Periventricular hemorrhagic infarction (PVHI) associated with intraventricular hemorrhage (IVH) in premature infants: Outcome at 2 years of age.

OBJECTIVE: Neonates can develop periventricular hemorrhagic infarction (PVHI) in association with intraventricular hemorrhage (IVH). The prognosis of this condition remains under debate. The aim of this study was to compare the neurological outcome at 2 years of age for neonates who have IVH with and without PVHI. METHODS: This retrospective single-center study (2010-2017) included all neonates who had at least a grade II IVH on ultrasound (US). The population was divided into two groups: Group 1 had grade II-III IVH without PVHI and Group 2 had grade II-III IVH with PVHI. All clinical and imaging (US and magnetic resonance images) data were reviewed. Neurological sequelae were classified as either mild (favorable outcome), or moderate or severe (unfavorable outcome), based on the follow-up report at the age of 2 years. RESULTS: A total of 25 of the 46 infants meeting the inclusion criteria were excluded due to missing clinical or imaging data at diagnosis or follow-up. Of the remaining 21 infants, all were preterm and had grade II-III IVH, either with PVHI (n = 10) or without (n = 11). The neurological prognosis was unfavorable for both groups. Except for the size of the lesion, no radiological findings appeared to be significantly associated with an unfavorable prognosis. Overall, 11 of the infants died early in life; i.e., the mortality rate was 52% (n = 11/21). The deaths were not due to neurological complications of prematurity. C-section was more common in the PVHI group. CONCLUSION: PVHI does not appear to be predictive of an unfavorable outcome. Lesion size may be a predictive factor for an unfavorable prognosis in children with PVHI.

E.U. paediatric MOG consortium consensus: Part 1 - Classification of clinical phenotypes of paediatric myelin oligodendrocyte glycoprotein antibody-associated disorders.

Over the past few years, increasing interest in the role of autoantibodies against myelin oligodendrocyte glycoprotein (MOG-abs) as a new candidate biomarker in demyelinating central nervous system diseases has arisen. MOG-abs have now consistently been identified in a variety of demyelinating syndromes, with a predominance in paediatric patients. The clinical spectrum of these MOG-ab-associated disorders (MOGAD) is still expanding and differs between paediatric and adult patients. This first part of the Paediatric European Collaborative Consensus emphasises the diversity in clinical phenotypes associated with MOG-abs in paediatric patients and discusses these associated clinical phenotypes in detail. Typical MOGAD presentations consist of demyelinating syndromes, including acute disseminated encephalomyelitis (ADEM) in younger, and optic neuritis (ON) and/or transverse myelitis (TM) in older children. A proportion of patients experience a relapsing disease course, presenting as ADEM followed by one or multiple episode(s) of ON (ADEM-ON), multiphasic disseminated encephalomyelitis (MDEM), relapsing ON (RON) or relapsing neuromyelitis optica spectrum disorders (NMOSD)-like syndromes. More recently, the disease spectrum has been expanded with clinical and radiological phenotypes including encephalitis-like, leukodystrophy-like, and other non-classifiable presentations. This review concludes with recommendations following expert consensus on serologic testing for MOG-abs in paediatric patients, the presence of which has consequences for long-term monitoring, relapse risk, treatments, and for counselling of patient and families. Furthermore, we propose a clinical classification of paediatric MOGAD with clinical definitions and key features. These are operational and need to be tested, however essential for future paediatric MOGAD studies.

E.U. paediatric MOG consortium consensus: Part 4 - Outcome of paediatric myelin oligodendrocyte glycoprotein antibody-associated disorders.

