Olivier Brissaud

Schizencephaly: clinical and imaging features in 30 infantile cases.

Schizencephaly is an uncommon structural disorder of cerebral cortical development, characterized by congenital clefts spanning the cerebral hemispheres from the pial surface to the lateral ventricles and lined by cortical gray matter. Either an antenatal environmental incident or a genetic origin could be responsible for this lesion which occurs between the third and fourth month of gestation. We report the clinical and cranial imaging features of 30 children, of whom 15 had unilateral and 15 had bilateral lesions. Their ages at the time of the first presentation ranged from 1 month to 10 years. They were thoroughly studied from clinical, epileptical, imaging and electroencephalographic (EEG) viewpoints. Five patients were investigated by cranial computed tomography (CT), eight by cranial magnetic resonance (MR) imaging, and 17 by both methods. The clinical features consisted of mild hemiparesis in 17 cases (57%), 12/17 were related to a unilateral phenotype (80% of all unilateral forms) and 5/17 to a bilateral phenotype. A tetraparesis was present in nine cases, all of which were due to a bilateral cleft. Bilateral forms were significantly associated with tetraparesis, whereas unilateral forms were associated with hemiparesis. Mental retardation was observed in 17 cases (57%), and was observed significantly more often in bilateral clefts (80%). When both hemispheres are involved, an absence of reorganization of the brain function between the two hemispheres leads to severe mental deficits, in addition to the cerebral anomaly itself. Eleven patients had seizures (seven from unilateral and three from bilateral forms). The degree of malformation was not related to the severity of epilepsy. Migration disorders, such as dysplasia or heterotopia, were observed in 30% of cases and are also important etiopathogenetic factors. The septum pellucidum was absent in 13 cases (43%), with septo-optical dysplasia in two cases. Corpus callosum dysgenesis was noted in 30% of cases. Four cases of mega cisterna magna were noted. Although familial cases and environmental factors have been previously reported, schizencephaly appears to be, in the majority of cases, sporadic.

Short-Term Effect of Erythropoietin on Brain Lesions and Aquaporin-4 Expression in a Hypoxic-Ischemic Neonatal Rat Model Assessed by Magnetic Resonance Diffusion Weighted Imaging and Immunohistochemistry

Erythropoietin (Epo) is an endogenous cytokine that regulates hematopoiesis and is widely used to treat anemia. In addition, it has recently increased interest in the neurosciences since the new concept of Epo as a neuroprotective agent has emerged. The potential protective effect of human recombinant Epo (r-hu-Epo) on a hypoxic-ischemic (HI) pup rat model was studied. Cerebral HI was obtained by permanent left carotid artery ligature of pups followed by a 2-h hypoxia. Three hours after carotid occlusion, brain lesions were assessed by magnetic resonance diffusion weighted imaging. Intraperitoneal administration of r-hu-Epo (30,000 U/kg dose) limited both the HI-induced brain lesion area and the decrease in apparent diffusion coefficient (ADC) in the lesion. To identify potential mechanisms underlying the effects of Epo, immunohistochemical detection of caspase-3 and water channel protein aquaporin-4 (AQP4) were performed. No early apoptosis was detected, but up-regulation of AQP4 expression was observed in HI pups that received r-hu-Epo compared with HI animals without treatment. This study demonstrates an early neuroprotective effect of Epo with regard to brain lesion area and ADC values. One possible mechanism of Epo for decreasing brain edema and cellular swelling could be a better clearance of water excess in brain tissue, a process possibly mediated by AQP4.

Patent Ductus Arteriosus in the Preterm Infant: A Survey of Clinical Practices in French Neonatal Intensive Care Units

Patent ductus arteriosus (PDA) is one of the most common problems in the care of premature infants, especially the extremely premature. There is no real consensus regarding the diagnostic criteria or treatment of a hemodynamically significant PDA. Its diagnosis, assessment, and treatment still remain challenges. Therefore, we investigated clinical practices in French tertiary neonatology centers regarding the management of PDA to compare their similarities and differences. We sent a questionnaire by email to the PDA specialist in every French tertiary neonatal intensive care unit. It contained 27 items regarding the unit’s structure, method of diagnosing PDA, and treatment choices. The completed questionnaire were returned via email and analyzed blindly. The questionnaire response rate was 87.5%, which allowed us to draw some conclusions regarding French clinical practices in the care of neonates with PDA. Although the diagnostic criteria are quite similar, the therapeutic practices are rather different across neonatal care units. We highlight the great variability in French clinical practices when it comes to treating PDA and underscore the necessity for harmonization of these practices, which could be achieved using multicenter, randomized studies to identify the advantages of one approach compared with another.

