Search

The study aimed to describe a case of a 7-year-old Caucasian girl who developed isolated chorea in her right upper and lower extremities progressively increasing over 2 years. This study is a case report and conducted at tertiary care center. A 7-year-old Caucasian girl who gradually developed worsening choreiform movements in her right upper and lower extremities over the course of 2 years until medical attention was sought. Literature review of children presenting with chorea as the only manifestation in moyamoya disease. A 7-year-old right-handed Caucasian girl presented with progressively worsening choreiform movements in her right upper and lower extremities affecting her fine motor skills and gait impairment There was no weakness, hyperreflexia, or spasticity on her neurological exam. Neuroimaging studies showed “ivy sign,” asymmetric prominence of vessels within the subarachnoid spaces overlying the left cerebral hemisphere with corresponding serpiginous T2 fluid-attenuated inversion recovery abnormality, but no parenchymal volume loss or diffusion restriction. Magnetic resonance angiography showed focal moderate-to-severe stenosis at the junction of the paraophthalmic and supraclinoid segments of the left internal carotid artery, with poststenotic dilatation. Angiography demonstrated focal stenosis involving the ophthalmic segment of the left internal carotid artery with 50% stenosis. There was compensation through a medium sized left posterior communicating artery as well as posterior cerebral artery to middle cerebral artery and anterior cerebral artery collateralization. Brain single-photon emission computerized tomography scan showed no evidence of perfusion defects in the cerebral hemispheres, basal ganglia, or thalami. She underwent successful revascularization procedure (left pial synangiosis) with resolution of her choreiform movements and normalization of her gait. Our case demonstrates that moyamoya disease should be suspected when evaluating a child with hemichorea and describes resolution of symptoms after revascularization surgery. Neuroimaging and vascular studies should be obtained in children with unilateral movement disorder.

OBJECTIVEBiopsies of brainstem lesions are performed to establish a diagnosis in the setting of an atypical clinical or radiological presentation, or to facilitate molecular studies. A better understanding of the safety and diagnostic yield of brainstem biopsies would help guide appropriate patient selection.METHODSAll patients who underwent biopsy of a brainstem lesion during the period from January 2011 to June 2019 were reviewed. Demographic, radiological, surgical, and outcome data were collected.RESULTSA total of 58 patients underwent 65 brainstem biopsies during the study period. Overall, the median age was 7.6 years (IQR 3.9–14.2 years). Twenty-two of the 65 biopsies (34%) were open, 42 (65%) were stereotactic, and 1 was endoscopic. In 3 cases (5%), a ventriculoperitoneal shunt was placed, and in 9 cases (14%), a posterior fossa decompression was performed during the same operative session as the biopsy. An intraoperative MRI (iMRI) was performed in 28 cases (43%). In 3 of these cases (11%), the biopsy was off target and additional samples were obtained during the same procedure. New neurological deficits were noted in 5 cases (8%), including sensory deficits, ophthalmoparesis/nystagmus, facial weakness, and hearing loss; these deficits persisted in 2 cases and were transient in 3 cases. A pseudomeningocele occurred in 1 patient; no patients developed a CSF leak or infection. In 8 cases (13%) an additional procedure was needed to obtain a diagnosis.CONCLUSIONSBrainstem biopsies are safe and effective. Target selection and approach should be a collaborative effort. iMRI can be used to assess biopsy accuracy in real time, thereby allowing any adjustment if necessary.

Transcranial magnetic stimulation (TMS) is a newer noninvasive language mapping tool that is safe and well-tolerated by children. We examined the accuracy of TMS-derived language maps in a clinical cohort by comparing it against functional magnetic resonance imaging (MRI)–derived language map. The number of TMS-induced speech disruptions and the volume of activation during functional MRI tasks were localized to Brodmann areas for each modality in 40 patients with epilepsy or brain tumor. We examined the concordance between TMS- and functional MRI–derived language maps by deriving statistical performance metrics for TMS including sensitivity, specificity, accuracy, and diagnostic odds ratio. Brodmann areas 6, 44, and 9 in the frontal lobe and 22 and 40 in the temporal lobe were the most commonly identified language areas by both modalities. Overall accuracy of TMS compared to functional MRI in localizing language cortex was 71%, with a diagnostic odds ratio of 1.27 and higher sensitivity when identifying left hemisphere regions. TMS was more accurate in determining the dominant hemisphere for language with a diagnostic odds ratio of 6. This study is the first to examine the accuracy of the whole brain language map derived by TMS in the largest cohort examined to date. While this comparison against functional MRI confirmed that TMS reliably localizes cortical areas that are not essential for speech function, it demonstrated only slight concordance between TMS- and functional MRI–derived language areas. That the localization of specific language cortices by TMS demonstrated low accuracy reveals a potential need to use concordant tasks between the modalities and other avenues for further optimization of TMS parameters.

OBJECTIVEStenoocclusive cerebral vasculopathy is an infrequent delayed complication of ionizing radiation. It has been well described with photon-based radiation therapy but less so following proton-beam radiotherapy. The authors report their recent institutional experience in evaluating and treating children with radiation-induced cerebral vasculopathy.METHODSEligible patients were age 21 years or younger who had a history of cranial radiation and subsequently developed vascular narrowing detected by MR arteriography that was significant enough to warrant cerebral angiography, with or without ischemic symptoms. The study period was January 2011 to March 2019.RESULTSThirty-one patients met the study inclusion criteria. Their median age was 12 years, and 18 (58%) were male. Proton-beam radiation therapy was used in 20 patients (64.5%) and photon-based radiation therapy was used in 11 patients (35.5%). Patients were most commonly referred for workup as a result of incidental findings on surveillance tumor imaging (n = 23; 74.2%). Proton-beam patients had a shorter median time from radiotherapy to catheter angiography (24.1 months [IQR 16.8–35.4 months]) than patients who underwent photon-based radiation therapy (48.2 months [IQR 26.6–61.1 months]; p = 0.04). Eighteen hemispheres were revascularized in 15 patients. One surgical patient suffered a contralateral hemispheric infarct 2 weeks after revascularization; no child treated medically (aspirin) has had a stroke to date. The median follow-up duration was 29.2 months (IQR 21.8–54.0 months) from the date of the first catheter angiogram to last clinic visit.CONCLUSIONSAll children who receive cranial radiation therapy from any source, particularly if the parasellar region was involved and the child was young at the time of treatment, require close surveillance for the development of vasculopathy. A structured and detailed evaluation is necessary to determine optimal treatment.

