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Objectives: To investigate the long-term impact of contrast-enhanced ultrasound (CEUS) on the treatment of patients with indeterminate renal masses., Methods: In this retrospective study, consecutive charts of all patients receiving renal CEUS at 1 of 2 academic medical centers between January 1, 2014, and December 31, 2018, were reviewed. Patients were included in the study if they had documented chronic renal disease (estimated glomerular filtration rate < 60 mL/min/1.73 m 2 ) or prior nephrectomy and received CEUS for a previously untreated renal mass., Results: A total of 215 lesions in 157 patients were used for analysis. Contrast-enhanced ultrasound provided a final treatment recommendation in 71.6% of lesions (154 of 215). Of these 154 lesions, 7.8% (12 of 154) were lost to follow-up despite CEUS suggesting malignancy; 15.6% (24 of 154) went directly for surgical intervention, with malignancy confirmed by pathologic results in 87.5% (21 of 24) of these cases; and the remaining 76.6% (118 of 154) were deemed benign and required no additional follow-up. Of the 118 lesions diagnosed by CEUS as benign and requiring no follow-up, none showed evidence of later renal cell carcinoma development and, only 5.1% (6 of 118) of the total population was referred for further cross-sectional imaging of the mass in question. In 28.4% of all lesions (61 of 215), CEUS resulted in a recommendation for surveillance imaging at a 6- to 12-month interval, and less than 10% (6 of 61) of these underwent additional cross-sectional imaging within the recommended 6 months after CEUS., Conclusions: These findings highlight the impact of CEUS on clinical treatment of indeterminate renal masses, including reducing the use of the potentially nephrotoxic contrast agents and providing a direct pathway to transplant., (© 2020 American Institute of Ultrasound in Medicine.)

Although cochlear implantation enables some children to attain age-appropriate speech and language development, communicative delays persist in others, and outcomes are quite variable and difficult to predict, even for children implanted early in life. To understand the neurobiological basis of this variability, we used presurgical neural morphological data obtained from MRI of individual pediatric cochlear implant (CI) candidates implanted younger than 3.5 years to predict variability of their speech-perception improvement after surgery. We first compared neuroanatomical density and spatial pattern similarity of CI candidates to that of age-matched children with normal hearing, which allowed us to detail neuroanatomical networks that were either affected or unaffected by auditory deprivation. This information enables us to build machine-learning models to predict the individual children's speech development following CI. We found that regions of the brain that were unaffected by auditory deprivation, in particular the auditory association and cognitive brain regions, produced the highest accuracy, specificity, and sensitivity in patient classification and the most precise prediction results. These findings suggest that brain areas unaffected by auditory deprivation are critical to developing closer to typical speech outcomes. Moreover, the findings suggest that determination of the type of neural reorganization caused by auditory deprivation before implantation is valuable for predicting post-CI language outcomes for young children., Competing Interests: Conflict of interest statement: G.F., N.M.Y., P.C.M.W., The Chinese University of Hong Kong, and the Ann & Robert H. Lurie Children's Hospital of Chicago disclose potential financial conflict of interest related to US patent application filed on December 21, 2017.

Purpose: The purpose of this study was to examine advanced diffusion-weighted magnetic resonance imaging (DW-MRI) models for differentiation of low- and high-grade tumors in the diagnosis of pediatric brain neoplasms., Methods: Sixty-two pediatric patients with various types and grades of brain tumors were evaluated in a retrospective study. Tumor type and grade were classified using the World Health Organization classification (WHO I-IV) and confirmed by pathological analysis. Patients underwent DW-MRI before treatment. Diffusion-weighted images with 16 b-values (0-3500 s/mm 2 ) were acquired. Averaged signal intensity decay within solid tumor regions was fitted using two-compartment and anomalous diffusion models. Intracellular and extracellular diffusion coefficients (D slow and D fast ), fractional volumes (V slow and V fast ), generalized diffusion coefficient (D), spatial constant (μ), heterogeneity index (β), and a diffusion index (index&#95;diff = μ × V slow /β) were calculated. Multivariate logistic regression models with stepwise model selection algorithm and receiver operating characteristic (ROC) analyses were performed to evaluate the ability of each diffusion parameter to distinguish tumor grade., Results: Among all parameter combinations, D and index&#95;diff jointly provided the best predictor for tumor grades, where lower D (p = 0.03) and higher index&#95;diff (p = 0.009) were significantly associated with higher tumor grades. In ROC analyses of differentiating low-grade (I-II) and high-grade (III-IV) tumors, index&#95;diff provided the highest specificity of 0.97 and D provided the highest sensitivity of 0.96., Conclusions: Multi-parametric diffusion measurements using two-compartment and anomalous diffusion models were found to be significant discriminants of tumor grading in pediatric brain neoplasms.

