Orbital biopsy findings consistent with granulomatosis polyangiitis in a 14-year-old boy with Crohn's disease.

Both granulomatous polyangiitis (GPA) and Crohn's disease (CD) can cause orbital inflammation though rarely coincide and can often be differentiated by presenting features and histological findings. Here, we report the clinical and therapeutic course of a 14-year-old White male with binocular diplopia caused by orbital myositis. Imaging and biopsy obtained at presentation revealed necrosis and necrotizing granulomatous vasculitis suspicious for GPA. He subsequently developed gastrointestinal symptoms and terminal ileitis consistent with CD. Orbital symptoms responded well to high-dose steroids and remained quiet on methotrexate maintenance therapy. While clinical history, thorough physical exam, and complete laboratory work-up are essential in the management of pediatric orbital myositis, orbital biopsy can prove critical for diagnosis and suitable treatment strategy.

Deadly Fungi: Invasive Fungal Rhinosinusitis in the Head and Neck.

Invasive fungal rhinosinusitis (IFRS) is a serious infection that is associated with high morbidity and mortality rates. The incidence of IFRS has been increasing, mainly because of the increased use of antibiotics and immunosuppressive drugs. Rhino-orbital cerebral mucormycosis has recently reemerged among patients affected by COVID-19 and has become a global concern. The detection of extrasinus involvement in its early stage contributes to improved outcomes; therefore, imaging studies are essential in establishing the degree of involvement and managing the treatment properly, especially in immunocompromised patients. The common sites of extrasinus fungal invasion are the intraorbital, cavernous sinus, and intracranial regions. Fungi spread directly to these regions along the blood vessels or nerves, causing devastating complications such as optic nerve ischemia or compression, optic neuritis or perineuritis, orbital cellulitis, cavernous sinus thrombosis, mycotic aneurysm, vasculitis, internal carotid arterial occlusion, cerebral infarction, cerebritis, and brain abscess. IFRS has a broad imaging spectrum, and familiarity with intra- and extrasinonasal imaging features, such as loss of contrast enhancement of the affected region, which indicates tissue ischemia due to angioinvasion of fungi, and the surrounding anatomy is essential for prompt diagnosis and management. The authors summarize the epidemiology, etiology, risk factors, and complications of IFRS and review the anatomy and key diagnostic imaging features of IFRS beyond the sinonasal regions. ©RSNA, 2022.

Imaging and health metrics in incidental cerebellar tonsillar ectopia: findings from the Adolescent Brain Cognitive Development Study (ABCD).

PURPOSE: Incidental cerebellar tonsillar ectopia (ICTE) that meets the radiographic criterion for Chiari malformation type I (CMI) is an increasingly common finding in the clinical setting, but its significance is unclear. The present study examined posterior cranial fossa (PCF) morphometrics and a broad range of health instruments of pediatric ICTE cases and matched controls extracted from the Adolescent Brain Cognitive Development (ABCD) dataset. METHODS: One-hundred-six subjects with ICTE and 106 matched controls without ICTE were identified from 11,411 anatomical MRI of healthy screened pediatric subjects from the ABCD project. Subjects were matched by sex, age, body mass index, race, and ethnicity. Twenty-two brain morphometrics and 22 health instruments were compared between the two groups to identify unrecognized CMI symptoms and assess the general health impact of ICTE. RESULTS: Twelve and 15 measures were significantly different between the ICTE and control groups for females and males, respectively. Notably, for females, the anterior CSF space was significantly smaller (p = 0.00005) for the ICTE group than controls. For males, the clivus bone length was significantly shorter (p = 0.0002) for the ICTE group compared to controls. No significant differences were found among the 22 health instruments between the two groups. CONCLUSION: This study demonstrated that pediatric ICTE subjects have similar PCF morphometrics to adult CMI. ICTE alone did not appear to cause any unrecognized CMI symptoms and had no impact on the subjects' current mental, physical, or behavioral health. Still, given their cranial and brain morphology, these cases may be at risk for adult-onset symptomatic CMI.

Intraoperative Phase Contrast MRI Analysis of Cerebrospinal Fluid Velocities During Posterior Fossa Decompression for Chiari I Malformation.

