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A growing body of evidence links abusive head trauma (AHT) to patterns of direct and indirect spinal injuries, such as spinal subdural hemorrhage (SDH). Identification of evidence of spinal injury such as spinal SDH plays a crucial role in the diagnosis and subsequent management of the index child with AHT and his or her siblings. In a value-based practice of medicine, it can be argued that adding spine imaging to identify spinal SDH in the workup of AHT adds value to both the short- and long-term management of the patient. This pictorial review describes the normal appearance of spinal SDH and challenges of identifying spinal SDH, and it explores the mechanism of spinal SDH development in AHT.

There is growing evidence of spine injury in abusive head trauma (AHT). Historically, spine injury was considered rare in AHT because of a lack of attributable clinical symptoms or signs and a lack of advanced imaging. Increased use of MRI in AHT has been instrumental in helping identify evidence of ligamentous injuries of the spine. These findings can be difficult to identify on autopsy because of the size and location of the ligaments. Because spinal injury in AHT mostly involves ligamentous and soft tissues and only rarely involves bony fractures, more than 90% of the injury findings are missed on CT or radiography of the spine. Investigation of these findings and the injury patterns should lead to a better understanding of the mechanism of spinal injury. In this pictorial review, we describe the various manifestations of spinal ligamentous injury in AHT, as seen on MRI, in children younger than 48 months.

Abusive head trauma (AHT) is the leading cause of fatal head injuries in children younger than 2 years. An intracranial pathology can exist even in the setting of a normal physical exam. A delay in the diagnosis of AHT can have serious life-threatening consequences for the child and increases the potential the child will be abused again. In this article, we review the traumatic subdural hematoma as well as various morpho-structural patterns of shearing injuries and thrombosis of intracranial bridging veins. This work serves as a summary of patterns of imaging features of intracranial venous injury in AHT, as described in the literature, to facilitate familiarity and early detection of abusive head trauma in the pediatric population. Essentially, in AHT there is a traumatic injury to the bridging vein with either partial or complete tear. This can secondarily result in thrombosis at the terminal end of the bridging vein with blood clots adjacent to the bridging vein.

Skull fractures are common in the pediatric population following head trauma and are estimated to occur post head trauma in 11% of children younger than 2 years. A skull fracture indicates potential underlying intracranial injury and might also help explain the mechanism of injury. Multiple primary and accessory sutures complicate the identification of non-depressed fractures in children younger than 2 years. Detection of linear skull fractures can be difficult on two-dimensional (2-D) CT and can be missed, particularly when the fracture is along the plane of image reconstruction. Knowledge of primary and accessory sutures as well as normal anatomical variants is of paramount importance in identifying pediatric skull fractures with a greater degree of confidence. Acute fractures appear as lucent cortical defects that do not have sclerotic borders, in contrast to sutures, which might demonstrate sclerotic margins. Three-dimensional (3-D) CT has increased sensitivity and specificity for detecting skull fractures and is essential in the evaluation of pediatric head CTs for distinguishing subtle fractures from sutural variants, especially in the setting of trauma. In this review, we present our experience of the use of 3-D reformats in head CT and its implications on the interpretation, especially in the setting of accidental or abusive head trauma.

Purpose Preoperative three-dimensional computed tomography is currently the gold standard imaging modality in patients with craniofacial anomalies. In these patients, bone structural evaluation is paramount for surgical planning and evaluation of brain parenchyma is often secondary. With the significant complexity of these patients, a majority of patients undergo multiple Computed Tomography (CT) studies from infancy into adulthood. This study presents an ultra-low-dose CT protocol that limits the radiation exposure per CT scan in patients with craniofacial anomalies. Material and methods A total of 200 consecutive patients who underwent head CT for preoperative evaluation of craniofacial anomalies at Tertiary Children's Hospital were included in the study. The kVp, (KiloVoltage Peak) mA (milliAnperage), CT dose index (CTDI), and dose-length product (DLP) were documented from the dose page. Patients were stratified based on age for determining age specific effective dose and for age matched comparison. The age specific effective dose was derived by using the established conversion factor as described in the paper. (1) Standard t test was performed to determine the statistical significance of radiation dose reduction. The Institutional Review Board approved the study and data was collected from 2012-2014. Findings Of the 200 patients assessed in our study, 90 patients had low-dose CT scans and 110 patients had ultra-low-dose CT scans of the head. All patients had diagnostic quality CT studies. The low-dose CT was performed at 120 kVp and 100 mA. The ultra-low-dose CT was performed at 80 kVp and fixed 80 mA. The minimum, maximum and mean effective dose before the introduction of the ultra-low-dose protocol was 0.8 mSv, 6.9 mSV and 2.82 mSv. The minimum, maximum and mean effective dose after the introduction of the ultra-low-dose protocol was 0.6 mSv, 3.8 mSV, and 1.37 mSv. The reduction in the effective radiation dose was statistically significant (standard t test; P = 0.0001). Conclusion Compared to the regular low-dose protocol, the ultra-low-dose CT protocol provided appropriate diagnostic images with a significantly decreased radiation dose.