Cortical stimulation mapping is the gold standard for presurgical language mapping; however, it cannot be reliably performed in very young patients. Language mapping using noninvasive modalities is also challenging in very young patients. Although utility of language mapping using power of high-gamma in electrocorticographic recordings was demonstrated in adults and older children, there is a gap of knowledge in the ability of this procedure for localizing language-specific cortex in very young patients. We describe a case of a 2-year-old patient who, to our knowledge, is the youngest person to undergo successful high-gamma electrocorticographic presurgical language mapping for localization of the expressive language cortex (Broca area). The surgical plan was to resect a cortical tuber within the left inferior frontal gyrus and there was a strong concern about postoperative language deficit after resection. Presurgical language mapping using noninvasive modalities were attempted without success. Cortical stimulation mapping was not feasible in this patient. Therefore, high-gamma electrocorticography was the only viable option for language mapping, and it successfully localized the expressive language cortex. The patient underwent surgery for resection of the IFG tuber based on results of high-gamma electrocorticography and had no postoperative language deficit. High-gamma electrocorticography can be used for localizing language-specific cortex, especially Broca’s area, in very young patients.

Introduction Flat panel imaging for emergent large vessel occlusion can be acquired prior to mechanical thrombectomy (MT). In this study, we examined patients undergoing MT with computed tomography angiography (CTA) to determine agreement on the site of occlusion and CTA collateral score (CS). Methods Flat Panel CTA (FP-CTA) was acquired before MT. Time between CTA and FP-CTA acquisition, site of occlusion, and CS were reported. Significant CS change was defined as >2-point change, or any change to/from a malignant profile (CS = 0 to CS > 0, or vice versa). Results Eleven patients (mean age, 60.8 years; NIHSS, 17; 55.0% female) were included; IV tPA was administered to 7. Intra-reader occlusion site, dichotomous CS, and continuous CS correlation between CTA and FP-CTA were 96.6%, 90.0%, and 86.6%, respectively. Inter-reader correlation for occlusion site was 93% for CTA and 100% for FP-CTA; dichotomous CS correlation was 87% for both CTA and FP-CTA; correlation of continuous CS was 77% for CTA and 87% for FP-CTA. Conclusion Standard CTA and FP-CTA have high intra and inter-reader correlation determining site of occlusion and CS in ELVO setting. This angiographic tool may have potential applications for both triage and patient selection.

Tinnitus is the perception of sound in the absence of an external source. It is a common symptom that can be related to hearing loss and other benign causes. However, tinnitus may be disabling and can be the only symptom in a patient with a central nervous system process disorder. History and physical examination are crucial first steps to determine the need for imaging. CT and MRI are useful in the setting of pulsatile tinnitus to evaluate for an underlying vascular anomaly or abnormality. If there is concomitant asymmetric hearing loss, neurologic deficit, or head trauma, imaging should be guided by those respective ACR Appropriateness Criteria® documents, rather than the presence of tinnitus. Imaging is not usually appropriate in the evaluation of subjective, nonpulsatile tinnitus that does not localize to one ear. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

MRI without and with contrast is the most accurate imaging method to determine whether a process is intrinsic or extrinsic to a nerve of the brachial or lumbosacral plexus. However, there are no Current Procedural Terminology codes to correspond to imaging studies of the brachial or lumbar plexus discretely. This assessment uses "MRI of the brachial plexus" or "MRI of the lumbosacral plexus" as independent entities given that imaging acquisition for the respective plexus differs in sequences and planes compared with those of a routine neck, chest, spine, or pelvic MRI, yet acknowledges the potential variability of ordering practices across institutions. In patients unable to undergo MRI, CT offers the next highest level of anatomic evaluation. In oncologic patients, PET/CT imaging can identify the extent of tumor involvement and be beneficial to differentiate radiation plexitis from tumor recurrence but provides limited resolution of the plexus itself. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer-reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

BACKGROUND Pineoblastomas are rare, supratentorial, primitive neuroectodermal tumors. OBJECTIVE To document outcomes with multimodal therapy and evaluate the impact that the degree of surgical resection has on outcome. METHODS A departmental brain tumor database was queried to identify all patients with pathologically proven pineoblastoma who were treated from January 1997 to June 2015 at St. Jude Children's Research Hospital. For each patient, we recorded demographic, pathological, radiological, surgical, and clinical follow-up data. The effect of degree of surgical resection on survival outcomes was analyzed. RESULTS Forty-one patients (21 male, 20 female) treated for pineoblastoma were identified. The median age at diagnosis was 5.5 years (range 0.4-28.1) and the median follow-up was 34.5 months. Nineteen patients experienced tumor relapse with a median progression-free survival of 11.3 months, and 18 ultimately succumbed to their disease. Patients who died or experienced treatment failure were younger (median, 2.69 vs 6.5 years, P = .026) and more likely to have metastatic disease at diagnosis (12 [63.2%] vs 5 [22.7%], P = .012). When analyzing only patients 5 years of age or older with focal disease at presentation, those who had a gross total resection or near-total resection-compared with subtotal resection or biopsy-had greater overall survival (75.18 vs 48.57 months), with no patients dying as a result of their cancer. CONCLUSION Poor prognostic variables for children with pineoblastoma include young age, metastatic disease at presentation, and tumor relapse. For patients older than 5 years with focal disease, maximal tumor resection should be the goal.

Accurate assessment of motor function is critical, particularly in the context of presurgical functional mapping. There are a few noninvasive tools available for preoperative motor mapping in older children and adults undergoing surgery for brain tumor or refractory epilepsy including fMRI, magnetoencephalography (MEG), and transcranial magnetic stimulation (TMS).1,2 However, assessing motor function is challenging in very young children. Direct cortical stimulation, a standard procedure in adults, is associated with a high incidence of intraoperative seizures, and is often unsuccessful in young children.3,4 Alternately, noninvasive motor mapping using MEG and fMRI attempted during sleep or under sedation primarily localize the somatosensory cortex from which the location of motor cortex is inferred.5,6 Furthermore, these methods have a low success rate (∼25%) and have risks associated with sedation.6 However, TMS has the advantage of mapping the motor cortex directly without requiring sedation. We have previously reported on successful mapping of the motor cortex in young children aged between 17 and 35 months.7 Here we describe a case of an 11-month-old infant who is, to our knowledge, the youngest person to undergo successful presurgical motor mapping with TMS.

Background and importance Presurgical mapping of eloquent cortex in young patients undergoing neurosurgery is critical for timely intervention, surgical planning, and minimizing postoperative deficits. However, invasive direct cortical stimulation has limited success in young children and noninvasive modalities, such as magnetoencephalography and functional MRI, require sedation, often precluding localization of critical language cortices. Transcranial magnetic stimulation (TMS), a noninvasive brain stimulation technique, is well suited to evaluate language areas in young children because it does not require the patient to remain still during mapping. Clinical presentation A 4-yr and 11-mo-old female patient diagnosed with epilepsia partialis continua of the right arm and face and right-sided weakness was evaluated at our institution. MRI findings and clinical examination led to the diagnosis of Rasmussen encephalitis involving left frontal lobe and insula. Language cortices were successfully identified in both hemispheres using TMS. The TMS findings aided in discussing with the family the risks of postsurgical deficits of left functional hemispherectomy, the definitive treatment for Rasmussen encephalitis. Postoperatively, the patient had intact speech and was seizure free. Conclusion We illustrate the feasibility and utility of TMS as a noninvasive functional mapping tool in this young child. The preoperative demonstration of bilateral language organization indicated a greater likelihood of preserved language functions postsurgery. We demonstrate that TMS is a safe and noninvasive tool to map language cortices in young children with serious epilepsy syndromes.

