent of resection (EOR) for language-eloquent high-grade glioma (HGG) must be balanced against the risk of neurological deficit. Predictors of postoperative decline in language remain poorly chara...OBJECTIVE: Maximizing extent of resection (EOR) for language-eloquent high-grade glioma (HGG) must be balanced against the risk of neurological deficit. Predictors of postoperative decline in language remain poorly characterized. This study aimed to evaluate intraoperative predictors of postoperative language decline, along with predictors of subsequent temporal recovery. METHODS: The authors conducted a single-center study of patients undergoing awake craniotomy for the resection of language-eloquent HGG utilizing diffusion tractography, 5-aminolevulinic acid (5-ALA), and subcortical stimulation (SCS) with intraoperative language testing. Cases were reviewed between January 2017 and November 2024. Data assessing intraoperative language function, SCS parameters, EOR, along with language deficit at the 48-hour, 2-week, and 3-month follow-ups were collected. Receiver operating characteristic curves and Youden's Index were used to identify optimal subcortical stimulatory thresholds predicative of postoperative deficit in respective language domains for individual fiber tracts, including the inferior fronto-occipital fasciculus (IFOF), arcuate fasciculus (AF) and superior longitudinal fasciculus (SLF), and inferior longitudinal fasciculus (ILF). RESULTS: A total of 130 patients (78 male, 52 female; mean age 57.1 years) were included. Postoperatively, a new or worsened language deficit was observed in 69 patients (53.1%). This was permanent (> 3 months) in 12 cases (9.2%). Clinical deterioration during awake language testing was a significant predictor of short-term (< 3 months) decline in language domains including semantic processing (IFOF) (RR 3.47, p = 0.0002), reading (ILF) (RR 21.4, p = 0.0025), and auditory naming/repetition (AF/SLF) (RR 6.98, p = 0.001). Factors associated with permanent postoperative language decline were the presence of preoperative speech deficit (RR 2.65, p = 0.020), intraoperative deterioration in ILF-related reading function (RR 8.92, p = 0.0407), and positive SCS of multiple white matter language tracts. Individual stimulation thresholds predictive of functional decline are presented for IFOF, AF, and ILF. CONCLUSIONS: This study evaluated multimodal resection of language-eloquent HGGs using awake mapping, 5-ALA, and tractography. It highlights the significant risk of transient decline in language function following eloquent tumor resection, particularly if a patient has an existing language deficit. The posterior ILF with its associated reading function appears to be most sensitive to decline and shows the least propensity for functional recovery. Deficits correlated with specific white matter tract involvement, especially when multiple tracts were affected. Proposed stimulation thresholds offer a novel guide for safer resections, supporting a multimodal strategy to balance maximal tumor removal with language preservation.
OBJECTIVE: Children with sickle cell disease (SCD) are at risk of developing cerebral vasculopathies, increasing the risk of stroke. While some vasculopathies are medically managed, surgical revascularization might be ne...OBJECTIVE: Children with sickle cell disease (SCD) are at risk of developing cerebral vasculopathies, increasing the risk of stroke. While some vasculopathies are medically managed, surgical revascularization might be needed in patients who also have moyamoya arteriopathy (MMA). The aim of this study was to characterize stroke timing and phenotype between children with homozygous SCD (HbSS) alone and those with HbSS and concurrent MMA. METHODS: A retrospective review of clinical and radiological information for pediatric patients (age < 21 years) with HbSS and cerebrovascular sequelae at a single institution (1997-2021) was conducted. Cerebrovascular sequelae included stroke, transient ischemic attack, elevated transcranial Doppler velocities, MMA, or other cerebral vessel stenosis. Patients with HbSS and MMA (HbSS/MMA group) were compared with patients with HbSS alone (HbSS group). RESULTS: Sixty-one children with HbSS were included; 35 had HbSS and MMA. Thirty-one patients in the HbSS/MMA group underwent surgical revascularization. The remaining patients were medically managed with chronic transfusion, hydroxyurea, and/or stem cell transplant. The incidence of first stroke detected on surveillance imaging peaked at 4-9 years of age for the HbSS group and 3-8 years for the HbSS/MMA group (p = 0.47). Children with concurrent MMA presented more often with symptomatic strokes (p < 0.05) and were diagnosed with MMA several years after their initial stroke (median 4.4, IQR 2.0-7.8). Although the HbSS and HbSS/MMA groups had a similar window of risk, children with HbSS and MMA had a higher stroke rate per person per year (0.15 vs 0.10). CONCLUSIONS: Children with HbSS and concurrent MMA have increased risk of stroke during early childhood relative to children with HbSS alone. Additionally, those with HbSS and MMA are at risk of delayed diagnosis after experiencing neurological deficit from symptomatic stroke. These findings represent an opportunity to optimize stroke prevention screening, targeting children as young as 3-8 years of age.