BACKGROUND: Cerebrospinal fluid (CSF) velocity at the craniovertebral junction (CVJ) is known to be altered in patients with Chiari I malformation (CMI), and normalization of CSF velocities is associated with symptom resolution. However, preoperative and intraoperative prediction methods have thus far failed to identify patients in whom CSF velocities can be normalized with posterior fossa decompression (PFD) without duraplasty. Phase contrast MRI (PC-MRI) may assist not only in diagnosis of CMI but also in guiding the intraoperative decision to perform duraplasty during PFD. PURPOSE: To use intraoperative MRI data to quantify changes in CSF hydrodynamics at the CVJ during each step of PFD with duraplasty (PFDD) in 12 consecutive patients. STUDY TYPE: Retrospective case series with all patients imaged before, during and after decompression, and all data analyzed postprocedure. POPULATION/SUBJECTS: Pediatric patients, mean age 14 years (range 4-18), undergoing PFD for CMI. FIELD STRENGTH/SEQUENCE: Intraoperative studies involved a dedicated 1.5T Siemens MRI imager. PC-MRI scans were in the axial plane at the CVJ. ASSESSMENT: Two observers assessed measurements. STATISTICAL TEST: The equality of matched pairs of observations was tested using the Wilcoxon matched-pairs signed-ranks test. RESULTS: Data analyses of the PC-MRI demonstrated a marked and immediate increase in CSF velocity at the posterior CVJ during PFDD. Mean cranially-directed velocities increased by a mean of 1.049cm/s (P = 0.028) from preincision to postoperative measurement. There was a mean 0.45 cm/s (P = 0.022) increase in mean cranial velocity from preincision to bone decompression scans, and a mean 0.48 cm/s (P = 0.018) increase in mean velocity from preincision to duraplasty. DATA CONCLUSION: In all subjects, significant increases in the mean and peak velocities of cranially- and caudally-directed velocities were observed from preincision to post-PFDD scans at the posterior CVJ. LEVEL OF EVIDENCE: 2 Technical Efficacy: Stage 4 J. Magn. Reson. Imaging 2020;51:1463-1470.

Clivus length distinguishes between asymptomatic healthy controls and symptomatic adult women with Chiari malformation type I.

PURPOSE: While the presence of cerebellar tonsillar descent in radiological images has been used as evidence of Chiari malformation type I (CMI), tonsillar ectopia alone is insufficient to identify individuals with symptomatic CMI. This study sought to identify differences in brain morphology between symptomatic CMI and healthy controls in adult females. METHODS: Two hundred and ten adult females with symptomatic CMI and 90 age- and body mass index-matched asymptomatic female controls were compared using seven brain morphometric measures visible on magnetic resonance images. The CMI and control groups were divided into four subgroups based on the tonsillar position (TP) relative to the foramen magnum: group 1 was made up of healthy controls with normal TP (TP < 0 mm); group 2 was comprised of control individuals with low-lying TP (1-5 mm); group 3 was comprised of symptomatic CMI patients with low-lying TP (1-5 mm); group 4 contained symptomatic CMI patients with severe tonsillar descent (6-13 mm). RESULTS: All morphometrics for symptomatic CMI with severe tonsillar descent were significantly different than those for both control groups. The CMI group with low-lying TP was significantly different for four measures when compared to controls with normal TP. However, only clivus length was statistically different between the CMI and healthy control groups with low-lying TP. CONCLUSION: This study demonstrates that clivus length distinguishes adult female healthy individuals with low-lying tonsils from those with symptomatic CMI. Further investigation is required to understand the importance of a shorter clivus length on CMI symptomatology and pathophysiology.

Quantification of changes in brain morphology following posterior fossa decompression surgery in women treated for Chiari malformation type 1.