Aicardi-Goutieres syndrome (AGS) is a rare, genetic inflammatory disease due to mutations in any of the seven genes discovered to date (TREX1, RNASEH2A, RNASEH2B, RNASEH2C, SAMHD1, ADAR, and IFIH1). Clinical onset is seen most commonly in utero or in infancy; irritability, feeding difficulties, jitteriness, microcephaly, abnormal movements, seizures, bone marrow suppression, and liver dysfunction are seen either during the neonatal age group or within the first few months of life with abrupt onset of neurologic regression and slowing of head growth. Diffusely abnormal white matters with swelling of frontal or temporal lobes, cerebral atrophy, and intracranial calcification are typical neuroradiologic abnormalities. However, ADAR mutation, a recently discovered AGS gene, can cause late-onset acute or subacute onset of severe dystonia and features of bilateral striatal necrosis on neuroimaging, in the absence of other typical features of AGS. We report a detailed description of a 5-year-old boy who had a recurrent encephalopathic presentation in the setting of infection. Magnetic resonance imaging (MRI) of brain revealed prominent and fairly symmetrical signal abnormalities in the cerebellar peduncles, thalamus, midbrain, and pons. His throat swab was positive for influenza B, and he was initially diagnosed with influenza encephalopathy. He had a recurrence after 18 months of his initial presentation, and his brain MRI showed extensive areas of signal abnormality similar to, but more extensive than, his previous scan. Extensive spinal cord swelling was also seen. His chronic skin finding was recognized as dyschromatosis symmetrica hereditaria (DSH), and genetic testing revealed compound heterozygous mutations of ADAR gene - causative for AGS. This is the first presentation of recurrent acute encephalopathy in the setting of documented ADAR mutation with the longest interval documented between two acute presentations. This is also the first documentation of extensive spinal cord involvement, which will expand its phenotype. This case also highlights the importance of early identification of DSH, a subtle but characteristic skin lesion of ADAR mutations, for prompt diagnosis of this rare condition.

Purpose: Research demonstrates that children receive twice as much medical radiation from Computed Tomography (CT) scans performed at non-pediatric facilities as equivalent CTs performed at pediatric trauma centers (PTCs). In 2014, AFMC outreach staff educated Emergency Department (ED) staff on appropriate CT imaging utilization to reduce unnecessary medical radiation exposure. We set out to determine the educational campaign’s impact on injured children received radiation dose. Methods: All injured children who underwent CT imaging and were transferred to a Level I PTC during 2010 to 2013 (pre-campaign) and 2015 (post-campaign) were reviewed. Patient demographics, mode of transportation, ED length of stay, scanned body region, injury severity score, and trauma center level were analyzed. Median effective radiation dose (ERD) controlled for each variable, pre-campaign and post-campaign, was compared using Wilcoxon rank sum test. Results: Three hundred eighty-five children under 17 years were transferred from 45 and 48 hospitals, pre- and post-campaign. Most (43%) transferring hospitals were urban or critical access hospitals (30%). Pre- and post-campaign patient demographics were similar. We analyzed 482 and 398 CT scans pre- and post-campaign. Overall, median ERD significantly decreased from 3.80 to 2.80. Abdominal CT scan ERD declined significantly from 7.2 to 4.13 (P-value 0.03). Head CT scan ERD declined from 3.27 to 2.45 (P-value < 0.0001). Conclusion: A statewide, CT scan educational campaign contributed to ERD decline (lower dose scans and fewer repeat scans) among transferred injured children seen at PTCs. State-level interventions are feasible and can be effective in changing radiology provider practices.

