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Craniosynostosis is when cranial sutures fuse prematurely. It causes cosmetic deformity and may limit brain growth with more serious consequences, such as hydrocephalus. It may involve one or more sutures, may be syndromic, and is thought most likely to result from genetic causes. Specific sutural closures result in reproducible cranial appearances and head shapes. Three-dimensional computed tomography has become the standard for preoperative diagnosis to allow surgical planning as well as postsurgical assessment. Early diagnosis allows early surgical management, which improves outcome. Nonsyndromic and syndromic craniosynostoses are illustrated in this pictorial review., (Crown Copyright © 2011. Published by Elsevier Inc. All rights reserved.)

Background: Multiple sclerosis (MS) diagnostic criteria incorporate MRI features that can be used to predict later diagnosis of MS in adults with acute CNS demyelination. To identify MRI predictors of a subsequent MS diagnosis in a paediatric population, we created a standardised scoring method and applied it to MRI scans from a national prospective incidence cohort of children with CNS demyelination., Methods: Clinical and MRI examinations were done at the onset of acute CNS demyelination and every 3 months in the first year after that, and at the time of a second demyelinating attack. MS was diagnosed on the basis of clinical or MRI evidence of relapsing disease. Baseline MRI scans were assessed for the presence of 14 binary response parameters. Parameters were assessed with a multiple tetrachoric correlation matrix. Univariate analyses and multivariable Cox proportional hazards models were used to identify predictors of MS., Findings: Between Sept 1, 2004, and June 30, 2010, 332 children and adolescents were assessed for eligibility. 1139 scans were available from 284 eligible participants who had been followed up for 3·9 (SD 1·7) years. 57 (20%) were diagnosed with MS after a median of 188 (IQR 144-337) days. Seven of 14 binary response parameters were retained. The presence of either one or more T1-weighted hypointense lesions (hazard ratio 20·6, 95% CI 5·46-78·0) or one or more periventricular lesions (3·34, 1·27-8·83) was associated with an increased likelihood of MS diagnosis (sensitivity 84%, specificity 93%, positive predictive value 76%, negative predictive value 96%). Risk for MS diagnosis was highest when both parameters were present (34·27, 16·69-70·38). Although the presence of contrast enhancement, cerebral white matter, intracallosal, and brainstem lesions was associated with MS in the univariate analyses, these parameters were not retained in the multivariable models., Interpretation: Specific MRI parameters can be used to predict diagnosis of MS in children with incident demyelination of the CNS. The ability to promptly identify children with MS will enhance timely access to care and will be important for future clinical trials in paediatric MS., Funding: Canadian Multiple Sclerosis Scientific Research Foundation., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

Introduction: Spinal cord lesions in adults with multiple sclerosis (MS) are thought to contribute to disability. The magnetic resonance imaging (MRI) appearance and clinical correlates of spinal cord lesions in children with MS have not been reported., Methods: T1-weighted pre- and post-gadolinium and T2-weighted TSE/FSE spine MR images of 36 children (age, 14.3 ± 3.3) with relapsing-remitting MS (annualized relapse rate, 0.7; disease duration, 7.5 ± 3.3 years) were analyzed for total lesion count, lesion location and length, intramedullary extent, and gadolinium enhancement. Clinical, demographic, laboratory, and MRI data were correlated., Results: Lesions preferentially involved the cervical region, were predominantly focal, and involved only a portion of the transverse cord diameter. However, ten of 36 patients demonstrated longitudinally extensive lesions. Children with the highest clinical relapse rate also tended to have more spinal cord lesions and were more likely to accrue new lesions on serial spinal scans., Conclusion: These preliminary data suggest that MS lesions of the spinal cord in children are radiographically similar to that of adult-onset MS--supporting a common biology of pediatric- and adult-onset disease. However, children with relapsing-remitting MS can also develop longitudinally extensive lesions, suggesting that such lesions may be less specific for diseases such as neuromyelitis optica in pediatric patients. All patients recovered well from spinal cord attacks, and the presence of spinal cord lesions in the first few years of disease did not correlate with physical disability. Measures of spinal cord atrophy and longer periods of observation are required to determine the impact of spinal cord involvement in pediatric-onset MS.

Juvenile nasopharyngeal angiofibroma is a well-described but rare benign tumor that mainly affects adolescent boys. Although it is capable of extravagant local extension and tissue destruction, the tumor is amenable to effective therapy. Ocular or visual complications have been little noted in the literature. We report a case whose sole symptom was unilateral transient visual impairment and findings were limited to a nonspecific optic disk edema., (Copyright (c) 2010 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.)