There is increasing knowledge on the role of antibodies against myelin oligodendrocyte glycoprotein (MOG-abs) in acquired demyelinating syndromes and autoimmune encephalitis in children. Better understanding and prediction of outcome is essential to guide treatment protocol decisions. Therefore, this part of the Paediatric European Collaborative Consensus provides an oversight of existing knowledge of clinical outcome assessment in paediatric MOG-ab-associated disorders (MOGAD). The large heterogeneity in disease phenotype, disease course, treatment and follow-up protocols is a major obstacle for reliable prediction of outcome. However, the clinical phenotype of MOGAD appears to be the main determinant of outcome. Patients with a transverse myelitis phenotype in particular are at high risk of accruing neurological disability (motor and autonomic), which is frequently severe. In contrast, having a single episode of optic neuritis any time during disease course is broadly associated with a lower risk of persistent disability. Furthermore, MOG-ab-associated optic neuritis often results in good functional visual recovery, although retinal axonal loss may be severe. The field of cognitive and behavioural outcome and epilepsy following demyelinating episodes has not been extensively explored, but in recent studies acute disseminated encephalomyelitis (-like) phenotype in the young children was associated with cognitive problems and epilepsy in long-term follow-up. In conclusion, main domains of importance in determining clinical outcome in paediatric MOGAD are visual, motor, autonomic and cognitive function. A standardised evaluation of these outcome domains in all children is of importance to allow adequate rehabilitation and follow-up.

Prognostic value of diffusion-weighted magnetic resonance imaging of brain in fetal growth restriction: results of prospective multicenter study.

OBJECTIVE: To measure prospectively apparent diffusion coefficient (ADC) values between 28 and 32 weeks of gestation in different cerebral territories of fetuses with estimated fetal weight (EFW) ≤ 5th centile, and analyze their association with adverse perinatal outcome. METHODS: This was a prospective study involving six tertiary-level perinatal centers. In the period 22 November 2016 to 11 September 2017, we included singleton, small-for-gestational-age (SGA) fetuses with EFW ≤ 5th percentile, between 28 and 32 weeks of gestation, regardless of the umbilical artery Doppler and maternal uterine artery Doppler findings. A fetal magnetic resonance imaging (MRI) examination with diffusion-weighted sequences (DWI) was performed within 14 days following inclusion and before 32 weeks. ADC values were calculated in the frontal and occipital white matter, basal ganglia and cerebellar hemispheres. An ultrasound examination was performed within 1 week prior to the MRI examination. The primary outcome was a composite measure of adverse perinatal outcome, defined as any of the following: perinatal death; admission to neonatal intensive care unit with mechanical ventilation > 48 h; necrotizing enterocolitis; Grade III-IV intraventricular hemorrhage; periventricular leukomalacia. A univariate comparison of median ADC values in all cerebral territories between fetuses with and those without adverse perinatal outcome was performed. The association between ADC values and adverse perinatal outcome was then analyzed using multilevel logistic regression models to adjust for other common prognostic factors for growth-restricted fetuses. RESULTS: MRI was performed in 64 patients, of whom five were excluded owing to fetal movement artifacts on DWI and two were excluded for termination of pregnancy with no link to fetal growth restriction (FGR). One intrauterine death occurred secondary to severe FGR. Among the 56 liveborn neonates, delivered at a mean ± SD gestational age of 33.6 ± 3.0 weeks, with a mean birth weight of 1441 ± 566 g, four neonatal deaths occurred. In addition, two neonates required prolonged mechanical ventilation, one of whom also developed necrotizing enterocolitis. Overall, therefore, seven out of 57 (12.3%) cases had an adverse perinatal outcome (95% CI, 3.8-20.8%). The ADC values in the frontal region were significantly lower in the group with adverse perinatal outcome vs those in the group with favorable outcome (mean values of both hemispheres, 1.68 vs 1.78 × 10-3 mm2 /s; P = 0.04). No significant difference in ADC values was observed between the two groups in any other cerebral territory. A cut-off value of 1.70 × 10-3 mm2 /s was associated with a sensitivity of 57% (95% CI, 18-90%), a specificity of 78% (95% CI, 63-88%), a positive predictive value of 27% (95% CI, 8-55%) and a negative predictive value of 93% (95% CI, 80-98%) for the prediction of adverse perinatal outcome. A mean frontal ADC value < 1.70 × 10-3 mm2 /s was not associated significantly with an increased risk of adverse perinatal outcome, either in the univariate analysis (P = 0.07), or when adjusting for gestational age at MRI and fetal sex (odds ratio (OR), 6.06 (95% CI, 0.9-37.1), P = 0.051) or for umbilical artery Doppler (OR, 6.08 (95% CI, 0.89-41.44)). CONCLUSION: This first prospective, multicenter, cohort study using DWI in the setting of SGA found lower ADC values in the frontal white-matter territory in fetuses with, compared with those without, adverse perinatal outcome. To determine the prognostic value of these changes, further standardized evaluation of the neurodevelopment of children born with growth restriction is required. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

Craniosynostosis and metabolic bone disorder. A review.