Abusive head trauma (AHT) is the leading cause of fatal head injuries in children younger than 2 years. An intracranial pathology can exist even in the setting of a normal physical exam. A delay in the diagnosis of AHT can have serious life-threatening consequences for the child and increases the potential the child will be abused again. In this article, we review the traumatic subdural hematoma as well as various morpho-structural patterns of shearing injuries and thrombosis of intracranial bridging veins. This work serves as a summary of patterns of imaging features of intracranial venous injury in AHT, as described in the literature, to facilitate familiarity and early detection of abusive head trauma in the pediatric population. Essentially, in AHT there is a traumatic injury to the bridging vein with either partial or complete tear. This can secondarily result in thrombosis at the terminal end of the bridging vein with blood clots adjacent to the bridging vein.

BACKGROUND AND PURPOSE: Anxiety and depression during pregnancy have been associated with an increased risk of adverse neurodevelopmental outcomes in offspring. We aimed to study the in utero effects of maternal anxiety and depression on early brain development. MATERIALS AND METHODS: Pregnant women were recruited at ∼36 weeks of gestation for this prospective study. They were assessed for anxiety symptoms by the State-Trait Anxiety Inventory and for depression symptoms by the Beck Depression Inventory, 2nd Edition. After delivery, infant underwent an MR imaging examination of the brain without sedation, including DTI, for evaluation of white matter (WM) development. Infant fractional anisotropy values, a putative marker of WM integrity, were correlated with the mothers’ State-Trait Anxiety Inventory and Beck Depression Inventory scores by using both tract-based spatial statistics and ROI methods. RESULTS: Thirty-four infants were included in this study. Both maternal State-Anxiety and Trait-Anxiety scores negatively correlated (P

BACKGROUND AND PURPOSE: The cesarean delivery rate has increased globally in the past few decades. Neurodevelopmental outcomes associated with cesarean delivery are still unclear. This study investigated whether cesarean delivery has any effect on the brain development of offspring. MATERIALS AND METHODS: A total of 306 healthy children were studied retrospectively. We included 3 cohorts: 2-week-old neonates (cohort 1, n = 32/11 for vaginal delivery/cesarean delivery) and 8-year-old children (cohort 2, n = 37/23 for vaginal delivery/cesarean delivery) studied at Arkansas Children9s Hospital, and a longitudinal cohort of 3-month to 5-year-old children (cohort 3, n = 164/39 for vaginal delivery/cesarean delivery) studied independently at Brown University. Diffusion tensor imaging, myelin water fraction imaging, voxel-based morphometry, and/or resting-state fMRI data were analyzed to evaluate white matter integrity, myelination, gray matter volume, and/or functional connectivity, respectively. RESULTS: While not all MR imaging techniques were shared across the institutions/cohorts, post hoc analyses showed similar results of potential effects of cesarean delivery. The cesarean delivery group in cohort 1 showed significantly lower white matter development in widespread brain regions and significantly lower functional connectivity in the brain default mode network, controlled for a number of potential confounders. No group differences were found in cohort 2 in white matter integrity or gray matter volume. Cohort 3 had significantly different trajectories of white matter myelination between groups, with those born by cesarean delivery having reduced myelin in infancy but normalizing with age. CONCLUSIONS: Cesarean delivery may influence infant brain development. The impact may be transient because similar effects were not observed in older children. Further prospective and longitudinal studies may be needed to confirm these novel findings.

Idiopathic intracranial hypertension (IIH) is a syndrome characterized by increased intracranial pressure (ICP), the absence of structural lesions on neuroimaging, and normal cerebrospinal fluid composition. Cerebral venous sinus thrombosis (CVST) is a common cause of increased ICP and can be differentiated from IIH with magnetic resonance venography. We describe a young woman with typical IIH who underwent lumbar puncture and was treated with a short course of high-dose corticosteroids followed by acetazolamide. She subsequently developed CVST, subarachnoid hemorrhage, and stroke. Risk factors that may have resulted in CVST are discussed.

Background Aicardi-Goutieres syndrome is an early-onset encephalopathy with presumed immune pathogenesis caused by inherited defects in nucleic acid metabolism. It is a model disease to study systemic autoimmunity, and there are many clinical, genetic, and basic science considerations that underline a possible overlap between Aicardi-Goutieres syndrome and systemic lupus erythematosus. Results We describe a 15-year-old girl with Aicardi-Goutieres syndrome due to compound heterozygous pathogenic variants in SAMHD1 (sterile alpha motif domain and HD domain-containing protein 1). Over time, she developed multiple autoimmune diseases (vitiligo, alopecia areata, immune thrombocytopenia, positive antithyroglobulin antibodies) without positive antinuclear antibody or features of systemic lupus erythematosus. Her thrombocytopenia was refractory to treatment with corticosteroids and intravenous immunoglobulin but responded to a standard course of rituximab. Conclusion This is the first report of a multiple autoimmune syndrome in a patient with molecularly proven Aicardi-Goutieres syndrome. This study illustrates an emerging pattern of the natural history of Aicardi-Goutieres syndrome characterized by early encephalopathic presentation followed by symptoms of systemic autoimmunity.