Objective: Determine the diagnostic usefulness of postimplantation 1.5 T magnetic resonance imaging (MRI) and review magnet-related MRI complications., Study Design: Retrospective chart review with additional review of MRIs., Setting: Tertiary care children's hospital., Patients: Twelve patients who underwent MRI after receiving a cochlear implant (CI)., Intervention: One or more episodes of 1.5 T MRI with CI in place., Main Outcome Measures: Occurrence of magnet-related complications; whether imaging was clinically useful., Results: The 12 patients underwent 23 episodes of MRI, including 13 episodes in 11 patients (18 ears) during which a magnet was present and 17 studies were obtained. Complications related to the magnet occurred during 4 of the 13 imaging episodes (30.8%), all during body or spine studies. Magnet torsion with reversal of polarity occurred in three devices; reduced magnet strength in one; and displacement of the magnet from its housing in one. One patient required surgical magnet replacement, whereas other headpiece retention problems were resolved without surgery. All studies but one brain with bilateral magnets were clinically useful., Conclusions: CI patients who undergo MRI with a magnet in situ may experience complications, especially when imaged below the head. Most complications may be resolved without surgery. Diagnostic usefulness of non-cranial MRI is not likely to be limited by presence of the magnet, while a magnet may prevent clinically useful brain imaging. Obtaining MRI with the magnet in situ avoids the cost and risks associated with multiple surgeries to remove and replace the magnet or the entire implant.

Objectives: This clinical study evaluated the pharmacokinetics (PK) and safety data of macrocyclic extracellular contrast agent gadobutrol in pediatric subjects aged younger than 2 years., Materials and Methods: Pediatric subjects (term newborns to those aged younger than 2 years) with normal renal function undergoing magnetic resonance imaging with gadobutrol (0.1 mmol/kg body weight [BW]) were prospectively enrolled in this open-label, multicenter clinical trial to evaluate PK as a primary end point. Plasma PK was analyzed using a population-based PK approach. Safety and qualitative efficacy (evaluation of images) were secondary end points. Safety and tolerability were assessed throughout study participation (approximately 7 days). Imaging efficacy variables were assessed by investigators., Results: Forty-four subjects were evaluated for safety and efficacy; 43 subjects were eligible for PK evaluation including 9 term newborns and infants aged younger than 2 months. Gadobutrol PK in pediatric subjects aged younger than 2 years were adequately described by a linear 2-compartmental model with elimination from the central compartment. Total median systemic exposure (area under the curve) of gadobutrol was estimated at 776 μmol · h/L (range, 544-1470 μmol · h/L). Simulated median concentration at 20 minutes after injection of gadobutrol (C20) was 339 μmol/L (range, 230-456 μmol/L). Safety and tolerability profile were similar to older populations. In 1 subject (2.3%), vomiting was reported as a mild adverse event related to gadobutrol, and there were no reported serious adverse events. The evaluation of gadobutrol-enhanced images provided improved diagnosis, increased confidence in diagnosis, and contributed to subject clinical management., Conclusions: The PK profile of gadobutrol in children aged younger than 2 years including newborns is similar to that in older children and adults. At the dose of 0.1 mmol/kg BW, gadobutrol had a favorable safety profile and was well tolerated with similar profile across the age range 0 to younger than 2 years and compared with older children and adults. Extrapolation of efficacy data from adults to the younger pediatric population, including term newborns, is justified. The recommended standard dose of gadobutrol (0.1 mmol/kg BW), as used in the population aged 2 years and older, is also appropriate in children aged younger than 2 years.