INTRODUCTION: Some metabolic bone disorders may result in the premature closure of one or more calvarial sutures during childhood, potentially leading to a cranioencephalic disproportion. The aim of this paper is to review the characteristics and consequences of craniosynostosis associated with metabolic disorder. MATERIAL AND METHODS: A review of the literature on metabolic forms of craniosynostosis was performed. RESULTS: The most common forms of craniosynostosis associated with metabolic bone disorder were isolated sagittal suture fusion with or without scaphocephaly, and sagittal suture fusion associated with coronal suture fusion (oxycephaly) or also with lambdoid suture fusion (pansynostosis). Synostosis may be well-tolerated, but in some subjects results in neurodevelopmental and functional impairment that is sometimes severe. CONCLUSION: The impact of metabolic synostosis is very variable, depending on the specific underlying metabolic disease, with a large spectrum of morphological and functional consequences. Diagnosis should be early and management should be carried out by a multidisciplinary team with expertise in both rare skeletal disorders and craniosynostosis. The impact of emergent medical therapies recently developed for some of these diseases will be assessed by systematic coherent follow-up of international registries.

Imaging features of atypical bleeds in young patients with hemophilia.

Hemarthroses and muscle bleeds are well-known and well-documented complications in pediatric and young adult hemophilia patients. In contrast, deep bleeds in atypical locations can be a diagnostic challenge, since clinicians and radiologists are often unfamiliar with their clinical and radiological features. Some atypical bleeds, however, can be life-threatening or severely disabling, highlighting the need for prompt, accurate diagnosis. Rare bleeds include central nervous system bleeds (including intracranial and spinal hematomas), urogenital bleeds, intra-abdominal bleeds (mesenteric and gastrointestinal wall hematomas) and pseudo tumors in unusual locations like the sinonasal cavities. Because clinical assessment can be difficult, clinicians and radiologists should be aware of the possibility of these rare complications in their hemophilia patients, so that they can avoid unnecessary invasive diagnostic and surgical procedures and institute prompt, appropriate treatment. The purpose of this review is to illustrate the imaging features of bleeds that occur in rare locations in young (i.e., children and young adults) patients with hemophilia to make the reader more familiar with these conditions.

Neonatal brain MRI: how reliable is the radiologist's eye?

INTRODUCTION: White matter (WM) analysis in neonatal brain magnetic resonance imaging (MRI) is challenging, as demonstrated by the issue of diffuse excessive high signal intensity (DEHSI). We evaluated the reliability of the radiologist's eye in this context. METHODS: Three experienced observers graded the WM signal intensity on axial T2-weighted 1.5T images from 60 different premature newborns on 2 occasions 4 weeks apart with a semi-quantitative classification under identical viewing conditions. RESULTS: The intra- and inter-observer correlation coefficients were fair to moderate (Fleiss' kappa between 0.21 and 0.60). CONCLUSION: This is a serious limitation of which we need to be aware, as it can lead to contradictory conclusions in the challenging context of term-equivalent age brain MRI in premature infants. These results highlight the need for a semiautomatic tool to help in objectively analyzing MRI signal intensity in the neonatal brain.

[Value of whole-body MRI in vertebral fractures]. / Intérêt de l'IRM corps entier dans les fractures vertébrales.

Chronic recurrent multifocal osteomyelitis (CRMO) is a rare autoinflammatory disease in children. Pathological vertebral fracture may be the first symptom revealing this disease. We describe the case of a 14-year-old boy, with no significant past medical history, who had a sudden dorsal pain after carrying a friend on his back. Plain radiographs and MRI showed fractures of the superior endplate of T5 and T6 associated with a mild degree of kyphosis. MRI allowed ruling out discitis. The diagnostic hypotheses raised were cancer (lymphoma, leukemia), Langerhans cell histiocytosis, osteogenesis imperfecta, and CRMO. A whole-body MRI (wbMRI) was performed and disclosed several clinically silent signal abnormalities in key sites of CRMO (pelvic bone and tibial metaphyses). We point out that CRMO should be systematically added to the list of possible diseases in case of vertebral fracture. In this perspective, wbMRI is a major noninvasive tool to assess the diagnosis of CRMO, and allows avoiding a bone biopsy in most cases.