Idiopathic intracranial hypertension often presents with cranial nerve VI paresis. Infrequently, other cranial nerve dysfunction may occur. We report a case of idiopathic intracranial hypertension that presented with bilateral cranial nerve III paresis. Review of the literature suggests that severe elevation of intracranial pressure may predispose patients with idiopathic intracranial hypertension to cranial nerve III dysfunction.

BACKGROUND AND PURPOSE: Studies on infants and children born preterm have shown that adequate gestational length is critical for brain white matter development. Less is known regarding how variations in gestational age at birth in term infants and children affect white matter development, which was evaluated in this study. MATERIALS AND METHODS: Using DTI tract-based spatial statistics methods, we evaluated white matter microstructures in 2 groups of term-born (≥37 weeks of gestation) healthy subjects: 2-week-old infants (n = 44) and 8-year-old children (n = 63). DTI parameters including fractional anisotropy, mean diffusivity, axial diffusivity, and radial diffusivity were calculated by voxelwise and ROI methods and were correlated with gestational age at birth, with potential confounding factors such as postnatal age and sex controlled. RESULTS: Fractional anisotropy values, which are markers for white matter microstructural integrity, positively correlated (P < .05, corrected) with gestational age at birth in most major white matter tracts/regions for the term infants. Mean diffusivity values, which are measures of water diffusivities in the brain, and axial and radial diffusivity values, which are markers for axonal growth and myelination, respectively, negatively correlated (P < .05, corrected) with gestational age at birth in all major white matter tracts/regions excluding the body and splenium of the corpus callosum for the term infants. No significant correlations with gestational age were observed for any tracts/regions for the term-born 8-year-old children. CONCLUSIONS: Our results indicate that longer gestation during the normal term period is associated with significantly greater infant white matter development (as reflected by higher fractional anisotropy and lower mean diffusivity, axial diffusivity, and radial diffusivity values); however, similar associations were not observable in later childhood.

Background Posterior reversible encephalopathy syndrome (PRES) is an acute neurotoxic syndrome that, although characteristically reversible, can result in long-term disability. Our aim was to identify the clinical and radiological factors that are unique to children with PRES compared with adults with the syndrome in a single center. Methods We retrospectively reviewed the clinical and radiological records of all patients with PRES admitted at a tertiary care medical center from 2007 to 2014. All patients who met the clinical and radiological criteria for PRES were dichotomized into children (less than 18 years) and adults (18 years or older) based on their age groups, and comparison of their baseline variables, clinical, laboratory, and imaging features was performed. Results During this study period, 19 pediatric patients and 100 adult patients with PRES were identified. On univariate analysis, factors significantly associated with pediatric patients with the syndrome were multiorgan failure (84.2% vs 50%, P = 0.006), temporal lobe involvement (63.3% vs 39%, P = 0.04), restricted diffusion (42.1% vs 18%, P = 0.02), and less likelihood of cerebellar involvement (21.1% vs 57%, P = 0.004). On bivariate logistic regression analysis, all these factors remained significantly associated with pediatric PRES; multiorgan failure (odds ratio: 5.80, 95% confidence interval: 1.45 to 29.41, P = 0.03), temporal lobe involvement (odds ratio: 5.08, 95% confidence interval: 1.17 to 22.17, P = 0.03), restricted diffusion (odds ratio: 2.48, 95% confidence interval: 1.61 to 10.10, P = 0.02), and less likely to have cerebellar involvement (odds ratio: 0.08, 95% confidence interval: 0.002 to 0.39, P = 0.002). Conclusions Factors unique to PRES in children compared with adults include a greater propensity with multi-organ failure, involvement of the temporal lobe, and restricted diffusion on imaging.