We report a case of an unsuspected ganglioneuroma of the choroid in a patient with neurofibromatosis type 1. A 5-year-old girl presented from an outside institution with right proptosis and glaucoma since birth. Magnetic resonance imaging was obtained and showed a cavernous sinus mass extending into the right orbit and multiple orbital lesions. Additionally, increased signal in the posterior globe of the right eye was noted, but its etiology was unclear at the time. She was lost to follow-up for 3 years and later returned with a blind painful eye. Enucleation was performed, and histopathology was significant for diffuse choroidal ganglioneuroma and advanced glaucoma. We report the atypical history, examination findings, and histopathology to support the diagnosis.

Maffucci syndrome is a rare disease process characterized by enchondromatosis with cutaneous hemangiomatosis. We report a 20-year-old woman with Maffucci syndrome with a 5-day history of diplopia. She was found to have a left sixth nerve palsy due to a parasellar chondrosarcoma. Three weeks later, the patient's diplopia spontaneously resolved. This unusual clinical course prompted us to review frequency of sixth nerve palsy with skull base neoplasms and the phenomenon of spontaneous resolution of diplopia.

Neuroimaging studies for persons in the National Collaborative Chicago-Based Congenital Toxoplasmosis Study (NCCCTS) with symptoms and signs referable to the spinal cord were reviewed. Three infants had symptomatic spinal cord lesions, another infant a Chiari malformation, and another infant a symptomatic peri-spinal cord lipoma. One patient had an unusual history of prolonged spinal cord symptoms presenting in middle age. Neuroimaging was used to establish her diagnosis and response to treatment. This 43 year-old woman with congenital toxoplasmosis developed progressive leg spasticity, weakness, numbness, difficulty walking, and decreased visual acuity and color vision without documented re-activation of her chorioretinal disease. At 52 years of age, spinal cord lesions in locations correlating with her symptoms and optic atrophy were diagnosed with 3 Tesla MRI scan. Treatment with pyrimethamine and sulfadiazine decreased her neurologic symptoms, improved her neurologic examination, and resolved her enhancing spinal cord lesions seen on MRI.

Ocular cross-sectional imaging is usually obtained as an adjunct to clinical ophthalmologic examination and ocular ultrasound. Computed tomography/magnetic resonance imaging (CT/MRI) are complimentary for ocular imaging and are performed for evaluation of the vitreous cavity, choroid, retina, sclera, and potential spaces and for the assessment of extension of disease beyond the globe into the orbit or brain. CT has superior spatial resolution aided by the natural contrast between bone, soft tissues, air, and fat. The short scanning time is advantageous to reduce motion effects and the need for sedation. CT is also the modality of choice for evaluation of traumatic injury and for visualization of foreign bodies. Potential clinical indications for MRI include staging of retinoblastoma and other causes of leukocoria, assessment of retinal or choroidal detachments for underlying retinal mass or hemorrhage, uveal melanoma, ocular metastases, choroidal hemangioma, and buphthalmus, staphyloma, and coloboma. Last, but not least, MRI has the advantage of no ionizing radiation., (Copyright © 2011. Published by Elsevier Inc.)