Background: MRI diagnostic criteria have not yet been adopted for pediatric multiple sclerosis (MS). MRI plays a pivotal role in supporting the diagnosis of MS in adults. We sought to quantitatively define the MRI features of pediatric MS, to determine features that distinguish MS from nondemyelinating relapsing childhood neurologic disorders, and to propose MRI criteria for lesion dissemination in space in children with MS., Methods: A retrospective analysis of MRI scans from 38 children with clinically definite MS and 45 children with nondemyelinating diseases with relapsing neurologic deficits (migraine, systemic lupus erythematosus) was performed. For each scan, T2/FLAIR hyperintense lesions were quantified and categorized according to location and size. Mean lesion counts in specific locations were compared between groups to derive diagnostic criteria. Validation of the proposed criteria was performed using MRI scans from a second independent MS cohort (n = 21)., Results: MRI lesion location and size categories differed between children with MS and nondemyelinating controls with a medium to large effect size for most variables. The presence of at least two of the following-five or more lesions, two or more periventricular lesions, or one brainstem lesion-distinguished MS from other nondemyelinating disease controls with 85% sensitivity and 98% specificity., Conclusions: We propose modifications to the currently established McDonald MRI criteria for lesion dissemination in space that will enhance the diagnostic accuracy of these criteria for multiple sclerosis in children.

Background: Acute disseminated encephalomyelitis (ADEM) is typically a monophasic demyelinating disorder. However, a clinical presentation consistent with ADEM can also be the first manifestation of multiple sclerosis (MS), particularly in children. Quantitative analyses of MRI images from children with monophasic ADEM have yet to be compared with those from children with MS, and MRI criteria capable of distinguishing ADEM from MS at onset have yet to be derived., Methods: A retrospective analysis of MRI scans obtained at first attack from 28 children subsequently diagnosed with MS and 20 children with ADEM was performed. T2/fluid-attenuated inversion recovery hyperintense lesions were quantified and categorized according to location, description, and size. T1-weighted images before and after administration of gadolinium were evaluated for the presence of black holes and for gadolinium enhancement. Mean lesion counts and qualitative features were compared between groups and analyzed to create a proposed diagnostic model., Results: Total lesion number did not differentiate ADEM from MS, but periventricular lesions were more frequent in children with MS. Combined quantitative and qualitative analyses led to the following criteria to distinguish MS from ADEM: any two of 1) absence of a diffuse bilateral lesion pattern, 2) presence of black holes, and 3) presence of two or more periventricular lesions. Using these criteria, MS patients at first attack could be distinguished from monophasic ADEM patients with an 81% sensitivity and a 95% specificity., Conclusions: MRI diagnostic criteria are proposed that may be useful in differentiating children experiencing the first attack of multiple sclerosis from those with monophasic acute disseminated encephalomyelitis.

Purpose: To retrospectively determine and compare the sensitivity and specificity of unenhanced and contrast material-enhanced computed tomography (CT) (reference standard) in the diagnosis of brain abnormalities and to evaluate any change in diagnosis that resulted from the contrast-enhanced study., Materials and Methods: This study was approved by the local research ethics board; the requirement for informed consent was waived. The authors reviewed the unenhanced and contrast-enhanced CT scans of the brain obtained in 353 children for indications other than trauma. There were 196 boys and 157 girls aged 0 months to 17.8 years. Scans were read independently by two pediatric neuroradiologists who were blinded to clinical information. The diagnosis for each scan was recorded according to the anatomic section (supratentorial, infratentorial, ventricles, and skull). The final diagnosis was classified as normal, abnormal, or equivocal. kappa Statistics, with 95% confidence intervals, were reported, and the sensitivity, specificity, positive predictive value, and negative predictive value were calculated., Results: Interreader agreement for different anatomic regions varied between good (kappa coefficient, 0.63) and very good (kappa coefficient, 0.88) for unenhanced and contrast-enhanced scans. Sensitivity, specificity, positive predictive value, and negative predictive value for unenhanced scans were 97%, 89%, 87%, and 97%, respectively. The use of contrast material led to a change in the original normal or equivocal diagnosis to an abnormal diagnosis for only five (2.7%) of the 183 normal unenhanced scans., Conclusion: Unenhanced CT of developing brains has high sensitivity and specificity in the diagnosis of pathologic findings. The use of intravenous contrast material after unenhanced CT of the brain in children did not change the diagnosis frequently., (Copyright (c) RSNA, 2007.)

We report a rare case of hypoplasia of the right internal carotid artery (ICA) with ipsilateral congenital Horner syndrome. The etiology and pathogenesis of hypoplasia of the ICA is not well understood. Multiple types of collateral flow have been reported to develop to maintain blood supply to the ipsilateral cerebral hemisphere. Although collateral flow may allow these patients to remain asymptomatic, we postulate that the enlarged posterior communicating artery (PcomA) in our patient caused mass effect on the cisternal segment of cranial nerve III causing intermittent mydriasis apart from Horner syndrome.