OBJECTMalignant tumors of the brainstem, excluding classic diffuse intrinsic pontine gliomas (DIPGs), are a very rare, heterogeneous group of neoplasms that have been infrequently described in the literature. In this paper, the authors present their experiences with treating these unique cancers.METHODSA retrospective chart review was conducted to identify eligible cases over a 15-year period. All tumors involving the pons were, by consensus, felt not to be DIPGs based on their neuroimaging features. Demographic information, pathological specimens, neuroimaging characteristics, surgical and nonsurgical management plans, and survival data were gathered for analysis.RESULTSBetween January 2000 and December 2014, 29 patients were identified. The mean age at diagnosis was 8.4 years (range 2 months to 25 years), and 17 (59%) patients were male. The most common presenting signs and symptoms were cranial neuropathies (n = 24; 83%), hemiparesis (n = 12; 41%), and ataxia or gait disturbance (n = 10; 34%). There were 18 glial and 11 embryonal tumors. Of the glial tumors, 5 were radiation-induced and 1 was a malignant transformation of a previously known low-grade tumor. Surgical intervention consisted of biopsy alone in 12 patients and some degree of resection in another 15 patients. Two tumors were diagnosed postmortem. The median overall survival for all patients was 196 days (range 15 to 3999 days). There are currently 5 (17%) patients who are still alive: 1 with an anaplastic astrocytoma and the remaining with embryonal tumors.CONCLUSIONSIn general, malignant non-DIPG tumors of the brainstem carry a poor prognosis. However, maximal cytoreductive surgery may be an option for select patients with focal tumors. Long-term survival is possible in patients with nonmetastatic embryonal tumors after multimodal treatment, most importantly maximal resection.

Background Weakness of the sixth cranial nerve is the most common cause of an ocular motor cranial nerve palsy. It is often difficult to identify a corresponding abnormality on neuroimaging to correlate with the clinical examination. Evidence acquisition High-resolution 3D skull base magnetic resonance imaging (MRI) allows for visualization of the sixth nerve along much of its course and may increase sensitivity for abnormalities in regions that previously were challenging to evaluate. In this review, the authors share their experience with high-resolution imaging of the sixth nerve. Results For each segment, anatomic features visible on high-resolution imaging are described along with relevant pathologic entities. Conclusions We present a segmental approach to high-resolution 3D MRI for evaluation of the sixth nerve from the nuclear to the orbital segment.

OBJECT Cervicomedullary tumors (CMTs) represent a heterogeneous group of intrinsic neoplasms that are typically low grade and generally carry a good prognosis. This single-institution study was undertaken to document the outcomes and current treatment philosophy for these challenging neoplasms. METHODS The charts of all pediatric patients with CMTs who received treatment at St. Jude Children’s Research Hospital between January 1988 and May 2013 were retrospectively reviewed. Demographic, surgical, clinical, radiological, pathological, and survival data were collected. Treatment-free survival and overall survival were estimated, and predictors of recurrence were analyzed. RESULTS Thirty-one children (16 boys, 15 girls) with at least 12 months of follow-up data were identified. The median age at diagnosis was 6 years (range 7 months-17 years) and the median follow-up was 4.3 years. Low-grade tumors (Grade I or II) were present in 26 (84%) patients. Thirty patients underwent either a biopsy alone or resection, with the majority of patients undergoing biopsy only (n = 12, 39%) or subtotal resection (n = 14, 45%). Only 4 patients were treated solely with resection; 21 patients received radiotherapy alone or in combination with other treatments. Recurrent tumor developed in 14 children (45%) and 4 died as a result of their malignancy. A high-grade pathological type was the only independent variable that predicted recurrence. The 5- and 10-year treatment-free survival estimates are 64.7% and 45.3%, respectively. The 5- and 10-year overall survival estimate is 86.7%. CONCLUSIONS Children with CMTs typically have low-grade neoplasms and consequently long-term survival, but high risk of recurrence. Therapy should be directed at achieving local tumor control while preserving and even restoring neurological function.

Despite the frequent occurrence of autism spectrum disorder (ASD) in tuberous sclerosis complex (TSC), the neu- rophysiological factors that distinguish children with TSC from children with TSC presenting with ASD symptoms remain unspecified. Growing evidence suggests that ASD may be characterized by atypical structural and functional connectivity be- tween specific cortical regions. In this exploratory study, we utilized magnetoencephalography to derive resting brain connec- tivity patterns, in an attempt to identify neurophysiological markers that may differentiate TSC children with ASD (n = 2) from TSC children without ASD (n = 2) and typically developing children (n = 2). Connectivity pattern analysis revealed that TSC children presenting with ASD symptoms can be distinguished from TSC and typically developing children by the presence of long-range, medial, anterior-posterior connections previously observed in adolescents with ASD. While preliminary in nature, our findings support the notion that altered functional connectivity may be a constituent characteristic of ASD and may enable prediction of which TSC children are likely to develop ASD and facilitate early behavioral and medical intervention.

Moyamoya disease and syndrome are typically described by their imaging features on cerebral angiography, however advances in noninvasive imaging has allowed improved anatomic and physiologic characterization of this vascular abnormality. Advanced imaging complements the role of cerebral angiography, and when used appropriately can provide valuable information regarding the diagnosis, treatment planning, and surveillance in patients with moyamoya. Techniques to be aware of include computed tomography, CT-angiography, Magnetic Resonance Imaging, MR Angiography (time-of-flight and dynamic contrast enhancemed angiography), and perfusion imaging (CT perfusion, arterial spin labeling perfusion, and nuclear medicine perfusion). With an appropriate understanding and implementation of noninvasive techniques, this disease process can be better understood allowing for improved patient management.

Identifying the normal relationship of the orbital rims to the globes is critical in planning surgical correction of craniofacial deformities affecting the orbit. This article illustrates a technical proof of principle and mathematical basis for a computed tomography-based measurement of the sagittal orbit-globe relationship. The technique does not require subject cooperation and is, therefore, optimal for pediatric craniofacial surgical treatment planning and outcome evaluation.

