Contemporary incidence of stroke (focal infarct and/or haemorrhage) determined by neuroimaging and neurodevelopmental disability at 12 months of age in neonates undergoing cardiac surgery utilizing cardiopulmonary bypass.

OBJECTIVES: When evaluated prospectively, acute brain injury is reported in up to 75% of neonates undergoing cardiopulmonary bypass (CPB), predominantly white matter injury rather than stroke. This study investigates the incidence of stroke (focal infarct and/or haemorrhage) detected by neuroimaging in contemporary clinical practice, whereby magnetic resonance imaging/computed tomography routinely occurs in response to clinical events, comparing those undergoing the Norwood procedure with those undergoing other neonatal procedures involving CPB, and defines the relationship between stroke and neurodevelopmental disability (NDD) at 12 months of age. METHODS: One hundred and twenty neonates underwent CPB between July 2011 and December 2014: 25 Norwood procedures and 95 non-Norwood procedures. Data were retrospectively collected including clinical data and 12-month neurodevelopmental assessment using the Bayley Scales of Infant and Toddler Development, Third Edition (BSID-III). RESULTS: Stroke was detected in 12% of neonates in current clinical practice: 24% of the Norwood group vs 8% of the non-Norwood group (P = 0.03). Significant predictors of stroke in the univariate analysis included the Norwood procedure, lowest operative temperature and use of extracorporeal membrane oxygenation (P < 0.05). The lowest operative temperature and use of extracorporeal membrane oxygenation remained significant in the multivariate analysis (P < 0.05). Fifty-seven percent were assessed using the BSID-III assessment, and 68% demonstrated NDD in at least 1 subscale. In neonates who suffered stroke, the incidence of NDD was significantly greater in 4/5 subscales compared with those with no injury (P < 0.05). The Norwood group had a significantly greater incidence of NDD in 2/5 subscales when compared with the non-Norwood group (P < 0.05). CONCLUSIONS: Stroke, established by neuroimaging in contemporary clinical practice, was detected in 12% of neonates having CPB, and those undergoing the Norwood procedure have a 3-fold risk of injury. Stroke was associated with NDD at 12 months of age.

Pineal cysts-A benign association with familial retinoblastoma.

Patients with familial/heritable retinoblastoma (RB) are at increased risk of developing second malignancies throughout life, including a pineoblastoma (trilateral RB [TRB]) in early childhood. Current guidelines recommend regular surveillance brain imaging for those with heritable RB until 5 years of age. The presence of pineal cysts has been reported in patients with RB. Pineal cysts are thought to arise due to focal degeneration of the pineal gland and can be found incidentally. The finding of pineal abnormalities including cysts in children with RB on imaging is disconcerting, as it raises the possibility of an underlying malignancy, specifically a pinealoblastoma. The authors reviewed the imaging findings and clinical significance of pineal cysts in 69 patients diagnosed with RB at our center between December 1999 and November 2015. Twenty-six patients had pineal cysts found on brain magnetic resonance imaging (MRI) scans performed either at diagnosis or follow-up. Thirty-eight of 69 patients had underlying heritable RB. Nineteen of 38 familial RB patients had a pineal cyst compared with 3 out of 26 with sporadic RB (P = .004). In the majority, the imaging characteristics and size of the cysts remained stable or resolved. In this cohort, pineal cysts were detected at significantly increased frequency in heritable RB. This may be a benign association or may reflect abnormal underlying biology of pineal tissue in individuals highly susceptible to malignancy. Imaging characteristics can be helpful in distinguishing between benign and malignant lesions. The presence of a pineal cyst in patients with unilateral disease may be a useful indicator of underlying heritable RB.

B Cell, Th17, and Neutrophil Related Cerebrospinal Fluid Cytokine/Chemokines Are Elevated in MOG Antibody Associated Demyelination.