Background and Purpose: We describe the rationale and safety of concurrent decompression of Chiari type 1 malformation (CM1) and untethering of the spinal cord. Spinal cord traction is considered one of the pathogenic mechanisms involved in the development of CM, and 14% of patients with CM1 have tethered cord syndrome (Milhorat et al., Surg Neurol 7:20-35, 2009; Roth, Neuroradiology 21:133-138, 1981; Royo-Salvador, Rev Neurol 24:937-959, 1996; Royo-Salvador et al., Acta Neurochir 147:515-523, 2005). Therefore, intraspinal anomalies that require intervention are commonly treated before surgical decompression of Chiari malformation (Cheng et al., Neurologist 8:357-362, 2002; Menezes, Pediatr Neurosurg 23:260-269, 1995; Milhorat et al., Surg Neurol 7:20-35, 2009; Royo-Salvador et al., Acta Neurochir 147:515-523, 2005; Schijman and Steinbok, Childs Nerv Syst 20:341-348, 2004; Yamada et al., Neurol Res 26:719-721, 2004). However, in the interval between the spinal cord untethering and the decompression surgery, patients may continue to suffer from the untreated symptoms of CM. In a series of four patients with concurrent severe and progressive symptoms referable to both conditions, we performed both surgeries simultaneously., Methods: Charts of four patients who underwent concurrent Chiari decompression and spinal cord untethering were reviewed., Results: All patients tolerated the procedures well without complication. They reported significant or complete early postsurgical resolution of headaches and ambulating difficulties. On average, patients started to walk on postoperative day 3 (3 ± 1 days) and were discharged on hospital day 6 (6 ± 1 days). No patient experienced a persistent subcutaneous or transcutaneous cerebrospinal fluid leak. Subsequent postoperative courses were uneventful., Conclusion: Concurrent Chiari decompression and untethering of the spinal cord is a feasible option and in some patients may be preferred in lieu of staged procedures.

Background: De novo constitutional chromosomal anomalies provide important insights into the genetic loci responsible for congenital neurological disorders. However, most phenotypic descriptions of patients with rare chromosomal abnormalities are published as individual case reports or small group studies, making genotype-phenotype correlations unclear. Moreover, many clinical genetic reports do not include neuroimaging., Methods: We conducted a retrospective case series study of all children who had genetic testing done at Children's Memorial Hospital in Chicago, Illinois between 1985 and 2006. The case series was selected from a database containing all chromosomal testing results, clinical data, and neuroimaging. Clinical examination results were assigned by board certified geneticists and/or neurologists and neuroimages were reviewed by both a neurologist or neuroradiologist and a blinded neurologist., Results: Of the 28,108 children in the series, we identified 34 children with novel or apparently novel de novo chromosomal abnormalities. Several of the cases represent potentially new genetic loci for neurological malformations and novel syndromic conditions., Conclusions: This study demonstrates the utility of large clinical databases in assessing genotype-phenotype correlations and mapping loci for congenital neurological disorders. We describe a case-series strategy to analyze existing databases to reveal new genotype-phenotype correlations., ((c) 2008 Wiley-Liss, Inc.)

Brainstem vascular malformations can sometimes simulate other conditions such as multiple sclerosis and pontine glioma. We report a case of brainstem cavernous angioma for which clinical course and radiologic appearance were suggestive of a pontine glioma. The diagnosis was uncertain until the clinical and radiologic evolution made it clear that the patient had a cavernous angioma. We suggest that brainstem angioma be considered as a differential diagnosis for pontine glioma.

The authors report two cases of ischemic stroke secondary to occlusive vasculopathy two decades after radiation therapy (RT) for medulloblastoma. Both patients underwent posterior fossa medulloblastoma partial resection, followed by craniospinal RT in which a cobalt 60 source was used; 40 Gy were given to the whole brain plus a 15-Gy boost to the posterior fossa. Both patients received multiagent chemotherapy, immediately following radiation therapy in the first case and after repeated craniotomy for recurrence 13 years after radiation in the second case. They experienced multiple sequelae from radiation and chemotherapy, including growth retardation and psychomotor delay. However, 20 years after treatment, they remained tumor free and able to work, until they presented with focal neurological deficits and seizures. Computerized tomography and magnetic resonance imaging of the brain in both cases showed no tumor recurrence, but did demonstrate ischemia in a posterior cerebral artery distribution. Cerebral angiography revealed multiple mid-sized arterial wall irregularities as well as focal stenoses consistent with a postirradiation vasculopathy. The pathophysiological mechanisms, radiological appearance, and incidence of this syndrome are reviewed from the literature.

In a case of alobar holoprosencephaly, a neonate who died several minutes after birth was found to have multiple facial and intracranial malformations, including cyclopia. Postmortem MR and CT findings included a single midline orbit, with two globes that contained separate lenses supplied by a single optic nerve. There were two separate superior orbital fissures and two separate lateral rectus muscles.