Objective To predict the postoperative language outcome using the support vector regression (SVR) and results of multimodal presurgical language mapping. Methods Eleven patients with epilepsy received presurgical language mapping using functional MRI (fMRI), magnetoencephalography (MEG), transcranial magnetic stimulation (TMS), and high-gamma electrocorticography (hgECoG), as well as pre- and postoperative neuropsychological evaluation of language. We constructed 15 (24–1) SVR models by considering the extent of resected language areas identified by all subsets of four modalities as input feature vector and the postoperative language outcome as output. We trained and cross-validated SVR models, and compared the cross-validation (CV) errors of all models for prediction of language outcome. Results Seven patients had some level of postoperative language decline and two of them had significant postoperative decline in naming. Some parts of language areas identified by four modalities were resected in these patients. We found that an SVR model consisting of fMRI, MEG, and hgECoG provided minimum CV error, although an SVR model consisting of fMRI and MEG was the optimal model that facilitated the best trade-off between model complexity and prediction accuracy. Conclusions A multimodal SVR can be used to predict the language outcome. Significance The developed multimodal SVR models in this study can be utilized to calculate the language outcomes of different resection plans prior to surgery and select the optimal surgical plan.

Introduction Flat Panel Imaging in the angiographic suite for patients with Emergent Large Vessel Occlusion (ELVO) can be acquired immediately prior to mechanical thrombectomy (MT). This can identifying patients who no longer need MT after long time delays or due to reperfusion with IV TPA. Additionally, these techniques may allow the angiography suite to serve as the initial diagnostic modality and treatment location for patients with ELVO. We performed a correlative study on patients who had received both acute stroke CT Angiography (CTA) and Flat Panel CTA (FP-CTA) to determine agreement on the site of occlusion and CTA collateral score (CS) in patients with ELVO. Methods This is a prospective pilot study of consecutive patients treated at a comprehensive stroke center. Patients with ELVO who underwent MT were studied with CTA in the emergency department on a Lightspeed VCT (GE Medical Systems). FP-CTA was then acquired before MT via an aortic arch pigtail injection. Post processing of images was performed on one of two interventional C-Arm CBCT systems: Siemens Artis Q Biplane or Zeego (Siemens Healthcare AG, Forchhiem Germany). Slice thickness was matched to ER CT thickness. Patient baseline characteristics and details of treatment and outcome were retrospectively collected. A blinded neuroradiologist read all paired CTA and FP-CTA datasets. Site of occlusion and the CS were rated and correlated. A change is CS between CTA and FP-CTA was defined as a greater than two point change. Time between CTA and FP-CTA acquisition was also recorded. Results Ten patients (mean age 60.8 years, NIHSS 17, 60% Female) were included in the study. IV TPA was administered to 70% of patients. There was 100% correlation of the site of occlusion: 8 MCA, 1 ICA, 1 Basilar. Average time between CTA and FP-CTA was 102 min (range 76–121). There was 90% correlation on CTA and FP-CTA CS with only a single patient with a 2 point decrease in CS. Conclusion FP-CTA correlates well with standard CTA in determining site of occlusion and CS in the setting of ELVO. This angiographic tool may have potential applications for both triage and patient selection of patients with ELVO. Disclosures L. Elijovich: 1; C; Siemens. 2; C; Stryker, Codman, Medtronic, Microvention, Penumbra. A. Choudhri: None. V. Inoa-Acosta: None. A. Arthur: 1; C; Siemens. 2; C; Microvention, Codman, Penumbra, Stryker, Medtronic. 4; C; Cerebrotech, Synchron. D. Hoit: 2; C; Medtronic, Microvention. C. Nickele: 1; C; Microvention. J. DiNitto: None.

Cortical Stimulation Mapping (CSM) and the Wada procedure have long been considered the gold standard for localizing motor and language-related cortical areas and for determining the language and memory-dominant hemisphere, respectively. In recent years, however, non-invasive methods such as magnetoencephalography (MEG), functional magnetic resonance imaging (fMRI), and transcranial magnetic stimulation (TMS) have emerged as promising alternatives to the aforementioned procedures, particularly in cases where the invasive localization of eloquent cortex has proven to be challenging. To illustrate this point, we will first introduce the evidence of the compatibility of invasive and non-invasive methods and subsequently outline the rationale and the conditions where the latter methods are applicable.

We describe a 14-year-old boy with a pilocytic astrocytoma of the left caudate head. Preoperative localization MR imaging (MRI) was performed in the operating room, and spatial distortion was noted felt to be related to head positioning relative to the isocenter of the magnetic field. The distortion artifact was subtle enough to be difficult to detect, but large enough to change the location of the lesion potentially leading to a non-diagnostic stereotactic biopsy. Repeat imaging after changing the head position to allow scanning closer to the isocenter of the magnetic field showed decreased distortion, an improvement greater than that using the manufacturer's distortion correction algorithm on the initial images. Intraoperative MRI, and its requisite limitations in positioning, requires vigilance to detect possible distortion that could alter surgical outcomes if not identified and corrected prospectively.

BACKGROUND AND PURPOSE: High-field-strength intraoperative MR imaging has emerged as a powerful adjunct for resection of brain tumors. However, its exact role has not been firmly established. We sought to determine the impact of 3T-intraoperative MRI on the surgical management of childhood CNS tumors. MATERIALS AND METHODS: We evaluated patient data from a single academic children's hospital during a consecutive 24-month period after installation of a 3T-intraoperative MRI. Tumor location, histology, surgical approach, operating room time, presence and volume of residual tumor, need for tumor and non-tumor-related reoperation, and anesthesia- and MR imaging–related complications were evaluated. Comparison with pre-intraoperative MRI controls was performed. RESULTS: One hundred ninety-four patients underwent intraoperative MRI–guided surgery. Of these, 168 were 18 years or younger (mean, 8.9 ± 5.0 years; 108 males/60 females). There were 65 posterior fossa tumors. The most common tumors were pilocytic astrocytoma ( n = 31, 19%), low-grade glioma ( n = 31, 19%), and medulloblastoma ( n = 20, 12%). An average of 1.2 scanning sessions was performed per patient (maximum, 3). There were no MR imaging–related safety issues. Additional tumor was resected after scanning in 21% of patients. Among patients with a preoperative goal of gross total resection, 93% achieved this goal. The 30-day reoperation rate was

Hypoxic-ischemic injury (HII) continues to be an important cause of neonatal mortality and morbidity. In recent years, the role of magnetic resonance (MR) imaging has increased by providing early detection to initiate preventive measures and assess the severity of tissue injury, and it often serves as a prognostic indicator. However, because of the subtle findings and temporal variability of signal abnormalities, the imaging diagnosis often remains troublesome, particularly for trainees and general radiologists who do not often encounter these findings. The imaging manifestations between term and preterm infants differ significantly; the imaging findings in term neonates are discussed. Two main patterns of HII have been described in term neonates: peripheral and basal ganglia-thalamus, with the predominant pattern in an affected infant dependent on the duration and severity of the insult. The peripheral pattern occurs in the setting of mild hypoxia or ischemia of prolonged duration, with predominant findings in the cerebral cortex and subcortical white matter along the intervascular boundary zones. The basal ganglia-thalamus pattern is most often secondary to a more severe hypoxic or ischemic event of short duration and manifests with abnormal hyperintensity on T1-weighted images and hypointensity on T2-weighted images in the posterolateral putamen and ventrolateral thalamus. Associated loss of normal hyperintensity on T1-weighted images and hypointensity on T2-weighted images in the posterior limb of the internal capsule may be present. Restricted diffusion and evolution of imaging findings may be seen in each of these regions, depending on when images are obtained. Advanced imaging techniques, including MR spectroscopy, may add valuable information and specificity, with an abnormal lactate peak often serving as an indicator of HII in term neonates.