PURPOSE: While 84% of patients surgically treated for Chiari malformation type 1 (CM1) demonstrate improved quality of life after posterior fossa decompression surgery, there are many risks associated with this surgery. Surgical planning to identify candidates likely to improve postoperatively may benefit from an improved understanding of morphological changes after decompression surgery. To evaluate these changes, we quantified 59 morphological parameters on 42 CM1 adult female patients before and after CM1 decompression surgery. METHODS: Fifty-nine morphological parameters in the posterior cranial fossa, cranio-cervical, and intracranial regions in the midsagittal plane were evaluated using 42 T1-weighted magnetic resonance images of female CM1 patients before and after surgery, and 42 healthy female controls. Morphological differences before and after surgery were compared through the development of a technique to establish the opisthion location, a key reference point not present after surgery. RESULTS: In addition to the expected reduction of the cranio-caudal dimension of the cerebellum, objective analyses showed a significant increase in the area of the cerebrospinal fluid spaces, posterior (6×) and inferior (2.6×) to the cerebellum (+ 112 ± 102 and + 140 ± 127 mm2, respectively). This increased area was primarily impacted by an average reduction in the occipital bone length of 24.5 ± 7.3 mm following surgery. Based on multiple angles, results demonstrated a 2°-4° anterior rotation of the cerebellum after surgery. CONCLUSION: Our results show that decompression surgery results in significant changes in the cerebellum and cerebrospinal fluid spaces. Further investigation should determine how these morphological changes impact clinical outcomes.

A morphometric assessment of type I Chiari malformation above the McRae line: A retrospective case-control study in 302 adult female subjects.

PURPOSE: Type I Chiari malformation (CMI) is a radiologically-defined structural dysmorphism of the hindbrain and posterior cranial fossa (PCF). Traditional radiographic identification of CMI relies on the measurement of the cerebellar tonsils in relation to the foramen magnum with or without associated abnormalities of the neuraxis. The primary goal of this retrospective study was to comprehensively assess morphometric parameters above the McRea line in a group of female CMI patients and normal controls. MATERIAL AND METHODS: Twenty-nine morphological measurements were taken on 302 mid-sagittal MR images of adult female CMI patients (n=162) and healthy controls (n=140). All MR images were voluntarily provided by CMI subjects through an online database and control participant images were obtained through the Human Connectome Project and a local hospital system. RESULTS: Analyses were performed on the full dataset of adult female MR images and a restricted dataset of 229 participants that were equated for age, race, and body mass index. Eighteen group differences were identified in the PCF area that we grouped into three clusters; PCF structures heights, clivus angulation, and odontoid process irregularity. Fourteen group differences persisted after equating our CMI and control groups on demographic characteristics. CONCLUSION: PCF structures reliably differ in adult female CMI patients relative to healthy controls. These differences reflect structural abnormalities in the osseous and soft tissue structures of the clivus, odontoid process, and cerebellum. Clinical and pathophysiological implications are discussed.

Advanced MRI to differentiate schwannomas and metastases in the cerebellopontine angle/internal auditory canal.

BACKGROUND AND PURPOSE: Differentiating schwannomas and metastases in the cerebellopontine angles (CPA)/internal auditory canals (IAC) can be challenging. This study aimed to assess the role of diffusion-weighted imaging (DWI) and dynamic contrast-enhanced MRI (DCE-MRI) to differentiate schwannomas and metastases in the CPA/IAC. METHODS: We retrospectively reviewed 368 patients who were diagnosed with schwannomas or metastases in the CPA/IAC between April 2017 and February 2022 in a single academic center. Forty-three patients had pretreatment DWI and DCE-MRI along with conventional MRI. Normalized mean apparent diffusion coefficient ratio (nADCmean) and DCE-MRI parameters of fractional plasma volume (Vp), flux rate constant (Kep), and forward volume transfer constant were compared along with patients' demographics and conventional imaging features between schwannomas and metastases as appropriate. The diagnostic performances and multivariate logistic regression analysis were performed using the significantly different values. RESULTS: Between 23 schwannomas (15 males; median 48 years) and 20 metastases (9 males; median 61 years), nADCmean (median: 1.69 vs. 1.43; p = .002), Vp (median: 0.05 vs. 0.20; p < .001), and Kep (median: 0.41 vs. 0.81 minute-1 ; p < .001) were significantly different. The diagnostic performances of nADCmean, Vp, and Kep were 0.77, 0.90, and 0.83 area under the curves, with cutoff values of 1.68, 0.12, and 0.53, respectively. Vp was identified as the most significant parameter for the tumor differentiation in the multivariate logistic regression analysis (p < .001). CONCLUSIONS: DWI and DCE-MRI can help differentiate CPA/IAC schwannomas and metastases, and Vp is the most significant parameter.