Purpose The long-term cancer risks for children exposed to radiologic images can be two to three times higher than for adults because children are more sensitive to radiation and have a longer lifetime in which to accumulate exposure from CT scans. Injured children often undergo repeat CT imaging if they are transferred from non-pediatric hospitals to a Level I pediatric trauma center (PTC). This study determined the impact of a statewide web-based image repository (WBIR) on repeat imaging among transferred injured children. Methods All injured children who underwent CT imaging and were transferred to the PTC in 2010 (pre-WBIR) and 2013 (post-WBIR) were included. Patient-level factors studied included demographics, body region of scan, Injury Severity Score, and Emergency Department (ED) disposition. Change from pre to post on rate of repeat imaging was assessed. Results Two hundred fifty-four and 233 children, with a median age of 7.3 years, were transferred to the Children's Hospital in 2010 and 2013, respectively. Repeat imaging levels at the PTC were lower post-WBIR than pre-WBIR (20% versus 33%, odds ratio [OR] 0.54, P = .005). Images of the head decreased most significantly (60% versus 33%, OR 0.33). Images performed at Level II and III trauma centers were repeated less often after WBIR. Conclusions The WBIR significantly reduced repeat imaging among injured children transferred to a PTC, especially children transferred from Level II and Level III trauma centers, children with lower-acuity injuries, and children with initial scans of the head. Radiation savings are expected to be beneficial to children.

Recent studies have shown associations between maternal obesity at pre- or early pregnancy and long-term neurodevelopment in children, suggesting in utero effects of maternal obesity on offspring brain development. In this study, we examined whether brain functional connectivity to the prefrontal lobe network is different in newborns from normal-weight or obese mothers. Thirty-four full-term healthy infants from uncomplicated pregnancies were included, with 18 born to normal-weight and 16 born to obese mothers. Two weeks after delivery, the infants underwent an magnetic resonance imaging (MRI) examination during natural sleep, which included structural imaging and resting-state functional MRI (fMRI) scans. Independent component analysis was used to identify the prefrontal lobe network, and dual regression was used to compare functional connectivity between groups. Infants born to normal-weight mothers had higher recruiting (P

Posterior reversible encephalopathy syndrome (PRES) is an acute neurotoxic syndrome that, although characteristically reversible, can be fatal or result in long-term disability in a subset of patients. Our aim was to identify factors associated with poor discharge outcome in PRES patients.We retrospectively reviewed the clinical and radiological records of all patients with PRES admitted at our tertiary care medical center from 2007 to 2014. They were divided based their modified Rankin Score at discharge and compared for their baseline variables, clinical, laboratory and imaging features. Poor outcome was defined by a modified Rankin scale 2-6 and was subdivided based on the primary mechanism that led to poor outcome.Out of 100 PRES subjects, 36% had poor discharge outcomes. Factors associated with poor outcomes on univariate analysis were history of diabetes mellitus, coma, high Charlson comorbidity index, post-transplantation, autoimmune condition, lack of systolic or diastolic hypertension, elevated blood urea nitrogen and involvement of the corpus callosum. On multivariate analysis, only prior diabetes mellitus odd ratio (OR) = 6.8 (95% CI 1.1-42.1, p = 0.04), corpus callosum involvement (OR = 11.7; 95% CI 2.4-57.4, p = 0.00) were associated with poor outcome. Poor outcome also correlated with increased length of hospital stay (OR = 7.9; 95% CI 1.3-49.7, p = 0.03).Large prospective studies incorporating serial blood glucose values and advanced imaging studies are required to validate these findings.

We report a case of subarachnoid haemorrhage resulting from a mycotic aortic aneurysm in a child with CHD. The patient previously underwent operations for CHD and had a subarachnoid haemorrhage of unknown cause before the scheduled re-operation. During the re-operation, a sealed rupture of an undiagnosed mycotic ascending aortic aneurysm was identified, and the causative organism was later identified as Streptococcus. A postoperative MRI indicated a partially thrombosed cerebral aneurysm. This case demonstrates that a mycotic aortic aneurysm can be a cause of intracranial haemorrhage in children.

A 13-year-old girl with infantile-onset self-resolving epilepsy and developmental delay had an unremarkable workup, including normal brain magnetic resonance imaging (MRI) and chromosomal microarray. During adolescence, she presented with features of early-onset parkinsonism: gait dyspraxia, freezing during walking, cogwheel rigidity in both upper extremities, and left arm dystonia. Repeat brain MRI showed iron deposition on the substantia nigra (SN) and basal ganglia, with hyperintense halo sign around a central linear hypointensity within the SN on the T1 imaging sequence. Whole-exome sequencing with trio revealed de novo heterozygote mutation in WDR45 to confirm the diagnosis of beta-propeller protein-associated neurodegeneration (BPAN). BPAN is a rare neurodegenerative with brain iron accumulation disorder with the pathognomonic halo sign. Preferential iron deposition over the SN compared to globus pallidus can distinguish this condition from other iron storage disorders. BPAN does not cause the radiologic eye of the tiger sign seen in other forms of iron storage disorders. Other types of childhood-onset parkinsonian disorders, such as PINK1-related Parkinson disease and Parkin-type Parkinson disease, do not have iron storage in the brain. This report describes a case of early-onset parkinsonism secondary to a mutation in WDR45. It underscores the importance of brain MRI to differentiate this condition from other childhood-onset parkinsonism and also other brain iron accumulation disorders. This report also shows iron deposition over the pituitary as a novel site of iron deposition in BPAN and emphasizes the presence of peri-dentate white matter volume loss and hyperintensity, which is another key radiologic abnormality associated with BPAN.