Advances in functional imaging have provided non-invasive techniques to probe brain organization of multiple constructs including language and memory. Because of high overall rates of agreements with older techniques, including Wada testing and cortical stimulation mapping (CSM), some have proposed that those approaches should be largely abandoned because of their invasiveness, and replaced with non-invasive functional imaging methods. High overall agreement, however, is based largely on concordant language lateralization in series dominated by cases of typical cerebral dominance. Advocating a universal switch from Wada testing and cortical stimulation mapping to fMRI or magnetoencephalography (MEG) ignores the differences in specific expertise across epilepsy centers, many of which often have greater skill with one approach rather than the other, and that Wada, CSM, fMRI, and MEG protocols vary across institutions resulting in different outcomes and reliability. Specific patient characteristics also affect whether Wada or CSM might influence surgical management, making it difficult to accept broad recommendations against currently useful clinical tools. Although the development of non-invasive techniques has diminished the frequency of more invasive approaches, advocating their use to replace Wada testing and CSM across all epilepsy surgery programs without consideration of the different skills, protocols, and expertise at any given center site is ill-advised.

Multiple sclerosis (MS) and its variants are inflammatory as well as neurodegenerative diseases that diffusely affect the central nervous system (CNS). There is a poor correlation between traditional imaging findings and symptoms in patients with MS. Current research in conventional magnetic resonance (MR) imaging of MS and related diseases includes optimization of hardware and pulse sequences and the development of automated and semiautomated techniques to measure and quantify disease burden. Advanced nonconventional MR techniques such as diffusion tensor and functional MR imaging probe the changes found in the CNS, and correlate these findings with clinical measures of disease.

The authors review the course and appearance of the major segments of the upper cranial nerves from their apparent origin at the brainstem through the proximal extraforaminal region, focusing on the imaging and anatomic features of particular relevance to high-resolution magnetic resonance imaging evaluation. Selected pathologic entities are included in the discussion of the corresponding cranial nerve segments for illustrative purposes.

Intramedullary spinal cord neoplasms (ISCN) in children provide diagnostic, treatment and management dilemmas. Resection results in the best chance for disease control, but the greatest risk of neurologic deficit. We hypothesize that diffusion tensor imaging (DTI) and diffusion tensor-fiber tracking (DT-FT) can help characterize margins of pediatric ISCN to aid in surgical planning. This HIPAA compliant retrospective study was performed after Institutional Review Board approval. Patients with ISCN from a single tertiary care pediatric institution were identified, and patients with preoperative DTI were evaluated. Ten patients (eight males and two females) with ISCN with preoperative DTI were identified. The mean age was 11.1 ± 6.2 years (range, 2–18 years). Eight tumors demonstrated DTI and DT-FT evidence of splayed cord tracts, and two demonstrated evidence of infiltration of cord tracts. The eight patients with splayed tracts underwent resection, with seven achieving gross-total resection and one subtotal resection. The two patients with infiltration of white matter tracts underwent biopsy of their lesion. DTI of pediatric ISCN can aid in defining the margins of the tumor and relationship to the intrinsic white matter structures of the spinal cord. Splaying and displacement of fiber tracts appears to predict a discrete margin to the tumor and resectability, whereas infiltration of the white matter tracts suggests biopsy may be more advisable.

In 1976 an infant boy initially presented with a choroidal vein of Galen malformation (cVOGM). Craniotomy and clipping of the anterior cerebral artery and posterior choroidal arteries was performed, which was reported in the literature 40 years ago. The patient remained asymptomatic until age 42 when he re-presented with an isolated intraventricular hemorrhage. Angiography demonstrated cVOGM with venous sinus occlusive disease leading to venous hypertension and subsequent intraventricular hemorrhage. The angiogram also demonstrated a ‘pseudo-nidus’ composed of multiple arterial-to-arterial anastomoses that had developed as a result of the original surgical treatment. We embolized a portion of the lesion to reduce the venous hypertension. The patient has been asymptomatic for more than 2 years. To our knowledge, this is the first reported case of a symptomatic neonatal VOGM treated with open surgery that required embolization as an adult for a delayed hemorrhagic presentation.

Background Tics and Tourette syndrome are common problems evaluated by both the general pediatrician and pediatric neurologist. The common comorbidities of tics are well known, but the severe neurological complications are rare and may not be appreciated. Methods This is a retrospective case series and literature review. Results We present here four adolescents with Tourette syndrome who had severe neurological complications secondary to motor tics. We provide the history, neurological examination, and radiological findings in addition to a review of previously reported cases of vascular and cervical cord complications associated with violent motor tics. Conclusions We highlight the importance of recognizing the presenting signs of these complications early and the need to vigorously treat violent motor tics to prevent significant neurological complications.

This article provides a summary of how to approach the imaging analysis of lesions of the anterior, central, and posterior skull base. The primary focus is tumors and tumor-mimickers, and representative examples are shown to differentiate the features of lesions that can occur in the same location.

Objectives The purpose of our study was to conduct an evidence-based evaluation of stress cardiac magnetic resonance imaging (MRI) in the diagnosis of coronary artery disease (CAD). Background Stress cardiac MRI has recently emerged as a noninvasive method in the detection of CAD, with 2 main techniques in use: 1) perfusion imaging; and 2) stress-induced wall motion abnormalities imaging. Methods We examined studies from January 1990 to January 2007 using MEDLINE and EMBASE. A study was included if it: 1) used stress MRI as a diagnostic test for CAD (≥50% diameter stenosis); and 2) used catheter X-ray angiography as the reference standard. Results Thirty-seven studies (2,191 patients) met the inclusion criteria, with 14 datasets (754 patients) using stress-induced wall motion abnormalities imaging and 24 datasets (1,516 patients) using perfusion imaging. Stress-induced wall motion abnormalities imaging demonstrated a sensitivity of 0.83 (95% confidence interval [CI] 0.79 to 0.88) and specificity of 0.86 (95% CI 0.81 to 0.91) on a patient level (disease prevalence = 70.5%). Perfusion imaging demonstrated a sensitivity of 0.91 (95% CI 0.88 to 0.94) and specificity of 0.81 (95% CI 0.77 to 0.85) on a patient level (disease prevalence = 57.4%). Conclusions In studies with high disease prevalence, stress cardiac MRI, using either technique, demonstrates overall good sensitivity and specificity for the diagnosis of CAD. However, limited data are available regarding use of either technique in populations with low disease prevalence.