Advanced Imaging and Computational Techniques for the Diagnostic and Prognostic Assessment of Malignant Gliomas.

ABSTRACT: Advanced imaging techniques provide a powerful tool to assess the intratumoral and intertumoral heterogeneity of gliomas. Advances in the molecular understanding of glioma subgroups may allow improved diagnostic assessment combining imaging and molecular tumor features, with enhanced prognostic utility and implications for patient treatment. In this article, a comprehensive overview of the physiologic basis for conventional and advanced imaging techniques is presented, and clinical applications before and after treatment are discussed. An introduction to the principles of radiomics and the advanced integration of imaging, clinical outcomes, and genomic data highlights the future potential for this field of research to better stratify and select patients for standard as well as investigational therapies.

Contrast Pediatric Brain Perfusion: Dynamic Susceptibility Contrast and Dynamic Contrast-Enhanced MR Imaging.

Magnetic resonance (MR) perfusion is a robust imaging technique that assesses the passage of blood through the cerebral vascular network using a variety of techniques. The applications of MR perfusion have been expanded and is well suited to investigate cerebrovascular diseases and cerebral neoplastic processes in pediatric patients. Assessment of brain perfusion can augment the information obtained on conventional MR imaging and provides additional information on the biological and physiologic features of pediatric brain tumors. Similarly, MR perfusion can help guide the management of a variety of pediatric cerebrovascular diseases, including acute ischemic stroke and Moyamoya syndrome.

Imaging Surveillance of Gliomas: Role of Basic and Advanced Imaging Techniques.

It is essential to be aware of widely accepted criteria for grading of treatment response in both high-grade and low-grade gliomas. These criteria primarily take into account responses of measurable and nonmeasurable lesions on T2-weighted, fluid-attenuated inversion recovery, and postcontrast images to determine a final category of response for the patient. The additional role that other advanced imaging techniques, such as diffusion and perfusion imaging, can play in the surveillance of these tumors is discussed in this article.

Brainstem hypoxic-ischemic lesions on MRI in infants treated with therapeutic cooling: effects on the length of stay and mortality.

OBJECTIVE: To test the hypothesis that brainstem hypoxic-ischemic injury on magnetic resonance imaging (MRI) would be independently associated with short-term outcomes in cooled asphyxiated infants. METHODS: A total of 90 consecutively cooled asphyxiated infants who survived to have brain MRI were reviewed. A neuroradiologist who was masked to outcomes evaluated MRI images for brainstem involvement. Outcomes were mortality and length of stay. RESULTS: Brainstem lesions were present on post-cooling brain MRI in 20 of the 90 infants (22%). Overall, four infants died before discharge, and all four had brainstem involvement. The infants with brainstem involvement had longer hospital stay (29 days, IQR 20-47 versus 16 days, IQR 10-26; P = 0.0001), compared to infants without brainstem lesions (n = 70); and upon multivariate analysis, brainstem involvement remained independently associated with prolonged hospital stay (ß = 12.4, P = 0.001). CONCLUSION: This study demonstrates the importance of recognizing brainstem injury for the prediction of short-term outcomes in cooled asphyxiated infants.

Stratification by Tumor Grade Groups in a Holistic Evaluation of Machine Learning for Brain Tumor Segmentation.

Accurate and consistent segmentation plays an important role in the diagnosis, treatment planning, and monitoring of both High Grade Glioma (HGG), including Glioblastoma Multiforme (GBM), and Low Grade Glioma (LGG). Accuracy of segmentation can be affected by the imaging presentation of glioma, which greatly varies between the two tumor grade groups. In recent years, researchers have used Machine Learning (ML) to segment tumor rapidly and consistently, as compared to manual segmentation. However, existing ML validation relies heavily on computing summary statistics and rarely tests the generalizability of an algorithm on clinically heterogeneous data. In this work, our goal is to investigate how to holistically evaluate the performance of ML algorithms on a brain tumor segmentation task. We address the need for rigorous evaluation of ML algorithms and present four axes of model evaluation-diagnostic performance, model confidence, robustness, and data quality. We perform a comprehensive evaluation of a glioma segmentation ML algorithm by stratifying data by specific tumor grade groups (GBM and LGG) and evaluate these algorithms on each of the four axes. The main takeaways of our work are-(1) ML algorithms need to be evaluated on out-of-distribution data to assess generalizability, reflective of tumor heterogeneity. (2) Segmentation metrics alone are limited to evaluate the errors made by ML algorithms and their describe their consequences. (3) Adoption of tools in other domains such as robustness (adversarial attacks) and model uncertainty (prediction intervals) lead to a more comprehensive performance evaluation. Such a holistic evaluation framework could shed light on an algorithm's clinical utility and help it evolve into a more clinically valuable tool.