Ovarian tumors in children and adolescents: a series of 41 cases.

OBJECTIVE: Pictorial review with a detailed semiological analysis of ovarian tumors in children and adolescents to provide a relevant diagnostic approach. PATIENTS AND METHODS: Retrospective study (2001-2011) of 41 patients under the age of 15 who underwent surgery for an ovarian mass with a definite pathological diagnosis. RESULTS: Sixty-two percent of the lesions were benign, 33% were malignant and 5% were borderline. Germ cell tumors were most frequent (77.5%), followed by sex cord stromal tumors (12.5%) and epithelial tumors (7.5%). Malignant tumors were more frequent in children between 0 and 2 years old. On imaging, calcifications and fat were specific for germ cell tumors; the presence of a mural nodule was predictive of a mature teratoma (P<0.001). Predictive factors for malignancy were clinical, including abdominal distension (P<0.01) or a palpable mass (P=0.05), biological, including increased hCG and/or AFP levels (P<0.001) and radiological, including tumors larger than 12 cm (P<0.05), tumoral hypervascularity (P<0.01) and voluminous ascites (P<0.01). CONCLUSION: This semiological analysis confirms the role of imaging in diagnosing the etiology of ovarian lesions in children and adolescents and emphasizes the importance identifying tumoral hypervascularity, which, in addition to classic criteria, is highly predictive of malignancy.

[Recent advances in newborn MRI]. / Progrès récents de l'imagerie par résonance magnétique (IRM) chez le nouveau-né

The accurate morphological exploration of the brain is a major challenge in neonatology that advances in magnetic resonance imaging (MRI) can now provide. MRI is the gold standard if an hypoxic ischemic pathology is suspected in a full term neonate. In prematures, the specific role of MRI remains to be defined, secondary to US in any case. We present a state of the art of hardware and software technical developments in MRI. The increase in magnetic field strength (3 tesla) and the emergence of new MRI sequences provide access to new information. They both have positive and negative consequences on the daily clinical data acquisition use. The semiology of brain imaging in full term newborns and prematures is more extensive and complex and thereby more difficult to interpret. The segmentation of different brain structures in the newborn, even very premature, is now available. It is now possible to dissociate the cortex and basal ganglia from the cerebral white matter, to calculate the volume of anatomical structures, which improves the morphometric quantification and the understanding of the normal and abnormal brain development. MRI is a powerful tool to analyze the neonatal brain. The relevance of the diagnostic contribution requires an adaptation of the parameters of the sequences to acquire and of the image processing methods.

[Medical care of brain malformative vascular diseases discovered during the pre- or neonatal period]. / Prise en charge des malformations vasculaires cérébrales découvertes en période ante- ou néonatale.

Three types of brain arteriovenous vascular malformations can be found during the neonatal period, according to their anatomical location. Vein of Galen malformations are the most common. The others are pial arteriovenous malformations or dural arteriovenous malformations, which include dural sinus malformations. They can be asymptomatic, but most often they are associated with different symptoms, related to their angioarchitecture or their effect on the brain. High-flow arteriovenous malformations can thus be responsible for heart failure. Local or regional venous hyperpressure exposes the patient to subacute or chronic brain lesions, or to hydrovenous disorders such as hydrocephalus. Some types of venous reflux can expose patients to brain hemorrhage. The treatment chosen for these vascular malformations and their consequences is transarterial or transvenous embolization, depending on the angioarchitecture and type of lesion. The schedule for the treatment will be determined according to the malformation type and its local or general effects on the brain. The aim of this article is to present the recommendations of the French National Referral Center for neurovascular malformations in children, in order to help clinicians and radiologists treat these patients during pre- or neonatal period.