Recent advances in prenatal imaging have made possible the in utero diagnosis of cleft lip and palate and associated deformities. Postnatal diagnosis of cleft lip is made clinically, but imaging still plays a role in detection of associated abnormalities, surgical treatment planning, and screening for or surveillance of secondary deformities. This article describes the clinical entities of cleft lip with or without cleft palate (CLP) and isolated cleft palate and documents their prenatal and postnatal appearances at radiography, ultrasonography (US), magnetic resonance (MR) imaging, and computed tomography (CT). Imaging protocols and findings for prenatal screening, detection of associated anomalies, and evaluation of secondary deformities throughout life are described and illustrated. CLP and isolated cleft palate are distinct entities with shared radiologic appearances. Prenatal US and MR imaging can depict clefting of the lip or palate and associated anomalies. While two- and three-dimensional US often can depict cleft lip, visualization of cleft palate is more difficult, and repeat US or fetal MR imaging should be performed if cleft palate is suspected. Postnatal imaging can assist in identifying associated abnormalities and dentofacial deformities. Dentofacial sequelae of cleft lip and palate include missing and supernumerary teeth, oronasal fistulas, velopharyngeal insufficiency, hearing loss, maxillary growth restriction, and airway abnormalities. Secondary deformities can often be found incidentally at imaging performed for other purposes, but detection is necessary because they may have considerable implications for the patient.

Objective To describe and analyze the chronological evolution of the radiological findings in seven children with febrile infection-related epilepsy syndrome. Methods This is a retrospective study describing the radiological findings and evolution in seven children with febrile infection-related epilepsy syndrome who presented from 2009 to 2013. The children all fit the defined clinical criteria for febrile infection-related epilepsy syndrome; all had a history of normal psychomotor development who presented with acute-onset catastrophic partial status epilepticus associated with a febrile illness or unspecific infectious process. The children were identified from the author's weekly review of the pediatric inpatient service, and then the data were collected and analyzed retrospectively. Results Six males and one female ranging from 3 months to 9 years of age presented with status epilepticus preceded by a febrile illness. Extensive investigations for infectious, autoimmune, and metabolic etiologies were unremarkable. Multiple antiepileptic medications were attempted, including drug-induced coma in all of them, with poor response. Immunotherapy with intravenous steroids or intravenous immunoglobulin (three patients had both) was tried in six of seven patients with a poor response. Ketogenic diet was initiated in four of seven patients with limited response. Serial magnetic resonance imaging studies, done from the initial presentation through 18 months of follow-up, showed evolution from normal imaging to severe cerebral atrophy. Progressive cytotoxic edema involving mostly bilateral hippocampi and temporal lobes was appreciated in one to three weeks. At one month from seizure onset, mild to moderate cerebral atrophy and hippocampal sclerosis was appreciated that continued to progress over the next year. After six to twelve months, most of the patients showed moderate to severe cerebral atrophy and by one year, cerebellar atrophy was also appreciated. Conclusion Febrile infection-related epilepsy syndrome is a devastating epilepsy syndrome of childhood without a diagnostic biologic marker. The magnetic resonance imaging findings appear to be progressive and typical. Thus, combined with the clinical course, imaging findings can help to confirm the diagnosis (until a biologic marker is found). This hopefully will allow multicentered treatment protocols in the future.

Objective The aim of the present study was to compare localization of the language cortex using cortical stimulation mapping (CSM), high gamma electrocorticography (hgECoG), functional magnetic resonance imaging (fMRI), and transcranial magnetic stimulation (TMS). Methods Language mapping using CSM, hgECoG, fMRI, and TMS were compared in nine patients with epilepsy. Considering CSM as reference, we compared language mapping approaches based on hgECoG, fMRI, and TMS using their sensitivity, specificity, and the results of receiver operating characteristic (ROC) analyses. Results Our results show that areas involved in language processing can be identified by hgECoG, fMRI, and TMS. The average sensitivity/specificity of hgECoG, fMRI, and TMS across all patients was 100%/85%, 50%/80%, and 67%/66%, respectively. The average area under the ROC curve of hgECoG, fMRI, and TMS across CSM-positive patients was 0.98, 0.76, and 0.68, respectively. Conclusions There is considerable concordance between CSM, hgECoG, fMRI, and TMS language mapping. Our results reveal that hgECoG, fMRI, and TMS are valuable tools for presurgical language mapping. Significance Language mapping on the basis of hgECoG, fMRI, and TMS can provide important additional information, therefore, these methods can be used in conjunction with CSM or as an alternative, when the latter is deemed impractical.

BackgroundAcute ischemic stroke (AIS) due to emergent large-vessel occlusion (ELVO) has a poor prognosis.ObjectiveTo examine the hypothesis that a better collateral score on pretreatment CT angiography (CTA) would correlate with a smaller final infarct volume and a more favorable clinical outcome after endovascular therapy (EVT).MethodsA retrospective chart review of the University of Tennessee AIS database from February 2011 to February 2013 was conducted. All patients with CTA-proven LVO treated with EVT were included. Recanalization after EVT was defined by Thrombolysis in Cerebral Infarction (TICI) score ≥2. Favorable outcome was assessed as a modified Rankin Score ≤3.ResultsFifty patients with ELVO were studied. The mean National Institutes of Health Stroke Scale score was 17 (2–27) and 38 of the patients (76%) received intravenous tissue plasminogen activator. The recanalization rate for EVT was 86.6%. Good clinical outcome was achieved in 32% of patients. Univariate predictors of good outcome included good collateral scores (CS) on presenting CTA (p=0.043) and successful recanalization (p=0.02). Multivariate analysis confirmed both good CS (p=0.024) and successful recanalization (p=0.009) as predictors of favorable outcome. Applying results of the multivariate analysis to our cohort we were able to determine the likelihood of good clinical outcome as well as predictors of smaller final infarct volume after successful recanalization.ConclusionsGood CS predict smaller infarct volumes and better clinical outcome in patients recanalized with EVT. These data support the use of this technique in selecting patients for EVT. Poor CS should be considered as an exclusion criterion for EVT as patients with poor CS have poor clinical outcomes despite recanalization.

Autopsy studies have described definitive traumatic avulsion of the oculomotor nerve from the brainstem; however, detailed characterization of mechanisms and localization of traumatic nerve injury has yet to be definitively described in vivo. We report the case of a 13-year-old girl in whom high-resolution magnetic resonance imaging confirmed irreversible injury to the left oculomotor nerve after trauma.