Morphometric changes at the craniocervical junction during childhood.

OBJECTIVE: Current understanding of how the pediatric craniocervical junction develops remains incomplete. Measurements of anatomical relationships at the craniocervical junction can influence clinical and surgical decision-making. The purpose of this analysis was to quantitatively define clinically relevant craniocervical junction measurements in a population of children with CT scans that show normal anatomy. METHODS: A total of 1458 eligible patients were identified from children between 1 and 18 years of age who underwent cervical spine CT scanning at a single institution. Patients were separated by both sex and age in years into 34 groups. Following this, patients within each group were randomly selected for inclusion until a target of 15 patients in each group had been reached. Each patient underwent measurement of the occipital condyle-C1 interval (CCI), pB-C2, atlantodental interval (ADI), basion-dens interval (BDI), basion-opisthion diameter (BOD), basion-axial interval (BAI), dens angulation, and canal diameter at C1. Mean values were calculated in each group. Each measurement was performed by two teams and compared for intraclass correlation coefficient (ICC). RESULTS: The data showed that CCI, ADI, BDI, and dens angulation decrease in magnitude throughout childhood, while pB-C2, PADI, BAI, and BOD increase throughout childhood, with an ICC of fair to good (range 0.413-0.912). Notably, CCI decreases continuously on coronal CT scans, whereas on parasagittal CT scans, CCI does not decrease until after age 9, when it shows a continuous decline similar to measurements on coronal CT scans. CONCLUSIONS: These morphometric analyses establish parameters for normal pediatric craniocervical spine growth for each year of life up to 18 years. The data should be considered when evaluating children for potential surgical intervention.

Evidence for sex differences in morphological abnormalities in type I Chiari malformation.

BACKGROUND AND PURPOSE: Relatively little is known about the influence of individual difference variables on the presentation of macro-level brain morphology in type I Chiari malformation (CMI). The goal of the present study is to examine how case-control differences in Chiari are affected by patient sex. MATERIALS AND METHODS: Patient-provided magnetic resonance images were acquired through the Chiari 1000 database. Twenty-four morphometric measurements were taken using mid-sagittal images of 104 participants (26 male CMI, 26 female CMI, 26 male controls, and 26 female controls) using internally developed and validated custom software, Morphpro. Case-control comparisons were conducted separately by sex using healthy controls matched by age and body mass index. Probability-based t-tests, effect sizes (Cohen's d), and confidence intervals were used to compare case-control differences separately by sex. RESULTS: Male and female case-control comparisons yielded largely the same trends of CMI-related morphometric abnormalities. Both groups yielded reductions in posterior cranial fossa (PCF) structure heights. However, there was evidence for greater PCF structure height reductions in male CMI patients as measured by Cohen's d. CONCLUSIONS: Case-control differences indicated strong consistency in the morphometric abnormalities of CMI malformation in males and females. However, despite the higher prevalence rates of CMI in females, the results from the present study suggest that male morphometric abnormalities may be greater in magnitude. These findings also provide insight into the inconsistent findings from previous morphometric studies of CMI and emphasize the importance of controlling for individual differences when conducting case-control comparisons in CMI.

Type I Chiari malformation, RBANS performance, and brain morphology: Connecting the dots on cognition and macrolevel brain structure.