Posterior reversible encephalopathy syndrome (PRES) (synonym: reversible posterior leukoencephalopathy syndrome, reversible posterior cerebral edema syndrome) is a neurotoxic state associated with sudden elevation in blood pres- sure. It presents initially with lethargy and somnolence but can also manifest as headache, confusion, hemianopsia, nausea vomiting, seizures, vision disturbance, paresis, and cortical blindness. Very often these features are short lived and are resolved both clinically and radiologically but occasionally this disease can progress to coma and other devastating permanent complica- tions. Several etiological factors have been described in literature including hypertensive encephalopathy, preeclampsia, eclamp- sia, infection, immunosuppression, autoimmune diseases, cyclosporine toxicity, drug overdose, trauma with aortic dissection, pheochromocytoma and Guillain-Barre syndrome (GBS). Particularly, very few cases have been reported in literature indicating GBS as a precipitating factor for PRES. Therefore, we present here a brief review of literature describing association between GBS and PRES along with detailed diagnostic and therapeutic description. This review suggests necessity for adequate clinical suspicion for PRES in GBS afflicted patient followed by prompt diagnostic and therapeutic intervention. Failure to treat promptly and adequately might lead to the catastrophic consequence both in terms of morbidity and mortality.

Use of cranial CT scans in children has been increasing, in part due to increased awareness of sports-related concussions. CT is the largest contributor to medical radiation exposure, a risk factor for cancer. Long-term cancer risks of CT scans can be two to three times higher for children than for adults because children are more radiosensitive and have a longer lifetime in which to accumulate exposure from multiple scans.To compare the radiation exposure injured children receive when imaged at nonpediatric hospitals (NPHs) versus pediatric hospitals.Injured children younger than 18 years who received a CT scan at a referring hospital during calendar years (CYs) 2010 and 2013 were included. Patient-level factors included demographics, mode of transportation, and Injury Severity Score, and hospital-level factors included region of state, radiology services, and hospital type and size. Our primary outcome of interest was the effective radiation dose.Four hundred eighty-seven children were transferred to the pediatric trauma center during CYs 2010 and 2013, with a median age of 7.2 years (interquartile range 5-13). The median effective radiation dose received at NPHs was twice that received at the pediatric trauma center (3.8 versus 1.6 mSv, P.001). Results were confirmed in independent and paired analyses, after controlling for mode of transportation, emergency department disposition, level of injury severity, and at the NPH trauma center level, hospital type, size, region, and radiology services location.NPHs have the potential to substantially reduce the medical radiation received by injured children. Pediatric CT protocols should be considered.

BACKGROUND: Brain injury is observed on cranial magnetic resonance imaging preoperatively in up to 50% of newborns with congenital heart disease. Newer imaging techniques such as diffusion tensor imaging provide sensitive measures of the white matter integrity. The objective of this study was to evaluate the diffusion tensor imaging analysis technique of tract-based spatial statistics in newborns with congenital heart disease. METHODS: Term newborns with congenital heart disease who would require surgery at less than 1 month of age were prospectively enrolled (n ¼ 19). Infants underwent preoperative and postoperative brain magnetic resonance imaging with diffusion tensor imaging. Tract-based spatial statistics, an objective whole-brain diffusion tensor imaging analysis technique, was used to determine differences in white matter fractional anisotropy between infant groups. Term control infants were also compared with congenital heart disease infants. Postmenstrual age was equivalent between congenital heart disease infant groups and between congenital heart disease and control infants. RESULTS: Ten infants had preoperative brain injury, either infarct or white matter injury, by conventional brain magnetic resonance imaging. The technique of tract-based spatial statistics showed significantly lower fractional anisotropy (P < 0.05, corrected) in multiple major white matter tracts in the infants with preoperative brain injury compared with infants without preoperative brain injury. Fractional anisotropy values increased in the white matter tracts from the preoperative to the postoperative brain magnetic resonance imaging correlating with brain maturation. Control infants had higher fractional anisotropy in multiple white matter tracts compared with infants with congenital heart disease. CONCLUSION: Tract-based spatial statistics is a valuable diffusion tensor imaging analysis technique that may have better sensitivity in detecting white matter injury compared with conventional brain magnetic resonance imaging in term newborns with congenital heart disease.