OBJECTIVE: Focused ultrasound (FUS) is a minimally invasive treatment option for essential tremor and tremor-dominant Parkinson's disease. Because thermal lesions are irreversible, it is critical to understand how lesion...OBJECTIVE: Focused ultrasound (FUS) is a minimally invasive treatment option for essential tremor and tremor-dominant Parkinson's disease. Because thermal lesions are irreversible, it is critical to understand how lesion placement relates to patient outcomes. The aim of this study was to determine whether patient outcomes can be predicted using real-time estimation of heating and the accumulated thermal dose derived from MR temperature imaging (MRTI). METHODS: MRTI, tremor control, and side effects were analyzed from 547 FUS thalamotomy treatments (481 patients, median age 74 years). Previously described methods were used to estimate the heating from 2D MRTI in different orientations and to generate phase maps from the MRTI data. After registration to an MRI atlas, we compared the thermal dose deposition of patients who reported side effects with that of patients who did not report side effects at time points ranging from 1 day to 1 year after FUS. Logistic regression and voxel-wise frequency mapping were used to compare the size and spatial extent of heating, thermal dose, and lesion segmentations to the frequency of different side effects and complete tremor control. RESULTS: Relationships between the frequency of side effects and tremor control were similar for thermal dose contours and lesion segmentations. Weakness and imbalance were more frequent when heating, dose contours, and lesions extended inferolaterally into the internal capsule. Sensory deficits and dysgeusia were more frequent with increased posterior extent, and dysarthria was more frequent with superior/medial/anterior extent. While most areas associated with complete tremor response overlapped with those associated with side effects, a small anterior region was identified in which complete tremor response without side effects was more frequent. Increased lesion area resulted in more side effects and complete tremor response at early and late time points, but the corresponding areas for dose contours were only related to side effects and only at 1 day after FUS. CONCLUSIONS: MRTI can provide similar results as lesion segmentations after estimating heating and the thermal dose in different orientations. Combining this approach with visualization of anatomy around the thalamus using filtered phase maps of MRTI is useful for real-time monitoring and optimization of FUS treatments.
OBJECTIVE: Moyamoya arteriopathy and middle aortic syndrome (MAS) are each rare, often progressive vascular diseases; their comorbid intersection increases their complexity because of the independent and contradictory de...OBJECTIVE: Moyamoya arteriopathy and middle aortic syndrome (MAS) are each rare, often progressive vascular diseases; their comorbid intersection increases their complexity because of the independent and contradictory demands on blood pressure. The management of pediatric patients with these comorbid conditions requires multidisciplinary consultation given the high risk involved. In the current study, the authors aim to describe the clinical and surgical history of pediatric patients with comorbid moyamoya arteriopathy and MAS and describe a multidisciplinary approach to care for these patients. METHODS: This study is a retrospective review of the clinical and radiological records of patients at Boston Children's Hospital who were treated with cerebral revascularization surgery for comorbid moyamoya arteriopathy and MAS from January 2004 to January 2024. Analysis of collected perioperative, surgical, inpatient, and follow-up data was conducted using R version 4.4.2 (R Foundation for Statistical Computing). RESULTS: Eleven patients, 5 male (45.5%), were included in the study. At presentation, 9 patients (81.8%) had symptoms from brain ischemia. Nine patients were on antihypertensive medications (median 2, IQR 1-2). All patients underwent surgery for moyamoya arteriopathy, and 6 patients (54.5%) also underwent surgical treatment for MAS. After moyamoya surgery, 4 patients (36.4%) required an intensified blood pressure regimen to achieve the same goal, whereas 3 patients (27.3%) had a reduced need for blood pressure medications. Four (66.7%) of the 6 patients who underwent MAS surgical treatment did so after moyamoya treatment. Perioperative stroke occurred after renal stent angioplasty before moyamoya surgery but also after aortic bypass following moyamoya surgery. CONCLUSIONS: Managing comorbid moyamoya arteriopathy and MAS is challenging, as patients have a higher risk of ischemic perioperative complications. These patients may have hypertension secondary to their aortic disease, and treating the aortic disease may correct the hypertension but with possible new relative hypotension and relative cerebral hypoperfusion. Consequently, moyamoya revascularization prior to aortic repair may be a strategy to reduce the risk of stroke.