BACKGROUND: Idiopathic descent of cerebellar tonsils into the cervical spine in Chiari malformation Type I (CMI) is typically associated with occipital headache. Accumulating evidence from experimental studies suggests cognitive effects of CMI. The aim of the current study was to examine the relationship between cognition and CMI using a battery of standardized neuropsychological and symptom inventory instruments. METHOD: Eighteen untreated adults with CMI, and 18 gender, age, and education matched healthy controls completed the Repeatable Battery for the Assessment of Neuropsychological Status (RBANS), and standardized measures of pain, mood, and disability. Morphometric measurements of key neural and osseous elements were also obtained from structural brain magnetic resonance images, for correlation with symptom outcomes. RESULTS: CMI patients exhibited deficits in RBANS attention, immediate memory, delayed memory, and total score. After controlling for pain and associated affective disturbance, the significant group effect for RBANS attention remained. CMI patients also presented seven morphometric differences comprising the cerebellum and posterior cranial fossa compartment that differed from healthy controls, some of which were associated with self-reported pain and disability. Notably, group differences in tonsillar position were associated with self-reported pain, disability, and delayed memory. CONCLUSION: Adult CMI is associated with domain-specific cognitive change, detectable using a standard clinical instrument. The extent of cognitive impairment is independent of pain or affective symptomatology and may be related to the key pathognomonic feature of the condition. (PsycINFO Database Record (c) 2019 APA, all rights reserved).

Predicting the need for home gavage or g-tube feeds in asphyxiated neonates treated with therapeutic hypothermia.

BACKGROUND: Asphyxiated infants treated with therapeutic cooling can have persistent oral feeding difficulty because of involvement of neural pathways in the brainstem, cortex, and basal ganglia. The goal is to predict the composite adverse outcome of death or persistent oral feeding difficulty using precooling/cooling attributes, and the severity and distribution of hypoxic-ischemic lesions, especially brainstem lesions on post-cooling brain magnetic resonance imaging (MRI). METHODS: Retrospective review of 86 asphyxiated infants cooled from January 2006 to August 2014. Persistent feeding difficulty was defined as needing feeding support (gastrostomy tube (g-tube) or home gavage feeds) after discharge. Clinical and laboratory risk factors, and the brain MRI abnormalities including the presence of brainstem lesions were compared between infants with and without adverse outcome using univariate analysis. Significant variables were then analyzed in a stepwise logistic regression (LR) model. RESULTS: Infants with adverse outcome (n = 31, 4 died pre-discharge) had longer hospital stay (26 days, interquartile range (IQR) 19-43 vs. 13 days, IQR 9-20; p < 0.01) and reached goal enteral feeds (oral/gavage) later (11 days vs. 8 days, p < 0.01) compared to 55 infants discharged on full oral feeds. The former infants were more likely to have cord pH ≤ 7.15, severely abnormal neurological examination, bleeding diathesis, continued need for ventilation, and positive MRI findings including brainstem lesions. In LR analysis, brainstem lesions on MRI (p = 0.00, odds ratio 19, 95% confidence interval 4-85) was independently associated with the adverse outcome. CONCLUSIONS: Brainstem involvement on post-cooling brain MRI was predictive of adverse outcome. Early identification of these infants may facilitate discussion of home feeding plans between clinicians and parents earlier, thereby potentially reducing the length of hospital stay.

A clinicoradiologic analysis of symptomatic craniofacial fibro-osseous lesions.

OBJECTIVE: To outline the clinical and radiologic aspects of symptomatic craniofacial fibro-osseous lesions and to study the appropriate surgical management with follow-up results. STUDY DESIGN AND SETTING: A retrospective review of 11 patients who underwent surgical treatment during 1985 to 2004 in a tertiary health care center. RESULTS: Most (72.7%) cases were of fibrous dysplasia and were under 25 years of age (72%). Maxilla was the most common bone involved (81%). Lateral rhinotomy was the main approach for surgical resection. One patient underwent bilateral optic nerve decompression (left side therapeutic and on right side prophylactic). Six (54.5%) cases had recurrences that were managed by either radical surgery (total maxillectomy) or by further shaving off the lesion. CONCLUSION: Treatment of craniofacial fibro-osseous lesions is highly individualized. A conservative approach may not be able to treat all cases of craniofacial fibro-osseous lesions. A more radical approach that includes a craniofacial resection or a total maxillectomy may be warranted in few cases.