BACKGROUND: Myelin oligodendrocyte glycoprotein antibody (MOG Ab) associated demyelination represents a subgroup of autoimmune demyelination that is separate from multiple sclerosis and aquaporin 4 IgG-positive NMO, and can have a relapsing course. Unlike NMO and MS, there is a paucity of literature on immunopathology and CSF cytokine/chemokines in MOG Ab associated demyelination. AIM: To study the differences in immunopathogenesis based on cytokine/chemokine profile in MOG Ab-positive (POS) and -negative (NEG) groups. METHODS: We measured 34 cytokines/chemokines using multiplex immunoassay in CSF collected from paediatric patients with serum MOG Ab POS [acute disseminated encephalomyelitis (ADEM = 8), transverse myelitis (TM = 2) n = 10] and serum MOG Ab NEG (ADEM = 5, TM = 4, n = 9) demyelination. We generated normative data using CSF from 20 non-inflammatory neurological controls. RESULTS: The CSF cytokine and chemokine levels were higher in both MOG Ab POS and MOG Ab NEG demyelination groups compared to controls. The CSF in MOG Ab POS patients showed predominant elevation of B cell related cytokines/chemokines (CXCL13, APRIL, BAFF and CCL19) as well as some of Th17 related cytokines (IL-6 AND G-CSF) compared to MOG Ab NEG group (all p<0.01). In addition, patients with elevated CSF MOG antibodies had higher CSF CXCL13, CXCL12, CCL19, IL-17A and G-CSF than patients without CSF MOG antibodies. CONCLUSION: Our findings suggest that MOG Ab POS patients have a more pronounced CNS inflammatory response with elevation of predominant humoral associated cytokines/chemokines, as well as some Th 17 and neutrophil related cytokines/chemokines suggesting a differential inflammatory pathogenesis associated with MOG antibody seropositivity. This cytokine/chemokine profiling provides new insight into disease pathogenesis, and improves our ability to monitor inflammation and response to treatment. In addition, some of these molecules may represent potential immunomodulatory targets.

ß-HCG Elevation in Wilms Tumor: An Uncommon Presentation.

Wilms tumor (nephroblastoma) is a readily diagnosed common abdominal tumor in children. Rarely, it may present with factors that may confound the diagnosis. We report a 6-year-old female child who presented with a rapidly growing and invasive abdominal mass with the histopathologic features of Wilms tumor associated with an elevated serum beta human chorionic gonadotropin, which has not been previously reported in this condition.

Longitudinal assessment of cognition and T2-hyperintensities in NF1: an 18-year study.

The developmental course of cognitive deficits in individuals with neurofibromatosis type 1 (NF1) is unclear. The objectives of this study were to determine the natural history of cognitive function and MRI T2-hyperintesities (T2H) from childhood to adulthood and to examine whether the presence of discrete T2H in childhood can predict cognitive performance in adulthood. We present cognitive and structural neuroimaging data from 18 patients with NF1 and five sibling controls assessed prospectively across an 18-year period. Longitudinal analyses revealed a significant increase in general cognitive function in patients with NF1 over the study period. Improvements were limited to individuals with discrete T2H in childhood. Patients without lesions in childhood exhibited a stable profile. The number of T2H decreased over time, particularly discrete lesions. Lesions located within the cerebral hemispheres and deep white matter were primarily stable, whereas those located in the basal ganglia, thalamus and brainstem tended to resolve. Our results support the hypothesis that resolution of T2H is accompanied by an improvement in general cognitive performance, possibly as a result of increased efficiency within white matter tracts.

Variability and accuracy in interpretation of consolidation on chest radiography for diagnosing pneumonia in children under 5 years of age.

BACKGROUND: Consolidation on chest radiography is widely used as the reference standard for defining pneumonia and variability in interpretation is well known but not well explored or explained. METHODS: Three pediatric sub-specialists (infectious diseases, radiology and respiratory medicine) viewed 3,033 chest radiographs in children aged under 5 years of age who presented to one Emergency Department (ED) with a febrile illness. Radiographs were viewed blind to clinical information about the child and blind to findings of other readers. Each chest radiograph was identified as positive or negative for consolidation. Percentage agreement and kappa scores were calculated for pairs of readers. Prevalence of consolidation and reader sensitivity/specificity was estimated using latent class analysis. RESULTS: Using the majority rule, 456 (15%) chest radiographs were positive for consolidation while the latent class estimate was 17%. The radiologist was most likely (21.3%) and respiratory physician least likely (13.7%) to diagnose consolidation. Overall percentage agreement for pairs of readers was 85-90%. However, chance corrected agreement between the readers was moderate, with kappa scores 0.4-0.6 and did not vary with patient characteristics (age, gender, and presence of chronic illness). Estimated sensitivity ranged from 0.71 to 0.81 across readers, and specificity 0.91 to 0.98. CONCLUSIONS: Overall agreement for identification of consolidation on chest radiographs was good, but agreement adjusted for chance was only moderate and did not vary with patient characteristics. Clinicians need to be aware that chest radiography is an imperfect test for diagnosing pneumonia and has considerable variability in its interpretation.