Supplemental Digital Content is available in the text., In this study, we have addressed the question of functional brain reorganization for language in the presence and absence of anatomical lesions in two patients with epilepsy using cortical stimulation mapping and high gamma (HG) activity in subdural grid recordings. In both, the expressive language cortex was defined as the cortical patch below the electrode(s) that when stimulated resulted in speech arrest, and during speech expression tasks generated HG activity. This patch fell within the borders of Broca’s area, as defined anatomically, in the case of the patient with a lesion, but outside that area in the other, lesion-free patient. Such results highlight the necessity for presurgical language mapping in all cases of surgery involving the language-dominant hemisphere and suggest that HG activity during expressive language tasks can be informative and helpful in conjunction with cortical stimulation mapping for expressive language mapping.

Intraoperative magnetic resonance imaging (iMRI) has emerged as an important tool in guiding the surgical management of children with brain tumors. Recent advances have allowed utilization of high field strength systems, including 3-tesla MRI, resulting in diagnostic-quality scans that can be performed while the child is on the operating table. By providing information about the possible presence of residual tumor, it allows the neurosurgeon to both identify and resect any remaining tumor that is thought to be safely accessible. By fusing the newly obtained images with the surgical guidance software, the images have the added value of aiding in navigation to any residual tumor. This is important because parenchyma often shifts during surgery. It also gives the neurosurgeon insight into whether any immediate postoperative complications have occurred. If any complications have occurred, the child is already in the operating room and precious minutes lost in transport and communications are saved. In this article we review the three main approaches to an iMRI system design. We discuss the possible roles for iMRI during intraoperative planning and provide guidance to help radiologists and neurosurgeons alike in the collaborative management of these children.

The main clinical application of functional neuroimaging is mapping cortical regions containing part of the circuitry necessary for somatosensory, motor, and language functions and assessing hemispheric dominance for both language and encoding operations of memory prior to several types of brain surgery. Presently, it is used in conjunction with the classical invasive methods of brain mapping. This chapter presents the case for replacing invasive methods with noninvasive ones given the limitations of the invasive methods that render them unjustifiable as “gold standards.” Evidence is presented that the efficacy of the two types of methods in reducing morbidity, facilitating surgical planning, and enhancing surgical outcome is comparable. Additional advantages of noninvasive presurgical brain mapping are also discussed. The chapter concludes that there are no compelling reasons for invasive mapping in most patients whenever noninvasive methods are available.

Objective Accurate noninvasive assessment of motor function using functional MRI (fMRI) and magnetoencephalography (MEG) is a challenge in patients who are very young or who are developmentally delayed. In such cases, passive mapping of the sensorimotor cortex is performed under sedation. We examined the feasibility of using transcranial magnetic stimulation (TMS) as a motor mapping tool in awake children younger than 3 years of age. Methods Six children underwent motor mapping with TMS while awake as well as passive sensorimotor mapping under conscious sedation with MEG during tactile stimulation (n = 5) and fMRI during passive hand movements (n = 4). Results Stimulation of the motor cortex via TMS successfully elicited evoked responses in contralateral hand muscles in 5 patients. The location of primary motor cortex in the precentral gyrus identified by TMS corresponded with the postcentral location of the primary sensory cortex identified by MEG in 2 patients and to the sensorimotor cortex identified by fMRI in 3 children. In this cohort, we demonstrate that TMS can illuminate abnormalities in motor physiology including motor reorganization. We also demonstrate the feasibility of using TMS-derived contralateral silent periods to approximate the location of motor cortex in the absence of an evoked response. When compared to chronological age, performance functioning level appears to be better in predicting successful mapping outcome with TMS. Conclusions Our findings indicate that awake TMS is a safe alternative to MEG and fMRI performed under sedation to localize the motor cortex and provides additional insight into the underlying pathophysiology and motor plasticity in toddlers.

Non-invasive functional evaluation of the brain complements structural MRI imaging and has largely supplanted invasive techniques such as awake craniotomy. Techniques used for functional mapping of the brain include BOLD-functional MRI (fMRI), magnetoencephalography (MEG), and transcranial magnetic stimulation (TMS). We describe the case of a right-handed patient with a lesion centered in the left inferior perirolandic cortex who underwent fMRI, MEG, and TMS on a single day to facilitate maximal lesion resection while preserving eloquent cortex and eloquent white matter tracts.

Rhombencephalosynapsis is a rare genetic aberration characterized by variable vermian hypoplasia/aplasia in conjunction with united cerebellar hemispheres. Genetic defects in the isthmic organizer at the mesencephalic–metencephalic junction are presumably responsible for the associated aqueductal stenosis. We performed a retrospective review of 20 children with rhombencephalosynapsis to evaluate for and emphasize the association of aqueductal stenosis and hydrocephalus. We retrospectively reviewed the MR and CT images of 20 children (0–11 years old) with rhombencephalosynapsis encountered at two academic children’s hospitals. Rhombencephalosynapsis spectrum severity was graded based on pre-existing literature. We analyzed examinations for ventriculomegaly and degree of aqueductal stenosis. The collicular distances were measured from the collicular apices. Imaging studies were also analyzed for malformations of cortical and cerebellar development. Thirteen of the 20 children (65%) with rhombencephalosynapsis presented with clinical or imaging evidence of hydrocephalus and aqueductal stenosis, principally involving the caudal cerebral aqueduct. All children with aqueductal stenosis had collicular fusion. All six children with complete rhombencephalosynapsis had aqueductal stenosis. The cerebral aqueduct varied from normal to stenotic in children with incomplete rhombencephalosynapsis. Corpus callosum dysgenesis was present in four children. Aqueductal stenosis in the setting of rhombencephalosynapsis is an under-recognized cause of noncommunicating hydrocephalus. Our findings support the hypothesis that a defect involving the common gene(s) responsible for the differentiation and development of both the roof plate and midline cerebellar primordium at the mesencephalon/first rhombomere junction may be responsible for the association of aqueductal stenosis and rhombencephalosynapsis.

Imaging in traumatic injury to the orbits plays an important role to identify malformation of the globe, retrobulbar pathology, such as hematoma, the presence of fractures, and identification of foreign bodies. MRI can be especially useful in characterizing soft tissue abnormalities without the use of ionizing radiation. The authors report a case of penetrating injury to the orbit with a retained foreign body where the graphite core of a pencil ("pencil lead") resulted in metal-like diamagnetic susceptibility artifact. This was proven to have no metallic components by CT and surgical exploration. MRI performed in the setting of penetrating injury could aid in localization of a graphite foreign body, and if there is a known graphite foreign body, evaluation of immediately adjacent structures may be obscured.