Successful treatment with rituximab for acute refractory thrombotic thrombocytopenic purpura related to acquired ADAMTS13 deficiency: A pediatric report and literature review

Objective: To report the case of a child with severe autoimmune thrombotic thrombocytopenic purpura (TTP) resistant to plasma exchange and steroids who was successfully treated with rituximab. Design: Case report and review of the literature on pediatric acquired TTP. The report was approved by an independent local ethics committee. Setting: Pediatric intensive care unit in a tertiary care childrens hospital. Patient: A 10-yr-old boy was referred to the emergency unit with fever, vomiting, confusion, hemolytic anemia, thrombocytopenia, and mild acute renal failure. An atypical hemolytic uremic syndrome was suspected, and plasma exchange was started urgently. The patient was refractory to plasma therapy and presented critical complications. After a diagnosis of acquired TTP attributable to anti-ADAMTS13 autoantibodies had been made, he was treated with rituximab, which resulted in a stable clinical remission. Interventions: Rituximab therapy. Measurements and Main Results: Clinical remission. Conclusions: TTP is a rare but life-threatening condition in children that is characterized by hemolytic anemia, thrombocytopenia, and signs of ischemic organ dysfunction. If renal involvement is present, TTP may be misdiagnosed as hemolytic uremic syndrome, but reliable screening for ADAMTS13 activity and anti-ADAMTS autoantibodies allow us to distinguish the two entities and provide adequate therapy.

Apport pronostique de la résonance magnétique cérébrale dans l’encéphalopathie hypoxique-ischémique du nouveau-né à terme : score d’imagerie, spectroscopie. Étude de 26 cas

UNLABELLED Neonatal hypoxic-ischemic encephalopathy remains a major cause of chronic disability in childhood. Early diagnosis and prognosis are necessary for the clinician to adapt the treatment. However, there is yet no reliable test to predict the patients evolution. OBJECTIVE The aim of our study was to evaluate the predictive value of a personal magnetic resonance imaging (MRI) scoring system and of magnetic resonance spectroscopy (MRS). MATERIAL AND METHODS We included 26 term newborns in condition of neonatal brain suffering. MR examination was performed during the first week of life for all patients and MRI and MRS data were collected. Standardised follow-up visits were made for all patients. Finally, prognostic value of the different criteria was evaluated with statistical tests. RESULTS Our MRI scoring system proved to be linked to prognosis. A high MRI score, abnormal signal in the internal capsule, white matter or basal ganglia abnormalities with diffusion imaging were associated with unfavourable outcome. These results confirmed the data of the literature concerning the MRI predictive value. Our study also confirmed prognostic interest of MR: particularly, ratios using lactate were significantly linked to prognosis in our study. Specificity of the elevation of these ratios was interesting but sensibility was less optimal. CONCLUSION We suggest using our MRI scoring system which associates standard MRI and diffusion imaging, which is significantly related to outcome. We confirm the prognostic value of MRS in this pathological situation. MR with diffusion sequence and spectroscopy, performed three to four days after birth appears to be an essential tool to manage these patients.

Severe transient ADAMTS13 deficiency in pneumococcal-associated hemolytic uremic syndrome

Thrombotic microangiopathies comprise different entities, including hemolytic uremic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP), and several other conditions. TTP is characterized by hemolytic anemia, thrombocytopenia, and multiorgan failure. TTP is the result of severe von Willebrand factor multimer cleaving protease (ADAMTS13) deficiency that is either inherited or the result of acquired autoantibodies. We report a critically ill 2-year-old girl with invasive pneumococcal disease associated HUS (p-HUS) whose condition was complicated by severe ADAMTS13 deficiency, without detectable inhibitor, in a context of multiple organ failure. The patient recovered with supportive treatment, and ADAMTS13 activity normalized without plasmatherapy. Severe ADAMTS13 deficiency appears to be a manifestation of transient endothelial cell injury in the context of severe sepsis, including invasive p-HUS. The choice of appropriate therapy should not be based on this finding.