The pathophysiology of posterior reversible encephalopathy syndrome (PRES) is incompletely understood; however, an underlying state of immune dysregulation and endothelial dysfunction has been proposed. We examined alterations of serum lactate dehydrogenase (LDH), a marker of endothelial dysfunction, relative to the development of PRES in patients receiving chemotherapy. A retrospective Institutional Review Board approved database of 88 PRES patients was examined. PRES diagnosis was confirmed by congruent clinical diagnosis and MRI. Clinical features at presentation were recorded. Serum LDH values were collected at three time points: prior to, at the time of, and following PRES diagnosis. Student's t-test was employed. LDH values were available during the course of treatment in 12 patients (nine women; mean age 57.8 years [range 33-75 years]). Chemotherapy-associated PRES patients were more likely to be normotensive (25%) versus the non-chemotherapy group (9%). LDH levels at the time of PRES diagnosis were higher than those before and after (p=0.0263), with a mean difference of 114.8 international units/L. Mean time intervals between LDH measurement prior to and following PRES diagnosis were 44.8 days and 51.4 days, respectively. Mean elapsed time between last chemotherapy administration and PRES onset was 11.1days. In conclusion, serum LDH, a marker of endothelial dysfunction, shows statistically significant elevation at the onset of PRES toxicity in cancer patients receiving chemotherapy. Our findings support a systemic process characterized by endothelial injury/dysfunction as a factor, if not the prime event, in the pathophysiology of PRES.

KCNQ2 mutations causing benign familial neonatal convulsions were initially described more than 10 years ago but more recently reports suggest that early infantile encephalopathy can also be caused by the same genetic mutation. We report an infant with de novo KCNQ2 mutation who presented with drug responsive myoclonic epilepsy in early infancy but later had a presentation consistent with West syndrome including hypsarrhythmia and electrodecrement pattern during spasms. This report highlights the importance of genomic screening in patients with infantile epileptic encephalopathy and the emergence of a broad phenotype of KCNQ2 mutations.

Early brain injury occurs in newborns with congenital heart disease (CHD) placing them at risk for impaired neurodevelopmental outcomes. Predictors for preoperative brain injury have not been well described in CHD newborns. This study aimed to analyze, retrospectively, brain magnetic resonance imaging (MRI) in a heterogeneous group of newborns who had CHD surgery during the first month of life using a detailed qualitative CHD MRI Injury Score, quantitative imaging assessments (regional apparent diffusion coefficient [ADC] values and brain volumes), and clinical characteristics. Seventy-three newborns that had CHD surgery at 8 ± 5 (mean ± standard deviation) days of life and preoperative brain MRI were included; 38 also had postoperative MRI. Thirty-four (34/73, 47%) had at least 1 type of preoperative brain injury, and 28/38 (74%) had postoperative brain injury. The 5-minute APGAR score was negatively associated with preoperative injury, but there was no difference between CHD types. Infants with intraparenchymal hemorrhage, deep gray matter injury, and/or watershed infarcts had the highest CHD MRI Injury Scores. ADC values and brain volumes were not different in infants with different CHD types, or in those with and without brain injury. In a mixed group of CHD newborns, brain injury was found preoperatively on MRI in almost 50%, and there were no significant baseline characteristic differences to predict this early brain injury, except 5-minute APGAR score. We conclude that all infants, regardless of CHD type, who require early surgery, should be evaluated with MRI as they are all at high risk for brain injury.

Trauma is the leading cause of morbidity and mortality in children. Computed tomography examinations play an important role in the management of patients with major trauma. Though abdominal trauma is less common compared to head injuries, the associated morbidity and mortality are substantial. It is easier to diagnose solid abdominal injuries than intestinal or mesenteric injuries on CT examinations. However, recognition of bowel injury is very important as a delay in diagnosis increases the morbidity and mortality. Hence, with every CT of the abdomen and pelvis, the radiologist must look for signs of bowel and mesenteric injury. This pictorial review presents various CT findings of blunt intestinal injury in children.