Correlations between polysomnographic and lateral airway radiograph measurements in paediatric obstructive sleep apnoea.

AIM: To evaluate the ability of lateral airway radiography (LAR) to assess adenoidal hypertrophy in children and correlate with the severity of obstructive sleep apnoea (OSA). METHODS: This cohort study was undertaken in 72 children who presented consecutively for evaluation of OSA to the outpatients of the Children's Hospital at Westmead. All children had LAR and overnight polysomnography (PSG). Five assessors, with varying experience, were blinded to the PSG results and independently analysed the LAR. Inter-rater reliability was determined for four published assessment methods; Hibbert, Johannesson, Fujioka and Cohen and Konak. We then compared the four LAR results with PSG-determined criteria for OSA. RESULTS: Using intraclass correlations, inter-rater correlations were moderate to high for all four standardised evaluations of LAR with values ranging from 0.51 to 0.96. With the radiologist taken as the 'gold standard', individual assessors ranged from 0.05 to 0.91. LAR correlated best with PSG determined obstructive apnoea hypopnea index and minimum oxygen saturation for the anterior airway measurement (Hibbert) with r-values of -0.25 and 0.25 respectively (P < 0.05). CONCLUSION: Amongst four methods of evaluating adenoid size, the anterior airway size correlated best with PSG variables of obstructive respiratory index and minimum oxygen saturation. However, the methods are not able to be used as a predictor for OSA.

The role of venous sinus outflow obstruction in pediatric idiopathic intracranial hypertension.

OBJECT: The authors examined the role of venous sinus obstruction in the etiology of idiopathic intracranial hypertension (IIH) by reviewing more than 200 MR venograms performed in suspected cases of IIH. METHODS: Individual MR venograms performed in cases of suspected IIH at the Children's Hospital at Westmead in Sydney, Australia, were reviewed. The authors excluded cases in which an intervention was performed before the scan or a structural cause for venous obstruction was identified. Cases with confirmed hydrocephalus were also excluded. For each of the 145 remaining scans, the authors completed a detailed review on a slice-by-slice basis of the 2D source images used to compile the rendered 3D MR venogram. The anatomical configuration of the dural venous sinuses and any areas of decreased flow in circulation were then noted. Where possible, they correlated their radiological findings with evidence of raised intracranial pressure based on LP opening pressures. They also reviewed a control group of 50 MR venograms. RESULTS: Seventy-six (52%) of 145 scans showed evidence of venous obstruction in the dominant-side circulation. Substantial nonphysiological collateral circulation was seen in 68% of cases with dominant-sided obstruction, suggesting a process of recanalization. In contrast, in the absence of dominant-sided obstruction, collateral circulation was uncommon. In 27 cases, CSF opening pressure measurements were available. In 20 cases the opening pressures were in excess of 20 cm H(2)O. Of those, 17 demonstrated evidence of dominant-sided venous outflow obstruction. Among those cases, the median opening pressure was 34 cm H(2)O. Dominant-sided venous outflow obstruction was seen in only 2 of 50 MR venograms in the control group. Furthermore, evidence of collateral circulation was also uncommon in the control group. There was a highly statistically significant difference between rates of dominant-sided venous obstruction in the suspected IIH and control groups (p ≤ 0.001). CONCLUSIONS: A majority of patients presenting for investigation of suspected IIH demonstrated evidence of dominant-sided venous obstruction on MR venogram. In addition there was a high correlation between elevated CSF opening pressures and dominant-sided venous sinus obstruction. This correlation was further supported by evidence of collateral recanalization in patients with elevated CSF pressures and dominant-sided venous obstruction. A control group of 50 MR venograms indicated that dominant-sided venous outflow obstruction is an unlikely incidental finding, and a highly statistically significant difference was found between rates of obstruction in the suspected IIH and control groups.

Neurosarcoidosis of the conus medullaris and cauda equina.

An uncommon case of neurosarcoidosis of the conus medullaris and cauda equina is described in a 24-year-old man with a 4-year history of urological symptoms and rash. Chest X-ray and biopsy confirmed sarcoidosis and MRI diagnosed conus medullaris involvement.