Targeted temperature management in the ICU: guidelines from a French expert panel

Over the recent period, the use of induced hypothermia has gained an increasing interest for critically ill patients, in particular in brain-injured patients. The term “targeted temperature management” (TTM) has now emerged as the most appropriate when referring to interventions used to reach and maintain a specific level temperature for each individual. TTM may be used to prevent fever, to maintain normothermia, or to lower core temperature. This treatment is widely used in intensive care units, mostly as a primary neuroprotective method. Indications are, however, associated with variable levels of evidence based on inhomogeneous or even contradictory literature. Our aim was to conduct a systematic analysis of the published data in order to provide guidelines. We present herein recommendations for the use of TTM in adult and paediatric critically ill patients developed using the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) method. These guidelines were conducted by a group of experts from the French Intensive Care Society (Société de Réanimation de Langue Française [SRLF]) and the French Society of Anesthesia and Intensive Care Medicine (Société Francaise d’Anesthésie Réanimation [SFAR]) with the participation of the French Emergency Medicine Association (Société Française de Médecine d’Urgence [SFMU]), the French Group for Pediatric Intensive Care and Emergencies (Groupe Francophone de Réanimation et Urgences Pédiatriques [GFRUP]), the French National Association of Neuro-Anesthesiology and Critical Care (Association Nationale de Neuro-Anesthésie Réanimation Française [ANARLF]), and the French Neurovascular Society (Société Française Neurovasculaire [SFNV]). Fifteen experts and two coordinators agreed to consider questions concerning TTM and its practical implementation in five clinical situations: cardiac arrest, traumatic brain injury, stroke, other brain injuries, and shock. This resulted in 30 recommendations: 3 recommendations were strong (Grade 1), 13 were weak (Grade 2), and 14 were experts’ opinions. After two rounds of rating and various amendments, a strong agreement from voting participants was obtained for all 30 (100%) recommendations, which are exposed in the present article.

Experts’ recommendations for the management of cardiogenic shock in children

Cardiogenic shock which corresponds to an acute state of circulatory failure due to impairment of myocardial contractility is a very rare disease in children, even more than in adults. To date, no international recommendations regarding its management in critically ill children are available. An experts’ recommendations in adult population have recently been made (Levy et al. Ann Intensive Care 5(1):52, 2015; Levy et al. Ann Intensive Care 5(1):26, 2015). We present herein recommendations for the management of cardiogenic shock in children, developed with the grading of recommendations’ assessment, development, and evaluation system by an expert group of the Groupe Francophone de Réanimation et Urgences Pédiatriques (French Group for Pediatric Intensive Care and Emergencies). The recommendations cover four major fields of application such as: recognition of early signs of shock and the patient pathway, management principles and therapeutic goals, monitoring hemodynamic and biological variables, and circulatory support (indications, techniques, organization, and transfer criteria). Major principle care for children with cardiogenic shock is primarily based on clinical and echocardiographic assessment. There are few drugs reported as effective in childhood in the medical literature. The use of circulatory support should be facilitated in terms of organization and reflected in the centers that support these children. Children with cardiogenic shock are vulnerable and should be followed regularly by intensivist cardiologists and pediatricians. The experts emphasize the multidisciplinary nature of management of children with cardiogenic shock and the importance of effective communication between emergency medical assistance teams (SAMU), mobile pediatric emergency units (SMUR), pediatric emergency departments, pediatric cardiology and cardiac surgery departments, and pediatric intensive care units.

Outbreak in newborns of methicillin-resistant Staphylococcus aureus related to the sequence type 5 Geraldine clone

We describe the first nosocomial outbreak of a toxic shock syndrome-positive methicillin-resistant Staphylococcus aureus (MRSA) sequence type 5 Geraldine clone. Infection control interventions that are usually successful were implemented to control the outbreak. Spread of this virulent MRSA strain highlights the need to be vigilant to MRSA antibiotic susceptibilities.

Sonography-guided positioning of intravenous long lines in neonates

OBJECTIVE In neonates, proper positioning of the tip of intravenous long lines (LL) is essential in order to prevent potential life-threatening complications. The gold standard for the evaluation of LL position in neonates is the chest X-ray with or without contrast. We performed a prospective study to assess the use of transthoracic ultrasonography (US) for the positioning of LL in neonates and to compare it to plain radiography. MATERIALS AND METHOD Thirty-six consecutive neonates requiring percutaneous LL over a period of 3 months were included in the study. Immediately after LL insertion, the position of its tip was verified using transthoracic US, followed by plain radiography. The two techniques were compared in terms of adequate placement and length of time between insertion and radiographic evaluation of the correct position. RESULTS The correlation between positioning by US and plain radiography was very good (r=0.97, r(2)=0.94, p<0.0001). The time needed to verify LL placement by US was shorter by a mean 15 min compared to plain radiography. CONCLUSION US can accurately guide LL tip positioning. We believe that because of the potential gain of time it offers and its lack of ionising radiation, it to be considered as an interesting tool for the positioning of LL in neonates. Yet more accurate results could be obtained with a better-trained staff.