The number of dirt bike injuries in children in the United States is increasing and poses a public health problem.The purpose of our study was to identify the imaging patterns of dirt bike injuries in children and associations with morbidity and mortality.The study included 85 children (83 boys, 2 girls)18 year of age (mean age 12.3 years, standard deviation 3 years) with dirt bike injury treated at a tertiary care pediatric hospital. Imaging studies and hospital medical records were reviewed. Outcomes were classified into the following categories: short-term disability, long-term disability or no follow-up available. Imaging studies were reviewed for head, torso, and extremity injuries. One-tailed z test for two proportions was used to determine significant differences between various proportions. Chi-square test with Yates correction was used to determine the significance of long-term disability with injury type.Long bone fractures were the most common injuries. Lower extremity fractures accounted for 79% of extremity fractures and were significantly more common than upper extremity fractures (p = 0.001). Head injuries included fractures (n = 9), brain contusion (n = 5), and meningeal hemorrhage (n = 2). Head injury was associated with long-term disability (p0.0001). All torso injuries were solitary.Long-term disability was associated with head injuries but not with torso or extremity injuries. Lower extremity injuries were significantly more common than upper extremity injuries. Torso solid organ injuries were uniformly solitary.

In the Neonatal Erythropoietin and Therapeutic Hypothermia Outcomes study, 9/20 erythropoietin-treated vs 12/24 placebo-treated infants with hypoxic-ischemic encephalopathy had acute brain injury. Among infants with acute brain injury, the injury volume was lower in the erythropoietin than the placebo group (P = .004). Higher injury volume correlated with lower 12-month neurodevelopmental scores. Trial registration ClinicalTrials.gov : NCT01913340 .

Osteopetrosis is a rare, heterogeneous group of inherited diseases characterized by increased bone mass and density due to defective osteoclasts, leading to failure in bone resorption. Pathological features are related to increase in bone mass density and altered craniofacial morphology. Cranial nerve compression due to narrowing of cranial foramina can lead to progressive blindness and deafness. Bone marrow failure can lead to pancytopenia and life-threatening infections. Early diagnosis is crucial due to a short window of opportunity for curative treatment and to prevent complications such as visual impairment. We present the case of a late-preterm infant who had recurrent hypocalcemic seizures and visual impairment. Genetic testing confirmed the diagnosis of autosomal recessive osteopetrosis.

Background Incontinentia pigmenti is a rare X-linked neurocutaneous disorder that can present in the neonatal period with seizures and encephalopathy. Brain magnetic resonance imaging and magnetic resonance angiography may reveal cerebral infarction and arteriopathy. Patient Description We describe a neonate with the typical rash of incontinentia pigmenti along with seizures and brain magnetic resonance imaging abnormalities. Results Brain magnetic resonance imaging, magnetic resonance angiography, and magnetic resonance spectroscopy at age one week revealed chronic and acute brain injury, arteriopathy of the small and medium-sized cerebral vessels, and elevation of a lactate metabolite peak. By age six months, her magnetic resonance angiogram had normalized. At age 2.5 years, she has well-controlled complex partial seizures, global developmental delay, and residual hemiplegia. Conclusion Despite extensive cerebral arteriopathy in association with incontinentia pigmenti, this girl had a relatively stable early clinical course, steady developmental progress over time, and seizures that have been well controlled. Later brain imaging revealed resolution of the arteriopathy.

Current convex tethering techniques for treatment of scoliosis have centered on anterior convex staples or polypropylene tethers. We hypothesized that an allograft tendon tether inserted via the costo-transverse foramen would correct an established spinal deformity. In the pilot study, six 8-week-old pigs underwent allograft tendon tethering via the costo-transverse foreman or sham to test the strength of the transplanted tendon to retard spine growth. After 4 months, spinal deformity in three planes was induced in all animals with allograft tendons. In the treatment study, the allograft tendon tether was used to treat established scoliosis in 11 8-week-old pigs (spinal deformity 50°). Once the deformity was observed (4 months) animals were assigned to either no treatment group or allograft tendon tether group and progression assessed by monthly radiographs. At final follow-up, coronal Cobb angle and maximum vertebral axial rotation of the treatment group was significantly smaller than the non-treatment group, whereas sagittal kyphosis of the treatment group was significantly larger than the non-treatment group. In sum, a significant correction was achieved using a unilateral allograft tendon spinal tether, suggesting that an allograft tendon tethering approach may represent a novel fusion-less procedure to correct idiopathic scoliosis. © 2016 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 35:183-192, 2017.