Background and purpose The best imaging selection technique for endovascular treatment (EVT) remains a topic of debate. Collateral scores (CS) on pre-treatment CT angiography (CTA) have been associated with favorable outcome. We hypothesized that low CS on pre-treatment CTA may predict a poor outcome after EVT in successfully recanalised patients with emergent large vessel occlusions (ELVO). Methods A retrospective chart review was performed for the University of Tennessee Health Sciences Center Acute Ischemic Stroke Database evaluating AIS patients presenting with CTA confirmed anterior circulation ELVO in a tertiary stroke center during a 3 year period. Only patients with successful recanalization (TICI 2 b or 3) after EVT were included in the analysis. A blinded neuroradiologist calculated the CTA CS and final infarct volume (FIV). Poor outcome after EVT was defined as symptomatic intracranial hemorrhage (SICH), cerebral edema requiring hypertonic treatment for ≥48 hours, hemicraniectomy, higher FIV, and poor clinical outcome of modified ranking scale (mRS)-score 3–6 at 3 months. Results 58 AIS patients with anterior circulation ELVO (mean age 63 ± 13 years, 48% male, median admission NIHSS-score: 17 points, IQR 14–21) had successful recanalization after EVT. Systemic thrombolysis was administered in 38 patients (65.5%). A total of 31 patients (53%) achieved favorable outcome (FO). There was no significant difference in rates of hemicraniectomy (p = 1.000) and SICH (p = 0.667) after EVT when compared to patients with low and good CS. Patients with low CS tended to have higher rates of cerebral edema requiring hypertonic treatment (30% vs 13%, p = 0.340) after EVT. Patients with low CS had greater FIV (111 ± 71 vs 41 ± 66 cm 3 , p = 0.007) and higher rates of poor clinical outcome (82% vs 39%, p = 0.017) in comparison to patients with high CS. A low CS was independently associated with poor clinical outcome (p = 0.048) in multiple logistic regression models adjusting for demographics, vascular risk factors, pretreatment SBP, admission NIHSS, intravenous thrombolysis, and onset to revascularization time. Conclusion Low CS on pre-treatment CTA was correlated with significantly worse outcome despite successful recanalization as evident by higher FIV and higher rates of poor clinical outcome. Poor CS should be considered an important variable in futures trials comparing the medical versus interventional management of patients with ELVO. Disclosures N. Goyal: None. S. Iftikhar: None. G. Tsivgoulis: None. Y. Khorchid: None. A. Choudhri: None. D. Hoit: None. A. Alexandrov: None. A. Arthur: None. L. Elijovich: None.

Acute aortic syndromes and traumatic aortic injury are often diagnosed on CT angiography, possibly requiring emergent intervention. Advances in handheld computing have created the possibility of viewing full DICOM datasets from a remote location. We evaluated the ability to diagnose and characterize acute aortic pathologies on CT angiograms of the thorax using an iPhone-based DICOM viewer. This study was performed after Institutional Review Board approval. Fifteen CT angiograms of the thorax in suspected acute aortic syndromes were evaluated by three blinded radiologists on a handheld device using a DICOM viewer. Studies were evaluated for the ability to identify and classify aortic dissection, transection, or intramural hematoma, measure aortic dimensions, and identify mediastinal hematoma, arch variants, and pulmonary pathology. Studies were compared to blinded interpretations on a dedicated PACS workstation. The aortic pathology was correctly identified as aortic transection/pseudoaneurysm (n = 5), type A dissection (n = 2), and type A intramural hematoma (n = 1) by all reviewers, with no false-positive interpretations. This represents a sensitivity and specificity of 100 %. Mediastinal hematoma (n = 6), pneumothorax (five right, three left), and arch vessel involvement (n = 2) were identified in all cases. There was 88.5 % accuracy in identifying arch variants. Measurement of the size of the involved aortic segment was similar on handheld device and PACS workstation; however the adjacent normal aorta was 1.2 ± 1.0 mm larger on the handheld device (p = 0.03). Handheld DICOM viewers may be useful for emergent consultations and triage, and may expedite preprocedure planning to reduce the time interval between diagnostic scan and therapeutic intervention.

PURPOSE Superselective intraophthalmic artery chemotherapy (SSIOAC) is being used for treatment of retinoblastoma; however, the hemodynamic consequences and toxicities are not fully known. We developed a nonhuman primate (NHP) model of SSIOAC and reported our clinical observations. For validation, we compared ophthalmic artery (OA) diameters between NHPs and children (

A 51-year-old woman with a history of migraine headaches was found to have an incidental right orbital mass on MRI during neurologic evaluation for headaches. The orbital mass was a well-defined, lobulated, intraosseous soft tissue lesion with circumscribed margins. Clinically, there was noted proptosis, tenderness to palpation, and slight limitation to right abduction. An orbitotomy with incisional biopsy revealed a lesion arising within the lateral orbital rim extending to the subperiosteal space. Intraoperative frozen sections indicated a low grade sarcoma, possibly metastatic. The extraosseous component was excised, and the bone was curetted until all visible tumor was removed. A diagnosis of chondromyxoid fibroma was made. The patient did well until 5 months postoperatively, when right-sided proptosis returned due to recurrent tumor. Repeat surgical resection with removal of the lateral orbital rim was performed. Histopathology was consistent with recurrent chondromyxoid fibroma.

The purpose of the study was to depict a subset of focal partial tears of the biceps brachii tendon, occurring at the entrance to the bicipital groove, which can be difficult to detect with MRI.The institutional review board approved this HIPAA-compliant study; informed consent was waived. The authors retrospectively reviewed imaging and medical records in 16 consecutive patients (12 men, 4 women; mean age, 57 years) who had prospective MRI diagnoses of tendinopathy and/or partial tearing of the intra-articular segment of the long head of the biceps brachii tendon (LHBT) at the entrance to the bicipital groove (restricted to within 1 cm of the groove entrance) and who also had surgical correlation within 4 months of imaging.Focal intrasubstance signal abnormality was noted in the tendons of 16 out of 16 (100%) patients. Focal tendon enlargement was noted in 8 out of 16 patients (50%). Fifteen out of 16 biceps partial tears (94%) were treated surgically. Shoulder pathology was restricted to the groove entrance in 4 out of 16 patients (25%).We depict a subset of focal partial tears of the biceps tendon, which can be difficult to detect on MRI because of their anatomical location at the entrance to the bicipital groove. Although they may coexist with other causes of shoulder pain, these lesions can also occur in isolation. In either case, they are potential causes of pain that can be addressed surgically.

Axenfeld-Rieger syndrome (ARS) is a genetic disorder representing a disease spectrum resulting from neural crest cell maldevelopment. Glaucoma is a common complication from the incomplete formation of the iridocorneal angle structures. Neural crest cells also form structures of the forebrain and pituitary gland, dental papillae, aortic arch walls, genitalia, and long bones; therefore, patients with ARS manifest a wide range of systemic findings. To our knowledge, detailed magnetic resonance imaging findings have not been previously reported. We report a case of a 19-month-old Indian male diagnosed with ARS with emphasis on magnetic resonance imaging findings of the globes, brain, teeth, and skull base.